Cochlear implantation in Waardenburg's syndrome

Children with Waardenburg's syndrome (WS) derive significant benefit from cochlear implantation (CI) and do so to an extent that is comparable to that of the general population of implanted children. Although we report on a relatively small cohort, our data are useful for counseling the parents of children with WS considering CI.

To present our experience with CI in patients with WS.

A retrospective record review was conducted for five children who underwent CI in our department between 1993 and 2004.

Children with WS comprised 1.9% of our entire pediatric CI population: four girls had a familial history of WS and the phenotype of WS Type I, and one boy met the criteria for WS Type II. They were all diagnosed as having bilateral profound sensorineural hearing loss 4-24 weeks after birth. Rehabilitation was initiated immediately and included bilateral fitting of hearing aids and intensive speech and language therapy. Otoscopic and temporal bone high-resolution CT findings were normal in all patients. At surgery, all children were found to have a patent cochlea, and the electrodes were implanted into the scala tympani without difficulty. After 1.3-10.2 years of implant use all children achieved open-set recognition of 2-syllable words, with an average score of 81%. Four of the 5 children achieved open-set recognition of monosyllabic words (average score 40%) and 3 achieved open-set recognition of words in sentences (average score 81%).