Hoyng C B, van Rijn P M, Deutman A F
Department of Ophthalmology, University of Nijmegen, The Netherlands.
Acta Ophthalmol Scand. 1996 Dec;74(6):639-41. doi: 10.1111/j.1600-0420.1996.tb00753.x.
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.
中心性晕轮状脉络膜营养不良（CACD）是一种罕见的遗传性疾病，可导致中年患者出现中心暗点。本文讨论了两例伴有感音神经性听力损失的CACD病例。一名62岁男性和一名51岁女性患者均主诉在数年中视力逐渐下降，随后出现听力损失。除了常规眼科和耳鼻喉科检查外，两名患者均接受了荧光素血管造影、（电）生理检查和听力测定检查。两名患者均发现色素上皮和脉络膜毛细血管有界限分明的萎缩区域。两名患者均在青春期后出现缓慢进展的感音神经性听力损失。到中年时听力恶化到需要佩戴助听器的程度。这两个病例表明，CACD可能与感音神经性听力损失有关。
