Maintenance of profound deaf model mouse and functional analysis of it's auditory system

当我们在我们封闭的ICR腹水小鼠群中遇到表现出盘旋运动的癫痫小鼠时，我们分离出一种自发突变的行为异常的动物。被命名为kuru2的突变小鼠对声音没有反应。为了阐明其听力能力，我们测量了小鼠的听觉脑干反应（ABR）。ABR表明小鼠的敏锐度严重受损。该小鼠表现出绕圈、共济失调步态和过度兴奋等异常行为，其遗传方式为常染色体隐性遗传。这一结果来自于对系谱和回交检验的探索。雄性或雌性突变体与野生型动物交配。将F1小鼠与突变小鼠回交。对100只子代的行为进行了分析。没有后代F1表现出听力障碍或异常行为。正常和失聪的老鼠是一比一。结果在两性中是相同的。我们推测该表型可能来自单个基因突变。由于这种动物有各种各样的症状，因此需要进行大规模的研究来阐明潜在的遗传学。

We isolated a spontaneous mutant animal with abnormal behaviors when we encountered epileptic mice exhibiting circling movement in our closed colony of ICR Ascites mice. The mutant mice, designated to kuru2, did not respond to sounds. In order to elucidate their hearing ability, we measured the auditory brain stem response (ABR) of the mice. ABR demonstrated that the mice were profoundly impaired acuity. Abnormal behaviors including circling, ataxic gait, and hyperirritability of the mouse were demonstrated.The genetic mode of the mouse was autosomal recessive. This result came from an exploration of the pedigree and backcross examinations. Either male or female mutants were mated with wild type animals. The F1 mice were backcrossed to the mutant mice. Behavior of 100 offspring were analyzed. None of the offspring F1 demonstrated hearing impairment or abnormal behaviors. Normal versus deaf mice was one to one. The result was same in the both sexes. We presumed that the phenotype might be from a single gene mutation. Since the animal has a wide variety of symptoms, a large-scale study will be required to elucidate the underlying genetics.