Nadol J B, Adams J C, Kim J R
Department of Otology and Laryngology, Harvard Medical School, Boston, MA, USA.
Acta Otolaryngol Suppl. 1995;519:47-59. doi: 10.3109/00016489509121870.
The pathogenesis of sensorineural hearing loss in Menière's disease and experimental endolymphatic hydrops is not fully understood. At the light microscopic level, there is poor correlation between the histopathology and loss of sensitivity and speech discrimination. The results of electron microscopic investigation of histopathology and alterations in immunoreactivity in the organ of Corti and lateral cochlear wall in the hydropic guinea pig are presented. Loss of outer and inner hair cells and spiral ganglion cells, particularly in the apical turn was evident by light microscopy. By electron microscopy, further evidence of degeneration was detected in the cuticular plate of outer hair cells, neural endings of both inner and outer hair cells, myelinated dendritic fibers, spiral ganglion cells, and types I and II fibrocytes of the lateral cochlear wall. There was a marked decrease in immunoreactivity for a variety of enzymes, calcium binding proteins, structural proteins, and integral membrane proteins of gap junctions, particularly among type I and type II fibrocytes of the lateral cochlear wall. The evidence suggests that dysfunction and degeneration of hair cells, afferent neurons and fibrocytes of the lateral cochlear wall are involved in the pathogenesis of hearing loss in endolymphatic hydrops.
梅尼埃病和实验性内淋巴积水所致感音神经性听力损失的发病机制尚未完全明了。在光学显微镜水平，组织病理学与听力敏感度及言语辨别力丧失之间的相关性较差。本文呈现了对积水豚鼠的耳蜗 Corti 器和蜗外侧壁进行组织病理学的电子显微镜研究结果以及免疫反应性改变。光学显微镜下可见外毛细胞、内毛细胞及螺旋神经节细胞缺失，尤其是在蜗顶明显。电子显微镜下，在外毛细胞的角质板、内毛细胞和外毛细胞的神经末梢、有髓树突纤维、螺旋神经节细胞以及蜗外侧壁的 I 型和 II 型纤维细胞中检测到了进一步的退变证据。多种酶、钙结合蛋白、结构蛋白和缝隙连接的整合膜蛋白的免疫反应性显著降低，特别是在蜗外侧壁的 I 型和 II 型纤维细胞之间。证据表明，毛细胞、传入神经元及蜗外侧壁纤维细胞的功能障碍和退变参与了内淋巴积水所致听力损失的发病机制。
