title: [The research of rehabilitation effect of cochlear implantation for deaf children with gene mutation].
abstract: OBJECTIVE
  To assess the evaluation on auditory rehabilitation effect for 42 deaf children with GJB2 gene mutation after cochlear implantation to provide a reference for the cochlear implant effect evaluation of such patients.
  
  
  METHODS
  To conduct the detection on common genetic deafness gene mutation hotspots of hearing impaired children with cochlear implantation. To conduct auditory rehabilitation effect evaluation on 42 cases of patients with GJB2 genetic deafness after 3 months, 6 months and 12 months of the operation respectively. The single factor repeated measure ANOVA was applied to analyze whether there were significant difference among the results of initial consonant of a Chinese syllable recognition at 3 different stages after the operation, the results of vowel of a Chinese syllable recognition at 3 different stages after the operation, and the results of two-syllable recognition at 3 different stages after the operation.
  
  
  RESULTS
  235delC is the high-incidence mutational site in 42 cases of patients with GJB2 genetic deafness, the total detection rate is up to 90.48%. There were significant differences in the initial consonant of a Chinese syllable recognition rate, the vowel of a Chinese syllable recognition rate, the two-syllable recognition rate as well as the vowel of a Chinese syllable recognition rate after 3 months, 6 months and 12 months of the operation (P < 0.01).
  
  
  CONCLUSIONS
  Cochlear implantation is a safe and effective measure for auditory reconstruction, it can help patients with GJB2 hereditary severe sensorineural deafness to improve auditory speech recognition.
authors.forename: Ying
authors.initials: Y
authors.lastname: Kong
authors.forename: Shuang
authors.initials: S
authors.lastname: Liang
authors.forename: Cuncun
authors.initials: C
authors.lastname: Ren
authors.forename: Xin
authors.initials: X
authors.lastname: Liu
authors.forename: Sha
authors.initials: S
authors.lastname: Liu
mesh_terms: D044466:Asian People; D002648:Child; D019929:Cochlear Implantation; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics*; D003638:Deafness / Q000235:genetics / Q000534:rehabilitation*; D006319:Hearing Loss, Sensorineural / Q000235:genetics / Q000534:rehabilitation; D006801:Humans; D007802:Language; D009154:Mutation
chemical_list: D017630:Connexins; C000607042:GJB2 protein, human; D000072259:Connexin 26