Cochlear implantation in Waardenburg syndrome: The Indian scenario.
CONCLUSIONS
Children with Waardenburg syndrome (WS) exhibiting normal inner ear anatomy, like those included in our cohort, derive significant benefit from cochlear implantation and results are comparable to those reported for the general population of implanted children.


OBJECTIVE
The patient population of WS accounts for approximately 2% of congenitally deaf children. The purpose of this retrospective case review was to describe the outcomes for those children with WS who have undergone cochlear implantation.


METHODS
On retrospective chart review, there were four cases with WS who underwent cochlear implantation. These cases were assessed for age at implantation, clinical and radiological features, operative and perioperative course, and performance outcomes. Auditory perception and speech production ability were evaluated using categories of auditory performance (CAP), meaningful auditory integration scales (MAIS), and speech intelligibility rating (SIR) during the follow-up period.


RESULTS
In this group of children with WS, with a minimum follow-up of 12 months, the CAP score ranged from 3 to 5, MAIS from 25 to 30, and SIR was 3. These scores are comparable with those of other cochlear implantees.
Deka
Ramesh Chandra
RC
Department of ENT, All India Institute of Medical Sciences, New Delhi, India. director@aiims.ac.in
Sikka
Kapil
K
Chaturvedy
Gaurav
G
Singh
Chirom Amit
CA
Venkat Karthikeyan
C
C
Kumar
Rakesh
R
Agarwal
Shivani
S
D001307:Auditory Perception / Q000502:physiology*; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000453:epidemiology / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D015994:Incidence; D007194:India / Q000453:epidemiology; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome; D014849:Waardenburg Syndrome / Q000453:epidemiology / Q000503:physiopathology / Q000601:surgery*