Lu C B, Schuknecht H F
ENT Department, Tainan Air Force General Hospital, Taiwan.
Am J Otol. 1994 Jan;15(1):74-85.
Quantitative histologic studies were performed on 15 temporal bones from eight adult persons who were known to have prelingual bilateral profound hearing loss. The pathologic changes are characterized by severe degeneration of the structures of the cochlear duct, often with degeneration of the vestibular sense organs, causing a reparative host response that features osteoneogenesis and fibrous proliferation followed by retrograde neuronal degeneration. The pathology is consistent with meningogenic bacterial or viral labyrinthitis that occurred subclinically or went undiagnosed. Bone and fibrous tissue are present in varying extent in the scala tympani of 12 of the 15 temporal bones. Six cochleae from four subjects with fibro-osseous proliferation in the scala tympani extending as far as the ascending part of the basal turn have neuronal populations ranging from 963 to 5,355 (mean 2,826, 8% of neonatal normal, 35,500). In three cochleae from two subjects with no fibro-osseous proliferation in any area of the scala tympani the neuronal population ranges from 11,322 to 20,484 (mean 15,438, 43% of neonatal normal). Relative to cochlear implantation, computed tomographic imaging provides a means for determining the extent of fibro-osseous proliferation in the scala tympani, which in turn alerts the surgeon to surgical obstacles to optimal implantation as well as providing a basis for judging the extent of loss of cochlear neuronal population.
对8名已知患有双侧先天性重度听力损失的成年人的15块颞骨进行了定量组织学研究。病理变化的特征是蜗管结构严重退化，前庭感觉器官常同时退化，引发机体的修复反应，其特征为骨生成和纤维增生，随后是逆行性神经元变性。这种病理情况与亚临床发生或未被诊断出的致脑膜炎性细菌或病毒性迷路炎一致。15块颞骨中有12块的鼓阶中存在不同程度的骨组织和纤维组织。4名受试者的6个蜗管中，鼓阶有纤维性骨增生并延伸至蜗底转的上升部分，其神经元数量在963至5355之间（平均2826，为新生儿正常数量35500的8%）。2名受试者的3个蜗管中，鼓阶任何区域均无纤维性骨增生，其神经元数量在11322至20484之间（平均15438，为新生儿正常数量的43%）。相对于人工耳蜗植入，计算机断层扫描成像提供了一种确定鼓阶中纤维性骨增生程度的方法，这反过来可以提醒外科医生注意最佳植入的手术障碍，并为判断蜗神经细胞数量损失的程度提供依据。
