The Journal of Laryngology & Otology

Review Articles

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

F Stelmaa1a2 c1 and M F Bhuttaa1a3

a1 MRC Harwell, Harwell Science and Innovation Campus, Didcot, UK

a2 Department of Otorhinolaryngology, University of Groningen, University Medical Centre Groningen, The Netherlands

a3 Nuffield Department of Surgical Sciences (University of Oxford) and Department of Otolaryngology Head and Neck Surgery, John Radcliffe Hospital, Oxford, UK


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.

(Accepted May 07 2013)

(Online publication January 14 2014)

Key words

  • Sensorineural Hearing Loss;
  • Ear, Inner;
  • Anatomy;
  • Mutations;
  • Genetic Research


c1 Address for correspondence: Dr F Stelma, MRC Harwell, Harwell Science and Innovation Campus, Didcot OX11 0RD, UK Fax: +44 (0) 1235 841172 E-mail:


  Dr F Stelma takes responsibility for the integrity of the content of the paper

Competing interests: None declared