Cochlear implants in genetic deafness

Genetic defects are one of the most important etiologies of severe to profound sensorineural hearing loss and play an important role in determining cochlear implantation outcomes.While the pathogenic mutation types of a number of deafness genes have been cloned,the pathogenesis mechanisms and their...

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Bibliographic Details
Published inJournal of otology (Beijing) Vol. 9; no. 4; pp. 156 - 162
Main Author Liu, Xuezhong
Format Journal Article
LanguageEnglish
Published Elsevier B.V 01.12.2014
Department of 0tolaryngology, University of Miami Miller School of Medicine, 1120 NW 14th Ave., Miami, FL 33136, USA
Subjects
Auditory neuropathy
Connexin Cx26/Cx30
Mitochondria genes
Usher syndrome
Waardenburg syndrome
Connexin Cx26/Cx30
Mitochondria genes
Waardenburg syndrome
Auditory neuropathy
Usher syndrome
Online AccessGet full text
ISSN1672-2930
DOI10.1016/j.joto.2015.01.001

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Summary:Genetic defects are one of the most important etiologies of severe to profound sensorineural hearing loss and play an important role in determining cochlear implantation outcomes.While the pathogenic mutation types of a number of deafness genes have been cloned,the pathogenesis mechanisms and their relationship to the outcomes of cochlear implantation remain a hot research area.The auditory performance is considered to be affected by the etiology of hearing loss and the number of surviving spiral ganglion cells,as well as others.Current research advances in cochlear implantation for hereditary deafness,especially the relationship among clinic-types,genotypes and outcomes of cochlear implantation,will be discussed in this review.
Bibliography:Xuezhong Liu;Department of Otolaryngology,University of Miami Miller School of Medicine
11-4883/R
ISSN:1672-2930
DOI:10.1016/j.joto.2015.01.001