title: Predictive factors and outcomes of cochlear implantation in patients with connexin 26 mutation: a comparative study.
abstract: OBJECTIVE
  To compare hearing outcomes in patients with connexin 26 (Cx 26) mutations undergoing cochlear implantation to age matched controls and to examine whether age at implantation, gender and type of mutation were correlated with hearing outcome.
  
  
  METHODS
  Retrospective chart review of 21 patients with Cx 26 mutations that underwent cochlear implantation compared to 18 age-matched controls. Patients' characteristics, type of mutation and pre- and postoperative short and long-term hearing thresholds, word and sentence scores were analyzed.
  
  
  RESULTS
  There was no statistically significant difference between the Cx 26 and control group in the mean short term and mean long term post-operative pure tone averages (PTA), speech reception thresholds (SRT), word and sentence scores. Gender, age at implantation and type of connexin 26 mutation did not predict hearing outcomes.
  
  
  CONCLUSIONS
  In patients with connexin 26 mutation, cochlear implantation provides an effective mean of auditory habilitation. Mutational status, age and gender do not seem to predict hearing outcomes.
authors.affiliation: Case Western Reserve University School of Medicine, Cleveland, OH, USA.
authors.forename: Hani M
authors.initials: HM
authors.lastname: Rayess
authors.affiliation: Ear, Nose, and Throat Institute, Case Western Reserve University, University Hospitals Case Medical Center, Cleveland, OH, USA.
authors.forename: Catherine
authors.initials: C
authors.lastname: Weng
authors.affiliation: Ear, Nose, and Throat Institute, Case Western Reserve University, University Hospitals Case Medical Center, Cleveland, OH, USA.
authors.forename: Gail S
authors.initials: GS
authors.lastname: Murray
authors.affiliation: Ear, Nose, and Throat Institute, Case Western Reserve University, University Hospitals Case Medical Center, Cleveland, OH, USA.
authors.forename: Cliff A
authors.initials: CA
authors.lastname: Megerian
authors.affiliation: Ear, Nose, and Throat Institute, Case Western Reserve University, University Hospitals Case Medical Center, Cleveland, OH, USA. Electronic address: maroun.semaan@uhhospitals.org.
authors.forename: Maroun T
authors.initials: MT
authors.lastname: Semaan
mesh_terms: D001301:Audiometry, Pure-Tone; D016022:Case-Control Studies; D002675:Child, Preschool; D019929:Cochlear Implantation; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics*; D003638:Deafness / Q000235:genetics* / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009154:Mutation; D012189:Retrospective Studies; D013067:Speech Perception; D016896:Treatment Outcome
chemical_list: D017630:Connexins; D000072259:Connexin 26