当前位置： SCI文献检索 > INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY期刊下所有文献 > High homogeneity in auditory outcome of pediatric CI-patients with mutations in Gap-Junction-Protein Beta2.

High homogeneity in auditory outcome of pediatric CI-patients with mutations in Gap-Junction-Protein Beta2.

Abstract：

OBJECTIVES:A controversy exists in the literature regarding better auditory performance of cochlear implanted children with mutations in the Gap-Junction-Protein Beta2 (GJB2) gene. This is partially due to insufficient numbers, lack of proper statistics, etc. As this represents a very important issue for the counselling clinician, the aim of the study was to analyze auditive outcome in GJB2-related hearing loss in relation to other causes and review the literature. METHODS:Retrospective study. 44 children with unilateral cochlea implants were assigned into 3 groups according to their cause of deafness: group A contained 13 patients with GJB2-related deafness, group B 15 with hereditary hearing loss and negative GJB2-screening, and group C 16 with a nonhereditary cause. The auditory outcome performance was evaluated by pure-tone audiograms (PTA), the Monosyllabic-Trochee-Polysyllabic-Word Test (MTP) and the Listening Progress Profile (LiP) according to the EARS-protocol (Evaluation of Auditory Response to Speech) pre- and postoperatively up to 6.5 years. Additionally the educational setting was considered. Statistical analysis included direct comparison by using mixed models and computing the variance to test for homogeneity. RESULTS:A trend towards better performance for the GJB2 group (vs. the other groups combined) was observed regarding PTA, even more pronounced for LiP/MTP. However, a significant p-value was not reached. A high homogeneity, expressed by a significant difference in the variance of MTP and LiP was observed in the GJB2 group. Differences in educational setting were not significant. CONCLUSIONS:The results of the study seem to support a better auditory performance of GJB2-patients with cochlea implants due to a cochlear origin of the defect. The significant homogeneity for this group is of paramount issue for the counselling clinician and a very important observation.

journal_name

Int J Pediatr Otorhinolaryngol

journal_title

International journal of pediatric otorhinolaryngology

authors

Reinert J,Honegger F,Gürtler N

doi

10.1016/j.ijporl.2010.04.002

subject

Has Abstract

pub_date

2010-07-01 00:00:00

pages

791-5

issue

7

eissn

0165-5876

issn

1872-8464

pii

S0165-5876(10)00174-6

journal_volume

74

pub_type

杂志文章

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