このエントリーをはてなブックマークに追加
ID 34221
FullText URL
Author
Kawasaki, Akihiro
Fukuda, Shoichiro
Abstract

Objective: While investigators have reported that patients with GJB2-associated deafness and cochlear implants have preferable language development, the mechanisms of this phenomenon remains unknown. The goat of the present study was to assess higher brain functions of patients with GJB2-retated and GJB2-unrelated deafness as a method of evaluating language development.
Methods: Eight children with cochlear implants were subjected to genetic testing for GJB2 and underwent the Raven colored progressive matrices test, Rey's auditory verbal learning test, Rey's complex figure test, the standardized Language test for aphasia, the picture vocabulary test, and the standardized comprehension test for abstract words.
Results:Three children were diagnosed with GJB2-related deafness, and five children were diagnosed with GJB2-unrelated deafness. All three GJB2-related cases demonstrated normal range higher brain functions and fair language development. By contrast, one GJB2-unrelated case showed a semantic disorder, another demonstrated a visual cognitive disorder with dyslexia, and another had attention deficit-hyperactivity disorder.
Conclusions:Children with GJB2-unrelated deafness showed a high frequency of heterogeneous disorders that can affect proper language development. This difference between children with GJB2-retated and GJB2-unrelated deafness may account for the improved language development in children with GJB2-related deafness and cochlear implants. Further, genetic diagnosis of the non-syndromic hearing toss represents a useful tool for the preoperative prediction of outcomes following a cochlear implant procedure.

Keywords
cochlear implant
higher brain function
GJB2
language development
non-syndromic hearing loss
learninng disability
Note
Digital Object Identifer:10.1016/j.ijporl.2006.01.010
Published with permission from the copyright holder. This is the institute's copy, as published in the Journal of International Journal of Pediatric Otorhinolaryngology, August 2006, Volume 70, Issue 8, Pages 1343-1349.
Publisher URL:http://dx.doi.org/10.1016/j.ijporl.2006.01.010
Direct access to Thomson Web of Science record
Copyright © 2006 Elsevier Ireland Ltd. All rights reserved.
Published Date
2006-8
Publication Title
International Journal of Pediatric Otorhinolaryngology
Volume
volume70
Issue
issue8
Start Page
1343
End Page
1349
Content Type
Journal Article
language
英語
Refereed
True
DOI
Web of Sience KeyUT
Submission Path
otorhinolaryngology/5