Study of pathogenesis of immune mediated inner ear disease

本研究探讨内耳免疫损伤动物模型的病理生理。将锁孔帽贝血青素（KLH）全身灌注豚鼠，并将KLH直接激发至内淋巴囊（ES）。内淋巴水肿（EH）最早在5小时内出现在囊部和基底部，并在2天内迅速发展。一周后EH逐渐消失。然而，9周后，EH在囊、基部和根尖部复发。ES的细胞反应从5小时开始明显发生，12小时达到最大。这些细胞是急性炎症细胞。1周后淋巴细胞和浆细胞占优势，且持续3个月以上。在急性炎症期，出血偶有发生，主要发生在淋巴周围区，此时常伴有感觉细胞变性。髓过氧化物酶最早在5小时内分布于ES、感觉毛细胞、螺旋韧带和血管纹，并在1天内达到最大值。刺激性自发性眼震发生约12小时，麻痹性眼震发生约48小时。大多数动物在2天后出现听阈升高和热反应抑制，随后恢复。一周内淋巴周围抗klh抗体水平升高与内耳形态生理损伤的严重程度密切相关。这些结果提示，ES中反复出现的免疫反应可能导致眩晕发作，类似于梅尼埃病。

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.