Usher综合征Ⅲ型是一罕见疾病,其特征是:色素性视网膜炎和进行性感觉神经性聋。1977年,Davenport和Omenn研究了本病,将其分为四型。Ⅰ型:重度先天性聋,10岁左右出现色素性视网膜炎,约占90%。Ⅱ型:中度至重度先天性聋,18～20岁出现色素性视网膜炎,约占10%。Ⅲ型:进行性听力减退,青春期出现明显的色素性视网膜炎。Ⅰ～Ⅲ型均显示常染色体隐性遗传。Ⅳ型符合X-连锁遗传并与Ⅱ型相似。Ⅰ型前庭反应消失,Ⅱ型前庭反应正常或减弱。Ⅲ型和Ⅳ型少见,不到1%。作者们报告1例Usher综合征Ⅲ型,患者为一9岁男孩,无中耳炎或其它病史。家族中无先天性畸形等。患孩有一妹,听力正常。父母亲无同血缘史。4岁时因讲话延迟于1972年2月就诊,测听显示双侧中度感觉神经性聋。给患孩配戴了双耳助听器并定期随访。1年后复查听力,又损失25～35分贝。声阻抗测听检查正常。两耳正常。7(1/2)岁时因视力障碍6个月去眼科检查,眼 Usher syndrome type III is a rare disease characterized by retinitis pigmentosa and progressive sensorineural deafness. In 1977, Davenport and Omenn studied the disease and divided it into four types. Type Ⅰ: Severe congenital deafness, retinitis pigmentosa occurs about 10 years old, accounting for about 90%. Type II: moderate to severe congenital deafness, 18 to 20 years of age appear retinitis pigmentosa, accounting for about 10%. Type III: progressive hearing loss, adolescent obvious retinitis pigmentosa. Ⅰ ~ Ⅲ type showed autosomal recessive inheritance. Type IV conformed to X-linked inheritance and similar to type II. Type Ⅰ vestibular reaction disappeared, type Ⅱ vestibular response was normal or weakened. Type Ⅲ and Ⅳ rare, less than 1%. The authors report 1 case of Usher syndrome type III, a 9-year-old boy without otitis media or other medical history. No congenital malformations in the family. Children have a sister, normal hearing. Parents have no history of blood. 4 years old due to speech delay in February 1972 treatment, audiometry showed bilateral moderate sensory nerve deafness. Wearing children’s hearing aids and regular follow-up. After 1 year review hearing, but also lost 25 ~ 35 decibels. Acoustic impedance audiometry test is normal. Normal ears. 7 (1/2) years old due to visual impairment 6 months to eye examination, eyes