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Cochlear Implantation in a Case of Auditory Neuropathy Spectrum Disorder with CAPOS Syndrome

Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder which characterized with normal outer hair cell function but disrupted neural synchrony in the afferent auditory pathway. CAPOS (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) syndrome can man...

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Autores principales: Atilgan, Atılım, Yuksel, Mustafa, Ciprut, Ayca
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Istanbul Medeniyet University 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7433731/
https://www.ncbi.nlm.nih.gov/pubmed/32821455
http://dx.doi.org/10.5222/MMJ.2019.53503
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author Atilgan, Atılım
Yuksel, Mustafa
Ciprut, Ayca
author_facet Atilgan, Atılım
Yuksel, Mustafa
Ciprut, Ayca
author_sort Atilgan, Atılım
collection PubMed
description Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder which characterized with normal outer hair cell function but disrupted neural synchrony in the afferent auditory pathway. CAPOS (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) syndrome can manifest itself with ANSD and this rare situation and audiological rehabilitation outcomes have not well documented in the literature. We aim to present a cochlear implant user subject with CAPOS syndrome and ANSD. A 14-year-old girl diagnosed with ANSD and CAPOS syndrome. She received unilateral cochlear implant (CI). Her hearing sensitivity and speech perception abilities have been improved with CI. Also, she has a good music perception ability measured with the Turkish version of Clinical Assessment of Music Perception Test. After detailed audiological evaluations, CI could be a good option for patients who have ANSD and CAPOS syndrome.
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spelling pubmed-74337312020-08-19 Cochlear Implantation in a Case of Auditory Neuropathy Spectrum Disorder with CAPOS Syndrome Atilgan, Atılım Yuksel, Mustafa Ciprut, Ayca Medeni Med J Case Report Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder which characterized with normal outer hair cell function but disrupted neural synchrony in the afferent auditory pathway. CAPOS (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) syndrome can manifest itself with ANSD and this rare situation and audiological rehabilitation outcomes have not well documented in the literature. We aim to present a cochlear implant user subject with CAPOS syndrome and ANSD. A 14-year-old girl diagnosed with ANSD and CAPOS syndrome. She received unilateral cochlear implant (CI). Her hearing sensitivity and speech perception abilities have been improved with CI. Also, she has a good music perception ability measured with the Turkish version of Clinical Assessment of Music Perception Test. After detailed audiological evaluations, CI could be a good option for patients who have ANSD and CAPOS syndrome. Istanbul Medeniyet University 2019 2019-09-27 /pmc/articles/PMC7433731/ /pubmed/32821455 http://dx.doi.org/10.5222/MMJ.2019.53503 Text en Copyright Istanbul Medeniyet University Faculty of Medicine. http://creativecommons.org/licenses/by-nc/4.0/ This journal is published by Logos Medical Publishing. Licenced by Creative Commons Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)
spellingShingle Case Report
Atilgan, Atılım
Yuksel, Mustafa
Ciprut, Ayca
Cochlear Implantation in a Case of Auditory Neuropathy Spectrum Disorder with CAPOS Syndrome
title Cochlear Implantation in a Case of Auditory Neuropathy Spectrum Disorder with CAPOS Syndrome
title_full Cochlear Implantation in a Case of Auditory Neuropathy Spectrum Disorder with CAPOS Syndrome
title_fullStr Cochlear Implantation in a Case of Auditory Neuropathy Spectrum Disorder with CAPOS Syndrome
title_full_unstemmed Cochlear Implantation in a Case of Auditory Neuropathy Spectrum Disorder with CAPOS Syndrome
title_short Cochlear Implantation in a Case of Auditory Neuropathy Spectrum Disorder with CAPOS Syndrome
title_sort cochlear implantation in a case of auditory neuropathy spectrum disorder with capos syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7433731/
https://www.ncbi.nlm.nih.gov/pubmed/32821455
http://dx.doi.org/10.5222/MMJ.2019.53503
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AT ciprutayca cochlearimplantationinacaseofauditoryneuropathyspectrumdisorderwithcapossyndrome