title: Long term speech perception after cochlear implant in pediatric patients with GJB2 mutations.
abstract: OBJECTIVE
  To determine the long term effect of cochlear implant (CI) in children with GJB2-related deafness in Japan.
  
  
  METHODS
  Genetic testing was performed on 29 children with CI. The speech perception in 9 children with GJB2 gene-related deafness fitted with CI was compared with those in matched 10 children who were diagnosed as having no genetic loci. The average follow-up period after CI was 55.9 months and 54.6 months, respectively.
  
  
  RESULTS
  A definitive inherited hearing impairment could be confirmed in 12 (41.4%) of the 29 CI children, including 10 with GJB2-related hearing impairment and 2 with SLC26A4-related hearing impairment. The results of IT-MAIS, word or speech perception testing under the noise, and development of speech perception and production testing using the Enjoji scale were slightly better for the GJB2 group after CI than for the control group without statistical significant difference.
  
  
  CONCLUSIONS
  The long-term results of this study show that CI is also effective in the development of speech performance after CI in Japanese children with GJB2-related hearing impairments as HL due to other etiologies.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, National Hospital Organization Ureshino Medical Center, Saga, Japan. y-haruo@f6.dion.ne.jp
authors.forename: Haruo
authors.initials: H
authors.lastname: Yoshida
authors.forename: Haruo
authors.initials: H
authors.lastname: Takahashi
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Kanda
authors.forename: Shin-Ichi
authors.initials: S
authors.lastname: Usami
mesh_terms: D002675:Child, Preschool; D019929:Cochlear Implantation; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics; D003638:Deafness / Q000235:genetics / Q000601:surgery*; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D026901:Membrane Transport Proteins / Q000235:genetics; D009154:Mutation; D013067:Speech Perception; D013069:Speech Reception Threshold Test; D000076988:Sulfate Transporters; D016896:Treatment Outcome
chemical_list: D017630:Connexins; C000607042:GJB2 protein, human; D026901:Membrane Transport Proteins; C085609:SLC26A4 protein, human; D000076988:Sulfate Transporters; D000072259:Connexin 26