Cochlear Implantation in Patients With Usher Syndrome Type IIa Increases Performance and Quality of Life

Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
Bas P HartelRonald J E Pennings

Abstract

Usher syndrome type IIa (USH2a) is characterized by congenital moderate to severe hearing impairment and retinitis pigmentosa. Hearing rehabilitation starts in early childhood with the application of hearing aids. In some patients with USH2a, severe progression of hearing impairment leads to insufficient speech intelligibility with hearing aids and issues with adequate communication and safety. Cochlear implantation (CI) is the next step in rehabilitation of such patients. This study evaluates the performance and benefit of CI in patients with USH2a. Retrospective case-control study to evaluate the performance and benefit of CI in 16 postlingually deaf adults (eight patients with USH2a and eight matched controls). Performance and benefit were evaluated by a speech intelligibility test and three quality-of-life questionnaires. Patients with USH2a with a mean age of 59 years at implantation exhibited good performance after CI. The phoneme scores improved significantly from 41 to 87% in patients with USH2a (p = 0.02) and from 30 to 86% in the control group (p = 0.001). The results of the questionnaire survey demonstrated a clear benefit from CI. There were no differences in performance or benefit between patients with USH2a and co...Continue Reading

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Citations

Oct 28, 2019·Cochlear Implants International·Leslie P Molina-RamirezGraeme C M Black
Feb 19, 2020·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Leslie P Molina-RamírezGraeme C M Black
Nov 2, 2019·Clinical and Experimental Otorhinolaryngology·Sang-Yeon LeeByung Yoon Choi
May 11, 2020·Ophthalmology and Therapy·Helene Lønborg-MøllerLine Kessel
Mar 17, 2020·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Leslie P Molina-RamírezGraeme C M Black
Oct 1, 2020·Therapeutic Advances in Ophthalmology·Maria TomsMariya Moosajee
May 1, 2021·International Journal of Molecular Sciences·Evan M de JoyaXuezhong Liu
Apr 26, 2021·Molecular Therapy : the Journal of the American Society of Gene Therapy·Kalyan DullaErwin van Wijk
Jul 3, 2021·Journal of Clinical Medicine·Camron DaviesAdrien A Eshraghi
Mar 23, 2020·Hearing Research·Gwenaelle G S Géléoc, Aziz El-Amraoui
May 28, 2021·Ear and Hearing·Eric NisenbaumXuezhong Liu

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