| Background:In mainland of our country, the incidence of congenital hearing loss is 1/1000-6/1000 in newborn. Most of the congenital hearing loss is sensorineural hearing loss (CSNHL). The primary pathogenies of non-genetic CSNHL are infection of microorganism and the use of ear-toxicity medicament in pregnancy and high-danger pregnancy. But up to now, many of CSNHL are still unclear.In 1979, McCabe firstly put forward a disease's name "autoimmune sensorineural hearing loss (ASNHL)". It was found that immune response against inner ear tissuses and cells happened. It also had the ability of immune responses to special antigen in inner ear. The immune response in inner ear could induce obvious functional and pathological trauma in labyrinthine (including cochlear and vestibule parts). It was found that the levels of antibodies against inner ear tissues antigens in serum ascended in ASNHL patients. IgG was the primary type of antibodies in serum. It was be found that the some of offsprings of the female ASNHL guinea pigs appeared sensorineural hearing loss in preterit experimantal research.Some of clinical studies about pregnancy with autoimmune disease discovered the IgG type of auto-antibodies in mother could transport to fetus through placenta-barrier, and then they may affect the growth of the system or organs in fetus or newborn.Up to now, there are no any of reports about the offspring of ASNHL happened CSNHL.Object:This experiment research was intended to investigate whether or not the autoimmune against inner ear could induce the hearing functional and labyrinth structural pathological impair in newborns and the characteristics of sensorineural hearing loss and inner ear pathological morphological changes. Then, based on the results of this study, it also wanted to explore a new way for etiological research of CSNHL.Methods:The female guinea pigs were immunized with crude inner ear antigens (CIEAgs) for establishing the animal model of ASNH, and they also had been continuously immunized in pregnancy until childbearing. At the same time, the control group was set up.After the birth, the auditory function was measured with EcochG, including acoustic nerve compound action potential (cAP) and cochlear microphonic potential (CM).The special antibodies in serum were measured with ELISA in all female guinea pigs before and after immunization with inner ear antigens and their offspring.Celloidin sections of the inner ear were observed under light microscope.The succinate dehydrogenase (SDH) in hair cells and stria vascularis stained by tetrazolium was observed with the frozen section of cochlea and surface preparation of the organ of Corti and the spiral ligament.Results:There are about 22.5% happened ASNHL in the mother, which manifested the abnormity of one or more item parameters of cAP and CM.The female ASNHL of mother showed broad pathologic damnification in inner ear, such as the effusion of inflammatic cells and a great deal of sediment in cochlea canal, the grain of hematin in cochlea bone walls and around blood vein, the undernourished stria vascularis, the denaturalization of spiral ganglion cells, and different degrees of endolymphatic hydrops or the atrophy of hair cells.The special antibodies against inner ear antigens in the serum of the female ASNHL guniea pigs and their offsprings ascended evidently.The activity of SDH in hair cells and stria vascularis of the female ASNHL guniea pigs depressed.About 30% of the offsprings of the female ASNHL guniea pigs happened CSNHL.The pathological changes in offspring of the female ASNHL guniea pigs mostly suffered denaturalization of spiral ganglion cells.The level of special antibodies in the serum of female ASNHL guniea pig's offspring was found ascened, but less evident than that of their mothers.The activity of SDH in hair cells and stria vascularis of the offsprings of the female ASNHL also depressed.Conclusions:About 30% of the offsprings happened CSNHL following the autoimmunity against inner ear in thei... |
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