title: Rehabilitation with cochlear implant in patient with harboyan syndrome.
abstract: 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.
authors.affiliation: Doctor of Science in Children's and Teenager's Health, Federal University of Rio Grande do Sul-UFRGS, Porto Alegre/RS, Brazil ; Professor of Speech-Language Pathology, Fatima's University (RS), Rio de Janeiro/RJ, Brazil.
authors.forename: Lauren Medeiros
authors.initials: LM
authors.lastname: Paniagua
authors.affiliation: Speech-Language Pathologist, Voice Specialist, Hospital de Clinicas de Porto Alegre, Porto Alegre/RS, Brazil.
authors.forename: Maria Elza Kazumi Yamaguti
authors.initials: ME
authors.lastname: Dorfman
authors.affiliation: Department of Otorhinolaryngology, University Hospital of Porto Alegre, Porto Alegre/RS, Brazil ; Graduate Program in Medicine: Surgery, Medical School, Federal University of Rio Grande do Sul, Porto Alegre/RS, Brazil.
authors.forename: Luiz
authors.initials: L
authors.lastname: Lavinsky
authors.affiliation: Audiologist, Professor of Federal University of Rio Grande do Sul (UFRGS), Porto Alegre/RS, Brazil.
authors.forename: Pricila
authors.initials: P
authors.lastname: Sleifer
keywords: cochlear implants; hearing loss; rehabilitation of speech and language disorders; sensorineural