oru.sePublications
Change search
Link to record
Permanent link

Direct link
BETA
Publications (10 of 245) Show all publications
Tranebjærg, L., Strenzke, N., Lindholm, S., Rendtorff, N. D., Poulsen, H., Khandelia, H., . . . Bitner-Glindzicz, M. (2018). The CAPOS mutation in ATP1A3 alters Na/K-ATPase function and results in auditory neuropathy which has implications for management. Human Genetics, 137(2), 111-127
Open this publication in new window or tab >>The CAPOS mutation in ATP1A3 alters Na/K-ATPase function and results in auditory neuropathy which has implications for management
Show others...
2018 (English)In: Human Genetics, ISSN 0340-6717, E-ISSN 1432-1203, Vol. 137, no 2, p. 111-127Article in journal (Refereed) Published
Abstract [en]

Cerebellar ataxia, areflexia, pes cavus, optic atrophy and sensorineural hearing impairment (CAPOS) is a rare clinically distinct syndrome caused by a single dominant missense mutation, c.2452G>A, p.Glu818Lys, in ATP1A3, encoding the neuron-specific alpha subunit of the Na+/K+-ATPase α3. Allelic mutations cause the neurological diseases rapid dystonia Parkinsonism and alternating hemiplegia of childhood, disorders which do not encompass hearing or visual impairment. We present detailed clinical phenotypic information in 18 genetically confirmed patients from 11 families (10 previously unreported) from Denmark, Sweden, UK and Germany indicating a specific type of hearing impairment-auditory neuropathy (AN). All patients were clinically suspected of CAPOS and had hearing problems. In this retrospective analysis of audiological data, we show for the first time that cochlear outer hair cell activity was preserved as shown by the presence of otoacoustic emissions and cochlear microphonic potentials, but the auditory brainstem responses were grossly abnormal, likely reflecting neural dyssynchrony. Poor speech perception was observed, especially in noise, which was beyond the hearing level obtained in the pure tone audiograms in several of the patients presented here. Molecular modelling and in vitro electrophysiological studies of the specific CAPOS mutation were performed. Heterologous expression studies of α3 with the p.Glu818Lys mutation affects sodium binding to, and release from, the sodium-specific site in the pump, the third ion-binding site. Molecular dynamics simulations confirm that the structure of the C-terminal region is affected. In conclusion, we demonstrate for the first time evidence for auditory neuropathy in CAPOS syndrome, which may reflect impaired propagation of electrical impulses along the spiral ganglion neurons. This has implications for diagnosis and patient management. Auditory neuropathy is difficult to treat with conventional hearing aids, but preliminary improvement in speech perception in some patients suggests that cochlear implantation may be effective in CAPOS patients.

Place, publisher, year, edition, pages
Springer, 2018
National Category
Medical Genetics
Identifiers
urn:nbn:se:oru:diva-64038 (URN)10.1007/s00439-017-1862-z (DOI)000425104600001 ()29305691 (PubMedID)2-s2.0-85040046811 (Scopus ID)
Note

Funding Agency:

German Research Foundation through the Leibniz Program

Available from: 2018-01-12 Created: 2018-01-12 Last updated: 2018-02-28Bibliographically approved
Tranebjaerg, L., Strenzke, N., Lindholm, S., Rendtorff, N. D., Poulsen, H., Khandelia, H., . . . Bitner-Glindzicz, M. (2018). The CAPOS mutation in ATP1A3 alters Na/K-ATPase function and results in auditory neuropathy which has implications for management (vol 137, pg 111, 2018). Human Genetics, 137(3), 279-280
Open this publication in new window or tab >>The CAPOS mutation in ATP1A3 alters Na/K-ATPase function and results in auditory neuropathy which has implications for management (vol 137, pg 111, 2018)
Show others...
2018 (English)In: Human Genetics, ISSN 0340-6717, E-ISSN 1432-1203, Vol. 137, no 3, p. 279-280Article in journal (Refereed) Published
Place, publisher, year, edition, pages
Springer, 2018
National Category
Medical Genetics
Identifiers
urn:nbn:se:oru:diva-66579 (URN)10.1007/s00439-018-1870-7 (DOI)000428560200008 ()29435658 (PubMedID)2-s2.0-85041914120 (Scopus ID)
Note

Funding Agency:

German Research Foundation through the Collaborative Research Center 889 

Available from: 2018-04-13 Created: 2018-04-13 Last updated: 2018-04-13Bibliographically approved
Tracewska-Siemiątkowska, A., Haer-Wigman, L., Bosch, D. G. M., Nickerson, D., Bamshad, M. J., van de Vorst, M., . . . Tranebjærg, L. (2017). An Expanded Multi-Organ Disease Phenotype Associated with Mutations in YARS. Genes, 8(12), Article ID E381.
Open this publication in new window or tab >>An Expanded Multi-Organ Disease Phenotype Associated with Mutations in YARS
Show others...
2017 (English)In: Genes, ISSN 2073-4425, E-ISSN 2073-4425, Vol. 8, no 12, article id E381Article in journal (Refereed) Published
Abstract [en]

Whole exome sequence analysis was performed in a Swedish mother-father-affected proband trio with a phenotype characterized by progressive retinal degeneration with congenital nystagmus, profound congenital hearing impairment, primary amenorrhea, agenesis of the corpus callosum, and liver disease. A homozygous variant c.806T > C, p.(F269S) in the tyrosyl-tRNA synthetase gene (YARS) was the only identified candidate variant consistent with autosomal recessive inheritance. Mutations in YARS have previously been associated with both autosomal dominant Charcot-Marie-Tooth syndrome and a recently reported autosomal recessive multiorgan disease. Herein, we propose that mutations in YARS underlie another clinical phenotype adding a second variant of the disease, including retinitis pigmentosa and deafness, to the spectrum of YARS-associated disorders.

Place, publisher, year, edition, pages
Basel, Switzerland: MDPI AG, 2017
Keyword
YARS, syndromic retinitis pigmentosa, whole exome sequencing
National Category
Medical Genetics
Identifiers
urn:nbn:se:oru:diva-63407 (URN)10.3390/genes8120381 (DOI)000419212400042 ()29232904 (PubMedID)2-s2.0-85038106245 (Scopus ID)
Note

Funding Agencies:

Stichting ODAS  2014-9 

Vereniging Bartimeus-Sonneheerdt  5781251 

Netherlands Organization for Scientific Research (TOP-grant)  91209047 

National Human Genome Research Institute  

National Heart, Lung and Blood Institute  HG006493 

Available from: 2017-12-19 Created: 2017-12-19 Last updated: 2018-01-25Bibliographically approved
Tingaud-Sequeira, A., Raldúa, D., Lavie, J., Mathieu, G., Bordier, M., Knoll-Gellida, A., . . . Babin, P. (2017). Functional validation of ABHD12 mutations in the neurodegenerative disease PHARC. Neurobiology of Disease, 98, 36-51
Open this publication in new window or tab >>Functional validation of ABHD12 mutations in the neurodegenerative disease PHARC
Show others...
2017 (English)In: Neurobiology of Disease, ISSN 0969-9961, E-ISSN 1095-953X, Vol. 98, p. 36-51Article in journal (Refereed) Published
Abstract [en]

ABHD12 mutations have been linked to neurodegenerative PHARC (polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, and early-onset cataract), a rare, progressive, autosomal, recessive disease. Although ABHD12 is suspected to play a role in the lysophosphatidylserine and/or endocannabinoid pathways, its precise functional role(s) leading to PHARC disease had not previously been characterized. Cell and zebrafish models were designed to demonstrate the causal link between an identified new missense mutation p.T253R, characterized in ABHD12 from a young patient, the previously characterized p.T202I and p.R352* mutations, and the associated PHARC. Measuring ABHD12 monoacylglycerol lipase activity in transfected HEK293 cells demonstrated inhibition with mutated isoforms. Both the expression pattern of zebrafish abhd12 and the phenotype of specific antisense morpholino oligonucleotide gene knockdown morphants were consistent with human PHARC hallmarks. High abhd12 transcript levels were found in the optic tectum and tract, colocalized with myelin basic protein, and in the spinal cord. Morphants have myelination defects and concomitant functional deficits, characterized by progressive ataxia and motor skill impairment. A disruption of retina architecture and retinotectal projections was observed, together with an inhibition of lens clarification and a low number of mechanosensory hair cells in the inner ear and lateral line system. The severe phenotypes in abhd12 knockdown morphants were rescued by introducing wild-type human ABHD12 mRNA, but not by mutation-harboring mRNAs. Zebrafish may provide a suitable vertebrate model for ABHD12 insufficiency and the study of functional impairment and potential therapeutic rescue of this rare, neurodegenerative disease.

Place, publisher, year, edition, pages
Elsevier, 2017
Keyword
Neurodegenerative disease, PHARC, ABHD1, Mutations, Cell and zebra fish models, Demyelinating polyneuropathy, Hearing loss, ataxia, retinitis pigmentosa
National Category
Medical Genetics Cell and Molecular Biology Neurology
Identifiers
urn:nbn:se:oru:diva-54703 (URN)10.1016/j.nbd.2016.11.008 (DOI)000394727300004 ()27890673 (PubMedID)2-s2.0-85001114593 (Scopus ID)
Note

Funding Agencies:

Agence Nationale de la Recherche (ANR)  2010BLAN112601/LIGENAX

Association Francaise contre les Myopathies (AFM)  14879/MNM2 2012

Conseil Regional d'Aquitaine (CRA)  2011-0151/LIGENAX

Pole de competitivite Prod'Innov

Association Strumpell-Lorrain (ASL)  2011-0135

Association Connaitre les syndromes cerebelleux (CSC)

French State in the frame of the "Investments for the future" Programme IdEx Bordeaux  ANR-10-IDEX-03-02

Lundbeck Foundation  32011

Widex AS

Linnaeus Centre for Research on Hearing and Deafness (HEAD): Excellence in the field of Cognitive Hearing Science  349-2007-8654

Available from: 2017-01-13 Created: 2017-01-13 Last updated: 2018-01-13Bibliographically approved
Widén, S., Båsjö, S., Möller, C. & Kähäri, K. (2017). Headphone listening habits and hearing thresholds in swedish adolescents. Noise & Health, 19(88), 125-132
Open this publication in new window or tab >>Headphone listening habits and hearing thresholds in swedish adolescents
2017 (English)In: Noise & Health, ISSN 1463-1741, E-ISSN 1998-4030, Vol. 19, no 88, p. 125-132Article in journal (Refereed) Published
Abstract [en]

Introduction: The aim of this study was to investigate self-reported hearing and portable music listening habits, measured hearing function and music exposure levels in Swedish adolescents. The study was divided into two parts.

Materials and Methods: The first part included 280 adolescents, who were 17 years of age and focused on self-reported data on subjective hearing problems and listening habits regarding portable music players. From this group, 50 adolescents volunteered to participate in Part II of the study, which focused on audiological measurements and measured listening volume.

Results: The results indicated that longer lifetime exposure in years and increased listening frequency were associated with poorer hearing thresholds and more self-reported hearing problems. A tendency was found for listening to louder volumes and poorer hearing thresholds. Women reported more subjective hearing problems compared with men but exhibited better hearing thresholds. In contrast, men reported more use of personal music devices, and they listen at higher volumes.

Discussion: Additionally, the study shows that adolescents listening for ≥3 h at every occasion more likely had tinnitus. Those listening at ≥85 dB LAeq, FF and listening every day exhibited poorer mean hearing thresholds, reported more subjective hearing problems and listened more frequently in school and while sleeping.

Conclusion: Although the vast majority listened at moderate sound levels and for shorter periods of time, the study also indicates that there is a subgroup (10%) that listens between 90 and 100 dB for longer periods of time, even during sleep. This group might be at risk for developing future noise-induced hearing impairments.

Place, publisher, year, edition, pages
Medknow Publications, 2017
Keyword
Adolescents, headphones, hearing symptoms, hearing thresholds, listening habits, portable music listening devices
National Category
Otorhinolaryngology
Research subject
Disability Science
Identifiers
urn:nbn:se:oru:diva-58061 (URN)10.4103/nah.NAH_65_16 (DOI)000404120500002 ()28615542 (PubMedID)2-s2.0-85021194011 (Scopus ID)
Available from: 2017-06-15 Created: 2017-06-15 Last updated: 2017-10-10Bibliographically approved
Kollén, L., Hörder, H., Möller, C. & Frändin, K. (2017). Physical functioning in older persons with dizziness: a population-based study. Aging Clinical and Experimental Research, 29(2), 197-205
Open this publication in new window or tab >>Physical functioning in older persons with dizziness: a population-based study
2017 (English)In: Aging Clinical and Experimental Research, ISSN 1594-0667, E-ISSN 1720-8319, Vol. 29, no 2, p. 197-205Article in journal (Refereed) Published
Abstract [en]

Background: Dizziness is one of the most prevalent symptoms in old age and tends to increase with age.

Aims: To report physical functioning, health-related aspects and gender differences in elderly persons with and without dizziness in a population-based sample of 75-year-olds.

Methods: A cross-sectional sample of 75-year-olds from Gothenburg, Sweden (n = 675, 398 women and 277 men) was examined by means of questionnaires and functional tests. The questions concerned dizziness/imbalance, physical activity level, walking habits, falls efficacy, number of falls, subjective health or general fatigue and medication. The tests included were self-selected and maximum gait speed, stair climbing capacity, one leg stance and grip strength.

Results: More women than men reported dizziness/imbalance (40 vs 30 %, p < 0.001). Persons with dizziness, compared to those without dizziness, less often regularly exercised at a moderate intensity level (summer: 62 vs 74 %, p < 0.001; winter: 41 vs 51 %, p < 0.001), less often took a daily walk (p < 0.05), had lower scores on the FES(S) (p < 0.001), more often reported general fatigue (p < 0.001), more often had fallen in the previous year (40 vs 23 %, p < 0.001) and had a higher intake of medical drugs (4.6 vs 3.3, p < 0.001). They also performed worse regarding gait speed, stair climbing and one leg stance (p < 0.001), but there was no difference in grip strength.

Conclusion: Older persons with dizziness are less physically active, have worse lower extremity function, are more often fallers and report lower self-rated health than persons without dizziness.

Place, publisher, year, edition, pages
Springer, 2017
Keyword
Vertigo, unsteadiness, elderly person, dynamic balance
National Category
Physiotherapy Geriatrics
Identifiers
urn:nbn:se:oru:diva-49872 (URN)10.1007/s40520-016-0567-9 (DOI)000400383800012 ()27086001 (PubMedID)2-s2.0-84963739329 (Scopus ID)
Note

Funding Agency:

Research and Development Council for Southern Gothenburg and Bohuslän

Available from: 2016-04-19 Created: 2016-04-19 Last updated: 2017-10-18Bibliographically approved
Liu, Y. P., Bosch, D. G. M., Siemiatkowska, A. M., Dahl Rendtorff, N., Boonstra, F. N., Möller, C., . . . Cremers, F. P. M. (2017). Putative digenic inheritance of heterozygous RP1L1 and C2orf71 null mutations in syndromic retinal dystrophy. Ophthalmic Genetics, 38(2), 127-132
Open this publication in new window or tab >>Putative digenic inheritance of heterozygous RP1L1 and C2orf71 null mutations in syndromic retinal dystrophy
Show others...
2017 (English)In: Ophthalmic Genetics, ISSN 1381-6810, E-ISSN 1744-5094, Vol. 38, no 2, p. 127-132Article in journal (Refereed) Published
Abstract [en]

Background: Retinitis pigmentosa (RP) is the most common cause of inherited retinal degeneration and can occur in non-syndromic and syndromic forms. Syndromic RP is accompanied by other symptoms such as intellectual disability, hearing loss, or congenital abnormalities. Both forms are known to exhibit complex genetic interactions that can modulate the penetrance and expressivity of the phenotype.

Materials and methods: In an individual with atypical RP, hearing loss, ataxia and cerebellar atrophy, whole exome sequencing was performed. The candidate pathogenic variants were tested by developing an in vivo zebrafish model and assaying for retinal and cerebellar integrity.

Results: Exome sequencing revealed a complex heterozygous protein-truncating mutation in RP1L1, p.[(Lys111Glnfs*27; Gln2373*)], and a heterozygous nonsense mutation in C2orf71, p.(Ser512*). Mutations in both genes have previously been implicated in autosomal recessive non-syndromic RP, raising the possibility of a digenic model in this family. Functional testing in a zebrafish model for two key phenotypes of the affected person showed that the combinatorial suppression of rp1l1 and c2orf71l induced discrete pathology in terms of reduction of eye size with concomitant loss of rhodopsin in the photoreceptors, and disorganization of the cerebellum.

Conclusions: We propose that the combination of heterozygous loss-of-function mutations in these genes drives syndromic retinal dystrophy, likely through the genetic interaction of at least two loci. Haploinsufficiency at each of these loci is insufficient to induce overt pathology.

Place, publisher, year, edition, pages
Philadelphia, USA: Taylor & Francis, 2017
Keyword
Cerebellum, ciliopathy, digenic inheritance, hearing loss, retinitis pigmentosa zebrafish
National Category
Medical Genetics Ophthalmology
Research subject
Genetics; Health and Medical Care Research
Identifiers
urn:nbn:se:oru:diva-49603 (URN)10.3109/13816810.2016.1151898 (DOI)000399550400005 ()27029556 (PubMedID)2-s2.0-84962086185 (Scopus ID)
Note

Funding agencies:

National Institutes of Health  DK072301, DK075972, HD042601

Vereniging Bartimeus-Sonneheerdt  5781251

Stichting ODAS 

Available from: 2016-04-01 Created: 2016-04-01 Last updated: 2018-01-10Bibliographically approved
Hartel, B. P., Löfgren, M., Huygen, P. L. M., Guchelaar, I., Lo-A-Njoe Kort, N., Sadeghi, A. M., . . . Pennings, R. J. E. (2016). A combination of two truncating mutations in USH2A causes more severe and progressive hearing impairment in Usher syndrome type IIa. Hearing Research, 339, 60-68
Open this publication in new window or tab >>A combination of two truncating mutations in USH2A causes more severe and progressive hearing impairment in Usher syndrome type IIa
Show others...
2016 (English)In: Hearing Research, ISSN 0378-5955, E-ISSN 1878-5891, Vol. 339, p. 60-68Article in journal (Refereed) Published
Abstract [en]

Objectives: Usher syndrome is an inherited disorder that is characterized by hearing impairment (HI), retinitis pigmentosa, and in some cases vestibular dysfunction. Usher syndrome type IIa is caused by mutations in USH2A. HI in these patients is highly heterogeneous and the present study evaluates the effects of different types of USH2A mutations on the audiometric phenotype. Data from two large centres of expertise on Usher Syndrome in the Netherlands and Sweden were combined in order to create a large combined sample of patients to identify possible genotype-phenotype correlations.

Design: A retrospective study on HI in 110 patients (65 Dutch and 45 Swedish) genetically diagnosed with Usher syndrome type IIa. We used methods especially designed for characterizing and testing differences in audiological phenotype between patient subgroups. These methods included Age Related Typical Audiograms (ARTA) and a method to evaluate the difference in the degree of HI developed throughout life between subgroups.

Results: Cross-sectional linear regression analysis of last-visit audiograms for the best hearing ear demonstrated a gradual decline of hearing over decades. The congenital level of HI was in the range of 16-33 dB at 0.25-0.5 kHz, and in the range of 51-60 dB at 1-8 kHz. The annual threshold deterioration was in the range of 0.4-0.5 dB/year at 0.25-2 kHz and in the range of 0.7-0.8 dB/year at 4-8 kHz. Patients with two truncating mutations, including homozygotes for the common c.2299delG mutation, developed significantly more severe HI throughout life than patients with one truncating mutation combined with one nontruncating mutation, and patients with two nontruncating mutations.

Conclusions: The results have direct implications for patient counselling in terms of prognosis of hearing and may serve as baseline measures for future (genetic) therapeutic interventions.

Place, publisher, year, edition, pages
Amsterdam, Netherlands: Elsevier, 2016
National Category
Otorhinolaryngology Neurology
Research subject
Oto-Rhino-Laryngology; Neurology
Identifiers
urn:nbn:se:oru:diva-50977 (URN)10.1016/j.heares.2016.06.008 (DOI)000383944300007 ()27318125 (PubMedID)2-s2.0-84975809636 (Scopus ID)
Note

Funding Agencies:

Innovatiefonds B13-200-2677

Fonds Nuts-Ohra 1303-009

Heinsius Houbolt Fonds

Netherlands Organisation for Health Research and Development (ZonMW Klinisch Fellowship) 90700388

Foundation Fighting Blindness C-CMM-0811-0547-RAD03

Netherlands Organisation for Scientific Research Veni-016.136.091

Netherlands Organisation for Health Research and Development (ZonMW E-rare grant) 40-42900-98-1006

Available from: 2016-06-21 Created: 2016-06-21 Last updated: 2017-11-28Bibliographically approved
Rönnåsen, B., Möller, K., Möller, C., Lyxell, B. & Anderzén Carlsson, A. (2016). Aspects of learning from the perspective of people with Alström syndrome. Disability and Rehabilitation: Assistive Technology, 38(7), 644-652
Open this publication in new window or tab >>Aspects of learning from the perspective of people with Alström syndrome
Show others...
2016 (English)In: Disability and Rehabilitation: Assistive Technology, ISSN 1748-3107, E-ISSN 1748-3115, Vol. 38, no 7, p. 644-652Article in journal (Refereed) Published
Abstract [en]

Purpose: The aim of the study was to explore aspects of learning, from a lifelong perspective, in individuals with Alstro ̈m syndrome (AS). AS is an autosomal recessive disorder causing early blindness, progressive sensorineural hearing loss, cardiomyopathy, endocrine disorders, metabolic dysfunction, and abbreviated lifespan.

Method: Eleven individuals with AS participated. The study had a qualitative explorative design, giving voice to the participants’ perspectives on their situation. Data were collected using semi-structured interviews, which were subjected to conventional (inductive) qualitative content analysis.

Results: The analysis revealed in the participants a quest for independence and an image of themselves as capable people willing to learn, but in constant need of support to continue learning throughout their lives to be as independent as possible.

Conclusion: Based on the levels of functioning, i.e. personal resources, revealed in the interviews, supervisors, caregivers, and teachers are encouraged to allow people with AS to be their own advocates, as they know best how, what, and with whom they learn, and what type of sensory material – tactile, auditory, visual, or a combination – is most helpful. Implications for RehabilitationIndividuals with AS strive for independence, and to be independent they need to continue to learn throughout their lives.Individuals with AS know best how they learn, and should be asked what modalities are the most effective for them.The tactile modality for learning will continue throughout life and should be emphasized early in the individual's education and rehabilitation.

Place, publisher, year, edition, pages
Taylor & Francis, 2016
Keyword
Alström syndrome (AS), deafblindness, dual sensory loss, learning, sensoneural progressive hearing loss, vision loss
National Category
Other Health Sciences Learning
Research subject
Disability Science
Identifiers
urn:nbn:se:oru:diva-47760 (URN)10.3109/09638288.2015.1055381 (DOI)000367902500005 ()26084572 (PubMedID)2-s2.0-84954546359 (Scopus ID)
Funder
Swedish Research Council
Note

Funding Agency:

Swedish Institute for Disability Research, Linnaeus Centre HEAD

Available from: 2016-01-25 Created: 2016-01-25 Last updated: 2017-11-30Bibliographically approved
Rudner, M., Dahlström, Ö., Skagerstrand, Å., Alvinzi, L., Thunberg, P., Sörqvist, P., . . . Möller, C. (2016). Does working memory training improve speech recognition in noise?. In: : . Paper presented at Neural Plasticity Workshop: Insights from Deafness and Language, London, UK, June 3-4, 2016.
Open this publication in new window or tab >>Does working memory training improve speech recognition in noise?
Show others...
2016 (English)Conference paper, Poster (with or without abstract) (Other academic)
Abstract [en]

Listening to speech in noise is often reported to be effortful, especially for individuals with hearing impairment, and many studies have shown that the ability to recognize speech in noise is positively associated with working memory capacity. We reasoned that if working memory capacity could be increased by training this might improve the ability to recognize speech in noise and modulate the neural activation associated with it. Adults with normal (NH) and impaired hearing (HI) were randomized to five weeks of CogMed QM training followed by five weeks of no training, or vice versa, according to a crossover design. Auditory and cognitive abilities were tested on four occasions: pre-training, T1; after 5 weeks, T2; after 10 weeks, T3 and after a further six months, T4. During fMRI scanning at T1, T2 and T3, the participants listened to stereotyped matrix type sentences in pink noise and competing talker noise at individually adapted 50% and 90% intelligibility levels as well as in quiet. Behavioural results show that although HI had worse auditory abilities than NH, there was no significant difference in cognitive ability, with the exception of phonological processing, which tended to be slower (cf Classon et al. 2013). Performance on most of cognitive tasks improved across sessions, although this could not be specifically attributed to training. We found no consistent pattern of correlations between working memory and the ability to understand speech in noise either before or after training. fMRI results did not reveal any significant effect of training and furthermore there was no significant effect of hearing status. However, there was a significant between group difference in activation of the left temporal gyrus (-44 -23 10) for the contrast speech in pink noise (across intelligibility levels) vs clear speech. There was also an interaction (p < .001 uncorrected) between group and testing occasion in the right superior frontal gyrus (7 58 16) for the contrast speech in noise (across types and levels) vs clear speech. Further, activation in left superior temporal gyrus (-56 -20 -2) correlated more strongly with intelligibility in NH compared HI participants.

These results suggest that even when cognitive abilities are matched and intelligibility is individually adjusted, there are differences relating to hearing impairment in the neural mechanisms supporting speech in noise processing. The pattern of results suggests hearing-related differences in bottom-up processing mechanisms across time and hearing-related differences in top-down mechanisms that change over time. However, we found no evidence that working memory training improves speech recognition in noise.

References:Classon, E., Rudner, M. & Rönnberg, J. (2013). Working memory compensates for hearing relatedphonological processing deficit. Journal of Communication Disorders, 46, 17-29.doi:10.1016/j.jcomdis.2012.10.001CogMed QM, developed by CogMed Cognitive Medical Systems AB, Stockholm, Sweden, 2006

National Category
Otorhinolaryngology
Identifiers
urn:nbn:se:oru:diva-63445 (URN)
Conference
Neural Plasticity Workshop: Insights from Deafness and Language, London, UK, June 3-4, 2016
Available from: 2017-12-19 Created: 2017-12-19 Last updated: 2017-12-19Bibliographically approved
Organisations
Identifiers
ORCID iD: ORCID iD iconorcid.org/0000-0001-6557-6359

Search in DiVA

Show all publications