Waardenburg syndrome as a challenging experience in pediatric cochlear implantation

Abstract

Background Waardenburg syndrome (WS) is a major cause of symptomatic sensorineural hearing loss, which accounts for 2–5% of patients with congenital hearing loss. Cochlear implantation (CI) has shown improvement in auditory perception and language skills of syndromic sensorineural hearing-impaired children and is now accepted as a gold standard treatment. Aim The aim of the present study was to evaluate the outcome of CI in cases with WS and to compare the results with nonsyndromic CI cases. Participants and methods A total of 268 children of less than 7 years of age underwent CI in the National Hearing and Speech Institute, and of these, six children, as group A, had WS, whereas the control group, as group B, consisted of 16 congenitally deaf children without any other comorbidities or inner ear anomalies. The following assessments were done: intraoperative impedance and auditory response threshold, and then at 3 and at 12 months, impedance, aided average pure tone threshold, Meaningful Auditory Integration Scale questionnaire, LittlEARS Auditory Questionnaire, and language assessment using modified Arabic preschool language scale-4. Results Intraoperative impedance, auditory response threshold, and neural response impedance were measured. Aided free field response at 500–4000 kHz and speech detection threshold were measured for both groups at 3 and 12 months after initiation. There was no statistically significant difference between both groups at all measured parameters. Meaningful Auditory Integration Scale questionnaire, LittlEARS Auditory Questionnaire, and language assessment using modified Arabic preschool language scale-4 revealed improvements in speech perception and production; however, comparison of the results between the WS group and the control group was insignificant. Conclusion CI is a good rehabilitation option for children with WS. The study indicates that children with WS benefit from CI similar to typically developing CI children.

Keywords

• cochlear implantation
• speech perception
• waardenburg syndrome