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常染色体遗传性骨硬化病(ADO)为一种遺传性骨代谢性疾病。本病以弥漫性、对称性和无骨缺损的骨硬化为特征,由于骨硬化与颅骨的孔道狭窄而常使脑神经受损害。作者对45万余居民作系统调查,发现本病共32例。其中14例按放射学的两型进行耳神经学表现(ONM)的研究。方法:全组摄取颅骨平片和岩骨断层片。测定内听道宽度并以年龄、性别与对照组配对比较。骨性外听道宽度<4mm为狭窄,<3mm为重度狭窄。耳神经学检查拟定正常范围为:SDS>84％;纯音听阈0.25～4KHz均<15dB;SRT在0.5～4KHz;HT声刺激≤100dB。声阻抗测定声顺值低于对照组50％
Autosomal Hereditary Osteosclerosis (ADO) is a hereditary disease of bone metabolism. The disease is characterized by diffuse, symmetrical and boneless osteopetrosis, which often impairs the cranial nerves due to osteosclerosis and narrowing of the cranial orifice. The author of 450,000 residents for a systematic survey found a total of 32 cases of the disease. Among them, 14 cases were studied by otology (ONM) according to two types of radiology. Methods: The whole group of cranial plain film and petrous bone fracture slice. The width of the internal auditory canal was measured and compared with the control group by age and sex. Skeletal external auditory canal width <4mm for stenosis, <3mm for severe stenosis. Otolaryngological examination to develop the normal range: SDS> 84%; tone threshold 0.25 ~ 4KHz are <15dB; SRT at 0.5 ~ 4KHz; HT sound stimulation ≤ 100dB. The acoustic impedance was lower than that of the control group