Cochlear implantation in common forms of genetic deafness

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

Research output: Contribution to journalReview articlepeer-review

28 Scopus citations


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 (US)
Pages (from-to)1107-1112
Number of pages6
JournalInternational Journal of Pediatric Otorhinolaryngology
Issue number10
StatePublished - Oct 2010


  • 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


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