title: Performance after cochlear implantation in DFNB1 patients.
abstract: OBJECTIVE
  To evaluate the speech perception and language development with cochlear implants (CI) of DFNB1 children in comparison with non-DFNB1 deaf children.
  
  
  METHODS
  Retrospective case series.
  
  
  METHODS
  Academic tertiary center.
  
  
  RESULTS
  Thirty-one congenitally deafened children, screened for GJB2 allele variants, were followed for an average 32 months after CI. With the use of age-appropriate testing, 75% of DFNB1 and 53% of non-DFNB1 children achieved open set word recognition (speech perception category [SPC] level 6). Multivariate analysis showed that SPC was primarily dependent on duration of CI use, but not on the cause of hearing loss. In Reynell language tests, DFNB1 children showed more consistent and quicker gains than non-DFNB1 children.
  
  
  CONCLUSIONS
  Although children with CI with DFNB1 show faster gains in Reynell scores, duration of CI use appears to have a greater effect on speech perception than DFNB1 status.
  
  
  CONCLUSIONS
  Identification of DFNB1 children is useful in counseling of CI outcomes.
authors: University of Miami Ear Institute, University of Miami Miller School of Medicine, Miami, FL 33136, USA.
authors: Sarah S
authors: SS
authors: Connell
authors: Simon I
authors: SI
authors: Angeli
authors: Hamlet
authors: H
authors: Suarez
authors: Annelle V
authors: AV
authors: Hodges
authors: Thomas J
authors: TJ
authors: Balkany
authors: Xue Z
authors: XZ
authors: Liu
mesh_terms: D000483:Alleles; D002648:Child; D002663:Child Language; D002675:Child, Preschool; D019929:Cochlear Implantation; D003054:Cochlear Implants; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics*; D003638:Deafness / Q000151:congenital / Q000235:genetics* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D005544:Forecasting; D014644:Genetic Variation / Q000235:genetics; D006579:Heterozygote; D006801:Humans; D007223:Infant; D007804:Language Development; D008297:Male; D009154:Mutation / Q000235:genetics; D012189:Retrospective Studies; D013060:Speech / Q000502:physiology; D013067:Speech Perception / Q000502:physiology; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D017630:Connexins; C000607042:GJB2 protein, human; D000072259:Connexin 26