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

Kunihiro Fukushima, Kennichi Sugata, Norio Kasai, Shouichirou Fukuda, Rie Nagayasu, Naomi Toida, Nobuhiko Kimura, Teruaki Takishita, Mehmet Gunduz, Kazunori Nishizaki

Research output: Contribution to journalArticlepeer-review

71 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)151-157
Number of pages7
JournalInternational Journal of Pediatric Otorhinolaryngology
Issue number2
Publication statusPublished - Feb 1 2002


  • Cochlear implantation
  • Connexin 26
  • GJB2
  • Language development
  • Mutation screening
  • Speech perception

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Otorhinolaryngology


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