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  • 1.
    Andin, Josefine
    et al.
    Department of Behavioural Sciences and Learning, Linköping University, Linköping, Sweden; Swedish Institute for Disability Research, Örebro University, Örebro, Sweden.
    Elwer, Åsa
    Department of Behavioural Sciences and Learning, Linköping University, Linköping, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Swedish Institute for Disability Research.
    Arithmetic in the adult deaf signing brain2020In: Journal of Neuroscience Research, ISSN 0360-4012, E-ISSN 1097-4547, Vol. 98, no 4, p. 643-654Article in journal (Refereed)
    Abstract [en]

    We have previously shown that deaf signers recruit partially different brain regions during simple arithmetic compared to a group of hearing non-signers, despite similar performance. Specifically, hearing individuals show more widespread activation in brain areas that have been related to the verbal system of numerical processing, i.e., the left angular and inferior frontal gyrus, whereas deaf individuals engaged brain areas that have been related to the quantity system of numerical processing, i.e., the right horizontal intraparietal sulcus. This indicates that compared to hearing non-signers, deaf signers can successfully make use of processes located in partially different brain areas during simple arithmetic. In this study, which is a conceptual replication and extension of the above-presented study, the main aim is to understand similarities and differences in neural correlates supporting arithmetic in deaf compared to hearing individuals. The primary objective is to investigate the role of the right horizontal intraparietal gyrus, the left inferior frontal gyrus, the hippocampus, and the left angular gyrus during simple and difficult arithmetic and how these regions are connected to each other. A second objective is to explore what other brain regions support arithmetic in deaf signers. Up to 34 adult deaf signers and the same amount of hearing non-signers will be enrolled in an functional magnetic resonance imaging study that will include simple and difficult subtraction and multiplication. Brain imaging data will be analyzed using whole-brain analysis, region of interest analysis and connectivity analysis. This is the first study to investigate neural underpinnings of arithmetic of different difficulties in deaf individuals.

  • 2.
    Andin, Josefine
    et al.
    Department of Behavioural Sciences and Learning, Linköping University, Linköping, Sweden.
    Elwér, Åsa
    Department of Behavioural Sciences and Learning, Linköping University, Linköping, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Audiological Research Center, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.
    Arithmetic in the signing brain: Differences and similarities in arithmetic processing between deaf signers and hearing non-signers2023In: Journal of Neuroscience Research, ISSN 0360-4012, E-ISSN 1097-4547, Vol. 101, no 1, p. 172-195Article in journal (Refereed)
    Abstract [en]

    Deaf signers and hearing non-signers have previously been shown to recruit partially different brain regions during simple arithmetic. In light of the triple code model, the differences were interpreted as relating to stronger recruitment of the verbal system of numerical processing, that is, left angular and inferior frontal gyrus, in hearing non-signers, and of the quantity system of numerical processing, that is, right horizontal intraparietal sulcus, for deaf signers. The main aim of the present study was to better understand similarities and differences in the neural correlates supporting arithmetic in deaf compared to hearing individuals. Twenty-nine adult deaf signers and 29 hearing non-signers were enrolled in an functional magnetic resonance imaging study of simple and difficult subtraction and multiplication. Brain imaging data were analyzed using whole-brain analysis, region of interest analysis, and functional connectivity analysis. Although the groups were matched on age, gender, and nonverbal intelligence, the deaf group performed generally poorer than the hearing group in arithmetic. Nevertheless, we found generally similar networks to be involved for both groups, the only exception being the involvement of the left inferior frontal gyrus. This region was activated significantly stronger for the hearing compared to the deaf group but showed stronger functional connectivity with the left superior temporal gyrus in the deaf, compared to the hearing, group. These results lend no support to increased recruitment of the quantity system in deaf signers. Perhaps the reason for performance differences is to be found in other brain regions not included in the original triple code model.

  • 3.
    Asp, Filip
    et al.
    Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Stockholm, Sweden; Department of Cochlear Implants M43, Karolinska University Hospital, Stockholm, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden; Department of ENT-Head Neck Surgery, County Council of Östergötland, Linköping, Sweden; Swedish Institute for Disability Research, Department of Behavioural Sciences and Learning, Linköping University, Linköping, Sweden.
    Karltorp, Eva
    Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Stockholm, Sweden; Department of Cochlear Implants M43, Karolinska University Hospital, Stockholm, Sweden.
    Harder, Henrik
    Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden; Department of ENT-Head Neck Surgery, County Council of Östergötland, Linköping, Sweden.
    Hergils, Leif
    Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden; Department of ENT-Head Neck Surgery, County Council of Östergötland, Linköping, Sweden.
    Eskilsson, Gunnar
    Department of Cochlear Implants, M43, Karolinska University Hospital, Stockholm, Sweden.
    Stenfelt, Stefan
    Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden; Swedish Institute for Disability Research, Department of Behavioural Sciences and Learning, Linköping University, Linköping, Sweden.
    A longitudinal study of the bilateral benefit in children with bilateral cochlear implants2015In: International Journal of Audiology, ISSN 1499-2027, E-ISSN 1708-8186, Vol. 54, no 2, p. 77-88Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: To study the development of the bilateral benefit in children using bilateral cochlear implants by measurements of speech recognition and sound localization.

    DESIGN: Bilateral and unilateral speech recognition in quiet, in multi-source noise, and horizontal sound localization was measured at three occasions during a two-year period, without controlling for age or implant experience. Longitudinal and cross-sectional analyses were performed. Results were compared to cross-sectional data from children with normal hearing.

    STUDY SAMPLE: Seventy-eight children aged 5.1-11.9 years, with a mean bilateral cochlear implant experience of 3.3 years and a mean age of 7.8 years, at inclusion in the study. Thirty children with normal hearing aged 4.8-9.0 years provided normative data.

    RESULTS: For children with cochlear implants, bilateral and unilateral speech recognition in quiet was comparable whereas a bilateral benefit for speech recognition in noise and sound localization was found at all three test occasions. Absolute performance was lower than in children with normal hearing. Early bilateral implantation facilitated sound localization.

    CONCLUSIONS: A bilateral benefit for speech recognition in noise and sound localization continues to exist over time for children with bilateral cochlear implants, but no relative improvement is found after three years of bilateral cochlear implant experience.

  • 4.
    Badache, Andreea C.
    et al.
    Örebro University, School of Health Sciences.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Audiological Research Center, Faculty of Medicine and Health, Örebro University, Sweden.
    Widén, Stephen
    Örebro University, School of Health Sciences.
    Fors, Stefan
    Aging Research Center, Karolinska Institutet and Stockholm University, Stockholm, Sweden; Centre for Epidemiology and Community Medicine, Region Stockholm, Stockholm, Sweden.
    Why are old-age disabilities decreasing in Sweden and Denmark? Evidence on the contribution of cognition, education, and sensory functions2023In: The journals of gerontology. Series B, Psychological sciences and social sciences, ISSN 1079-5014, E-ISSN 1758-5368, Vol. 78, no 3, p. 483-495Article in journal (Refereed)
    Abstract [en]

    OBJECTIVES: Improvements in educational attainment, cognitive and sensory functions, and a decline in the prevalence of disabilities have been observed in older adults in Sweden and Denmark. In the present study, it was investigated whether better cognition, higher educational attainment, and improved sensory function among older adults aged 60 and older in these countries have contributed to decreasing rates of old-age disabilities.

    METHODS: The analyses were based on repeated cross-sectional data from the Survey of Health, Ageing, and Retirement in Europe, for 2004-2017 period. Descriptive data was used to benchmark the declining prevalence of disabilities, improving cognitive and sensory function, and increased educational level. The association between time and disabilities was analyzed with logistic regression models and the contribution of the improved cognitive, education and sensory function to the declining prevalence of old-age disabilities was estimated using the Karlson-Holm-Breen (KHB) method for mediation analysis.

    RESULTS: The analysis suggests that the declining prevalence of old-age disabilities in Sweden and Denmark between 2004 and 2017, can largely be attributed to improved cognitive function and vision, and to a lesser extent by education and hearing ability.

    DISCUSSION: These findings raise important questions about the causal mechanisms producing the associations between cognition, education, and sensory functions and disability in older age. Future studies should explore the causal nature of the associations between these mediators, and old-age disabilities. In addition, they should explore whether these findings differ across regional and cultural contexts and over different time periods.

  • 5.
    Badache, Andreea
    et al.
    Örebro University, School of Health Sciences.
    Hachem, Hany
    Örebro University, School of Humanities, Education and Social Sciences.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Swedish Institute of Disability Research, Örebro, Sweden.
    The perspectives of successful ageing among older adults aged 75+: a systematic review with a narrative synthesis of mixed studies2023In: Ageing & Society, ISSN 0144-686X, E-ISSN 1469-1779, Vol. 43, p. 1203-1239Article, review/survey (Refereed)
    Abstract [en]

    Older adults 75 and above are a fast-growing segment of the population. However, few studies have investigated what it means to age successfully from their perspective. This group of older adults face challenges that might characteristically differ from younger older adults. Therefore, the aim of this study was to conduct a systematic review of the perspectives of older adults aged 75 and above regarding what it means to age successfully and to summarise the findings through a narrative synthesis. We also aimed to provide a snapshot of inhibitors and facilitators to achieve successful ageing. A systematic review of the 75+ older adults’ perspectives was conducted across PubMed, CINAHL, Scopus, Web of Science and PsycINFO. Qualitative, quantitative and mixed-methods original peer-reviewed studies were included. After reviewing 4,661 articles, 15 articles met the inclusion criteria and 15 themes were identified. They ranged from biomedical components such as physical functioning to psychosocial components such as relationships, reflections on life and past experiences, preparations for death and environmental factors. The findings revealed that as people age, their definition of successful ageing changes, expanding the current conceptualisation of successful ageing by including additional factors that can act as facilitators and/or inhibitors, such as death and environmental factors. The findings also highlight the need for further research on theory development by considering age-related differences and the perspectives of under-studied populations.

  • 6.
    Badache, Andreea
    et al.
    Örebro University, School of Health Sciences.
    Rehnberg, Johan
    Aging Research Center, Karolinska Institutet and Stockholm University, Stockholm, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences.
    Widén, Stephen
    Örebro University, School of Health Sciences.
    Fors, Stefan
    Aging Research Center, Karolinska Institutet and Stockholm University, Stockholm, Sweden; Centre for Epidemiology and Community Medicine, Region Stockholm, Stockholm, Sweden; Department of Public Health Sciences, Stockholm University, Stockholm, Sweden.
    Longitudinal associations between sensory and cognitive functioning in adults 60 years or older in Sweden and Denmark2024In: Archives of gerontology and geriatrics (Print), ISSN 0167-4943, E-ISSN 1872-6976, Vol. 121, article id 105362Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: The objective of this study is to explore the bidirectional, longitudinal associations between self-reported sensory functions (hearing/vision) and cognitive functioning among older adults in Sweden and Denmark during the period 2004-2017.

    METHODS: The study is based on data from The Survey of Health, Ageing and Retirement in Europe and consists of 3164 persons aged 60 to 93 years. Within-person associations between sensory and cognitive functions were estimated using random intercept cross-lagged panel models.

    RESULTS: The results indicated that cognitive and sensory functions were associated within their respective domains over time. The results on the bidirectional associations between sensory functions and cognition over time showed weak and statistically non-significant estimates.

    CONCLUSION: Our study showed no clear evidence for cross-lagged effects between sensory functions and cognitive functioning. Important to note, however, is that using longitudinal data to estimate change within persons is a demanding statistical test and various factors may have contributed to the absence of conclusive evidence in our study. We discuss several of these factors.

  • 7.
    Badache, Andreea-Corina
    et al.
    Örebro University, School of Health Sciences. Swedish Institute for Disability Research.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Audiological Research Center, Faculty of Medicine, and Health, Örebro University, Örebro Sweden .
    Widén, Stephen
    Örebro University, School of Health Sciences.
    Fors, Stefan
    Aging Research Center, Karolinska Institutet and Stockholm University, Stockholm, Sweden; Centre for Epidemiology and Community Medicine, Region Stockholm, Stockholm, Sweden; Department of Public Health Sciences, Stockholm University, Stockholm, Sweden.
    A descriptive epidemiological study of the prevalence of self-reported sensory difficulties by age group, sex, education, disability, and migration status in Sweden in 2020Manuscript (preprint) (Other academic)
  • 8.
    Badache, Andreea-Corina
    et al.
    Örebro University, School of Health Sciences. Swedish Institute for Disability Research.
    Rehnberg, Johan
    Aging Research Center, Karolinska Institutet and Stockholm University, Stockholm, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Audiological Research Center, Faculty of Medicine, and Health, Örebro University, Örebro Sweden .
    Widén, Stephen
    Örebro University, School of Health Sciences.
    Fors, Stefan
    Aging Research Center, Karolinska Institutet and Stockholm University, Stockholm, Sweden; Centre for Epidemiology and Community Medicine, Region Stockholm, Stockholm, Sweden; Department of Public Health Sciences, Stockholm University, Stockholm, Sweden.
    Longitudinal associations between sensory and cognitive functioning in adults 60 years or older in Sweden and DenmarkManuscript (preprint) (Other academic)
  • 9.
    Bergman, Pia
    et al.
    Department of Otorhinolaryngology, Jönköping County hospital, Jönköping, Sweden; Division of Neuro and Inflammation Science, Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden; Department of Otorhinolaryngology in Linköping, Anaesthetics, Operations and Specialty Surgery Center, Region Östergötland, Linköping, Sweden.
    Lyxell, Björn
    Department of Special Needs Education, Oslo University, Oslo, Norway; Linnaeus Centre HEAD, Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    Harder, Henrik
    Department of Otorhinolaryngology in Linköping, Anaesthetics, Operations and Specialty Surgery Center, Region Östergötland, Linköping, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Division of Neuro and Inflammation Science, Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden; Department of Otorhinolaryngology in Linköping, Anaesthetics, Operations and Specialty Surgery Center, Region Östergötland, Linköping, Sweden; Linnaeus Centre HEAD, Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    The outcome of unilateral cochlear implantation in adults: Speech recognition, health-related quality of life and level of anxiety and depression: a one- and three-year follow up study2020In: International Archives of Otorhinolaryngology, ISSN 1809-9777, E-ISSN 1809-4864, Vol. 24, no 3, p. 338-346Article in journal (Refereed)
    Abstract [en]

    Introduction: Hearing impairment is a common disease worldwide, with a comprehensive impact, and cochlear implantation (CI) is an intervention for profound hearing impairment.

    Objective: To study the outcome one and three years after unilateral CI on hearing, health-related quality of life and level of depression and anxiety, and the correlation between the outcomes. Second, to study whether age, gender, etiology, operated side, residual hearing or cognitive performance can predict the outcome.

    Methods: A prospective longitudinal study including adults with profound postlingual hearing impairment, with respect to hearing (speech recognition), health-related quality of life (Health Utilities Index 3) and level of depression and anxiety (Hospital Anxiety and Depression scale), pre-CI, and one and three years post-CI. The total sample was composed of 40 participants (40% of men), with a mean age of 71 years.

    Results: Speech recognition and the overall health-related quality of life improved one year post-CI (p ¼ 0.000), without correlation (ρ¼ 0.27), and with no difference three years post-CI. The hearing attribute (in the health-related quality of life instrument) improved one and three years post-CI (p ¼ 0.000). The level of anxiety did not change one and three years post-CI. The level of depression improved one year post-CI (p ¼ 0.036), and deteriorated three years post-CI (p ¼ 0.031). Age, etiology, operated side, residual hearing and cognitive performance did not predict the outcome, but the female gender did significantly improve speech recognition compared with men (p ¼ 0.009).

    Conclusion: The CI significantly improved speech recognition, health-related quality of life and level of depression one year post-CI without mutual correlation, and women performed significantly better than men. There were no further improvements three years post-CI, apart from the hearing attribute.

    Download full text (pdf)
    The Outcome of Unilateral Cochlear Implantation in Adults: Speech Recognition, Health-Related Quality of Life and Level of Anxiety and Depression: a One- and Three-Year Follow-Up Study
  • 10.
    Boisvert, Isabelle
    et al.
    Centre for Language Sciences, Macquarie University, Sydney NSW, Australia; HEARing Cooperative Research Centre, Melbourne VIC, Australia; Linnaeus Centre HEAD, Swedish Institute for Disability Research, Linnaeus University, Växjö, Sweden.
    Lyxell, Björn
    Linnaeus Centre HEAD, Swedish Institute for Disability Research, Linnaeus University, Växjö, Sweden; Department of Behavioural Sciences and Learning, Division of Technical Audiology, Linköping University, Linköping, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Linnaeus Centre HEAD, Swedish Institute for Disability Research, Linnaeus University, Växjö, Sweden; Department of Clinical and Experimental Medicine, Division of Technical Audiology, Linkoping University, Linkoping, Sweden; Department of ENT-Head Neck Surgery UHL, County Council of Östergötland, Linköping, Sweden.
    McMahon, Catherine M.
    Centre for Language Sciences, Macquarie University, Sydney NSW, Australia; HEARing Cooperative Research Centre, Melbourne VIC, Australia.
    Dowell, Richard C.
    HEARing Cooperative Research Centre, Melbourne VIC, Australia; Department of Audiology and Speech Pathology, University of Melbourne, Melbourne, Australia; Department of Audiology, Royal Victorian Eye and Ear Hospital, Melbourne VIC, Australia.
    Choice of ear for cochlear implantation in adults with monaural sound-deprivation and unilateral hearing aid2012In: Otology and Neurotology, ISSN 1531-7129, E-ISSN 1537-4505, Vol. 33, no 4, p. 572-579Article in journal (Refereed)
    Abstract [en]

    OBJECTIVES: To identify whether speech recognition outcomes are influenced by the choice of ear for cochlear implantation in adults with bilateral hearing loss who use a hearing aid in 1 ear but have long-term auditory deprivation in the other.

    STUDY DESIGN: Retrospective matched cohort study. Speech recognition results were examined in 30 adults with monaural sound deprivation. Fifteen received the implant in the sound-deprived ear and 15 in the aided ear.

    SETTING: Tertiary referral centers with active cochlear implant programs.

    PATIENTS: Adults with bilateral hearing loss and a minimum of 15 years of monaural sound deprivation who received a cochlear implant after meeting the traditional implantation criteria of the referral centers.

    INTERVENTION: Cochlear implantation with devices approved by the U.S. Food and Drug Administration.

    MAIN OUTCOME MEASURE(S): Paired comparisons of postoperative monosyllabic word recognition scores obtained with the implant alone and in the usual listening condition (CI alone or bimodal).

    RESULTS: With the cochlear implant alone, individuals who received the implant in a sound-deprived ear obtained poorer scores than individuals who received the implant in the aided ear. There was no significant difference, however, in speech recognition results for the 2 groups when tested in their usual listening condition. In particular, poorer speech recognition scores were obtained with the cochlear implant alone by individuals using bimodal hearing.

    CONCLUSION: Similar clinical outcomes of cochlear implantation can be achieved by adults with a long-term monaural sound deprivation when comparing the usual listening condition, irrespective of whether the implant is in the sound-deprived or in the aided ear.

  • 11.
    Drakskog, Cecilia
    et al.
    The Division of ENT Diseases, CLINTEC, Karolinska Institutet, Stockholm, Sweden.
    de Klerk, Nele
    The Division of ENT Diseases, CLINTEC, Karolinska Institutet, Stockholm, Sweden.
    Westerberg, Johanna
    Division of Neuro and Inflammation Science,Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden; Department of Otorhinolaryngologyin Linköping, Anaesthetics, Operations and Specialty Surgery Centre, Region Östergötland, Linköping, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Division of Neuro and Inflammation Science,Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden; Department of Otorhinolaryngologyin Linköping, Anaesthetics, Operations and Specialty Surgery Centre, Region Östergötland, Linköping, Sweden.
    Georén, Susanna Kumlien
    The Division of ENT Diseases, CLINTEC, Karolinska Institutet, Stockholm, Sweden.
    Cardell, Lars Olaf
    The Division of ENT Diseases, CLINTEC, Karolinska Institutet, Stockholm, Sweden; Department of ENT Diseases, Karolinska University Hospital, Stockholm, Sweden.
    Extensive qPCR analysis reveals altered gene expression in middle ear mucosa from cholesteatoma patients2020In: PLOS ONE, E-ISSN 1932-6203, Vol. 15, no 9, article id e0239161Article in journal (Refereed)
    Abstract [en]

    The middle ear is a small and hard to reach compartment, limiting the amount of tissue that can be extracted and the possibilities for studying the molecular mechanisms behind diseases like cholesteatoma. In this paper 14 reference gene candidates were evaluated in the middle ear mucosa of cholesteatoma patients and two different control tissues.ACTBandGAPDHwere shown to be the optimal genes for the normalisation of target gene expression when investigating middle ear mucosa in multiplex qPCR analysis. Validation of reference genes usingc-MYCexpression confirmed the suitability ofACTBandGAPDHas reference genes and showed an upregulation ofc-MYCin middle ear mucosa during cholesteatoma. The occurrence of participants of the innate immunity,TLR2andTLR4, were analysed in order to compare healthy middle ear mucosa to cholesteatoma. Analysis ofTLR2andTLR4showed variable results depending on control tissue used, highlighting the importance of selecting relevant control tissue when investigating causes for disease. It is our belief that a consensus regarding reference genes and control tissue will contribute to the comparability and reproducibility of studies within the field.

  • 12.
    Elmazoska, Iris
    et al.
    Örebro University, School of Health Sciences.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences.
    Granberg, Sarah
    Örebro University, School of Health Sciences.
    Widén, Stephen
    Örebro University, School of Health Sciences.
    Associations between recreational noise exposure and hearing function in adolescents and young adults: a systematic reviewIn: Article in journal (Refereed)
  • 13.
    Elmazoska, Iris
    et al.
    Örebro University, School of Health Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Sweden.
    Granberg, Sarah
    Örebro University, School of Health Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Sweden; School of Medical Sciences, Örebro University, Sweden.
    Widén, Stephen
    Örebro University, School of Health Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Sweden.
    Associations Between Recreational Noise Exposure and Hearing Function in Adolescents and Young Adults: A Systematic Review2024In: Journal of Speech, Language and Hearing Research, ISSN 1092-4388, E-ISSN 1558-9102Article, review/survey (Refereed)
    Abstract [en]

    PURPOSE: There is an increasing concern regarding hazardous recreational noise exposure among adolescents and young adults. Daily exposure to loud sound levels over a long period of time can increase the risk of noise-induced hearing loss. The full extent of the impact of recreational noise on hearing is not yet fully understood. The purpose of this review was to synthesize research that investigated hearing function in relation to recreational noise exposure in adolescents and young adults.

    METHOD: A systematic literature search of five databases covering the years 2000-2023 was performed. The articles included investigated audiological measurements of hearing function in relation to recreational noise exposure.

    RESULTS: Four hundred sixty records were identified, of which 20 met the inclusion criteria and were included in the results. This review showed that although some recreational noise activities can be potentially harmful, there is an unclear relationship between exposure and outcome. Some findings indicated hearing threshold shifts or reduced otoacoustic emission amplitudes after recreational noise exposure, but most changes were short term and in the extended high-frequency range.

    CONCLUSIONS: There seemed to be inconsistencies regarding the utilization of methods of measuring exposure and outcome between studies. This might be one reason for the differing results in studies on the reported impact on hearing function from recreational noise exposure. To draw more certain conclusions about long-term effects, there is a need for longitudinal research that utilizes sound level measurements to assess low and high degrees of recreational noise exposure in relation to hearing function.

    SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.25114193.

  • 14.
    Frodlund, Jonas
    et al.
    Department of Otorhinolaryngology, Anaesthetics, Operations and Specialty Surgery Center, Region Östergötland, Linköping, Sweden.
    Harder, Henrik
    Department of Otorhinolaryngology,Anaesthetics, Operations and Specialty Surgery Center, Region Östergötland, Linköping, Sweden.
    Mäki-Torkko, Elina
    Department of Otorhinolaryngology, Anaesthetics, Operations and Specialty Surgery Center, Region Östergötland, Linköping, Sweden; Division of Neuro and Inflammation Science, Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden; Linneaus Centre HEAD, Linköping University, Linköping, Sweden.
    Ledin, Torbjörn
    Department of Otorhinolaryngology, Linköping, Anaesthetics, Operations and Specialty Surgery Center, Region Östergötland, Linköping, Sweden; Division of Neuro and Inflammation Science, Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden.
    Vestibular Function After Cochlear Implantation: A Comparison of Three Types of Electrodes2016In: Otology and Neurotology, ISSN 1531-7129, E-ISSN 1537-4505, Vol. 37, no 10, p. 1535-1540Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: To investigate the vestibular function after cochlear implantation with different types of electrode arrays.

    STUDY DESIGN: Retrospective cohort study.

    SETTING: Academic tertiary referral center.

    MATERIALS AND METHODS: Forty three adults underwent first cochlear implantation. Three consecutive series of patients: Group 1 (n = 13) implanted with a precurved electrode, Group 2 (n = 15) implanted with a straight electrode, Group 3 (n = 15) implanted with a flexible electrode. Patient's vestibular functions were assessed with pre- and postoperative caloric testing using videonystagmography (VNG). The postoperative reduction of the maximum slow phase velocity (MSPV) in the implanted ear was evaluated. Medical charts were reviewed to evaluate the occurrence of late onset of postoperative vestibular symptoms.

    RESULTS: Mean reduction of MSPV was 7.6/s (standard deviation [SD] 8.0) in Group 1, 23.1/s (SD 16.6) in Group 2, and 0.1/s (SD 18.5) in Group 3. Significant difference was found between Group 1 and 2 (p < 0.030) and between Group 2 and 3 (p < 0.001). Group 2 showed a higher prevalence of late onset of clinical vertigo (28.6%) than Group 1 (7.7%) and 3 (6.7%).

    CONCLUSION: In this prospective study, significantly larger reductions of caloric responses were found in subjects implanted with a straight electrode compared with subjects implanted with a precurved or flexible electrode. These findings seem to correlate to a higher prevalence of postoperative vertigo.

  • 15.
    Frånlund, Karin
    et al.
    Department of Otorhinolaryngology, Linköping University Hospital, Linköping, Sweden; Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden.
    Lindehammar, Hans
    Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden; Department of Clinical Neurophysiology, Linköping University Hospital, Linköping, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden.
    Hergils, Leif
    Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden.
    Cortical auditory evoked potentials (P1 latency) in children with cochlear implants in relation to clinical language tests2023In: International Journal of Audiology, ISSN 1499-2027, E-ISSN 1708-8186Article in journal (Refereed)
    Abstract [en]

    Objective: To study the correlation between P1 latency and the results of clinical language tests (Reynell III and TROG-2), the latter were used as they are recommended for follow-up assessments of children with cochlear implants (Cis) by the Swedish National Quality Register for children with hearing impairment.

    Design: A clinical cohort study.Study sample - Cross-sectional and consecutive sampling of 49 children with CIs coming for clinical follow-up assessment from March 2017 - December 2019.

    Results: For all children tested, there was a significant negative correlation (Spearman's rho= -0.403, p = 0.011) between hearing age and P1 latency. A significant correlation between P1 latency and the Reynell III result (Spearman's rho =  -0.810, p = 0.015) was found. In the TROG-2 group, there was no significant correlation between their P1 latency and their language test results (Spearman's rho -0.239, p = 0.196).

    Conclusion: This method seems to be feasible and easily accepted. The study was conducted in a heterogeneous group of children that we meet daily in our clinic. The results indicated that P1 latency has a negative correlation with language development among our youngest patients fitted with CIs and might be a clinical tool to assess the maturation of central auditory pathways.

  • 16.
    Hannula, Samuli
    et al.
    Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Bloigu, Risto
    Medical Informatics Group, University of Oulu, Oulu, Finland.
    Majamaa, Kari
    Department of Clinical Medicine, Neurology, University of Oulu, Oulu, Finland.
    Sorri, Martti
    Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland; Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Audiogram configurations among older adults: prevalence and relation to self-reported hearing problems2011In: International Journal of Audiology, ISSN 1499-2027, E-ISSN 1708-8186, Vol. 50, no 11, p. 793-801Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: There are only a few population-based epidemiological studies on audiogram configurations among adults. The aim of this study was to investigate the prevalence of different audiogram configurations among older adults. In addition, audiogram configurations among subjects reporting hearing problems were examined.

    DESIGN: Cross-sectional, population-based, unscreened epidemiological study among older adults.

    STUDY SAMPLE: The subjects (n = 850), aged 54-66 years, were randomly sampled from the population register. A questionnaire survey, an otological examination, and pure-tone audiometry were performed.

    RESULTS: The most prevalent audiogram configuration among men was high-frequency steeply sloping (65.3% left ear, 51.2% right ear) and among women, high-frequency gently sloping (33.0% left ear, 31.5% right ear). There were significantly more flat configurations among women than among men. Unclassified audiograms were common especially among women (17.5%). Subjects reporting hearing difficulties, difficulties in following conversation in noise, or tinnitus, more often had a high-frequency steeply sloping configuration than those not reporting.

    CONCLUSIONS: High-frequency sloping audiogram configurations were common among older adults, and a high-frequency steeply sloping configuration was common among those reporting hearing problems.

  • 17.
    Hannula, Samuli
    et al.
    Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Bloigu, Risto
    Medical Informatics Group, University of Oulu, Oulu, Finland.
    Majamaa, Kari
    Department of Clinical Medicine, Neurology, University of Oulu, Oulu, Finland.
    Sorri, Martti
    Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland; Department of Clinical and Experimental Medicine/Technical Audiology, Faculty of Health Sciences, Linköping University, Linköping, Sweden; Department of ENT-Head Neck Surgery UHL, County Council of Östergötland, Linköping, Sweden.
    Ear diseases and other risk factors for hearing impairment among adults: an epidemiological study2012In: International Journal of Audiology, ISSN 1499-2027, E-ISSN 1708-8186, Vol. 51, no 11, p. 833-840Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: To investigate the prevalence of ear diseases, other otological risk factors potentially affecting hearing, and noise exposure among adults. Furthermore, subject-related factors possibly associated with hearing impairment (HI), i.e. handedness, eye color, and susceptibility to sunburn, were studied.

    DESIGN: A cross-sectional, unscreened, population-based, epidemiological study among adults.

    STUDY SAMPLE: The subjects (n = 850), aged 54-66 years, were randomly sampled from the population register. A questionnaire survey, an otological examination, and pure-tone audiometry were performed.

    RESULTS: Chronic middle-ear disease (both active and inactive) was the most common ear disease with a prevalence of 5.3%, while the prevalence of otosclerosis was 1.3%, and that of Ménière's disease, 0.7%. Noise exposure was reported by 46% of the subjects, and it had no effect on hearing among those with no ear disease or other otological risk factors for HI. Dark eye color and non-susceptibility to sunburn were associated with HI among noise-exposed subjects.

    CONCLUSIONS: Common ear diseases and other otological risk factors constitute a major part of the etiologies of HI among adults. Contrary to previous studies, noise exposure turned out to have only marginal effect on hearing among those with no otological risk factors.

  • 18.
    Hannula, Samuli
    et al.
    Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Bloigu, Risto
    Medical Informatics Group, University of Oulu, Oulu, Finland.
    Majamaa, Kari
    Department of Clinical Medicine, Neurology, University of Oulu, Oulu, Finland.
    Sorri, Martti
    Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland; Department of Clinical and Experimental Medicine/Technical Audiology, Linköping University, Linköping, Sweden; Department of ENT-Head Neck Surgery UHL, County Council of Östergötland, Linköping, Sweden.
    Self-reported hearing problems among older adults: prevalence and comparison to measured hearing impairment2011In: Journal of american academy of audiology, ISSN 1050-0545, E-ISSN 2157-3107, Vol. 22, no 8, p. 550-559Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: There are not many population-based epidemiological studies on the association between self-reported hearing problems and measured hearing thresholds in older adults. Previous studies have shown that the relationship between self-reported hearing difficulties and measured hearing thresholds is unclear and, according to our knowledge, there are no previous population-based studies reporting hearing thresholds among subjects with hyperacusis.

    PURPOSE: The aim was to investigate the prevalence of self-reported hearing problems, that is, hearing difficulties, difficulties in following a conversation in noise, tinnitus, and hyperacusis, and to compare the results with measured hearing thresholds in older adults.

    RESEARCH DESIGN: Cross-sectional, population-based, and unscreened.

    STUDY SAMPLE: Random sample of subjects (n=850) aged 54-66 yr living in the city of Oulu (Finland) and the surrounding areas.

    DATA COLLECTION AND ANALYSIS: Otological examination, pure tone audiometry, questionnaire survey

    RESULTS: The prevalence of self-reported hearing problems was 37.1% for hearing difficulties, 43.3% for difficulties in following a conversation in noise, 29.2% for tinnitus, and 17.2% for hyperacusis. More than half of the subjects had no hearing impairment, or HI (BEHL[better ear hearing level]0.5-4 kHz<20 dB HL) even though they reported hearing problems. Subjects with self-reported hearing problems, including tinnitus and hyperacusis, had significantly poorer hearing thresholds than those who did not report hearing problems. Self-reported hearing difficulties predicted hearing impairment in the pure-tone average at 4, 6, and 8 kHz, and at the single frequency of 4 kHz.

    CONCLUSIONS: The results indicate that self-reported hearing difficulties are more frequent than hearing impairment defined by audiometric measurement. Furthermore, self-reported hearing difficulties seem to predict hearing impairment at high frequencies (4-8 kHz) rather than at the frequencies of 0.5-4 kHz, which are commonly used to define the degree of hearing impairment in medical and legal issues.

  • 19.
    Hannula, Samuli
    et al.
    Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Mäki-Torkko, Elina
    Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland; Department of Clinical and Experimental, Medicine/Technical Audiology, Linköping University, Linköping, Sweden.
    Majamaa, Kari
    Department of Clinical Medicine, Neurology, University of Oulu, Oulu, Finland.
    Sorri, Martti
    Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Hearing in a 54- to 66-year-old population in northern Finland.2010In: International Journal of Audiology, ISSN 1499-2027, E-ISSN 1708-8186, Vol. 49, no 12, p. 920-927Article in journal (Refereed)
    Abstract [en]

    There are only a few large, population-based epidemiological studies on hearing impairment (HI) in adults. The objective of this study was to investigate the prevalence of HI and possible differences between ears in older adults. The subjects (n = 850), aged 54-66 years, were randomly sampled from the population register. A questionnaire survey, an otological examination, and pure-tone audiometry were performed. Another questionnaire was mailed to collect information on non-participants. The prevalence of HI averaged over the frequencies of 0.5, 1, 2, and 4 kHz for the better ear ≥20 dB HL was 26.7% (men: 36.8%, women: 18.4%). There was no difference between left and right ear pure-tone averages over the frequencies 0.5, 1, 2, and 4 kHz (PTA(0.5-4 kHz)), but a significant difference of -0.8 dB HL was found for the low frequencies 0.125, 0.25, and 0.5 kHz (PTA(0.125-0.5 kHz)), and 4.4 dB HL for the high frequencies over 4, 6, and 8 kHz (PTA(4-8 kHz)). In conclusion, HI was a highly prevalent finding in this age group.

  • 20.
    Hansson, Kristina
    et al.
    Department of Logopedics, Phoniatrics and Audiology, Clinical Sciences, Lund University, Lund, Sweden.
    Sahlén, Birgitta
    Department of Logopedics, Phoniatrics and Audiology, Clinical Sciences, Lund University, Lund, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Otorhinolaryngology, Section of Audiology, Linköping University Hospital, Linköping, Sweden.
    Can a 'single hit' cause limitations in language development?: A comparative study of Swedish children with hearing impairment and children with specific language impairment2007In: International journal of language and communication disorders, ISSN 1368-2822, E-ISSN 1460-6984, Vol. 42, no 3, p. 307-323Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: Studies of language in children with mild-to-moderate hearing impairment (HI) indicate that they often have problems in phonological short-term memory (PSTM) and that they have linguistic weaknesses both in vocabulary and morphosyntax similar to children with specific language impairment (SLI). However, children with HI may be more likely than children with SLI to acquire typical language skills as they get older. It has been suggested that the more persisting problems in children with SLI are due to a combination of factors: perceptual, cognitive and/or linguistic.

    AIMS: The main aim of this study was to explore language skills in children with HI in comparison with children with SLI, and how children with both HI and language impairment differ from those with non-impaired spoken language skills.

    METHODS & PROCEDURES: PSTM, output phonology, lexical ability, receptive grammar and verb morphology were assessed in a group of children with mild-to-moderate HI (n = 11) and a group of children with SLI (n = 12) aged 5 years 6 months to 9 years 0 months.

    OUTCOMES & RESULTS: The HI group tended to score higher than the SLI group on the language measures, although few of the differences were significant. The children with HI had their most obvious weaknesses in PSTM, vocabulary, receptive grammar and inflection of novel verbs. The subgroup of children with HI (five out of 10) who also showed evidence of grammatical output problems was significantly younger than the remaining children with HI. Correlation analysis showed that the language variables were not associated with age, whereas hearing level was associated with PSTM.

    CONCLUSIONS: Children with HI are at risk for at least a delay in lexical ability, receptive grammar and grammatical production. The problems seen in the HI group might be explained by their low-level perceptual deficit and weak PSTM. For the SLI group the impairment is more severe. From a clinical perspective an important conclusion is that the language development in children with even mild-to-moderate HI deserves attention and support.

  • 21.
    Hendrickx, Jan-Jaap
    et al.
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium; Department of Otorhinolaryngology, University of Oulu, Oulu, Finland; Department of Otorhinolaryngology, UMC Nijmegen, Nijmegen, Netherlands; Department of Otolaryngology, University Hospital Antwerp, Wilrijkstraat 10, Antwerp, Belgium.
    Huyghe, Jeroen R.
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Demeester, Kelly
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium.
    Topsakal, Vedat V.
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium.
    Van Eyken, Els
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Fransen, Erik
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Hannula, Samuli
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Jensen, Mona
    Department of Audiology, Bispebjerg Hospital, Copenhagen, Denmark.
    Tropitzsch, Anke S.
    Department of Otorhinolaryngology, University of Tübingen, Tübingen, Germany.
    Bonaconsa, Amanda
    Department of Oto-surgery, University Hospital Padova, Padova, Italy.
    Mazzoli, Manuela
    Department of Oto-surgery, University Hospital Padova, Padova, Italy.
    Espeso, Angeles
    Welsh Hearing Institute, Cardiff University, Cardiff, United Kingdom.
    Verbruggen, Katia
    Department of Otorhinolaryngology, University Hospital of Gent, Gent, Belgium.
    Huyghe, Joke
    Department of Otorhinolaryngology, University Hospital of Gent, Gent, Belgium.
    Huygen, Patrick L.M.
    Department of Otorhinolaryngology, UMC Nijmegen, Nijmegen, Netherlands.
    Kremer, Hannie Ph.
    Department of Otorhinolaryngology, UMC Nijmegen, Nijmegen, Netherlands.
    Kunst, S J
    Department of Otorhinolaryngology, UMC Nijmegen, Nijmegen, Netherlands.
    Manninen, Minna
    Department of Otorhinolaryngology, University of Tampere, Tampere, Finland.
    Diaz-Lacava, Amalia N.
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Germany.
    Steffens, Michael
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Germany.
    Parving, Agnete
    Department of Audiology, Bispebjerg Hospital, Copenhagen, Denmark.
    Pyykkö, Ilmari V.
    Department of Otorhinolaryngology, University of Tampere, Tampere, Finland.
    Dhooge, Ingeborg Johanna Maria
    Department of Otorhinolaryngology, University Hospital of Gent, Gent, Belgium.
    Stephens, Dafydd
    Welsh Hearing Institute, Cardiff University, Cardiff, United Kingdom.
    Orzan, Eva
    Department of Oto-surgery, University Hospital Padova, Padova, Italy.
    Pfister, Markus H.F.
    Department of Otorhinolaryngology, University of Tübingen, Tübingen, Germany.
    Bille, Michael
    Department of Audiology, Bispebjerg Hospital, Copenhagen, Denmark.
    Sorri, Martti J.
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Cremers, Cor Wrj R.J.
    Department of Otorhinolaryngology, UMC Nijmegen, Nijmegen, Netherlands.
    Van Laer, Lut
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Van Camp, Guy V.
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Wienker, Thomas F.
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Germany.
    Van de Heyning, Paul
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium.
    Familial aggregation of tinnitus: a European multicentre study2007In: B-ENT, ISSN 1781-782X, Vol. 3, no Suppl 7, p. 51-60Article in journal (Refereed)
    Abstract [en]

    INTRODUCTION AND AIM: Tinnitus is a common condition affecting approximately 20% of the older population. There is increasing evidence that changes in the central auditory system following cochlear malfunctioning are responsible for tinnitus. To date, few investigators have studied the influence of genetic factors on tinnitus. The present report investigates the presence of a familial effect in tinnitus subjects.

    METHODS: In a European multicentre study, 198 families were recruited in seven European countries. Each family had at least 3 siblings. Subjects were screened for causes of hearing loss other than presbyacusis by clinical examination and a questionnaire. The presence of tinnitus was evaluated with the question "Nowadays, do you ever get noises in your head or ear (tinnitus) which usually last longer than five minutes". Familial aggregation was tested using three methods: a mixed model approach, calculating familial correlations, and estimating the risk of a subject having tinnitus if the disorder is present in another family member.

    RESULTS: All methods demonstrated a significant familial effect for tinnitus. The effect persisted after correction for the effect of other risk factors such as hearing loss, gender and age. The size of the familial effect is smaller than that for age-related hearing impairment, with a familial correlation of 0.15.

    CONCLUSION: The presence of a familial effect for tinnitus opens the door to specific studies that can determine whether this effect is due to a shared familial environment or the involvement of genetic factors. Subsequent association studies may result in the identification of the factors responsible. In addition, more emphasis should be placed on the effect of role models in the treatment of tinnitus.

  • 22.
    Hendrickx, Jan-Jaap
    et al.
    University Department of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, University of Antwerp, Antwerp, Belgium; Department of Medical Genetics, University of Antwerp, Antwerp, Belgium; StatUa Statistics Center, University of Antwerp, Antwerp, Belgium; Department of Otorhinolaryngology, University Hospital of Gent, Gent, Belgium.
    Huyghe, Jeroen R.
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Topsakal, Vedat
    University Department of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, University of Antwerp, Antwerp, Belgium.
    Demeester, Kelly
    University Department of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, University of Antwerp, Antwerp, Belgium.
    Wienker, Thomas F.
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
    Laer, Lut Van
    Eyken, Els Van
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Fransen, Erik
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium; StatUa Statistics Center, University of Antwerp, Antwerp, Belgium.
    Mäki-Torkko, Elina
    Institute of Clinical Medicine, Department of Otorhinolaryngology, University of Oulu, Finland.
    Hannula, Samuli
    Institute of Clinical Medicine, Department of Otorhinolaryngology, University of Oulu, Finland.
    Parving, Agnete
    Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Northern Ostrobothnia Hospital District, Finland.
    Jensen, Mona
    Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Northern Ostrobothnia Hospital District, Finland.
    Tropitzsch, Anke
    Department of Audiology, Bispebjerg Hospital, Copenhagen, Denmark.
    Bonaconsa, Amanda
    Department of Otorhinolaryngology, University of Tubingen, Tubingen, Germany.
    Mazzoli, Manuela
    Department of Otorhinolaryngology, University of Tubingen, Tubingen, Germany.
    Espeso, Angeles
    Department of Otosurgery, University Hospital Padova, Padova, Italy.
    Verbruggen, Katja
    Welsh Hearing Institute, University Hospital of Wales, Cardiff, United Kingdom.
    Huyghe, Joke
    Welsh Hearing Institute, University Hospital of Wales, Cardiff, United Kingdom.
    Huygen, Patrick L. M.
    Department of Otorhinolaryngology, University Hospital of Gent, Gent, Belgium; Department of Otorhinolaryngology, Head and Neck Surgery, Radboud Universiteit Nijmegen Medical Center, Netherlands.
    Kremer, Hannie
    Department of Otorhinolaryngology, University Hospital of Gent, Gent, Belgium; Department of Otorhinolaryngology, Head and Neck Surgery, Radboud Universiteit Nijmegen Medical Center, Netherlands; Donders Institute for Brain, Cognition and Behaviour, Nijmegen Center for Molecular Life Sciences, Radboud University Nijmegen, Nijmegen, Netherlands.
    Kunst, Sylvia J.
    Department of Otorhinolaryngology, University Hospital of Gent, Gent, Belgium; Department of Otorhinolaryngology, Head and Neck Surgery, Radboud Universiteit Nijmegen Medical Center, Netherlands.
    Diaz-Lacava, Amalia N.
    Pharmacoepidemiology in Neurodegenerative Disorders, German Center for Neurodegenerative Diseases (DZNE), Bonn, Germany; Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
    Steffens, Michael
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
    Pyykkö, Ilmari
    Department of Otorhinolaryngology, University of Tampere, Tampere, Finland.
    Dhooge, Ingeborg
    Welsh Hearing Institute, University Hospital of Wales, Cardiff, United Kingdom.
    Stephens, Dafydd
    Department of Otosurgery, University Hospital Padova, Padova, Italy.
    Orzan, Eva
    Audiology and Otolaryngology Unit, Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy.
    Pfister, Markus H. F.
    Department of Audiology, Bispebjerg Hospital, Copenhagen, Denmark.
    Bille, Michael
    Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Northern Ostrobothnia Hospital District, Finland.
    Sorri, Martti
    Institute of Clinical Medicine, Department of Otorhinolaryngology, University of Oulu, Finland.
    Cremers, Cor W. R. J.
    Department of Otorhinolaryngology, University Hospital of Gent, Gent, Belgium; Department of Otorhinolaryngology, Head and Neck Surgery, Radboud Universiteit Nijmegen Medical Center, Netherlands.
    Camp, Guy Van
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    de Heyning, Paul Van
    University Department of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, University of Antwerp, Antwerp, Belgium.
    Familial aggregation of pure tone hearing thresholds in an aging European population2013In: Otology and Neurotology, ISSN 1531-7129, E-ISSN 1537-4505, Vol. 34, no 5, p. 838-844Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: To investigate the familial correlations and intraclass correlation of age-related hearing impairment (ARHI) in specific frequencies. In addition, heritability estimates were calculated.

    STUDY DESIGN: Multicenter survey in 8 European centers.

    SUBJECTS: One hundred ninety-eight families consisting of 952 family members, screened by otologic examination and structured interviews. Subjects with general conditions, known to affect hearing thresholds or known otologic cause were excluded from the study.

    RESULTS: We detected familial correlation coefficients of 0.36, 0.37, 0.36, and 0.30 for 0.25, 0.5, 1, and 2 kHz, respectively, and correlation coefficients of 0.20 and 0.18 for 4 and 8 kHz, respectively. Variance components analyses showed that the proportion of the total variance attributable to family differences was between 0.32 and 0.40 for 0.25, 0.5, 1, and 2 kHz and below 0.20 for 4 and 8 kHz. When testing for homogeneity between sib pair types, we observed a larger familial correlation between female than male subjects. Heritability estimates ranged between 0.79 and 0.36 across the frequencies.

    DISCUSSION: Our results indicate that there is a substantial shared familial effect in ARHI. We found that familial aggregation of ARHI is markedly higher in the low frequencies and that there is a trend toward higher familial aggregation in female compared with male subjects.

  • 23.
    Hesser, Hugo
    et al.
    Department of Behavioural Sciences and Learning, Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    Gustafsson, Tore
    Department of Behavioural Sciences and Learning, Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    Lundén, Charlotte
    Department of Behavioural Sciences and Learning, Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    Henrikson, Oskar
    Department of Behavioural Sciences and Learning, Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    Fattahi, Kidjan
    Department of Behavioural Sciences and Learning, Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    Johnsson, Erik
    Department of Behavioural Sciences and Learning, Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    Zetterqvist Westin, Vendela
    Department of Behavioural Sciences and Learning, Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    Carlbring, Per
    Department of Psychology, Umeå University, Umeå, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Clinical and Experimental Medicine, Division of Technical Audiology, Linköping University, Linköping, Sweden; Department of ENT-Head Neck Surgery UHL, County Council of Östergötland, Linköping, Sweden.
    Kaldo, Viktor
    Department of Clinical Neuroscience, Psychiatric Section, Karolinska Institutet, Stockholm, Sweden.
    Andersson, Gerhard
    Department of Behavioural Sciences and Learning, Swedish Institute for Disability Research, Linköping University, Linköping, Sweden; Department of Clinical Neuroscience, Psychiatric Section, Karolinska Institutet, Stockholm, Sweden.
    A randomized controlled trial of Internet-delivered cognitive behavior therapy and acceptance and commitment therapy in the treatment of tinnitus2012In: Journal of Consulting and Clinical Psychology, ISSN 0022-006X, E-ISSN 1939-2117, Vol. 80, no 4, p. 649-661Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: Our aim in this randomized controlled trial was to investigate the effects on global tinnitus severity of 2 Internet-delivered psychological treatments, acceptance and commitment therapy (ACT) and cognitive behavior therapy (CBT), in guided self-help format.

    METHOD: Ninety-nine participants (mean age = 48.5 years; 43% female) who were significantly distressed by tinnitus were recruited from the community. Participants were randomly assigned to CBT (n = 32), ACT (n = 35), or a control condition (monitored Internet discussion forum; n = 32), and they were assessed with standardized self-report measures (Tinnitus Handicap Inventory; Hospital Anxiety and Depression Scale; Quality of Life Inventory; Perceived Stress Scale; Tinnitus Acceptance Questionnaire) at pre-, posttreatment (8 weeks), and 1-year follow-up.

    RESULTS: Mixed-effects linear regression analysis of all randomized participants showed significant effects on the primary outcome (Tinnitus Handicap Inventory) for CBT and for ACT compared with control at posttreatment (95% CI [-17.03, -2.94], d = 0.70, and 95% CI [-16.29, -2.53], d = 0.68, respectively). Within-group effects were substantial from pretreatment through 1-year-follow-up for both treatments (95% CI [-44.65, -20.45], d = 1.34), with no significant difference between treatments (95% CI [-14.87, 11.21], d = 0.16).

    CONCLUSIONS: Acceptance-based procedures may be a viable alternative to traditional CBT techniques in the management of tinnitus. The Internet can improve access to psychological interventions for tinnitus.

  • 24.
    Hjertman, Heléne
    et al.
    Department of Otorhinolaryngology in Östergötland, Sweden; Department of Biomedical and Clinical Sciences, Linköping University, Sweden; Linnaeus Centre HEAD and Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    Hällgren, Mathias
    Department of Otorhinolaryngology in Östergötland, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Linnaeus Centre HEAD and Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    Stenfelt, Stefan
    Department of Biomedical and Clinical Sciences, Linköping University, Sweden; Linnaeus Centre HEAD and Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    The Swedish hearing in noise test for children, HINT-C2021In: International Journal of Pediatric Otorhinolaryngology, ISSN 0165-5876, E-ISSN 1872-8464, Vol. 141, article id 110509Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: The aim of this study was to develop and evaluate a Swedish version of the Hearing In Noise Test for Children (HINT-C).

    DESIGN: In the first part, the Swedish HINT lists for adults was evaluated by children at three signal to noise ratios (SNRs), -4, -1 and +2 dB. Lists including sentences not reaching 50% recognition at +2 dB SNR were excluded and the rest constituted the HINT-C. In the second part, HINT-C was evaluated in children and adults using an adaptive procedure to determine the SNR for 50% correctly repeated sentences. Study Sample In the first part, 112 children aged 6-11 years participated while another 28 children and 9 adults participated in the second part.

    RESULTS: Eight out of 24 tested adult HINT lists did not reach the inclusion criteria. The remaining 16 lists formed the Swedish HINT-C which was evaluated in children 6-11 years old. A regression analysis showed that the predicted SNR threshold (dB) was 0.495-0.365*age (years + months/12) and the children reached the mean adult score at an age of 10.5 years.

    CONCLUSIONS: A Swedish version of HINT-C was developed and evaluated in children six years and older.

  • 25.
    Huttunen, Kerttu
    et al.
    Faculty of Humanities, Logopedics, and Child Language Research Center, University of Oulu, Finland; PEDEGO Research Unit, University of Oulu, Finland; MRC Oulu, Oulu, Finland; Oulu University Hospital, Department of Otorhinolaryngology, Head and Neck Surgery, Oulu, Finland.
    Erixon, Elsa
    Uppsala University, Department of Surgical Sciences, Section of Otorhinolaryngology and Head and Neck Surgery, Uppsala, Sweden.
    Löfkvist, Ulrika
    University of Oslo, Department of Special Needs Education, Oslo, Norway; Karolinska Institute, Department of Clinical Science, Intervention and Technology, Stockholm, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Audiological Research Center.
    The impact of permanent early-onset unilateral hearing impairment in children: A systematic review2019In: International Journal of Pediatric Otorhinolaryngology, ISSN 0165-5876, E-ISSN 1872-8464, Vol. 120, p. 173-183Article, review/survey (Refereed)
    Abstract [en]

    Background: Decision-making on treatment and (re)habilitation needs to be based on clinical expertise and scientific evidence. Research evidence for the impact of permanent unilateral hearing impairment (UHI) on children's development has been mixed and, in some of the reports, based on fairly small, heterogeneous samples. Additionally, treatment provided has been highly variable, ranging from no action taken or watchful waiting up to single-sided cochlear implantation. Published information about the effects of treatment has also been heterogeneous. Moreover, earlier reviews and meta-analyses published on the impact of UHI on children's development have generally focused on select areas of development.

    Objectives: This systematic review aimed to summarize the impact of children's congenital or early onset unilateral hearing impairment on listening and auditory skills, communication, speech and language development, cognitive development, educational achievements, psycho-social development, and quality of life.

    Methods: Literature searches were performed to identify reports published from inception to February 16th, 2018 with the main electronic bibliographic databases in medicine, psychology, education, and speech and hearing sciences as the data sources. PubMed, CINALH, ERIC, LLBA, PsychINFO, and ISI Web of Science were searched for unilateral hearing impairment with its synonyms and consequences of congenital or early onset unilateral hearing impairment. Eligible were articles written in English, German, or Swedish on permanent unilateral hearing impairments that are congenital or with onset before three years of age. Hearing impairment had to be of at least a moderate degree with PTA >= 40 dB averaged over frequencies 0.5 to 2 or 0.5-4 kHz, hearing in the contralateral ear had to have PTA(0.5-2 kHz) or PTA(0.5-4 kHz) <= 20 dB, and consequences of unilateral hearing impairment needed to be reported in an unanimously defined population in at least one of the areas the review focused on.

    Four researchers independently screened 1618 abstracts and 566 full-text articles for evaluation of study eligibility. Eligible full-text articles were then reviewed to summarize the results and assess the quality of evidence. Additionally, data from 13 eligible case and multi-case studies, each having less than 10 participants, were extracted to summarize their results.

    Quality assessment of evidence was made adapting the Grades of Recommendations, Assessment, Development, and Evaluation (GRADE) process, and reporting of the results adheres to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standards.

    Results: Three articles with the quality of evidence graded as very-low to low, fulfilled the eligibility criteria set. Due to the heterogeneity of the articles, only a descriptive summary could be generated from the results. Unilateral hearing impairment was reported to have a negative impact on preverbal vocalization of infants and on sound localization and speech perception both in quiet and in noise.

    Conclusions: No high-quality studies of consequences of early-onset UHI in children were found. Inconsistency in assessing and reporting outcomes, the relatively small number of participants, low directness of evidence, and the potential risk of confounding factors in the reviewed studies prevented any definite conclusions. Further well-designed prospective research using larger samples is warranted on this topic.

  • 26.
    Huyghe, Jeroen R.
    et al.
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Fransen, Erik
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium; StatUA Statistics Center, University of Antwerp, Antwerp, Belgium.
    Hannula, Samuli
    Institute of Clinical Medicine, Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Van Laer, Lut
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Van Eyken, Els
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Institute of Clinical Medicine, Department of Otorhinolaryngology, University of Oulu, Oulu, Finland; Department of Clinical and Experimental Medicine/Technical Audiology, Linköping University, Linköping, Sweden.
    Aikio, Pekka
    Thule Institute, University of Oulu, Oulu, Finland.
    Sorri, Martti
    Institute of Clinical Medicine, Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Huentelman, Matthew J.
    Neurogenomics Division, Translational Genomics Research Institute, Phoenix AZ, USA.
    Van Camp, Guy
    Department of Medical Genetics, University of Antwerp,Antwerp, Belgium.
    A genome-wide analysis of population structure in the Finnish Saami with implications for genetic association studies2011In: European Journal of Human Genetics, ISSN 1018-4813, E-ISSN 1476-5438, Vol. 19, no 3, p. 347-352Article in journal (Refereed)
    Abstract [en]

    The understanding of patterns of genetic variation within and among human populations is a prerequisite for successful genetic association mapping studies of complex diseases and traits. Some populations are more favorable for association mapping studies than others. The Saami from northern Scandinavia and the Kola Peninsula represent a population isolate that, among European populations, has been less extensively sampled, despite some early interest for association mapping studies. In this paper, we report the results of a first genome-wide SNP-based study of genetic population structure in the Finnish Saami. Using data from the HapMap and the human genome diversity project (HGDP-CEPH) and recently developed statistical methods, we studied individual genetic ancestry. We quantified genetic differentiation between the Saami population and the HGDP-CEPH populations by calculating pair-wise F(ST) statistics and by characterizing identity-by-state sharing for pair-wise population comparisons. This study affirms an east Asian contribution to the predominantly European-derived Saami gene pool. Using model-based individual ancestry analysis, the median estimated percentage of the genome with east Asian ancestry was 6% (first and third quartiles: 5 and 8%, respectively). We found that genetic similarity between population pairs roughly correlated with geographic distance. Among the European HGDP-CEPH populations, F(ST) was smallest for the comparison with the Russians (F(ST)=0.0098), and estimates for the other population comparisons ranged from 0.0129 to 0.0263. Our analysis also revealed fine-scale substructure within the Finnish Saami and warns against the confounding effects of both hidden population structure and undocumented relatedness in genetic association studies of isolated populations.

  • 27.
    Huyghe, Jeroen R.
    et al.
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Fransen, Erik
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Hannula, Samuli
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Van Laer, Lut
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Van Eyken, Els
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Lysholm-Bernacchi, Alana
    Neurogenomics Division, Translational Genomics Research Institute, Phoenix AZ, USA.
    Aikio, Pekka
    Thule Institute, University of Oulu, Oulu, Finland.
    Stephan, Dietrich A.
    Neurogenomics Division, Translational Genomics Research Institute, Phoenix AZ, USA.
    Sorri, Martti
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Huentelman, Matthew J.
    Neurogenomics Division, Translational Genomics Research Institute, Phoenix AZ, USA.
    Van Camp, Guy
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Genome-wide SNP analysis reveals no gain in power for association studies of common variants in the Finnish Saami2010In: European Journal of Human Genetics, ISSN 1018-4813, E-ISSN 1476-5438, Vol. 18, no 5, p. 569-574Article in journal (Refereed)
    Abstract [en]

    The Saami from Fennoscandia are believed to represent an ancient, genetically isolated population with no evidence of population expansion. Theoretical work has indicated that under this demographic scenario, extensive linkage disequilibrium (LD) is generated by genetic drift. Therefore, it has been suggested that the Saami would be particularly suited for genetic association studies, offering a substantial power advantage and allowing more economic study designs. However, no study has yet assessed this claim. As part of a GWAS for a complex trait, we evaluated the relative power for association studies of common variants in the Finnish Saami. LD patterns in the Saami were very similar to those in the non-African HapMap reference panels. Haplotype diversity was reduced and, on average, levels of LD were higher in the Saami as compared with those in the HapMap panels. However, using a 'hidden' SNP approach we show that this does not translate into a power gain in association studies. Contrary to earlier claims, we show that for a given set of common SNPs, genomic coverage attained in the Saami is similar to that in the non-African HapMap panels. Nevertheless, the reduced haplotype diversity could potentially facilitate gene identification, especially if multiple rare variants play a role in disease etiology. Our results further indicate that the HapMap is a useful resource for genetic studies in the Saami.

  • 28.
    Huyghe, Jeroen R.
    et al.
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Van Laer, Lut
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Hendrickx, Jan-Jaap
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium; Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands.
    Fransen, Erik
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Demeester, Kelly
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium.
    Topsakal, Vedat
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium.
    Kunst, Sylvia
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands.
    Manninen, Minna
    Department of Otorhinolaryngology, University of Tampere, Tampere, Finland.
    Jensen, Mona
    Department of Audiology, Bispebjerg Hospital, Copenhagen, Denmark.
    Bonaconsa, Amanda
    Department of Oto-surgery, University Hospital Padova, Padova, Italy.
    Mazzoli, Manuela
    Department of Oto-surgery, University Hospital Padova, Padova, Italy.
    Baur, Manuela
    Department of Otorhinolaryngology, University of Tübingen, Tübingen, Germany.
    Hannula, Samuli
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Espeso, Angeles
    Welsh Hearing Institute, Cardiff University, Cardiff, UK.
    Van Eyken, Els
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Flaquer, Antonia
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
    Becker, Christian
    Cologne Center for Genomics (CCG) and Institute for Genetics, University of Cologne, Cologne, Germany.
    Stephens, Dafydd
    Welsh Hearing Institute, Cardiff University, Cardiff, UK.
    Sorri, Martti
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Orzan, Eva
    Department of Oto-surgery, University Hospital Padova, Padova, Italy.
    Bille, Michael
    Department of Audiology, Bispebjerg Hospital, Copenhagen, Denmark.
    Parving, Agnete
    Department of Audiology, Bispebjerg Hospital, Copenhagen, Denmark.
    Pyykkö, Ilmari
    Department of Otorhinolaryngology, University of Tampere, Tampere, Finland.
    Cremers, Cor W. R. J.
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands.
    Kremer, Hannie
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, NL-6500 HB Nijmegen, the Netherlands; Nijmegen Centre for Molecular Life Sciences, Nijmegen, The Netherlands.
    Van de Heyning, Paul H.
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium.
    Wienker, Thomas F.
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
    Nürnberg, Peter
    Cologne Center for Genomics (CCG) and Institute for Genetics, University of Cologne, Cologne, Germany; Cologne Excellence Cluster on Cellular Stress Responses in Aging-associated Diseases (CECAD), University of Cologne, D-50674Cologne, Germany.
    Pfister, Markus
    Department of Otorhinolaryngology, University of Tübingen, Tübingen, Germany.
    Van Camp, Guy
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Genome-wide SNP-based linkage scan identifies a locus on 8q24 for an age-related hearing impairment trait2008In: American Journal of Human Genetics, ISSN 0002-9297, E-ISSN 1537-6605, Vol. 83, no 3, p. 401-407Article in journal (Refereed)
    Abstract [en]

    Age-related hearing impairment (ARHI), or presbycusis, is a very common multifactorial disorder. Despite the knowledge that genetics play an important role in the etiology of human ARHI as revealed by heritability studies, to date, its precise genetic determinants remain elusive. Here we report the results of a cross-sectional family-based genetic study employing audiometric data. By using principal component analysis, we were able to reduce the dimensionality of this multivariate phenotype while capturing most of the variation and retaining biologically important features of the audiograms. We conducted a genome-wide association as well as a linkage scan with high-density SNP microarrays. Because of the presence of genetic population substructure, association testing was stratified after which evidence was combined by meta-analysis. No association signals reaching genome-wide significance were detected. Linkage analysis identified a linkage peak on 8q24.13-q24.22 for a trait correlated to audiogram shape. The signal reached genome-wide significance, as assessed by simulations. This finding represents the first locus for an ARHI trait.

  • 29.
    Ibertsson, Tina
    et al.
    Department of Logopedics, Phoniatrics and Audiology, Clinical Sciences, Lund University, Lund, Sweden.
    Hansson, Kristina
    Department of Logopedics, Phoniatrics and Audiology, Clinical Sciences, Lund University, Lund, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Otolaryngology, Section of Audiology, Linköping University Hospital, Linköping, Sweden.
    Willstedt-Svensson, Ursula
    Department of Logopedics, Phoniatrics and Audiology, Clinical Sciences, Lund University, Lund, Sweden.
    Sahlen, Birgitta
    Department of Logopedics, Phoniatrics and Audiology, Clinical Sciences, Lund University, Lund, Sweden.
    Deaf teenagers with cochlear implants in conversation with hearing peers2009In: International journal of language and communication disorders, ISSN 1368-2822, E-ISSN 1460-6984, Vol. 44, no 3, p. 319-337Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: This study investigates the use of requests for clarification in conversations between teenagers with a cochlear implant (CI) and hearing peers. So far very few studies have focused on conversational abilities in children with CI.

    AIMS: The aim was to explore co-construction of dialogue in a referential communication task and the participation of the teenagers with CI in comparison with individually matched hearing children and teenagers (HC) by studying the use of requests for clarification.

    METHODS & PROCEDURES: Sixteen conversational pairs participated: eight pairs consisting of a child with CI and his/her hearing conversational partner (CIP); and eight pairs consisting of an HC and a conversational partner (HCP). The conversational pairs were videotaped while carrying out a referential communication task requiring the description of two sets of pictures depicting faces. The dialogues were transcribed and analysed with respect to the number of words and turns, the time it took for each pair to complete the tasks, and the occurrence and different types of requests for clarification that were used in each type of conversational pair and in each type of dialogue.

    OUTCOMES & RESULTS: The main finding was that the teenagers with CI produced significantly more requests for clarification than the HCs. The most frequently used type of request for clarification in all dialogues was request for confirmation of new information. Furthermore, there was a trend for the teenagers with CI to use this type of request more often than the HC. In contrast, the teenagers with CI used significantly fewer requests for confirmation of already given information and fewer requests for elaboration than the HC.

    CONCLUSIONS & IMPLICATIONS: The deaf teenagers with CI in the study seem to be equally collaborative and responsible conversational partners as the hearing teenagers. The interpretation is that certain conditions in this study facilitate their participation in conversation. Such conditions might be a calm environment, a task that is structured and without time limits and that the partner is well known to the teenager with CI.

  • 30.
    Karlsson, Elin
    et al.
    Örebro University, School of Health Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Örebro Sweden; Swedish Institute of Disability Research (SIDR), Örebro University, Örebro Sweden .
    Manchaiah, Vinaya
    Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine, Aurora, Colorado, USA; UCHealth Hearing and Balance, University of Colorado Hospital, Aurora, Colorado, USA; Virtual Hearing Lab, Collaborative initiative between University of Colorado School of Medicine and University of Pretoria, Aurora, Colorado, USA; Department of Speech-Language Pathology and Audiology, University of Pretoria, South Africa; Department of Speech and Hearing, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, India .
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Audiological Research Centre.
    Granberg, Sarah
    Örebro University, School of Health Sciences. Audiological Research Centre.
    Gustafsson, Johanna
    Örebro University, School of Health Sciences. Audiological Research Centre.
    Widén, Stephen
    Örebro University, School of Health Sciences. Audiological Research Centre.
    Hearing and Functioning in Everyday Life Questionnaire (HFEQ): Construct validity, internal consistency reliability and floor and ceiling effectsManuscript (preprint) (Other academic)
  • 31.
    Karlsson, Elin
    et al.
    Örebro University, School of Health Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Örebro, Sweden; Swedish Institute of Disability Research (SIDR), Örebro University, Örebro, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.
    Granberg, Sarah
    Örebro University, School of Health Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.
    Widén, Stephen
    Örebro University, School of Health Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.
    Manchaiah, Vinaya
    Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine, Aurora, Colorado, USA; UCHealth Hearing and Balance, University of Colorado Hospital, Aurora, Colorado, USA; Virtual Hearing Lab, Collaborative Initiative Between University of Colorado School of Medicine and University of Pretoria, Aurora, Colorado, USA; Department of Speech-Language Pathology and Audiology, University of Pretoria, Pretoria, South Africa; Department of Speech and Hearing, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, India.
    Swarnalatha Nagaraj, Vinay
    Audiology Group, Institute of Neuromedicine and Movement Sciences, Norwegian University of Science and Technology, Trondheim, Norway.
    Pichora-Fuller, Kathleen M.
    Department of Psychology, University of Toronto, Mississauga, Ontario, Canada.
    Selb, Melissa
    ICF Research Branch and Swiss Paraplegic Research, Nottwil, Switzerland.
    Swanepoel, De Wet
    Virtual Hearing Lab, Collaborative Initiative Between University of Colorado School of Medicine and University of Pretoria, Aurora, Colorado, USA; Department of Speech-Language Pathology and Audiology, University of Pretoria, Pretoria, South Africa.
    Yerraguntla, Krishna
    Department of Speech and Hearing, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, India.
    Gustafsson, Johanna
    Örebro University, School of Health Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.
    Hearing and Functioning in Everyday Life Questionnaire: Development and Validation of an ICF-Based Instrument2023In: Ear and Hearing, ISSN 0196-0202, E-ISSN 1538-4667, Vol. 44, no 6, p. 1498-1506Article in journal (Refereed)
    Abstract [en]

    OBJECTIVES: Self-assessment instruments are commonly used in audiological rehabilitation. However, several studies highlight the lack of multidimensionality in existing outcome measures, with the consequence that they only partially capture aspects of functioning in everyday life for people living with hearing loss. This study aimed to develop and investigate the content validity of a self-assessment instrument based on the validated Brief International Classification of Functioning, Disability, and Health Core Set for Hearing Loss.

    DESIGN: The design was a two-part instrument development study. The first part focused on the item-generation process of the instrument, named the Hearing and Functioning in Everyday Life Questionnaire (HFEQ) during an experts' workshop. The second part focused on international content validation of the instrument using group interviews. Strategic sampling was used and 30 adults with hearing loss from India, South Africa, and the United States participated in the group interviews.

    RESULTS: The expert's workshop resulted in the first version of the HFEQ containing 30 items. The results from group interviews show that the content of the HFEQ was considered to be valid concerning its relevance, comprehensiveness, and comprehensibility. A majority (73%) of the HFEQ items were perceived by the participants as relevant and easy to comprehend. For the remaining 27% of the items, the content was perceived to be relevant in all countries, but some terms and expressions were reported to require rewording or clearer examples. These modifications will be made in the next step of the development process.

    CONCLUSION: Content validation of the HFEQ demonstrates promising results, with participants perceiving the content as relevant and comprehensible. Further psychometric validation is required to investigate other psychometric properties, such as construct validity and reliability. The HFEQ has the potential to become a valuable new instrument for assessing everyday functioning in people with hearing loss in audiological rehabilitation and in research.

  • 32.
    Karlsson, Elin
    et al.
    Örebro University, School of Health Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Örebro Sweden; Swedish Institute of Disability Research (SIDR), Örebro University, Örebro Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Audiological Research Centre.
    Granberg, Sarah
    Örebro University, School of Health Sciences. Audiological Research Centre.
    Widén, Stephen
    Örebro University, School of Health Sciences. Audiological Research Centre.
    Manchaiah, Vinaya
    Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine, Aurora, Colorado, USA; UCHealth Hearing and Balance, University of Colorado Hospital, Aurora, Colorado, USA; Virtual Hearing Lab, Collaborative initiative between University of Colorado School of Medicine and University of Pretoria, Aurora, Colorado, USA; Department of Speech-Language Pathology and Audiology, University of Pretoria, South Africa; Department of Speech and Hearing, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, India .
    Vinay, M.
    Audiology Group, Institute of Neuromedicine and Movement Sciences, Norwegian University of Science and Technology, Trondheim, Norway.
    Pichora-Fuller, Kathleen
    Department of Psychology, University of Toronto, Mississauga, Ontario, Canada .
    Selb, Melissa
    ICF Research Branch and Swiss Paraplegic Research, Nottwil, Switzerland .
    Swanepoel, De Wet
    Virtual Hearing Lab, Collaborative initiative between University of Colorado School of Medicine and University of Pretoria, Aurora, Colorado, USA; Department of Speech-Language Pathology and Audiology, University of Pretoria, South Africa.
    Yerraguntla, Krishna
    Department of Speech and Hearing, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, India.
    Gustafsson, Johanna
    Örebro University, School of Health Sciences. Audiological Research Centre.
    Hearing and Functioning in Everyday Life Questionnaire (HFEQ): Development and Validation of an ICF-based InstrumentManuscript (preprint) (Other academic)
  • 33.
    Karlsson, Elin
    et al.
    Örebro University, School of Health Sciences. Faculty of Medicine and Health, Audiological Research Centre, Örebro University, Örebro, Sweden; Swedish Institute of Disability Research (SIDR), Örebro University, Örebro, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Faculty of Medicine and Health, Audiological Research Centre, Örebro University, Örebro, Sweden; Swedish Institute of Disability Research (SIDR), Örebro University, Örebro, Sweden.
    Widén, Stephen
    Örebro University, School of Health Sciences. Faculty of Medicine and Health, Audiological Research Centre, Örebro University, Örebro, Sweden; Swedish Institute of Disability Research (SIDR), Örebro University, Örebro, Sweden.
    Gustafsson, Johanna
    Örebro University, School of Health Sciences. Faculty of Medicine and Health, Audiological Research Centre, Örebro University, Örebro, Sweden; Swedish Institute of Disability Research (SIDR), Örebro University, Örebro, Sweden.
    Manchaiah, Vinaya
    Department of Speech and Hearing Sciences, Lamar University, Beaumont, TX, USA.
    Mahomed-Asmail, Faheema
    Department of Speech-Language Pathology and Audiology, University of Pretoria, Pretoria, South Africa.
    Swanepoel, De Wet
    Department of Speech-Language Pathology and Audiology, University of Pretoria, Pretoria, South Africa.
    Yerraguntla, Krishna
    Department of Speech and Hearing, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, India.
    Granberg, Sarah
    Örebro University, School of Health Sciences. Faculty of Medicine and Health, Audiological Research Centre, Örebro University, Örebro, Sweden; Swedish Institute of Disability Research (SIDR), Örebro University, Örebro, Sweden.
    Validation of the Brief International Classification of Functioning, Disability and Health (ICF) core set for hearing loss: an international multicentre study2021In: International Journal of Audiology, ISSN 1499-2027, E-ISSN 1708-8186, Vol. 60, no 6, p. 412-420Article in journal (Refereed)
    Abstract [en]

    Objective: Hearing loss (HL) affects the everyday functioning of millions of people worldwide. The Brief International Classification of Functioning Disability and Health (ICF) core sets for HL was developed to meet the complex health care needs of adults with HL. Because the brief core set for HL has not yet been validated internationally, this study aimed to investigate its validity from an international perspective.

    Design: A cross-sectional validation study based on data from structured interviews with adults with HL.

    Study sample: Participants (n = 571) from India, South Africa, Sweden and the US were included.

    Results: A six-factor solution explained 71% of the variance, focussing on issues related to communication, the social environment, participation in society, health care services, support, relationships and emotions (α = 0.915). Three ICF categories demonstrated low reliability - temperament and personality functions, seeing functions and school education.

    Conclusion: The Brief ICF core set for HL is valid for adults with HL internationally. However, to further increase its international validity, we recommend adding the categories d920 recreation and leisure and replacing d850 school education with the more inclusive block, d810-d839 education.

  • 34.
    Karlsson, Elin
    et al.
    Örebro University, School of Health Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Audiological Research Centre, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.
    Widén, Stephen
    Örebro University, School of Health Sciences.
    Gustafsson, Johanna
    Örebro University, School of Health Sciences.
    Manchaiah, Vinaya
    Department of Speech and Hearing Sciences, Lamar University, Beaumont, TX, USA.
    Mahomed-Asmail, Faheema
    Department of Speech-Language Pathology and Audiology, University of Pretoria, Pretoria, South Africa.
    Yerraguntla, Krishna
    Department of Speech and Hearing, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, India.
    Granberg, Sarah
    Örebro University, School of Health Sciences.
    Validation of the Brief (ICF) core set for hearing loss2021Conference paper (Other academic)
  • 35.
    Kytövuori, Laura
    et al.
    Medical Research Center Oulu, Oulu University Hospital, University of Oulu, Oulu, Finland; Research Unit of Clinical Neuroscience, University of Oulu, Oulu, Finland; Department of Neurology, Oulu University Hospital, Oulu, Finland.
    Hannula, Samuli
    Medical Research Center Oulu, Oulu University Hospital, University of Oulu, Oulu, Finland; Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Oulu, Finland; PEDEGO Research Unit, University of Oulu, Oulu, Finland.
    Mäki-Torkko, Elina
    Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Oulu, Finland; Department of Clinical and Experimental Medicine/Technical Audiology, Faculty of Health Sciences, Linköping University, Linköping, Sweden; Department of ENT-Head Neck Surgery, Region Östergötland, Linköping, Sweden.
    Sorri, Martti
    Medical Research Center Oulu, Oulu University Hospital, University of Oulu, Oulu, Finland; Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Oulu, Finland; PEDEGO Research Unit, University of Oulu, Oulu, Finland.
    Majamaa, Kari
    Medical Research Center Oulu, Oulu University Hospital, University of Oulu, Oulu, Finland; Research Unit of Clinical Neuroscience, University of Oulu, Oulu, Finland; Department of Neurology, Oulu University Hospital, Oulu, Finland.
    A nonsynonymous mutation in the WFS1 gene in a Finnish family with age-related hearing impairment2017In: Hearing Research, ISSN 0378-5955, E-ISSN 1878-5891, Vol. 355, p. 97-101Article in journal (Refereed)
    Abstract [en]

    Wolfram syndrome (WS) is caused by recessive mutations in the Wolfram syndrome 1 (WFS1) gene. Sensorineural hearing impairment (HI) is a frequent feature in WS and, furthermore, certain mutations in WFS1 cause nonsyndromic dominantly inherited low-frequency sensorineural HI. These two phenotypes are clinically distinct indicating that WFS1 is a reasonable candidate for genetic studies in patients with other phenotypes of HI. Here we have investigated, whether the variation in WFS1 has a pathogenic role in age-related hearing impairment (ARHI). WFS1 gene was investigated in a population sample of 518 Finnish adults born in 1938-1949 and representing variable hearing phenotypes. Identified variants were evaluated with respect to pathogenic potential. A rare mutation predicted to be pathogenic was found in a family with many members with impaired hearing. Twenty members were recruited to a segregation study and a detailed clinical examination. Heterozygous p.Tyr528His variant segregated completely with late-onset HI in which hearing deteriorated first at high frequencies and progressed to mid and low frequencies later in life. We report the first mutation in the WFS1 gene causing late-onset HI with audiogram configurations typical for ARHI. Monogenic forms of ARHI are rare and our results add WFS1 to the short list of such genes.

  • 36.
    Laugen, Nina Jakhelln
    et al.
    Department of Psychology, Norwegian University of Science and Technology (NTNU), Trondheim, Norway.
    Erixon, Elsa
    Department of Surgical Sciences, Uppsala University, Uppsala, Sweden.
    Huttunen, Kerttu
    Research Unit of Logopedics and Child Language Research Center, Faculty of Humanities, University of Oulu, Oulu, Finland; Department of Otorhinolaryngology, Head and Neck Surgery, Oulu University Hospital, Oulu, Finland; Medical Research Center Oulu, University of Oulu, Oulu, Finland.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences.
    Löfkvist, Ulrika
    Department of Public Health and Caring Sciences, Uppsala University, Uppsala, Sweden; Department of Clinical Science, Intervention and Technology (CLINTEC), Karolinska Institute, Stockholm, Sweden.
    Newborn Hearing Screening and Intervention in Children with Unilateral Hearing Impairment: Clinical Practices in Three Nordic Countries2021In: Journal of Clinical Medicine, E-ISSN 2077-0383, Vol. 10, no 21, article id 5152Article in journal (Refereed)
    Abstract [en]

    Studies have limitedly considered children with early-identified unilateral hearing impairment (UHI), and clinical practices regarding screening, diagnostics and habilitation in this group are rarely documented. In this study, routines for newborns with UHI from screening to diagnostics and habilitation were explored in Norway, Sweden and Finland. An online survey was sent to hospitals responsible for the hearing diagnostics of children requesting information about their practices regarding congenital UHI. Responses covered 95% of the children born in the three included countries. The results revealed large variations in ways of organising healthcare and in clinical decisions regarding hearing screening, diagnostics and habilitation of children with congenital UHI. Finally, implications for policy making and research are also discussed.

  • 37.
    Lohi, Venla
    et al.
    Institute of Clinical Medicine, Department of Otorhinolaryngology, University of Oulu, Oulu, Finland; Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Oulu, Finland.
    Hannula, Samuli
    Institute of Clinical Medicine, Department of Otorhinolaryngology, University of Oulu, Oulu, Finland; Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Oulu, Finland.
    Ohtonen, Pasi
    Departments of Surgery and Anesthesiology, Oulu University Hospital, Oulu, Finland.
    Sorri, Martti
    Institute of Clinical Medicine, Department of Otorhinolaryngology, University of Oulu, Oulu, Finland; Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Oulu, Finland.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Clinical and Experimental Medicine/Technical Audiology, Faculty of Health Sciences, Linköping University, Linköping, Sweden.
    Hearing impairment among adults: the impact of cardiovascular diseases and cardiovascular risk factors2015In: International Journal of Audiology, ISSN 1499-2027, E-ISSN 1708-8186, Vol. 54, no 4, p. 265-73Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: To investigate the influence of cardiovascular diseases on hearing impairment (HI) among adults. Furthermore, to seek other potential risk factors for HI, such as smoking, obesity, and socioeconomic class.

    DESIGN: A cross-sectional, unscreened, population-based, epidemiological study among adults.

    STUDY SAMPLE: The subjects (n = 850), aged 54-66 years, were randomly sampled from the population register. A questionnaire survey, an otological examination, and pure-tone audiometry were performed.

    RESULTS: Cardiovascular diseases did not increase the risk for HI in a propensity-score adjusted logistic regression model: OR 1.24, 95% CI 0.79 to 1.96 for HI defined by better ear hearing level (BEHL), and OR 1.48, 95% CI 0.96 to 2.28 for HI defined by worse ear hearing level (WEHL), in the 0.5-4 kHz frequency range. Heavy smoking is a risk factor for HI among men (BEHL: OR 1.96, WEHL: OR 1.88) and women (WEHL: OR 2.4). Among men, obesity (BEHL, OR 1.85) and lower socioeconomic class (BEHL: OR 2.79, WEHL: OR 2.28) are also risk factors for HI.

    CONCLUSION: No significant association between cardiovascular disease and HI was found.

  • 38.
    Lohi, Venla
    et al.
    PEDEGO Research Unit, University of Oulu, Oulu, Finland; Medical Research Center Oulu, Oulu University Hospital, University of Oulu, Oulu, Finland; Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Oulu, Finland.
    Ohtonen, Pasi
    Division of Operative Care and Medical Research Center Oulu, Oulu University Hospital and University of Oulu, Oulu, Finland.
    Aikio, Pekka
    Thule Institute, University of Oulu, Oulu, Finland.
    Sorri, Martti
    PEDEGO Research Unit, University of Oulu, Oulu, Finland; Medical Research Center Oulu, Oulu University Hospital, University of Oulu, Oulu, Finland.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. PEDEGO Research Unit, University of Oulu, Oulu, Finland; Medical Research Center Oulu, Oulu University Hospital, University of Oulu, Oulu, Finland; Örebro University Hospital, Örebro, Sweden.
    Hannula, Samuli
    PEDEGO Research Unit, University of Oulu, Oulu, Finland; Medical Research Center Oulu, Oulu University Hospital, University of Oulu,Oulu, Finland; Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Oulu, Finland.
    Hearing impairment is common among Saami adults in Northern Finland2017In: International Journal of Circumpolar Health, ISSN 1239-9736, E-ISSN 2242-3982, Vol. 76, no 1, article id 1398004Article in journal (Refereed)
    Abstract [en]

    The Saami are the only indigenous population in Europe and their traditional living area is northern Scandinavia. Hearing impairment (HI) among Saami has not been studied before. The objective was to investigate the presence and type of HI among Saami adults, aged 49-77 years (median age 61 years), living in northern Finland. In addition, the presence of self-reported hearing difficulties, difficulties to hear in background noise and tinnitus were studied. An epidemiological, cross-sectional study encompassing a structured interview, otological examination and audiometry was performed. Bilateral HI was present in 42.9% of men and 29.4% of women, when HI was defined as a pure tone average (PTA) of at least 20 dB hearing level (HL) or more at the frequencies of 0.5, 1, 2 and 4 kHz. In one or both ears (worse ear hearing level, WEHL0.5,1,2,4≥20 dB HL) HI was present in 61.8% of men and 42.2% of women. Sensorineural high frequency hearing impairment was found to be most common. Nearly half (46.9%) of the study subjects reported hearing problems and more than half (55.6%) reported difficulties in following conversation in background noise. Measured HI and subjective hearing difficulties are common among the Saami adults. The healthcare personnel working in this area should be aware of the hearing problems of the Saami population.

    ABBREVIATIONS: ARHI, Age-related hearing impairment; PTA, Pure tone average; HI, Hearing impairment; HL, Hearing level; BEHL, Better ear hearing level; WEHL, Worse ear hearing level; CI, Confidence interval.

  • 39.
    Lohi, Venla
    et al.
    Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Oulu, Finland; PEDEGO Research Unit, University of Oulu, Finland and Medical Research Centre Oulu, Oulu, Finland.
    Ohtonen, Pasi
    Division of Operative Care and Medical Research Centre Oulu, Oulu University Hospital and University of Oulu, Oulu, Finland.
    Sorri, Martti
    PEDEGO Research Unit, University of Oulu, Finland and Medical Research Centre Oulu, Oulu, Finland.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. PEDEGO Research Unit, University of Oulu, Finland and Medical Research Centre Oulu, Oulu, Finland.
    Hannula, Samuli
    Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Oulu, Finland; PEDEGO Research Unit, University of Oulu, Finland and Medical Research Centre Oulu, Oulu, Finland.
    Prevalence and incidence of hearing impairment among adults: a 13-year follow-up study2021In: International Journal of Audiology, ISSN 1499-2027, E-ISSN 1708-8186, Vol. 60, no 9, p. 687-694Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: The aim of this study was to investigate the prevalence and incidence of hearing impairment (HI) in a longitudinal setting among adults.

    DESIGN: An unscreened, population-based epidemiological 13-year follow-up study. Study sample: 850 randomly sampled 54 to 66-year-old baseline participants, of whom 559 participated in the follow-up study at the age of 68 to 79 years. A questionnaire-based interview, an otological examination and pure-tone audiometry were performed.

    RESULTS: The overall prevalence of HI was 70.3%, defined by better ear hearing level (BEHL)  ≥ 20 dB in the 0.5-4 kHz frequency range. The prevalence was higher among men (78.6%) than among women (63.7%). The overall incidence rate for HI was 45.8 per 1000 person years and the 13-year cumulative incidence was 60.9%. The incidence was higher among men and older participants.

    CONCLUSION: HI is highly prevalent and incident among older adults in Northern Finland.

  • 40.
    Lohi, Venla
    et al.
    Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Oulu, Finland; PEDEGO Research Unit, University of Oulu, Oulu, Finland; Medical Research Center Oulu, Oulu, Finland.
    Ohtonen, Pasi
    Division of Operative Care and Oulu University Hospital and Research Unit of Surgery, Anesthesia and Intensive care, University of Oulu, Oulu, Finland.
    Sorri, Martti
    PEDEGO Research Unit, University of Oulu, Oulu, Finland; Medical Research Center Oulu, Oulu, Finland.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. PEDEGO Research Unit, University of Oulu, Oulu, Finland; Medical Research Center Oulu, Oulu, Finland; Audiological Research Center, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.
    Hannula, Samuli
    Department of Otorhinolaryngology and Head and Neck Surgery, Oulu University Hospital, Oulu, Finland; PEDEGO Research Unit, University of Oulu, Oulu, Finland; Medical Research Center Oulu, Oulu, Finland.
    The impact of cardiovascular diseases on hearing deterioration: a 13-year follow-up study2022In: International Journal of Audiology, ISSN 1499-2027, E-ISSN 1708-8186, Vol. 61, no 10, p. 826-831Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: To study the impact of cardiovascular diseases (CVDs) on hearing deterioration among ageing adults in a longitudinal setting. Furthermore, to describe the pure tone threshold changes at the 0.125-8 kHz frequency range over 13 years.

    DESIGN: A population-based follow-up study.

    STUDY SAMPLE: A random sample of 850 adults, of whom 559 participated in the follow-up study. Otological examination, a structured interview, and pure tone audiometry were conducted. Multivariate regression models were used to estimate the effect of CVD (participants had at least one cardiovascular condition) on hearing deterioration of the better ear hearing level (BEHL), defined as a change in the pure-tone average (PTA) of the frequencies 0.5, 1, 2, and 4 kHz and separately at the lower (0.125, 0.25, and 0.5 kHz) and higher (4, 6, and 8 kHz) frequencies.

    RESULTS: In the multivariable-adjusted analysis, the BEHL change at 13 years was 0.7 dB greater among participants with CVD (p = 0.3). The mean BEHL change during the 13-year follow-up was 12.0 dB (95% CI 11.4-12.6) among all participants.

    CONCLUSIONS: No significant association between CVD and hearing threshold changes was found.

  • 41.
    Lyxell, Björn
    et al.
    Swedish Institute for Disability Research, Linköping University, Linköping, Sweden; Department of Behavioural Sciences, Linköping University, Linköping, Sweden; Department of Behavioural Sciences, Linköping University, Linköping, Sweden.
    Sahlén, Birgitta
    Department of Clinical Sciences, Lund University, Lund, Sweden.
    Wass, Malin
    Swedish Institute for Disability Research, Linköping University, Linköping, Sweden; Department of Behavioural Sciences, Linköping University, Linköping, Sweden;.
    Ibertsson, Tina
    Department of Clinical Sciences, Lund University, Lund, Sweden.
    Larsby, Birgitta
    Swedish Institute for Disability Research, Linköping University, Linköping, Sweden; Department of Clinical and Experimental Medicine, Lund University, Lund, Sweden.
    Hällgren, Mathias
    Swedish Institute for Disability Research, Linköping University, Linköping, Sweden; Department of Clinical and Experimental Medicine, Lund University, Lund, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Behavioural Sciences, Linköping University, Linköping, Sweden.
    Cognitive development in children with cochlear implants: relations to reading and communication2008In: International Journal of Audiology, ISSN 1499-2027, E-ISSN 1708-8186, Vol. 47, no Suppl 2, p. S47-S52Article in journal (Refereed)
    Abstract [en]

    The purpose of the present article is to present an overview of a set of studies conducted in our own laboratory on cognitive and communicative development in children with cochlear implants (CI). The results demonstrate that children with CIs perform at significantly lower levels on the majority of the cognitive tasks. The exceptions to this trend are tasks with relatively lower demands on phonological processing. A fairly high proportion of the children can reach a level of reading comprehension that matches hearing children, despite the fact that they have relatively poor phonological skills. General working memory capacity is further correlated with the type of questions asked in a referential communication task. The results are discussed with respect to issues related to education and rehabilitation.

  • 42.
    Lyxell, Björn
    et al.
     Department of Behavioural Sciences and Learning, Linköping University, Sweden, Linköping University, Sweden;  Linnaeus Centre HEAD, The Swedish Institute for Disability Research, Linköping University, Sweden.
    Wass, Malin
     Department of Behavioural Sciences and Learning, Linköping University, Sweden, Linköping University, Sweden;  Linnaeus Centre HEAD, The Swedish Institute for Disability Research, Linköping University, Sweden.
    Sahlén, Birgitta
     Linnaeus Centre HEAD, The Swedish Institute for Disability Research, Linköping University, Sweden; Department of Clinical Sciences, Lund University, Sweden.
    Samuelsson, Christina
     Department of Clinical and Experimental Medicine, Linköping University, Sweden.
    Asker-Arnason, Lena
    Department of Clinical Sciences, Lund University, Sweden.
    Ibertsson, Tina
     Department of Clinical Sciences, Lund University, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences.  Linnaeus Centre HEAD, The Swedish Institute for Disability Research, Linköping University, Sweden; Department of Clinical and Experimental Medicine, Linköping University, Sweden.
    Larsby, Birgitta
     Linnaeus Centre HEAD, The Swedish Institute for Disability Research, Linköping University, Sweden;  Department of Clinical and Experimental Medicine, Linköping University, Sweden.
    Hällgren, Mathias
    Linnaeus Centre HEAD, The Swedish Institute for Disability Research, Linköping University, Sweden; Department of Clinical and Experimental Medicine, Linköping University, Sweden.
    Cognitive development, reading and prosodic skills in children with cochlear implants2009In: Scandinavian Journal of Psychology, ISSN 0036-5564, E-ISSN 1467-9450, Vol. 50, no 5, p. 463-474Article in journal (Refereed)
    Abstract [en]

    This report summarizes some of the results of studies in our laboratory exploring the development of cognitive, reading and prosodic skills in children with cochlear implantation (CI). The children with CI performed at significantly lower levels than the hearing comparison group on the majority of cognitive tests, despite showing levels of nonverbal ability. The differences between children with CI and hearing children were most pronounced on tasks with relatively high phonological processing demands, but they were not limited to phonological processing. Impairment of receptive and productive prosody was also evident in children with CI. Despite these difficulties, 75% of the children with CI reached a level of reading skill comparable to that of hearing children. The results are discussed with respect to compensation strategies in reading.

  • 43.
    Lyxell, Björn
    et al.
    The Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    Wass, Malin
    The Swedish Institute for Disability Research, Linköping University, Linköping, Sweden; .
    Sahlén, Birgitta
    Department of Clinical Sciencies, Lund University, Lund, Sweden.
    Uhlén, Inger
    Department of Clinical Sciencie, Intervention and Technology, Karolinska Institute, Stockholm, Sweden.
    Samuelsson, Christina
    Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden.
    Asker-Árnason, Lena
    Department of Clinical Sciencies, Lund University, Lund, Sweden.
    Ibertsson, Tina
    Department of Clinical Sciencies, Lund University, Lund, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. The Swedish Institute for Disability Research, Linköping University, Linköping, Sweden; Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden.
    Larsby, Birgitta
    The Swedish Institute for Disability Research, Linköping University, Linköping, Sweden; Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden.
    Hällgren, Mathias
    The Swedish Institute for Disability Research, Linköping University, Linköping, Sweden; Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden.
    Development of cognitive and reading skills in deaf children with CIs2011In: Cochlear Implants International, ISSN 1467-0100, E-ISSN 1754-7628, Vol. 12, no Suppl 1, p. S98-S100Article in journal (Refereed)
  • 44.
    Mackey, Allison R.
    et al.
    Karolinska Institutet, Department of Clinical Science Intervention and Technology, Division of Ear, Nose and Throat Diseases, Karolinska University Hospital, Huddinge, Stockholm, Sweden.
    Bussé, Andrea M. L.
    Department of Otorhinolaryngology and Head and Neck Surgery and Department of Ophthalmology, Erasmus University Medical Center, Rotterdam, The Netherlands.
    Del Vecchio, Valeria
    Department of Neuroscience, University of Padua, Bologna, Italy; Unit of Audiology, Department of Neuroscience, Reproductive Sciences and Dentistry, University of Naples Federico II, Naples, Italy.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Audiological Research Centre.
    Uhlén, Inger M.
    Karolinska Institutet, Department of Clinical Science Intervention and Technology, Division of Ear, Nose and Throat Diseases, Karolinska University Hospital, Huddinge, Stockholm, Sweden.
    Protocol and programme factors associated with referral and loss to follow-up from newborn hearing screening: a systematic review2022In: BMC Pediatrics, ISSN 1471-2431, E-ISSN 1471-2431, Vol. 22, no 1, article id 473Article, review/survey (Refereed)
    Abstract [en]

    BACKGROUND: An effective newborn hearing screening programme has low referral rate and low loss to follow-up (LTFU) rate after referral from initial screening. This systematic review identified studies evaluating the effect of protocol and programme factors on these two outcomes, including the screening method used and the infant group.

    METHODS: Five databases were searched (latest: April 2021). Included studies reported original data from newborn hearing screening and described the target outcomes against a protocol or programme level factor. Studies were excluded if results were only available for one risk condition, for each ear, or for < 100 infants, or if methodological bias was observed. Included studies were evaluated for quality across three domains: sample, screening and outcome, using modified criteria from the Ottawa-Newcastle and QUADAS-2 scales. Findings from the included studies were synthesised in tables, figures and text.

    RESULTS: Fifty-eight studies reported on referral rate, 8 on LTFU rate, and 35 on both. Only 15 studies defined LTFU. Substantial diversity in referral and LTFU rate was observed across studies. Twelve of fourteen studies that evaluated screening method showed lower referral rates with aABR compared to TEOAE for well babies (WB). Rescreening before hospital discharge and screening after 3 days of age reduced referral rates. Studies investigating LTFU reported lower rates for programmes that had audiologist involvement, did not require fees for step 2, were embedded in a larger regional or national programme, and scheduled follow-up in a location accessible to the families. In programmes with low overall LTFU, higher LTFU was observed for infants from the NICU compared to WB.

    CONCLUSION: Although poor reporting and exclusion of non-English articles may limit the generalisability from this review, key influential factors for referral and LTFU rates were identified. Including aABR in WB screening can effectively reduce referral rates, but it is not the only solution. The reported referral and LTFU rates vary largely across studies, implying the contribution of several parameters identified in this review and the context in which the programme is performed. Extra attention should be paid to infants with higher risk for hearing impairment to ensure their return to follow-up.

  • 45.
    Mäki-Torkko, Elina
    et al.
    Örebro University, School of Medical Sciences. University Hospital of Lund, Department of Otorhinolaryngology, Lund, Sweden.
    Brorsson, Bengt
    Statens Beredning for Medicinsk Utvärdering, Stockholm, Sweden.
    Davis, Adrian
    MRC Institute of Hearing Research, Nottingham, United Kingdom.
    Mair, Iain W. S.
    Ullevål Sykehus, Oslo, Norway.
    Myhre, Kurt I.
    Senter for Medisinsk Metodevurdering, Oslo, Norway.
    Roine, Risto P.
    Finnish Office for Health Care Technology Assessment, Helsinki, Finland.
    Parving, Agnete
    Department of Audiology, Bispebjerg Hospital, Copenhagen, Denmark.
    Rosenhall, Ulf
    Department of Audiology, Karolinska Hospital, Stockholm, Sweden.
    Sorri, Martti J.
    Department of Otorhinolaryngology, University of Oulu, Finland.
    Stilvén, Staffan
    Danish Institute for Health Technology Assessment, Copenhagen, Denmark.
    Hearing impairment among adults: extent of the problem and scientific evidence on the outcome of hearing aid rehabilitation2001In: Scandinavian audiology. Supplementum, ISSN 0107-8593, Vol. 30, no 54Article in journal (Refereed)
    Abstract [en]

    Scientific surveys on current and estimated prevalence of hearing impairment (HI) in adult populations (> or = 18 years of age) in Denmark, Finland, Norway, Sweden and the United Kingdom, and scientific reports on the outcome of hearing aid (HA) rehabilitation worldwide were reviewed. Only a few of the studies meet strict scientific criteria, and many locally clinically relevant studies cannot be generalized to larger populations. Population-based studies indicate an increase in prevalence of HI with age, but because of differences in study populations and available national population statistics, the studies do not allow reliable comparisons between countries or estimation of future prevalence of HI. Studies on HA prescription or outcomes do not provide uniform data in favour of non-linear amplification, but they do show some subject preference for the newer technology. No conclusions can be drawn regarding the degree of HI and the effects of amplification. The literature review alone gives only limited information regarding the extent of the problem of HI in adult populations in the target countries. Similarly, only a few studies on HA outcome meet strict scientific criteria and even fewer studies correlate rehabilitation outcome with the degree of HI, disability or handicap.

  • 46.
    Mäki-Torkko, Elina Margareetta
    et al.
    Örebro University Hospital. Department of Clinical and Experimental Medicine/Technical Audiology, Linköping University, Linköping, Sweden; Department of ENT-Head Neck Surgery, County Council of Östergötland, Linköping, Sweden; Linnaeus Centre HEAD, Linköping University, Linköping, Sweden.
    Vestergren, Sara
    Department of Behavioural Sciences and Learning, Linköping University, Linköping, Sweden.
    Harder, Henrik
    Department of ENT-Head Neck Surgery, County Council of Östergötland, Linköping, Sweden.
    Lyxell, Björn
    Linnaeus Centre HEAD, Linköping University, Linköping, Sweden; Department of Behavioural Sciences and Learning, Linköping University, Linköping, Sweden; Swedish Institute for Disability Research, Linköping University, Linköping, Sweden.
    From isolation and dependence to autonomy - expectations before and experiences after cochlear implantation in adult cochlear implant users and their significant others2015In: Disability and Rehabilitation, ISSN 0963-8288, E-ISSN 1464-5165, Vol. 37, no 6, p. 541-547Article in journal (Refereed)
    Abstract [en]

    Purpose: The aim of the study was to examine pre-operative expectations and the postoperative experiences related to cochlear implants (CI) in CI-users and their significant others.

    Methods: A questionnaire was used and the responses were analysed by means of The Qualitative Content Analysis. All adults implanted between 1992 and 2010, who had had their implants for a minimum of 12 months (n = 120) were contacted. Response rate was high (90.8%), and all-inclusive answers were received from 101 CI-users (84.2%).

    Results: The overall sense of increased well-being and life satisfaction was described as having lived in two different worlds, one with the auditory stimulation and one without. In the overall sense of increased well-being and satisfaction three interwoven subcategories, alienation - normality, fear - autonomy, and living a social life emerged. When CI-users and their significant others recalled the time prior to receiving the CI, a sense of fear was present with origins in the concern for the respondents' (CI-users) ability to cope and care independently in society. Conversely, after the implantation both parties emphasized the notion of a distinct transformation within the CI-user towards autonomy. Communication was highlighted as a large part of living social life.

    Conclusion: The CI increases well-being and satisfaction for both CI-users and their significant others, which is especially evident regarding enhanced autonomy, normality and living social life.

  • 47.
    Uusimaa, Johanna
    et al.
    Department of Paediatrics, University of Oulu, Oulu; Clinical Research Center, Oulu University Hospital, Oulu.
    Moilanen, Jukka S
    Department of Clinical Genetics, University of Oulu, Oulu; Institute of Medical Technology, University of Tampere, Tampere.
    Vainionpää, Leena
    Department of Paediatrics, University of Oulu, Oulu.
    Tapanainen, Päivi
    Department of Paediatrics, University of Oulu, Oulu.
    Lindholm, Päivi
    Department of Otorhinolaryngology, University of Oulu, Oulu; Department of Child Psychiatry, University of Oulu, Oulu.
    Nuutinen, Matti
    Department of Paediatrics, University of Oulu, Oulu.
    Löppönen, Tuija
    Department of Clinical Genetics, University of Oulu, Oulu; Department of Paediatrics, University of Kuopio, Kuopio, Finland.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Otorhinolaryngology, University of Oulu, Oulu; Department of Otorhinolaryngology, Linköping University Hospital, Linköping, Sweden.
    Rantala, Heikki
    Department of Paediatrics, University of Oulu, Oulu.
    Majamaa, Kari
    Clinical Research Center, Oulu University Hospital, Oulu; Department of Neurology, University of Oulu, Oulu; Department of Neurology, University of Turku, Turku, Finland.
    Prevalence, segregation, and phenotype of the mitochondrial DNA 3243A>G mutation in children2007In: Annals of Neurology, ISSN 0364-5134, E-ISSN 1531-8249, Vol. 62, no 3, p. 278-287Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: We studied the prevalence, segregation, and phenotype of the mitochondrial DNA 3243A>G mutation in children in a defined population in Northern Ostrobothnia, Finland.

    METHODS: Children with diagnoses commonly associated with mitochondrial diseases were ascertained. Blood DNA from 522 selected children was analyzed for 3243A>G. Children with the mutation were clinically examined. Information on health history before the age of 18 years was collected from previously identified adult patients with 3243A>G. Mutation segregation analysis in buccal epithelial cells was performed in mothers with 3243A>G and their children whose samples were analyzed anonymously.

    RESULTS: Eighteen children were found to harbor 3243A>G in a population of 97,609. A minimum estimate for the prevalence of 3243A>G was 18.4 in 100,000 (95% confidence interval, 10.9-29.1/100,000). Information on health in childhood was obtained from 37 adult patients with 3243A>G. The first clinical manifestations appearing in childhood were sensorineural hearing impairment, short stature or delayed maturation, migraine, learning difficulties, and exercise intolerance. Mutation analysis from 13 mothers with 3243A>G and their 41 children gave a segregation rate of 0.80. The mothers with heteroplasmy greater than 50% tended to have offspring with lower or equal heteroplasmy, whereas the opposite was true for mothers with heteroplasmy less than or equal to 50% (p = 0.0016).

    INTERPRETATION: The prevalence of 3243A>G is relatively high in the pediatric population, but the morbidity in children is relatively low. The random genetic drift model may be inappropriate for the transmission of the 3243A>G mutation.

  • 48.
    Van Eyken, Els
    et al.
    Department of Medical Genetics, University of Antwerp (UA), Antwerp, Belgium.
    Van Camp, Guy V.
    Department of Medical Genetics, University of Antwerp (UA), Antwerp, Belgium.
    Fransen, Erik
    Department of Medical Genetics, University of Antwerp (UA), Antwerp, Belgium.
    Topsakal, Vedat V.
    Department of Otorhinolaryngology, University Hospital of Antwerp (UZA), Antwerp, Belgium.
    Hendrickx, Jan Jaap
    Department of Otorhinolaryngology, University Hospital of Antwerp (UZA), Antwerp, Belgium.
    Demeester, Kelly
    Department of Otorhinolaryngology, University Hospital of Antwerp (UZA), Antwerp, Belgium.
    Van de Heyning, Paul
    Department of Otorhinolaryngology, University Hospital of Antwerp (UZA), Antwerp, Belgium.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Hannula, Samuli
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Sorri, Martti J.
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Jensen, Mona
    Department of Audiology, Bispebjerg Hospital (BBH), Copenhagen, Denmark.
    Parving, Agnete
    Department of Audiology, Bispebjerg Hospital (BBH), Copenhagen, Denmark.
    Bille, Michael
    Department of Audiology, Bispebjerg Hospital (BBH), Copenhagen, Denmark.
    Baur, Manuela
    Department of Otorhinolaryngology, University of Tuebingen, Tuebingen, Germany.
    Pfister, Markus H.F.
    Department of Otorhinolaryngology, University of Tuebingen, Tuebingen, Germany.
    Bonaconsa, Amanda
    Department of Oto-surgery, University Hospital Padova, Padova, Italy.
    Mazzoli, Manuela
    Department of Oto-surgery, University Hospital Padova, Padova, Italy.
    Orzan, Eva
    Department of Oto-surgery, University Hospital Padova, Padova, Italy.
    Espeso, Angeles
    Welsh hearing Institute, University Hospital of Wales, Cardiff, UK.
    Stephens, Dafydd
    Welsh hearing Institute, University Hospital of Wales, Cardiff, UK.
    Verbruggen, Katia
    Department of Otorhinolaryngology, University Hospital of Gent (UZ), Gent, Belgium.
    Huyghe, Joke
    Department of Otorhinolaryngology, University Hospital of Gent (UZ), Gent, Belgium.
    Dhooge, Ingeborg
    Department of Otorhinolaryngology, University Hospital of Gent (UZ), Gent, Belgium.
    Huygen, Patrick L.M.
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands.
    Kremer, Hannie Ph .
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands.
    Cremers, Cor Wrj R.J.
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands.
    Kunst, Sylvia J.W.
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands.
    Manninen, Minna
    Department of Otorhinolaryngology, University of Tampere, Tampere, Finland.
    Pyykkö, Ilmari V.
    Department of Otorhinolaryngology, University of Tampere, Tampere, Finland.
    Lacava, Amalia Diaz
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Germany.
    Steffens, Michael
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Germany.
    Wienker, Thomas F.
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Germany.
    Van Laer, Lut
    Department of Medical Genetics, University of Antwerp (UA), Antwerp, Belgium.
    Contribution of the N-acetyltransferase 2 polymorphism NAT2*6A to age-related hearing impairment2007In: Journal of Medical Genetics, ISSN 0022-2593, E-ISSN 1468-6244, Vol. 44, no 9, p. 570-578Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: Age-related hearing impairment (ARHI) is the most common sensory impairment in older people, affecting 50% of those aged 80 years. The proportion of older people is increasing in the general population, and as a consequence, the number of people affected with ARHI is growing. ARHI is a complex disorder, with both environmental and genetic factors contributing to the disease. The first studies to elucidate these genetic factors were recently performed, resulting in the identification of the first two susceptibility genes for ARHI, NAT2 and KCNQ4.

    METHODS: In the present study, the association between ARHI and polymorphisms in genes that contribute to the defence against reactive oxygen species, including GSTT1, GSTM1 and NAT2, was tested. Samples originated from seven different countries and were combined into two test population samples, the general European population and the Finnish population. Two distinct phenotypes for ARHI were studied, Z(low) and Z(high), representing hearing in the low and high frequencies, respectively. Statistical analysis was performed for single polymorphisms (GSTM1, GSTT1, NAT2*5A, NAT2*6A, and NAT2*7A), haplotypes, and gene-environment and gene-gene interactions.

    RESULTS: We found an association between ARHI and GSTT1 and GSTM1 in the Finnish population sample, and with NAT2*6A in the general European population sample. The latter finding replicates previously published data.

    CONCLUSION: As replication is considered the ultimate proof of true associations in the study of complex disorders, this study provides further support for the involvement of NAT2*6A in ARHI.

  • 49.
    Van Eyken, Els
    et al.
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Van Laer, Lut
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Fransen, Erik
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Topsakal, Vedat
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium.
    Hendrickx, Jan-Jaap
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium.
    Demeester, Kelly
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium.
    Van de Heyning, Paul
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Hannula, Samuli
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Sorri, Martti
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Jensen, Mona
    Department of Audiology, Bispebjerg Hospital (BBH) H:S, Copenhagen, Denmark.
    Parving, Agnete
    Department of Audiology, Bispebjerg Hospital (BBH) H:S, Copenhagen, Denmark.
    Bille, Michael
    Department of Audiology, Bispebjerg Hospital (BBH) H:S, Copenhagen, Denmark.
    Baur, Manuela
    Department of Otorhinolaryngology, University of Tüebingen, Tüebingen, Germany;.
    Pfister, Markus
    Department of Otorhinolaryngology, University of Tüebingen, Tüebingen, Germany.
    Bonaconsa, Amanda
    Department of Oto-surgery, University Hospital Padova, Padova, Italy.
    Mazzoli, Manuela
    Department of Oto-surgery, University Hospital Padova, Padova, Italy.
    Orzan, Eva
    Department of Oto-surgery, University Hospital Padova, Padova, Italy.
    Espeso, Angeles
    Welsh hearing Institute, University Hospital of Wales, Cardiff, U.K..
    Stephens, Dafydd
    Welsh hearing Institute, University Hospital of Wales, Cardiff, U.K..
    Verbruggen, Katia
    Department of Otorhinolaryngology, University Hospital of Gent, Gent, Belgium.
    Huyghe, Joke
    Department of Otorhinolaryngology, University Hospital of Gent, Gent, Belgium.
    Dhooge, Ingeborg
    Department of Otorhinolaryngology, University Hospital of Gent, Gent, Belgium.
    Huygen, Patrick
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
    Kremer, Hannie
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
    Cremers, Cor
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
    Kunst, Sylvia
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
    Manninen, Mina
    Department of Otorhinolaryngology, University of Tampere, Tampere, Finland.
    Pyykkö, Ilmari
    Department of Otorhinolaryngology, University of Tampere, Tampere, Finland.
    Rajkowska, Elzbieta
    Department of Audiology and Phoniatrics, Nofer Institute of Occupational Medicine, Lodz, Poland.
    Pawelczyk, Malgorzata
    Department of Audiology and Phoniatrics, Nofer Institute of Occupational Medicine, Lodz, Poland.
    Sliwinska-Kowalska, Mariola
    Department of Audiology and Phoniatrics, Nofer Institute of Occupational Medicine, Lodz, Poland.
    Steffens, Michael
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
    Wienker, Thomas
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
    Van Camp, Guy
    Department of Medical Genetics, University of Antwerp, the Netherlands.
    The contribution of GJB2 (Connexin 26) 35delG to age-related hearing impairment and noise-induced hearing loss2007In: Otology and Neurotology, ISSN 1531-7129, E-ISSN 1537-4505, Vol. 28, no 7, p. 970-975Article in journal (Refereed)
    Abstract [en]

    HYPOTHESIS: The common GJB2 (Connexin 26) 35delG mutation might contribute to the development of age-related hearing impairment (ARHI) and noise-induced hearing loss (NIHL).

    BACKGROUND: GJB2, a gene encoding a gap junction protein expressed in the inner ear, has been suggested to be involved in the potassium recycling pathway in the cochlea. GJB2 mutations account for a large number of individuals with nonsyndromic recessive hearing loss, with 35delG being the most frequent mutation in populations of European origin. Other genes involved in potassium homeostasis have been suggested to be associated with ARHI and NIHL, and distortion product otoacoustic emission distortions indicative of hearing loss alterations have been found in 35delG carriers.

    METHOD: We genotyped 35delG in two distinct sample sets: an ARHI sample set, composed of 2,311 Caucasian samples from nine different centers originating from seven different countries with an age range between 53 and 67 years, and an NIHL sample set consisting of 702 samples from the two extremes of a noise-exposed Polish sample.

    RESULTS: After statistical analysis, we were unable to detect an association between 35delG and ARHI, nor between 35delG and NIHL.

    CONCLUSION: Our findings indicate that there is no increased susceptibility in 35delG carriers for the development of ARHI or NIHL.

  • 50.
    Van Laer, Lut
    et al.
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Huyghe, Jeroen R.
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Hannula, Samuli
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Van Eyken, Els
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Stephan, Dietrich A.
    Neurogenomics Division, Translational Genomics Research Institute, Phoenix AZ, United States.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Aikio, Pekka
    Thule Institute, University of Oulu, Oulu, Finland.
    Fransen, Erik
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Lysholm-Bernacchi, Alana
    Neurogenomics Division, Translational Genomics Research Institute, Phoenix AZ, United States.
    Sorri, Martti
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Huentelman, Matthew J.
    Neurogenomics Division, Translational Genomics Research Institute, Phoenix AZ, United States.
    Van Camp, Guy
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    A genome-wide association study for age-related hearing impairment in the Saami2010In: European Journal of Human Genetics, ISSN 1018-4813, E-ISSN 1476-5438, Vol. 18, no 6, p. 685-693Article in journal (Refereed)
    Abstract [en]

    This study aimed at contributing to the elucidation of the genetic basis of age-related hearing impairment (ARHI), a common multifactorial disease with an important genetic contribution as demonstrated by heritability studies. We conducted a genome-wide association study (GWAS) in the Finnish Saami, a small, ancient, genetically isolated population without evidence of demographic expansion. The choice of this study population was motivated by its anticipated higher extent of LD, potentially offering a substantial power advantage for association mapping. DNA samples and audiometric measurements were collected from 352 Finnish Saami individuals, aged between 50 and 75 years. To reduce the burden of multiple testing, we applied principal component (PC) analysis to the multivariate audiometric phenotype. The first three PCs captured 80% of the variation in hearing thresholds, while maintaining biologically important audiometric features. All subjects were genotyped with the Affymetrix 100 K chip. To account for multiple levels of relatedness among subjects, as well as for population stratification, association testing was performed using a mixed model. We summarised the top-ranking association signals for the three traits under study. The top-ranked SNP, rs457717 (P-value 3.55 x 10(-7)), was associated with PC3 and was localised in an intron of the IQ motif-containing GTPase-activating-like protein (IQGAP2). Intriguingly, the SNP rs161927 (P-value 0.000149), seventh-ranked for PC1, was positioned immediately downstream from the metabotropic glutamate receptor-7 gene (GRM7). As a previous GWAS of a European and Finnish sample set already suggested a role for GRM7 in ARHI, this study provides further evidence for the involvement of this gene.

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