Zajtchuk J T, Lindsay J R
Ann Otol Rhinol Laryngol. 1975 May-Jun;84(3 Pt 1):350-8. doi: 10.1177/000348947508400311.
The temporal bone report of an operated case of osteogenesis imperfecta tarda is presented. Histological examination confirmed the presence of bilateral fixation of the footplate by otosclerosis as the cause of the conductive hearing loss. Fragility of bony septae in the mastoid and of the stapedial crura were observed. Sensorineural impairment in later years with a reduction in neural elements in the cochlea appear related to the extent and activity of the otosclerotic foci. Additional temporal bone reports of three cases of osteogenesis imperfecta congenita show lack of deposition of the skein-like bone in the endochondral layer, sparse bony septae in marrow spaces and deficiency of the perosteal layer. The stapedial crura were thin and in two cases both were deformed and fractured.
本文呈现了一例晚发型成骨不全症手术病例的颞骨报告。组织学检查证实，镫骨足板双侧固定是由耳硬化症引起的传导性听力损失的原因。观察到乳突内骨间隔和镫骨脚的脆弱性。晚年出现的感音神经性损害以及耳蜗内神经元数量减少似乎与耳硬化病灶的范围和活动有关。另外三例先天性成骨不全症的颞骨报告显示，软骨内骨层缺乏绞丝状骨沉积，骨髓腔内骨间隔稀疏，骨膜层不足。镫骨脚很细，其中两例镫骨脚均有变形和骨折。
