title: Association of GJB2 gene mutation with cochlear implant performance in genetic non-syndromic hearing loss.
abstract: OBJECTIVE
  To analyze the association of GJB2 gene mutations with cochlear implant performance in children.
  
  
  METHODS
  Sixty-five consecutive children who underwent cochlear implantation due to congenital profound senseurineural hearing between 2006 and 2008 were included in the study. In children, GJB2 gene mutation analysis was performed. Their auditory performance was assessed using MAIS, MUSS and LittlEARS tests.
  
  
  RESULTS
  Twenty-two of sixty-five patients GJB2 mutations, and 35delG was the most frequent mutation. No significant difference was found between the auditory performance of mutation positive and negative children after one year follow up (p>0.05).
  
  
  CONCLUSIONS
  GJB2 gene mutations do not impact on the outcome of cochlear implantation.
authors: Department of Otolaryngology, Gazi University Faculty of Medicine,06510 Besevler, Cankaya, Ankara, Turkey. dr.recepkaramert@yahoo.com
authors: Recep
authors: R
authors: Karamert
authors: Yildirim A
authors: YA
authors: Bayazit
authors: Senay
authors: S
authors: Altinyay
authors: Akın
authors: A
authors: Yılmaz
authors: Adnan
authors: A
authors: Menevse
authors: Ozan
authors: O
authors: Gokdogan
authors: Cagil
authors: C
authors: Gokdogan
authors: Ayca
authors: A
authors: Ant
mesh_terms: D000293:Adolescent; D001299:Audiometry; D002648:Child; D002675:Child, Preschool; D003054:Cochlear Implants; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics*; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000151:congenital / Q000235:genetics* / Q000628:therapy*; D006801:Humans; D007223:Infant; D008297:Male; D009154:Mutation; D016896:Treatment Outcome
chemical_list: D017630:Connexins; C000607042:GJB2 protein, human; D000072259:Connexin 26