title: Influence of DFNB1 status on expressive language in deaf children with cochlear implants.
abstract: OBJECTIVE
  The objective of this study was to compare the language growth of children with connexin-related deafness (DFNB1) who received cochlear implants versus the language growth of implanted children with non-DFNB1 deafness.
  
  
  METHODS
  A prospective longitudinal observational study and analysis.
  
  
  METHODS
  Two tertiary referral centers.
  
  
  METHODS
  There were 37 children with severe-to-profound hearing loss who received cochlear implants before the age of 5 years.
  
  
  METHODS
  A standardized language measure, the section for expressive language of the Reynell Developmental Language Scale was used to assess expressive language skills at 2 times postimplantation (14 and 57 mo postimplantation). Molecular screening for DFNB1 gene variants.
  
  
  METHODS
  Language quotient (LQ) scores (i.e., age-equivalent score obtained on the Reynell Developmental Language Scale divided by the child's chronological age), results of genotyping.
  
  
  RESULTS
  The mean language age at the second time interval (mean ± standard deviation, 51.8 ± 13 mo) was greater than at the first testing session (mean ± standard deviation, 19 ± 8 mo, p < 0.001, Wilcoxon signed rank test). When divided by genotype, DFNB1 children exhibited a higher LQ and less variability in scores than non-DFNB1 children at the second testing interval (Wilcoxon sign rank test, p = 0.0034). A regression analysis (linear-fit by least squares) conducted on 26 children with preimplantation audiometric data showed that DFNB1 status was the independent variable with greater predictive effect on LQ at the second testing interval, followed by age at implantation (R2 = 0.35, p = 0.0479).
  
  
  CONCLUSIONS
  Deaf children who received cochlear implants before the age of 5 years and use oral communication show substantial improvement in language abilities. In this study, DFNB1 children who use cochlear implants show greater gains in expressive language than non-DFNB1 children, independent of residual hearing, age at implantation, and duration of implant use.
authors.affiliation: Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida 33136, USA. sangeli@med.miami.edu
authors.forename: Simon I
authors.initials: SI
authors.lastname: Angeli
authors.forename: Hamlet
authors.initials: H
authors.lastname: Suarez
authors.forename: Alina
authors.initials: A
authors.lastname: Lopez
authors.forename: Thomas J
authors.initials: TJ
authors.lastname: Balkany
authors.forename: Xue Z
authors.initials: XZ
authors.lastname: Liu
mesh_terms: D002648:Child; D002675:Child, Preschool; D003054:Cochlear Implants; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics*; D005260:Female; D005838:Genotype; D006319:Hearing Loss, Sensorineural / Q000235:genetics* / Q000601:surgery; D006801:Humans; D007223:Infant; D007802:Language; D008297:Male; D011446:Prospective Studies; D013060:Speech
chemical_list: D017630:Connexins; C000607042:GJB2 protein, human; D000072259:Connexin 26