TONAL AUDIOLOGICAL PERFORMANCE EVALUATION AFTER COCHLEAR IMPLANTATION IN CHILDREN WITH GJB2 GENE RELATED HEARING LOSS

Abstract

Introduction: Audiological performance in cochlear implanted children may vary depending of the etiology of the deafness, one of which is GJB2 gene mutations. Aim: In this study we evaluated the auditory performance by pure tone audiogram testing after unilateral cochlear implantation for children with GJB2 related mutations and to compare the results with the ones from children that did not show any mutations in the GJB2 gene. Material and Methods: We have determined the auditory tonal thresholds in 28 patients and we calculated the average of these thresholds for the frequencies of 500, 1000, 2000 and 4000Hz. We have taken into consideration the first tonal thresholds that averaged at 35dB or better for the given frequencies. Results and Discussions: Comparison between the average tonal thresholds between the two groups show that even though children from the GJB2 mutation related deafness group need a slightly longer period of time to reach minimal conversational level thresholds, the dB value of these levels is lower than the ones in the second group. Conclusions: This can prove to be a useful tool for the clinician regarding cochlear implant counselling and for the patient in terms of better speech understanding skills and oral communication with parents and educational staff.

Keywords

• audiological evaluation
• cochlear implant
• connexin 26
• deafness
• GJB2 mutation