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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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Detalles Bibliográficos
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
Descripción
Sumario: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.