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
