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遗传因素引起的内耳疾病早为人们所认识,常见类型是隐性遗传。由于许多环境因素使得遗传对人类先天性耳聋的影响难以弄清。作者以 Jerker 突变小鼠为动物模型,以具有频率特性的听性脑干反应阈值为指标(范围2～31.5kHz),对小鼠遗传性耳聋的规律进行了研究。作者认为,近交系小鼠的遗传背景和等位基因对听力的影响可以排除,可作为研究单基因突变对内耳致畸作用的良好模型。小鼠内耳畸形可分为三类:包括形态发生、神经上皮和耳蜗球囊异常,与人类有相似之处。Jerkre系突变是神经上皮异常的典型代表,为第四对
Genetic diseases caused by ear disease as early as people know, the common type is recessive. Due to many environmental factors, genetic influence on human congenital deafness is difficult to understand. In this study, Jerker mutant mice were used as animal models, and the frequency of auditory brainstem response threshold (frequency range 2 ~ 31.5 kHz) was used as an index to study the law of hereditary deafness in mice. The author believes that the inbred mice genetic background and alleles on the hearing effect can be ruled out, can be used as a single gene mutation on the inner ear teratogenic effect of a good model. Mouse inner ear deformity can be divided into three categories: including morphogenesis, abnormal neuroepithelial and cochlear balloon, and human similarities. Jerkre mutation is a typical representative of neuroepithelial abnormalities, the fourth pair