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Cochlear Implantation for Children With GJB2 ‐Related Deafness
Author(s) -
Cullen Robert D.,
Buchman Craig A.,
Brown Carolyn J.,
Copeland Ben J.,
Zdanski Carlton,
Pillsbury Harold C.,
Shores Carol G.
Publication year - 2004
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1097/00005537-200408000-00019
Subject(s) - habilitation , medicine , audiology , cochlear implant , cochlear implantation , auditory neuropathy , sensorineural hearing loss , hearing loss , congenital hearing loss , speech perception , retrospective cohort study , perception , surgery , psychology , philosophy , neuroscience , humanities
Objectives/Hypothesis: 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. Study Design: 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. Conclusion: 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.