Study of pathogenesis of immune mediated inner ear disease

本研究探讨了内耳免疫损伤动物模型的病理生理学。用匙孔血蓝蛋白（KLH）对豚鼠进行全身性预致敏，并用KLH直接激发内淋巴囊（ES）。内淋巴积水（EH）最早出现在球囊和基底弯，并在2天内迅速发展。1周后EH逐渐消失。但9周后球囊、基底及顶弯内均出现EH。ES细胞反应从5h开始明显，12h达到高峰。这些细胞是急性炎症细胞。一周后，淋巴细胞和浆细胞占优势，并持续3个月以上。急性炎症期出血主要发生在外淋巴区，并伴有感觉细胞变性。髓过氧化物酶在胚胎干细胞、感觉毛细胞、螺旋韧带和血管纹中的分布最早于5h，1d内达高峰。刺激性自发性眼震发生在12小时后，麻痹性眼震发生在48小时后。大多数动物在2天后出现听阈升高和热反应抑制，此后恢复。一周内外淋巴抗KLH抗体水平的升高与内耳形态生理学损伤的严重程度相关。结果提示ES反复免疫反应可能导致类似梅尼埃病的眩晕发作。

This study investigated pathophysiology of inner ear immune-injury in the animal model. Guineapigs were systemically presensitized with keyhole limpet hemocyanin (KLH) and were directly challenged with KLH to the endolymphatic sac (ES). Endolymphatic hydrops (EH) appeared in the saccule and the basal turn as soon as 5 hours and rapidly developed within 2 days. After one week, EH gradually disappeared. However, after 9 weeks, EH reccurred in the saccule, basal and apical turn. Cellular reaction in the ES obviously ocurred from 5 hours and reached maximum at 12 hours. These cells were acute inflammatory cells. After one week, lymphocytes and plasma cells were prodominant and remained for more than 3 months. In the acute inflammatory phase, hemorrhage occasionally occured mainly in the perilymphatic region in which case degeneration of sensory cells often accompanied. Myeloperoxidase distributed in the ES, sensory hair cells, spiral ligament and stria vasularis as soon as 5 hours and reached maximum within one day. Irritative spontaneous nystagmus occurred nearly after 12 hours and paralytic nystagmus followed nearly for 48 hours. Elevation of hearing threshold as well as suppression of caloric response occurred after 2 days and thereafter recoverd in most animals. Elevation in perilymphatic anti-KLH antibody levels within one week correlated well to the severity of morphophsyiological damage of the inner ear. These results suggested that recurrent immune reaction in the ES may lead to cause attack of vertigo, similar to Meniere's disease.