CENTRAL AREOLAR CHOROIDAL DYSTROPHY AND SLOWLY PROGRESSIVE SENSORINEURAL HEARING-LOSS

Central areolar choroidal dystrophy (CACD) is a rare hereditary disord er leading to a central scotoma in middle-aged patients. Two cases of CACD in association with perceptive hearing loss are discussed. A 62-y ear-old male and a 51-year-old female patient complained of visual det erioration and subsequent hearing loss over several years. In addition to routine ophthalmic and otorhinolaryngological examination both pat ients underwent fluorescein angiography (electro)physiological examina tion and audiometry examination. A demarcated area of atrophy of pigme nt epithelium and choriocapillaris was found in both patients. A slowl y progressive sensorineural hearing loss after adolescence was found i n both patients. The hearing deteriorated to such a level at middle ag e that hearing aids were neccessary. These two cases show that CACD ma y be associated with perceptive hearing loss.