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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Detalhes bibliográficos
Autores: Paniagua, Lauren Medeiros, Dorfman, Maria Elza Kazumi Yamaguti, Lavinsky, Luiz, Sleifer, Pricila
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2013
País:Brasil
Recursos:Universidade Federal do Rio Grande do Sul (UFRGS)
Repositorio:Repositório Institucional da UFRGS
Idioma:inglés
OAI Identifier:oai:www.lume.ufrgs.br:10183/111923
Acesso em linha:http://hdl.handle.net/10183/111923
Access Level:acceso abierto
Palavra-chave:Envelhecimento
Implante coclear
Perda auditiva
Reabilitação dos transtornos da fala e da linguagem
Cochlear implants
Hearing loss
Sensorineural
Rehabilitation of speech and language disorders
Descrição
Resumo: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.