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成骨不全的特征与骨质变脆,蓝巩膜及进行性听力减退。听力减退常开始于10至20多岁,可为单纯的传导性或混合性耳聋,高频感音性耳聋很常见,传导性听力减退的原因为镫骨足板固定,有时可见镫骨骨折。根据病理学、生物化学、临床遗传学,成骨不全和耳硬化症是两种完全不同的疾病。本文分析了荷兰58耳成骨不全镫骨手术后部分耳听力减退的病例,并对其病因进行回顾性分析。本组成骨不全47例,男17,女30例,共58耳,于1960～1988年行镫骨手术,4耳作过修正手术。10例(男3,女7)12耳术后出现听力减退(失败组),文章分析了听力减退和第一次镫骨手术时的年龄、骨折次数、
The characteristics of osteogenesis imperfecta and bone fragility, blue sclera and progressive hearing loss. Hearing loss often starts in the 10 to 20 years of age, can be purely conductive or mixed deafness, high-frequency sensorineural hearing loss is common, conductive hearing loss due to the stapes foot plate fixation, sometimes visible tarsal fracture. According to pathology, biochemistry, clinical genetics, osteogenesis imperfecta and otosclerosis are two completely different diseases. In this paper, we analyzed the cases of partial aural hearing loss after 58 cases of osteotomies in the Netherlands with osteogenesis imperfecta and retrospectively analyzed its etiology. The composition of the bone in 47 cases, 17 males and 30 females, a total of 58 ears, in the period from 1960 to 1988 stapes operation, 4 ears for revision surgery. 10 cases (male 3, female 7) 12 ears after hearing loss (failure group), the article analyzes the hearing loss and the first stapes operation age, number of fractures,