Good cochlear implantation outcomes in subjects with mono-allelic WFS1-associated sensorineural hearing loss – a case series.
Objective: This study aimed to evaluate long-term cochlear implant (CI) outcomes in individuals with mono-allelic pathogenic variants in WFS1, which is associated with both Wolfram-like syndrome and DFNA6/14/38. Design: Retrospective case series. Study sample: Seven CI recipients, ranging from eight...
| Publicado en: | International Journal of Audiology Vol. 64; no. 8; pp. 863 - 872 |
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| Autores principales: | , , , , , |
| Formato: | research tables/charts Journal Article |
| Publicado: |
Taylor & Francis Ltd
Aug2025
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=187005085&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 187005085 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 14992027 JW2 jtl: International Journal of Audiology issn: 14992027 maglogo: Y pubinfo: dt: Aug2025 vid: 64 iid: 8 pid: 377 pub: Taylor & Francis Ltd place: Philadelphia, Pennsylvania artinfo: ui: 187005085 180356341 187005085 187005085 10.1080/14992027.2024.2411579 187005085 ppf: 863 ppct: 9 formats: fmt: – @attributes: type: T – @attributes: type: P tig: atl: Good cochlear implantation outcomes in subjects with mono-allelic WFS1-associated sensorineural hearing loss – a case series. aug: au: Fehrmann, M. L. A. Lanting, C. P. Haer-Wigman, L. Mylanus, E. A. M. Huinck, W. J. Pennings, R. J. E. affil: Department of Otorhinolaryngology, Radboud University Medical Center, Nijmegen, The Netherlands sug: subj: Hearing Loss, Sensorineural Familial and Genetic Cochlear Implant Treatment Outcomes Wolfram Syndrome Alleles Genetic Variation Dutch Persons Human Male Female Retrospective Design Record Review Nonexperimental Studies Netherlands Evoked Potentials, Auditory, Brainstem Genotype Phenotype Audiometry Descriptive Statistics Data Analysis Software Mann-Whitney U Test Middle Age Aged Confidence Intervals Speech Perception Acoustic Stimulation Funding Source Middle Aged: 45-64 years Aged: 65+ years Male Female ab: Objective: This study aimed to evaluate long-term cochlear implant (CI) outcomes in individuals with mono-allelic pathogenic variants in WFS1, which is associated with both Wolfram-like syndrome and DFNA6/14/38. Design: Retrospective case series. Study sample: Seven CI recipients, ranging from eight months to 58 years of age, were included in the study, including four with Wolfram-like syndrome and three with DFNA6/14/38. A total of ten cochlear implantations were performed among these subjects. Results: At one-year post-implantation, a mean phoneme score of 90 ± 9% at 65 dB SPL in quiet was found, which remained stable up to ten years post-implantation with a mean phoneme score of 94 ± 6%. Despite these excellent outcomes, one subject achieved no speech recognition with CI and eventually became a non-user. This individual had a prolonged absence of auditory stimulation prior to implantation and encountered multiple challenges during rehabilitation. Conclusion: Individuals with Wolfram-like syndrome or DFNA6/14/38 demonstrate consistently good outcomes following implantation, which remain stable over time. These findings affirm cochlear implantation as an effective rehabilitation option for these individuals. Furthermore, the stable and good CI outcomes contradict the suggested link between WFS1-associated sensorineural hearing loss and auditory neuropathy. pubtype: Academic Journal doctype: research tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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