OBJECTIVE: Waardenburg syndrome is an autosomal-dominant syndrome characterized 
by dystopia canthorum, hyperplasia of the eyebrows, heterochromia irides, a 
white forelock, and sensorineural hearing loss in 20% to 55% of patients. This 
patient population accounts for approximately 2% of congenitally deaf children. 
The purpose of this retrospective case review was to describe the outcomes for 
those children with Waardenburg syndrome who have undergone cochlear 
implantation.
METHODS: Pediatric cochlear implant recipients with documented evidence of 
Waardenburg syndrome underwent retrospective case review. All patients received 
their cochlear implants at the study institution followed by outpatient auditory 
habilitation. Charts were reviewed for etiology and duration of deafness, age at 
time of cochlear implantation, perioperative complications, duration of use, and 
performance outcomes. Results of standard tests batteries for speech perception 
and production administered as a part of the patients' auditory habilitation 
were reviewed.
RESULTS: Seven patients with Waardenburg syndrome and cochlear implants were 
identified. The average age at implantation was 37 months (range, 18-64 months) 
and the average duration of use was 69 months (range, 12-143 months). All of 
these patients are active users of their devices and perform very well after 
implantation. There were no major complications in this small group of patients.
CONCLUSIONS: Children with congenital sensorineural hearing loss without other 
comorbidities (e.g., developmental delay, inner ear malformations) perform well 
when they receive cochlear implantation and auditory habilitation. Patients with 
Waardenburg syndrome can be expected to have above-average performance after 
cochlear implantation.