The effect of GJB2 allele variants on performance after cochlear implantation

Paul W Bauer; Ann E Geers; Christine Brenner; Jean S Moog; Richard J H Smith

doi:10.1097/00005537-200312000-00015

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The effect of GJB2 allele variants on performance after cochlear implantation

Journal article

Peer reviewed

The effect of GJB2 allele variants on performance after cochlear implantation

Paul W Bauer, Ann E Geers, Christine Brenner, Jean S Moog and Richard J H Smith

The Laryngoscope, Vol.113(12), pp.2135-2140

12/2003

DOI: 10.1097/00005537-200312000-00015

PMID: 14660916

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Abstract

It has been hypothesized that etiology of hearing loss may serve as an independent variable in performance after cochlear implantation. To test this hypothesis, the authors identified pediatric cochlear implant recipients with gap junction protein beta2 (GJB2)-related deafness. The study examines performance outcomes associated with GJB2 deafness-causing allele variants. Pediatric cochlear implant patients were screened for GJB2 allele variants; statistical comparisons were made with prospectively obtained performance measures. From 181 children who participated in a nationwide cochlear implant research program, 122 children were identified with congenital nonsyndromic sensorineural hearing loss and invited to participate. Screening for GJB2 allele variants was completed for 55 children. The children were homogeneous with respect to age (8 or 9 y) and age at implant (before age 5 y). All patients have previously undergone a prospective regimented battery of performance measures. Performance measures were compared between 22 children with and 33 children without mutations to determine whether GJB2 status was a significant predictor of cochlear implant outcomes. Reading and cognitive outcomes were significantly dependent on connexin status. The group of children who tested positive for GJB2-related deafness scored significantly higher on a nonverbal cognitive measure, Block Design, and on a measure of reading comprehension. The isolated insult to the cochlea created by GJB2 allele variants allows for preservation of central cognitive function. Better reading performance is seen in children with GJB2-related deafness.

Prospective Studies

Humans

Connexins - genetics

Male

Treatment Outcome

Cognition

Connexin 26

Genetic Variation

Hearing Loss - genetics

Cochlear Implantation

Reading

Alleles

Hearing Loss - rehabilitation

Female

Child

Details

Title: Subtitle: The effect of GJB2 allele variants on performance after cochlear implantation
Creators: Paul W Bauer - Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390-9035, USA. paul.bauer@utsouthwestern.edu
Ann E Geers
Christine Brenner
Jean S Moog
Richard J H Smith
Resource Type: Journal article
Publication Details: The Laryngoscope, Vol.113(12), pp.2135-2140
DOI: 10.1097/00005537-200312000-00015
PMID: 14660916
NLM abbreviation: Laryngoscope
ISSN: 0023-852X
eISSN: 1531-4995
Publisher: United States
Grant note: R01 DC 02842 / NIDCD NIH HHS
Language: English
Date published: 12/2003
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
Record Identifier: 9984006429002771

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