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  • 1.
    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
    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.
    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.

  • 2.
    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.

  • 3.
    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.

  • 4.
    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.

  • 5.
    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.

  • 6.
    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.

  • 7.
    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.

  • 8.
    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.

  • 9.
    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.

  • 10.
    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.

  • 11.
    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.

  • 12.
    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.

  • 13.
    Kytövuori, Laura
    et al.
    Medical Research Center Oulu, Oulu University Hospital and 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 and 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, Sweden.
    Sorri, Martti
    Medical Research Center Oulu, Oulu University Hospital and 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 and 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.

  • 14.
    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.

  • 15.
    Lohi, Venla
    et al.
    PEDEGO Research Unit, University of Oulu, Oulu, Finland; Medical Research Center Oulu, University of Oulu and Oulu University Hospital, 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, University of Oulu and Oulu University Hospital, Oulu, Finland.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. PEDEGO Research Unit, University of Oulu, Oulu, Finland; Medical Research Center Oulu, University of Oulu and Oulu University Hospital, Oulu, Finland; Örebro University Hospital, Örebro, Sweden.
    Hannula, Samuli
    PEDEGO Research Unit, University of Oulu, Oulu, Finland; Medical Research Center Oulu, University of Oulu and Oulu University Hospital, 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.

  • 16.
    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.

  • 17.
    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.

  • 18.
    Lyxell, Björn
    et al.
    The Swedish Institute for Disability Research, Linköping University, Sweden.
    Wass, Malin
    The Swedish Institute for Disability Research, Linköping University, 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, Sweden; Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden.
    Larsby, Birgitta
    The Swedish Institute for Disability Research, Linköping University, Sweden; Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden.
    Hällgren, Mathias
    The Swedish Institute for Disability Research, Linköping University, 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)
  • 19.
    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.

  • 20.
    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.

  • 21.
    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.

  • 22.
    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.

  • 23.
    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.

  • 24.
    Van Laer, Lut
    et al.
    Department of Medical Genetics, University 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.
    Huyghe, Jeroen R.
    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; Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands.
    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.
    Jensen, Mona
    Department of Audiology, Bispebjerg Hospital, Copenhagen, Denmark.
    Demeester, Kelly
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium.
    Baur, Manuela
    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 Ghent, Ghent, Belgium.
    Huyghe, Joke
    Department of Otorhinolaryngology, University Hospital of Ghent, Ghent, Belgium.
    Huygen, Patrick
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands.
    Kunst, Sylvia
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands.
    Manninen, Minna
    Department of Otorhinolaryngology, University of Tampere, Tampere, Finland.
    Konings, Annelies
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    Diaz-Lacava, Amalia N.
    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.
    Wienker, Thomas F.
    Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
    Pyykkö, Ilmari
    Department of Otorhinolaryngology, University of Tampere, Tampere, Finland.
    Cremers, Cor W. R. J.
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands.
    Kremer, Hannie
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands.
    Dhooge, Ingeborg
    Department of Otorhinolaryngology, University Hospital of Ghent, Ghent, Belgium.
    Stephens, Dafydd
    Welsh Hearing Institute, Cardiff University, Cardiff, United Kingdom.
    Orzan, Eva
    Department of Oto-Surgery, University Hospital Padova, Padova, Italy.
    Pfister, Markus
    Department of Otorhinolaryngology, University of Tübingen, Tübingen, Germany.
    Bille, Michael
    Department of Audiology, Bispebjerg Hospital, Copenhagen, Denmark.
    Parving, Agnete
    Department of Audiology, Bispebjerg Hospital, Copenhagen, Denmark.
    Sorri, Martti
    Department of Otorhinolaryngology, University of Oulu, Oulu, Finland.
    Van de Heyning, Paul H.
    Department of Otorhinolaryngology, University Hospital of Antwerp, Antwerp, Belgium.
    Van Camp, Guy
    Department of Medical Genetics, University of Antwerp, Antwerp, Belgium.
    The grainyhead like 2 gene (GRHL2), alias TFCP2L3, is associated with age-related hearing impairment2008In: Human Molecular Genetics, ISSN 0964-6906, E-ISSN 1460-2083, Vol. 17, no 2, p. 159-169Article in journal (Refereed)
    Abstract [en]

    Age-related hearing impairment (ARHI) is the most prevalent sensory impairment in the elderly. ARHI is a complex disease caused by an interaction between environmental and genetic factors. The contribution of various environmental factors has been relatively extensively studied. In contrast, investigations to identify the genetic risk factors have only recently been initiated. In this paper we describe the results of an association study performed on 2418 ARHI samples derived from nine centers from seven European countries. In 70 candidate genes, a total of 768 tag single nucleotide polymorphisms (SNPs) were selected based on HAPMAP data. These genes were chosen among the monogenic hearing loss genes identified in mice and men in addition to several strong functional candidates. After genotyping and data polishing, statistical analysis of all samples combined resulted in a P-value that survived correction for multiple testing for one SNP in the GRHL2 gene. Other SNPs in this gene were also associated, albeit to a lesser degree. Subsequently, an analysis of the most significant GRHL2 SNP was performed separately for each center. The direction of the association was identical in all nine centers. Two centers showed significant associations and a third center showed a trend towards significance. Subsequent fine mapping of this locus demonstrated that the majority of the associated SNPs reside in intron 1. We hypothesize that the causative variant may change the expression levels of a GRHL2 isoform.

  • 25.
    Wass, Malin
    et al.
    The Swedish Institute for Disability Research, Linköping and the Department of Behavioural Sciences and Learning, Linköping University, Linköping, Sweden.
    Ibertsson, Tina
    Department of Logopedics, Phoniatrics and Audiology, Lund University, Lund, Sweden.
    Lyxell, Björn
    The Swedish Institute for Disability Research, Linköping and the Department of Behavioural Sciences and Learning, Linköping University, Linköping, Sweden.
    Sahlén, Birgitta
    Department of Logopedics, Phoniatrics and Audiology, Lund University, Lund, Sweden.
    Hällgren, Mathias
    Department of Clinical and Experimental Medicine, Division of Technical Audiology, Linköping University, Linköping, Sweden.
    Larsby, Birgitta
    Department of Clinical and Experimental Medicine, Division of Technical Audiology, Linköping University, Swede.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Department of Otolaryngology/Section of Audiology, Linköping University Hospital, Linköping, Linköping, Sweden.
    Cognitive and linguistic skills in Swedish children with cochlear implants - measures of accuracy and latency as indicators of development2008In: Scandinavian Journal of Psychology, ISSN 0036-5564, E-ISSN 1467-9450, Vol. 49, no 6, p. 559-76Article in journal (Refereed)
    Abstract [en]

    The purpose of the present study was to examine working memory (WM) capacity, lexical access and phonological skills in 19 children with cochlear implants (CI) (5;7-13;4 years of age) attending grades 0-2, 4, 5 and 6 and to compare their performance with 56 children with normal hearing. Their performance was also studied in relation to demographic factors. The findings indicate that children with CI had visuospatial WM capacities equivalent to the comparison group. They had lower performance levels on most of the other cognitive tests. Significant differences between the groups were not found in all grades and a number of children with CI performed within 1 SD of the mean of their respective grade-matched comparison group on most of the cognitive measures. The differences between the groups were particularly prominent in tasks of phonological WM. The results are discussed with respect to the effects of cochlear implants on cognitive development.

  • 26.
    Wass, Malin
    et al.
    Linköping University Hospital, Linköping, Sweden.
    Lyxell, Björn
    The Swedish Institute for Disability Research, Linköping, Sweden.
    Sahlé, Birgitta
    Lund University, Lund, Sweden.
    Asker-Árnason, Lena
    Lund University, Lund, Sweden.
    Ibertsson, Tina
    Lund university, Lund, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Linköping University, Linköping, Sweden.
    Hällgren, Mathias
    Linköping University Hospital, Linköping, Sweden.
    Larsby, Birgitta
    Linköping University Hospital, Linköping, Sweden.
    Cognitive skills and reading ability in children with cochlear implants.2010In: Cochlear Implants International, ISSN 1467-0100, E-ISSN 1754-7628, Proceedings of the 9th European Symposium on Paediatric Cochlear Implantation, Warsaw, 2009, Vol. 11, no Suppl 1:1-6, p. 395-398Article in journal (Refereed)
  • 27.
    Zeitooni, Mehrnaz
    et al.
    Division of Technical Audiology, Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden.
    Mäki-Torkko, Elina
    Örebro University, School of Medical Sciences. Division of Technical Audiology, Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden; Department of ENT-Head Neck Surgery, Region Östergötland, Linköping, Sweden.
    Stenfelt, Stefan
    Division of Technical Audiology, Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden.
    Binaural Hearing Ability With Bilateral Bone Conduction Stimulation in Subjects With Normal Hearing: Implications for Bone Conduction Hearing Aids.2016In: Ear and Hearing, ISSN 0196-0202, E-ISSN 1538-4667, Vol. 37, no 6, p. 690-702Article in journal (Refereed)
    Abstract [en]

    OBJECTIVES: The purpose of this study is to evaluate binaural hearing ability in adults with normal hearing when bone conduction (BC) stimulation is bilaterally applied at the bone conduction hearing aid (BCHA) implant position as well as at the audiometric position on the mastoid. The results with BC stimulation are compared with bilateral air conduction (AC) stimulation through earphones.

    DESIGN: Binaural hearing ability is investigated with tests of spatial release from masking and binaural intelligibility level difference using sentence material, binaural masking level difference with tonal chirp stimulation, and precedence effect using noise stimulus.

    RESULTS: In all tests, results with bilateral BC stimulation at the BCHA position illustrate an ability to extract binaural cues similar to BC stimulation at the mastoid position. The binaural benefit is overall greater with AC stimulation than BC stimulation at both positions. The binaural benefit for BC stimulation at the mastoid and BCHA position is approximately half in terms of decibels compared with AC stimulation in the speech based tests (spatial release from masking and binaural intelligibility level difference). For binaural masking level difference, the binaural benefit for the two BC positions with chirp signal phase inversion is approximately twice the benefit with inverted phase of the noise. The precedence effect results with BC stimulation at the mastoid and BCHA position are similar for low frequency noise stimulation but differ with high-frequency noise stimulation.

    CONCLUSIONS: The results confirm that binaural hearing processing with bilateral BC stimulation at the mastoid position is also present at the BCHA implant position. This indicates the ability for binaural hearing in patients with good cochlear function when using bilateral BCHAs.

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