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Updated: Mar 31, 2026

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Features of autosomal recessive non-syndromic hearing impairment: a review to serve as a reference
A M M Oonk1,2, P L M Huygen3, H P M Kunst3,4
1Department of Otorhinolaryngology, Hearing & Genes, Radboud University Medical Center, Nijmegen, The Netherlands. Anne.Oonk@radboudumc.nl.
Objective:
Non-syndromic sensorineural hearing impairment is inherited in an autosomal recessive fashion in 75-85% of cases. To date, 61 genes with this type of inheritance have been identified as related to hearing impairment, and the genetic heterogeneity is accompanied by a large variety of clinical characteristics. Adequate counselling on a patient's hearing prognosis and rehabilitation is part of the diagnosis on the genetic cause of hearing impairment and, in addition, is important for the psychological well-being of the patient.
Type Of Review:
Traditional literature review.
Data Source:
All articles describing clinical characteristics of the audiovestibular phenotypes of identified genes and related loci have been reviewed.
Conclusion:
This review aims to serve as a summary and a reference for counselling purposes when a causative gene has been identified in a patient with a non-syndromic autosomal recessively inherited sensorineural hearing impairment.
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