Cochlear Implantation in Children with Usher's Syndrome: A South Asian Experience.

Indian Journal of Otolaryngology and Head and Neck Surgery : Official Publication of the Association of Otolaryngologists of India
Geetha NairMohan Kameswaran

Abstract

Usher's syndrome is an autosomal recessive disorder characterized by dual sensory impairment involving both the ears and eyes. Cochlear implantation paves a way to restore hearing loss in such individuals but poor vision among these patients poses additional challenges for the habilitationists. This study aimed to compare the habilitation outcomes and hearing-related quality of life scores of cochlear implantees having Usher syndrome with age-matched cochlear implantees with no such syndromic association. 27 patients aged 1-6 years with Usher syndrome underwent cochlear Implantation over a period of 10 years from 2006 to 2016 and were included in this study along with an age-matched cohort of 30 implantees with no additional disabilities. Category of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores were compared at 3, 6, 9 and 12 months respectively. Glasgow Benefit Inventory and Health Utility Index (HUI 3.0) questionnaires were used to assess the hearing-related quality of life in both groups at 1 year post implantation. There was significant difference in CAP and SIR scores between children with Usher's syndrome and the control group (p < 0.05). The overall scores in terms of quality of life as well ...Continue Reading

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