1858年Von Graeffe首先报告色素性视网膜炎(RP)合并耳聋。随后,临床上有关上述二病的综合征报告日多,其中最熟悉的是Usher氏综合征,它表现为先天性感音聋及随后发生色素性视网膜炎。该病通常是由常染色体隐性遗传所致,少数病例的听力下降是进行性的。作者对17名被证实患有色素性视网膜炎的病人进行听力学研究,其中9名发现有显著 In 1858, Von Graeffe first reported retinitis pigmentosa (RP) with deafness. Subsequently, the clinical symptoms of the above two syndromes reported more days, of which the most familiar is Usher’s syndrome, which is manifested as congenital sensorineural hearing loss and subsequent retinitis pigmentosa. The disease is usually caused by autosomal recessive inheritance, a few cases of hearing loss is progressive. The authors performed audiological studies on 17 patients with retinitis pigmentosa, of which 9 were found to be significant