遗传性进行性感音神经性聋少见,作者发现两个家系患者,一家系3代7例发病,为常染色体显性遗传。另一家系4代4例发病,为显性遗传。两家系内发病年龄基本一致,在8～10岁或10余岁。由于起病时语言发育已完成且起病后听力减退为渐进性,故本病患者一般聋而不哑,临床听力学检查为两侧对称性 Hereditary sensory neurotic deafness rare, the authors found that two families of patients, a family of 3 generations of 7 cases of onset, autosomal dominant. Another family 4 cases of 4 cases of onset, dominant inheritance. The age of onset of the two families is basically the same, at 8 to 10 years old or over 10 years old. As the onset of language development has been completed and the onset of hearing loss as progressive, so the general deafness of patients with non-dumb, the clinical auditory examination for bilateral symmetry