Cochlear Implantation in Children with Usher’s Syndrome: A South Asian Experience
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 were statistically different (p < 0.05). Though there was improvement in speech and language acquisition after cochlear Implantation this was found to be of lesser extent than the normative cohort. These children with additional visual disabilities required intensive, individualized therapy catering to their complex needs. Their family’s perception of expected benefit from cochlear Implantation was guarded and needed to be appraised in detail prior to surgery. This experience helped prepare an institutional protocol for counseling such implantees in future.
KeywordsUsher syndrome Cochlear implant (CI) Category of Auditory Performance (CAP) Speech Intelligibility Rating (SIR) Hearing Related Quality of Life (HR-QOL) Glasgow Benefit Inventory (GBI) Health Utility Index version 3 (HUI 3.0)
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
Ethical committee approval obtained on 24th June 2016.
- 1.Usher CH (1935) Bowman’s lecture: on a few hereditary eye affections. Trans Ophthalmol Soc UK 55:164–245Google Scholar
- 13.Young NM, Johnson JC, Mets MB, Hain TC (1995) Cochlear implants in young children with Usher’s syndrome. Ann Otol Rhinol Laryngol 9(2):34–45Google Scholar
- 15.Waltzmann S, Jareen M et al (2013) Outcomes for children with deaf-blindness with cochlear implants: a multisite observational study. Otol Neurotol 34(3):13–21Google Scholar
- 17.Pennings RJ et al (2006) Audiologic performance and benefit of cochlear implantation in Usher syndrome type-I. The Laryngoscope 116(5):717–722. https://doi.org/10.1097/01.mlg.0000205167.08415.9e CrossRefPubMedGoogle Scholar
- 19.Fitzpatrick R, Davey C, Buxton MJ, Jones DR (1998) Evaluating patient based outcome measures for use in clinical trials. Health Technol Assess (Winch, Engl) 2(14):1–74Google Scholar
- 22.Institute of Hearing Research. The Glasgow Health Status Questionnaires Manual 1998. http://www.ihr.mrc.ac.uk. Accessed 1 Sept 2019