Cochlear implantation in children with syndromic deafness.

Abstract

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.

• Authors: Stephen J Broomfield, Iain A Bruce, Lise Henderson, Richard T Ramsden, Kevin M J Green
• Journal: International journal of pediatric otorhinolaryngology (Int J Pediatr Otorhinolaryngol) Vol. 77 Issue 8 Pg. 1312-6 (Aug 2013) ISSN: 1872-8464 [Electronic] Ireland
• PMID: 23773333 (Publication Type: Journal Article)
• Copyright: Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Topics

• Abnormalities, Multiple (pathology, psychology, surgery)
• Adolescent
• Child
• Child, Preschool
• Cochlear Implantation (rehabilitation)
• Cochlear Implants
• Female
• Hearing Loss, Sensorineural (complications, pathology, therapy)
• Humans
• Male
• Retrospective Studies
• Speech Perception
• Syndrome
• Treatment Outcome