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

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.