title: Performance of speech perception after cochlear implantation in DFNB1 patients.
abstract: CONCLUSIONS
  There were no apparent differences in speech performance after cochlear implantation between patients with biallelic GJB2 and/or GJB6 mutations and those with deafness of unknown aetiology. These data have important implications for the selection of prognostic indicators of the outcome of cochlear implantation.
  
  
  OBJECTIVE
  To compare performance after cochlear implantation in children with mutations in GJB2 (connexin 26) and/or GJB6 (connexin 30) and children with deafness of unknown aetiology.
  
  
  METHODS
  Genetic analysis and speech performance evaluation was determined in 24 patients with (n=11) and without (n=13) biallelic GJB2 and/or GJB6 mutations who underwent cochlear implantation. Speech perception skills were measured 12 and 24 months after surgery. Each patient was classified in accordance with the speech perception category (SPC).
  
  
  RESULTS
  Overall, the two groups showed similar significant improvement in speech perception after implantation.
authors.affiliation: Instituto de Investigaciones en Ingenieria Genetica y Biologia Molecular, Consejo Nacional de Investigaciones Cientificas y Tecnicas, Universidad de Buenos Aires, Buenos Aires, Argentina. dalamon@dna.uba.ar
authors.forename: Viviana
authors.initials: V
authors.lastname: Dalamón
authors.forename: Vanesa
authors.initials: V
authors.lastname: Lotersztein
authors.forename: Marcela
authors.initials: M
authors.lastname: Lipovsek
authors.forename: Agustina
authors.initials: A
authors.lastname: Bèherán
authors.forename: Maria Elena
authors.initials: ME
authors.lastname: Mondino
authors.forename: Fernando
authors.initials: F
authors.lastname: Diamante
authors.forename: Norma
authors.initials: N
authors.lastname: Pallares
authors.forename: Vicente
authors.initials: V
authors.lastname: Diamante
authors.forename: Ana Belen
authors.initials: AB
authors.lastname: Elgoyhen
mesh_terms: D001302:Audiometry, Speech; D016022:Case-Control Studies; D002675:Child, Preschool; D019929:Cochlear Implantation; D000072259:Connexin 26; D000075302:Connexin 30; D017630:Connexins / Q000235:genetics*; D004252:DNA Mutational Analysis; D003638:Deafness / Q000151:congenital* / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009154:Mutation; D013067:Speech Perception
chemical_list: D000075302:Connexin 30; D017630:Connexins; C000607042:GJB2 protein, human; C491020:GJB6 protein, human; D000072259:Connexin 26