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

AbstractOBJECTIVE:
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.
AuthorsKunihiro Fukushima, Kennichi Sugata, Norio Kasai, Shouichirou Fukuda, Rie Nagayasu, Naomi Toida, Nobuhiko Kimura, Teruaki Takishita, Mehmet Gunduz, Kazunori Nishizaki
JournalInternational journal of pediatric otorhinolaryngology (Int J Pediatr Otorhinolaryngol) Vol. 62 Issue 2 Pg. 151-7 (Feb 01 2002) ISSN: 0165-5876 [Print] Ireland
PMID11788148 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Connexins
  • GJB2 protein, human
  • Connexin 26
Topics
  • Audiometry
  • Child
  • Child Development
  • Child, Preschool
  • Cochlear Implantation
  • Cognition
  • Connexin 26
  • Connexins (genetics)
  • DNA Mutational Analysis
  • Deafness (genetics, therapy)
  • Humans
  • Infant
  • Language Development
  • Male
  • Mutation (genetics)
  • Predictive Value of Tests
  • Speech Intelligibility
  • Vocabulary

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