Gene expression profiles of the cochlea and vestibular endorgans: localization and function of genes causing deafness

Ann Otol Rhinol Laryngol. 2015 May;124 Suppl 1:6S-48S. doi: 10.1177/0003489415575549. Epub 2015 Mar 26.


Objectives: We sought to elucidate the gene expression profiles of the causative genes as well as the localization of the encoded proteins involved in hereditary hearing loss.

Methods: Relevant articles (as of September 2014) were searched in PubMed databases, and the gene symbols of the genes reported to be associated with deafness were located on the Hereditary Hearing Loss Homepage using localization, expression, and distribution as keywords.

Results: Our review of the literature allowed us to systematize the gene expression profiles for genetic deafness in the inner ear, clarifying the unique functions and specific expression patterns of these genes in the cochlea and vestibular endorgans.

Conclusions: The coordinated actions of various encoded molecules are essential for the normal development and maintenance of auditory and vestibular function.

Keywords: deafness; gene expression; immunocytochemistry; in situ hybridization; localization.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Cochlea / metabolism*
  • Deafness / genetics*
  • Gene Expression Profiling*
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization
  • Vestibule, Labyrinth / metabolism*