Related Experiment Video
Updated: May 10, 2026
06:04
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Cochlear implantation in children with syndromic deafness.
Stephen J Broomfield1, Iain A Bruce, Lise Henderson
1Department of Otolaryngology Head & Neck Surgery, North Bristol NHS Trust, Southmead Road, Bristol BS10 5NB, United Kingdom. steve_broomfield@hotmail.com
International Journal of Pediatric Otorhinolaryngology
|June 19, 2013
Summary
Cochlear implantation outcomes in children with syndromic deafness are often excellent, but results vary. Individual assessment is crucial due to potential additional disabilities and diverse outcomes within syndrome groups.
Area of Science:
- Otolaryngology
- Genetics
- Pediatrics
Background:
- Syndromic deafness presents unique challenges for cochlear implantation.
- Children with syndromic deafness are increasingly considered for cochlear implants.
Purpose of the Study:
- To examine the outcomes of cochlear implantation in children with syndromic deafness.
- To assess the impact of additional disabilities and syndrome variations on implant success.
Main Methods:
- Retrospective case series of 38 children with syndromic deafness undergoing cochlear implantation.
- Outcome measures included Bench-Kowal-Bamford (BKB) speech reception scores and Geers and Moog Speech Reception Scores (SRS).
Main Results:
- Syndromes included Waardenburg, Usher, Pendred, Jervell and Lange-Nielsen, CHARGE, and others.
- After 19 months, BKB scores ranged from 46-100% (median 87%).
- 55% achieved SRS level six, and 24% achieved level five, with significant outcome variation.
Conclusions:
- Cochlear implantation can yield excellent results in syndromic deafness.
- Additional disabilities are common and influence outcomes.
- Individualized assessment is essential due to variable outcomes within and between syndrome groups.