Alport综合征是伴有家族遗传性先天性出血性肾炎的遗传性感音神经性耳聋综合征,也称为家族性出血性肾炎或家族遗传性出血性肾炎并耳聋综合征。以进行性肾功能减退、血尿和双侧对称性感音聋为特征。我科于1980年3月间曾见1家3例(1母2子),作了听力计检查,现报道如下。病例摘要例1:蒋×,女性,51岁。21年前(1959年)尿检发现有红细胞(+)和蛋白(++),持续至今,症状不严重,有轻度听力减退,但对话无困难。有时有耳鸣,有间歇短暂头 Alport syndrome is a hereditary sensorineural deafness syndrome with familial hereditary hemorrhagic nephritis, also known as familial hemorrhagic nephritis or familial hemorrhagic nephritis and deafness syndrome. With progressive renal dysfunction, hematuria and bilateral symmetrical acoustic hearing loss is characterized. My Section in March 1980 had seen a 3 cases (1 mother 2 children), made a hearing test, are reported below. Case Summary Example 1: Chiang ×, female, 51 years old. 21 years ago (1959) urine test found red blood cells (+) and protein (++), until now, the symptoms are not serious, mild hearing loss, but the dialogue without difficulties. Tinnitus sometimes, intermittent head short