Central areolar choroidal dystrophy (CACD) is a rare hereditary disorder leading to a central scotoma in middle-aged patients. Two cases of CACD in association with perceptive hearing loss are discussed. A 62-year-old male and a 51-year-old female patient complained of visual deterioration and subsequent hearing loss over several years. In addition to routine ophthalmic and otorhinolaryngological examination both patients underwent fluorescein angiography, (electro)physiological examination and audiometry examination. A demarcated area of atrophy of pigment epithelium and choriocapillaris was found in both patients. A slowly progressive sensorineural hearing loss after adolescence was found in both patients. The hearing deteriorated to such a level at middle age that hearing aids were necessary. These two cases show that CACD may be associated with perceptive hearing loss.