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Rehabilitation with Cochlear Implant in Patient with Harboyan Syndrome

Background Harboyan syndrome, defined as congenital corneal dystrophy associated with progressive sensorineural hearing loss, was first described by Harboyan in 1971. It is a hereditary disease manifested by eye lesions consistent with corneal endothelial dystrophy and progressive sensorineural hear...

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Autores principales: Paniagua, Lauren Medeiros, Dorfman, Maria Elza Kazumi Yamaguti, Lavinsky, Luiz, Sleifer, Pricila
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Thieme Publicações Ltda 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4399191/
https://www.ncbi.nlm.nih.gov/pubmed/25992045
http://dx.doi.org/10.1055/s-0033-1351669
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author Paniagua, Lauren Medeiros
Dorfman, Maria Elza Kazumi Yamaguti
Lavinsky, Luiz
Sleifer, Pricila
author_facet Paniagua, Lauren Medeiros
Dorfman, Maria Elza Kazumi Yamaguti
Lavinsky, Luiz
Sleifer, Pricila
author_sort Paniagua, Lauren Medeiros
collection PubMed
description Background Harboyan syndrome, defined as congenital corneal dystrophy associated with progressive sensorineural hearing loss, was first described by Harboyan in 1971. It is a hereditary disease manifested by eye lesions consistent with corneal endothelial dystrophy and progressive sensorineural hearing loss. There is bilateral symmetric progressive hearing loss, which may be either dominant or recessive. Objective To report a case of a patient with a diagnosis of Harboyan syndrome. Case Report A 25-year-old woman with profound bilateral sensorineural hearing loss, showing poor hearing performance while using a personal sound amplification device, underwent hearing rehabilitation with a cochlear implant. Conclusion Rehabilitation was imperative in this case. The cochlear implant has proven to be the best therapeutic option, providing the patient with a better quality of life.
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spelling pubmed-43991912015-05-19 Rehabilitation with Cochlear Implant in Patient with Harboyan Syndrome Paniagua, Lauren Medeiros Dorfman, Maria Elza Kazumi Yamaguti Lavinsky, Luiz Sleifer, Pricila Int Arch Otorhinolaryngol Article Background Harboyan syndrome, defined as congenital corneal dystrophy associated with progressive sensorineural hearing loss, was first described by Harboyan in 1971. It is a hereditary disease manifested by eye lesions consistent with corneal endothelial dystrophy and progressive sensorineural hearing loss. There is bilateral symmetric progressive hearing loss, which may be either dominant or recessive. Objective To report a case of a patient with a diagnosis of Harboyan syndrome. Case Report A 25-year-old woman with profound bilateral sensorineural hearing loss, showing poor hearing performance while using a personal sound amplification device, underwent hearing rehabilitation with a cochlear implant. Conclusion Rehabilitation was imperative in this case. The cochlear implant has proven to be the best therapeutic option, providing the patient with a better quality of life. Thieme Publicações Ltda 2013-10 /pmc/articles/PMC4399191/ /pubmed/25992045 http://dx.doi.org/10.1055/s-0033-1351669 Text en © Thieme Medical Publishers
spellingShingle Article
Paniagua, Lauren Medeiros
Dorfman, Maria Elza Kazumi Yamaguti
Lavinsky, Luiz
Sleifer, Pricila
Rehabilitation with Cochlear Implant in Patient with Harboyan Syndrome
title Rehabilitation with Cochlear Implant in Patient with Harboyan Syndrome
title_full Rehabilitation with Cochlear Implant in Patient with Harboyan Syndrome
title_fullStr Rehabilitation with Cochlear Implant in Patient with Harboyan Syndrome
title_full_unstemmed Rehabilitation with Cochlear Implant in Patient with Harboyan Syndrome
title_short Rehabilitation with Cochlear Implant in Patient with Harboyan Syndrome
title_sort rehabilitation with cochlear implant in patient with harboyan syndrome
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4399191/
https://www.ncbi.nlm.nih.gov/pubmed/25992045
http://dx.doi.org/10.1055/s-0033-1351669
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