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Usher综合征是童年后以聋盲为主的一种疾病,其主要特征是先天性聋和色素性视网膜炎,有的还有精神和前庭功能障碍,约占总人口的十万分之三,先天性聋的3～6%。根据临床症状和遗传类型,目前将该征分为4型,其第3型就是以进行性聋为特征,为双侧耳蜗性聋,在出生时即有,在少儿时期就可达到需行康复训练的严重程度,但全聋者很罕见。
Usher syndrome is a childhood deafness-based disease, its main features are congenital deaf and pigmented retinitis, and some mental and vestibular dysfunction, accounting for about three hundred thousandths of the total population, Congenital deafness 3 ~ 6%. According to clinical symptoms and genetic types, the current classification of the syndrome is divided into 4, the third is characterized by progressive deafness, bilateral cochlear deafness, that is, at birth, can be achieved in childhood rehabilitation The severity of training, but the deaf are rare.