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目的:证实针对内耳组织抗原特异性抗体通过胎盘可造成子鼠先天性自身免疫性感音神经性聋。方法:同种粗制内耳抗原(CIEAgs)免疫豚鼠,造成自身免疫性感音神经性聋(ASNHL)动物模型。ELISA法测定示其血清抗体水平升高,耳蜗电图(ECochG)示其听神经复合动作电位和(或)耳蜗微音器电位阈值或伪阈升高。取其血清(含特异性抗内耳组织抗原抗体)持续转移免疫妊娠豚鼠,采用ECochG测试被动免疫妊娠母鼠和子鼠的听觉功能,再采用颞骨火棉胶切片和苏木精-伊红染色,光镜观察内耳组织形态学改变。结果:各组出现听觉损伤的动物是:采用ASNHL模型动物血清被动免疫的8只妊娠母鼠中有5只(7耳)和其所产子鼠8只中有6只(10耳),采用非ASNHL模型动物血清免疫的妊娠豚鼠所产子鼠5只中有1只(2耳)。出现听力损失的被动免疫母鼠和子鼠内耳病理形态学改变主要为螺旋神经节细胞变性和Rosenthal管中炎性细胞(以单个核细胞为主)浸润,部分动物出现膜迷路积水。结论:同种体液转移免疫可造成自身免疫性内耳病变,子鼠产生听觉功能障碍主要是由于母鼠所产生的特异性抗内耳组织抗原抗体经胎盘到达子鼠体内所致。
OBJECTIVE: To demonstrate that congenital autoimmune sensorineural deafness can be induced in the offspring through the placenta by targeting antigen-specific antibodies in the inner ear tissue. Methods: Guinea pigs were immunized with the same kind of crude inner ear antigen (CIEAgs), resulting in an animal model of autoimmune sensorineural hearing loss (ASNHL). ELISA method showed elevated levels of serum antibodies, ECochG showed its auditory nerve compound action potential and / or cochlear microphter threshold or pseudo-threshold increased. The serum of guinea pigs (including anti-inner ear antigen antibody) was continuously transferred to immunize pregnant guinea pigs. The auditory function of passive immunized pregnant rats and offspring mice was detected by ECochG. The temporal bone flagellation and hematoxylin-eosin staining Microscopic observation of inner ear tissue morphological changes. Results: Animals with auditory impairment in each group were: 5 of 8 pregnant rats (7 ears) passive immunized with ASNHL animal serum and 6 (10 ears) of 8 produced offspring, There were 1 (2 ears) out of 5 rats in the guinea pigs immunized with ASNHL model animal serum. Pathological changes in the inner ear of passive immunized mice and offspring of hearing-impaired mice were mainly caused by degeneration of spiral ganglion cells and infiltration of inflammatory cells (dominated by mononuclear cells) in Rosenthal tube, and some animals showed labyrinthine hydrops. CONCLUSION: Allogeneic humoral immune transfusion can cause autoimmune inner ear lesion. The auditory dysfunction of the offspring is mainly caused by the anti-inner ear antigen antibody produced by the female rats reaching the offspring through the placenta.