- 期刊
Mondini氏內耳發育不全的顳骨組織病理發現
Temporal Bone Histopathologic Findings of Mondini Dysplasia
摘要
Mondini氏內耳發育不良其病變爲內耳骨性迷路及膜性迷路結構的不正常，此疾病常合併其他器官的病變。本科報告病例爲26個月的小男孩，是因先天性心臟病去世，經死後15小時屍體解剖，且取出顳骨，經固定、脫鈣、中和、脫水、賽璐錠(celloidin)包埋、硬化後，以20μ的厚度，水平方位連續切片。其屍體解剖結果發現爲先天性心臟病，肝臟腫大，無脾臟，而顳骨病理組織切片發現爲Mondini氏內耳發育不良。主要變化爲(1)耳蝸融合成圓形狀，骨螺旋板(osseous spiral lamina)變形；(2)柯氏器(organ of corti)只有少量的上皮細胞，無毛髮細胞；(3)半規管變形；(4)內聽道篩板(lamina cribrosa)缺損；(5)耳蝸與前庭廣泛相通；(6)鐙骨足板細薄不規則及鐙骨發育不全；(7)顏面神經管裂縫及裸露。
並列摘要
Mondini dysplasia is characterized by bony and membranous anomalies of the inner ear exhibiting a wide range of morphologic abnormality. Mondini dysplasia may occur in association with anomalies in other organs. A 26-month-old boy died of congenital heart disease. His temporal bone was harvested within 15 hours after death, processed and embedded in celloidin. Sections were cut in the horizontal plane at a thickness of 20μm and stained with H & E for light microscopic study. The autopsy findings of this boy include heart disease, hepatomegaly and asplenia. The temporal bone pathologic findings were: (1) the cochlea is a round space with deformed osseous spiral lamina; (2) the organ of Corti is a small mound of epithelial cell with no hair cells; (3) the semicircular canals are deformed; (4)the bony plate of the lamina cribrosa is deficient in the internal auditory canal; (5) the cochlea is wide communication with the vestibule; (6) the stapes footplate is thin and irregular, the stapes is in incomplete development; (7) the facial nerve canal is dehiscent and the facial nerve exposed.