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Updated: May 10, 2026
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
Insights
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.
Objective:
To examine the outcome of cochlear implantation in children with syndromic deafness, who are increasingly being considered for cochlear implants and who represent a unique challenge to the cochlear implant team.
Method:
In this retrospective case series in a tertiary referral cochlear implant centre, we describe a series of 38 children with a clinical syndrome causing deafness who have undergone cochlear implantation. The outcome measures are Bench-Kowal-Bamford (BKB) speech reception score (range 0-100%) and speech perception ability using the Geers and Moog Speech Reception Score (SRS) (range from 0; no speech perception, to 6; open set recognition of words).
Results:
The syndromes identified were Waardenburg syndrome (n = 10), Usher syndrome (n = 9), Pendred syndrome (n = 7), Jervell and Lange-Nielsen syndrome (n = 5), CHARGE syndrome (n = 2), and 1 each of Stickler, CINCA (Chronic Infantile Neurological Cutaneous and Articular), Bartter, Down, and Donnai-Barrow syndromes. After a minimum of 19 months following implantation, BKB was measurable in 20 of 38 patients, and ranged from 46 to 100% in quiet (median 87%, mean 81%). Eighteen children (55%) achieved a SRS at level six, and a further 8 (24%) achieved level five. There was significant variation of outcome between and within syndrome groups.
Conclusions:
Additional disabilities are frequently encountered when considering children for cochlear implantation, and may be part of a recognised syndrome. Outcome is often excellent but can be variable even within the same syndrome group, and such children are therefore assessed on an individual basis to ensure a realistic expectation.