Cochlear implantation in common forms of genetic deafness

Richard J. Vivero, Kenneth Fan, Simon I Angeli, Thomas J. Balkany, Xue Z Liu

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Genetic factors are among the main etiologies of severe to profound hearing loss and may play an important role in cochlear implantation (CI) outcomes. While genes for common forms of deafness have been cloned, efforts to correlate the functional outcome of CIs with a genetic form of deafness carried by the patient have been largely anecdotal to date. It has been suggested that the differences in auditory performance may be explained by differences in the number of surviving spiral ganglion cells, etiology of hearing loss, and other factors. Knowledge of the specific loci and mutations involved in patients who receive cochlear implants may elucidate other factors related to CI performance. In this review article, current knowledge of cochlear implants for hereditary hearing loss will be discussed with an emphasis on relevant clinical genotype-phenotype correlations.

Original languageEnglish
Pages (from-to)1107-1112
Number of pages6
JournalInternational Journal of Pediatric Otorhinolaryngology
Volume74
Issue number10
DOIs
StatePublished - Oct 1 2010

Fingerprint

Cochlear Implantation
Deafness
Hearing Loss
Cochlear Implants
Spiral Ganglion
Genetic Association Studies
Mutation
Genes

Keywords

  • Cochlear implantation
  • Genetic deafness
  • GJB2
  • GJB3
  • Hearing loss
  • Jervell and Lange-Nielson
  • Usher syndrome
  • Wardenburg syndrome

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Pediatrics, Perinatology, and Child Health

Cite this

Cochlear implantation in common forms of genetic deafness. / Vivero, Richard J.; Fan, Kenneth; Angeli, Simon I; Balkany, Thomas J.; Liu, Xue Z.

In: International Journal of Pediatric Otorhinolaryngology, Vol. 74, No. 10, 01.10.2010, p. 1107-1112.

Research output: Contribution to journalArticle

Vivero, Richard J. ; Fan, Kenneth ; Angeli, Simon I ; Balkany, Thomas J. ; Liu, Xue Z. / Cochlear implantation in common forms of genetic deafness. In: International Journal of Pediatric Otorhinolaryngology. 2010 ; Vol. 74, No. 10. pp. 1107-1112.
@article{1df66802e99344b09022a9754808ddd6,
title = "Cochlear implantation in common forms of genetic deafness",
abstract = "Genetic factors are among the main etiologies of severe to profound hearing loss and may play an important role in cochlear implantation (CI) outcomes. While genes for common forms of deafness have been cloned, efforts to correlate the functional outcome of CIs with a genetic form of deafness carried by the patient have been largely anecdotal to date. It has been suggested that the differences in auditory performance may be explained by differences in the number of surviving spiral ganglion cells, etiology of hearing loss, and other factors. Knowledge of the specific loci and mutations involved in patients who receive cochlear implants may elucidate other factors related to CI performance. In this review article, current knowledge of cochlear implants for hereditary hearing loss will be discussed with an emphasis on relevant clinical genotype-phenotype correlations.",
keywords = "Cochlear implantation, Genetic deafness, GJB2, GJB3, Hearing loss, Jervell and Lange-Nielson, Usher syndrome, Wardenburg syndrome",
author = "Vivero, {Richard J.} and Kenneth Fan and Angeli, {Simon I} and Balkany, {Thomas J.} and Liu, {Xue Z}",
year = "2010",
month = "10",
day = "1",
doi = "10.1016/j.ijporl.2010.06.010",
language = "English",
volume = "74",
pages = "1107--1112",
journal = "International Journal of Pediatric Otorhinolaryngology",
issn = "0165-5876",
publisher = "Elsevier Ireland Ltd",
number = "10",

}

TY - JOUR

T1 - Cochlear implantation in common forms of genetic deafness

AU - Vivero, Richard J.

AU - Fan, Kenneth

AU - Angeli, Simon I

AU - Balkany, Thomas J.

AU - Liu, Xue Z

PY - 2010/10/1

Y1 - 2010/10/1

N2 - Genetic factors are among the main etiologies of severe to profound hearing loss and may play an important role in cochlear implantation (CI) outcomes. While genes for common forms of deafness have been cloned, efforts to correlate the functional outcome of CIs with a genetic form of deafness carried by the patient have been largely anecdotal to date. It has been suggested that the differences in auditory performance may be explained by differences in the number of surviving spiral ganglion cells, etiology of hearing loss, and other factors. Knowledge of the specific loci and mutations involved in patients who receive cochlear implants may elucidate other factors related to CI performance. In this review article, current knowledge of cochlear implants for hereditary hearing loss will be discussed with an emphasis on relevant clinical genotype-phenotype correlations.

AB - Genetic factors are among the main etiologies of severe to profound hearing loss and may play an important role in cochlear implantation (CI) outcomes. While genes for common forms of deafness have been cloned, efforts to correlate the functional outcome of CIs with a genetic form of deafness carried by the patient have been largely anecdotal to date. It has been suggested that the differences in auditory performance may be explained by differences in the number of surviving spiral ganglion cells, etiology of hearing loss, and other factors. Knowledge of the specific loci and mutations involved in patients who receive cochlear implants may elucidate other factors related to CI performance. In this review article, current knowledge of cochlear implants for hereditary hearing loss will be discussed with an emphasis on relevant clinical genotype-phenotype correlations.

KW - Cochlear implantation

KW - Genetic deafness

KW - GJB2

KW - GJB3

KW - Hearing loss

KW - Jervell and Lange-Nielson

KW - Usher syndrome

KW - Wardenburg syndrome

UR - http://www.scopus.com/inward/record.url?scp=77956474070&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77956474070&partnerID=8YFLogxK

U2 - 10.1016/j.ijporl.2010.06.010

DO - 10.1016/j.ijporl.2010.06.010

M3 - Article

C2 - 20655117

AN - SCOPUS:77956474070

VL - 74

SP - 1107

EP - 1112

JO - International Journal of Pediatric Otorhinolaryngology

JF - International Journal of Pediatric Otorhinolaryngology

SN - 0165-5876

IS - 10

ER -