Better speech performance in cochlear implant patients with GJB2-related deafness

Int J Pediatr Otorhinolaryngol. 2002 Feb 1;62(2):151-7. doi: 10.1016/s0165-5876(01)00619-x.

Abstract

Objective: We applied mutation screening in seven cochlear implant users to identify those persons with GJB2-related deafness to determine whether etiology of deafness was predictive of speech performance after implantation.

Methods: Direct sequence of GJB2 was conducted over seven cochlear implant users with prelingual hearing impairment and their speech, language and cognitive performance was examined.

Results: The three persons with GJB2-related deafness had a mean vocabulary of 1243 words compared to a mean vocabulary of 195 words in the four children with GJB2-unrelated deafness, although the number of patients examined here was limited. The developmental quotient (DQ) of cognitive ability also was higher in those children with GJB2-related deafness.

Conclusions: These preliminary results suggest that better speech performance after cochlear implantation may be observed in persons with GJB2-related deafness. In the future, detailed phenotypic studies and mutation screening for non-syndromic hearing loss may play an important role in the preoperative assessment of prelingually-deafened children.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Audiometry
  • Child
  • Child Development
  • Child, Preschool
  • Cochlear Implantation*
  • Cognition
  • Connexin 26
  • Connexins / genetics*
  • DNA Mutational Analysis
  • Deafness / genetics*
  • Deafness / therapy
  • Humans
  • Infant
  • Language Development
  • Male
  • Mutation / genetics
  • Predictive Value of Tests
  • Speech Intelligibility*
  • Vocabulary

Substances

  • Connexins
  • GJB2 protein, human
  • Connexin 26