Cochlear implantation in pediatric patients with Cockayne Syndrome.

Abstract

Cockayne Syndrome (CS) is a rare, autosomal recessive disorder characterized by a spectrum of phenotypic abnormalities, including progressive sensorineural hearing loss (SNHL) that involves both peripheral and central components. To date, a single series of CS patients undergoing cochlear implant (CI) placement has been reported; this study reports on additional previously unreported pediatric CI recipients. Subjective benefits were noted early after activation in both patients, and speech perception scores improved over time as well, varying from 42 to 70% (versus 0-12% previously). Thus, we report that cochlear implantation in pediatric patients with CS can be effective in the management of progressive SNHL.

• Authors: Renae D Van Wyhe, Claudia V Emery, Robert A Williamson
• Journal: International journal of pediatric otorhinolaryngology (Int J Pediatr Otorhinolaryngol) Vol. 106 Pg. 64-67 (Mar 2018) ISSN: 1872-8464 [Electronic] Ireland
• PMID: 29447894 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2017. Published by Elsevier B.V.

Topics

• Child
• Cochlear Implantation (methods)
• Cochlear Implants
• Cockayne Syndrome (surgery)
• Female
• Hearing Loss, Sensorineural (surgery)
• Humans
• Magnetic Resonance Imaging
• Male
• Retrospective Studies
• Tomography, X-Ray Computed
• Treatment Outcome