Cochlear implantation for children with GJB2-related deafness.
OBJECTIVE
Mutations in GJB2 are a common cause of congenital sensorineural hearing loss. Many children with these mutations receive cochlear implants for auditory habilitation. The purpose of the study was to compare the speech perception performance of cochlear implant patients with GJB2-related deafness to patients without GJB2-related deafness.


METHODS
Retrospective case review.


METHODS
Pediatric cochlear implant recipients who have been tested for GJB2 mutation underwent chart review. All patients received cochlear implantation at a tertiary referral center, followed by outpatient auditory habilitation. Charts were reviewed for cause and duration of deafness, age at time of cochlear implantation, intraoperative and postoperative complications, duration of use, and current age. Results of standard tests of speech perception administered as a part of the patients' auditory habilitation were reviewed.


RESULTS
Twenty patients with GJB2 mutations were compared with 27 patients without GJB2 mutations. There was no statistical difference between patients with and without GJB2-related congenital sensorineural hearing loss with regard to open-set and closed-set speech recognition performance at 12, 24, and 36 months after cochlear implantation. Surgical complications were uncommon.


CONCLUSIONS
Pediatric patients with congenital sensorineural hearing loss without other comorbid conditions (eg, developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. The presence or absence of GJB2 mutation does not appear to impact speech recognition performance at 12, 24, and 36 months after implantation.
W. Paul Biggers, MD, Carolina Children's Communication Disorders Program, Department of Otolaryngology--Head and Neck Surgery, University of North Carolina, Chapel Hill, North Carolina, USA.
Robert D
RD
Cullen
Craig A
CA
Buchman
Carolyn J
CJ
Brown
Ben J
BJ
Copeland
Carlton
C
Zdanski
Harold C
HC
Pillsbury
Carol G
CG
Shores
D002675:Child, Preschool; D019929:Cochlear Implantation; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics*; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000151:congenital / Q000235:genetics* / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D009154:Mutation; D011183:Postoperative Complications; D013067:Speech Perception
D017630:Connexins; C000607042:GJB2 protein, human; D000072259:Connexin 26