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Int Arch Otorhinolaryngol 2013; 17(04): 403-406
DOI: 10.1055/s-0033-1351669
Case Report
Thieme Publicações Ltda Rio de Janeiro, Brazil

Rehabilitation with Cochlear Implant in Patient with Harboyan Syndrome

Lauren Medeiros Paniagua
1   Doctor of Science in Children's and Teenager's Health, Federal University of Rio Grande do Sul-UFRGS, Porto Alegre/RS, Brazil
2   Professor of Speech-Language Pathology, Fatima's University (RS), Rio de Janeiro/RJ, Brazil
,
Maria Elza Kazumi Yamaguti Dorfman
3   Speech-Language Pathologist, Voice Specialist, Hospital de Clinicas de Porto Alegre, Porto Alegre/RS, Brazil
,
Luiz Lavinsky
4   Department of Otorhinolaryngology, University Hospital of Porto Alegre, Porto Alegre/RS, Brazil
5   Graduate Program in Medicine: Surgery, Medical School, Federal University of Rio Grande do Sul, Porto Alegre/RS, Brazil
,
Pricila Sleifer
6   Audiologist, Professor of Federal University of Rio Grande do Sul (UFRGS), Porto Alegre/RS, Brazil
› Author Affiliations
Further Information

Publication History

22 April 2011

15 April 2013

Publication Date:
13 September 2013 (online)

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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.

Keywords

cochlear implants - hearing loss - sensorineural - rehabilitation of speech and language disorders
 

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