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Non-syndromic hereditary sensorineural hearing loss: review of the genes involved

Published online by Cambridge University Press:  14 January 2014

F Stelma  and
M F Bhutta
F Stelma*
Affiliation:
MRC Harwell, Harwell Science and Innovation Campus, Didcot, UK Department of Otorhinolaryngology, University of Groningen, University Medical Centre Groningen, The Netherlands
M F Bhutta
Affiliation:
MRC Harwell, Harwell Science and Innovation Campus, Didcot, UK Nuffield Department of Surgical Sciences (University of Oxford) and Department of Otolaryngology Head and Neck Surgery, John Radcliffe Hospital, Oxford, UK
*
Address for correspondence: Dr F Stelma, MRC Harwell, Harwell Science and Innovation Campus, Didcot OX11 0RD, UK Fax: +44 (0) 1235 841172 E-mail: [email protected]
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Abstract

Background:

Hereditary sensorineural hearing loss is the most frequently occurring birth defect. It has profound effects for the individual and is a substantial burden on society. Insight into disease mechanisms can help to broaden therapeutic options and considerably lower lifetime social costs. In the past few decades, the identification of genes that can cause this type of hearing loss has developed rapidly.

Objective:

This paper provides a concise overview of the currently known genes involved in non-syndromic hereditary hearing loss and their function in the inner ear.

Keywords

Sensorineural Hearing LossEar, InnerAnatomyMutationsGenetic Research
Type
Review Articles
Information
The Journal of Laryngology & Otology , Volume 128 , Issue 1 , January 2014 , pp. 13 - 21
Copyright
Copyright © JLO (1984) Limited 2014 

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