Hearing and speech performance after cochlear implantation in children with Waardenburg syndrome

Abstract

Waardenburg syndrome (WS) is a rare autosomal-dominant syndrome that can be presented with sensorineural hearing loss. In this report, we describe the outcomes of three children with WS at zero, three, nine, twelve and sixty months after cochlear implant (CI) fitting. The outcomes were assessed using IT-MAIS (Infant-Toddler Meaningful Auditory Integration Scale - younger than 5 year), MAIS (Meaningful Auditory Integration Scale - older than 5 year), MUSS (Meaningful Use of Speech Scale), and categories of auditory performance and speech intelligibility. The results showed an improvement in auditory and language performance over time, two patients who used CI for 5 years achieved 100% in IT-MAIS and MUSS tests. In addition, both were able to understand sentences in open set and achieve fluent speech. Moreover, both reached fluency on auditory and language performance scale. The third patient with 50 months of follow-up and in the 48 months evaluation, is in category 5 of auditory performance and 3 of speech intelligibility. We concluded that all children who had low levels of hearing and language before cochlear implant have improved hearing and language skills after implantation and rehabilitation.