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

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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.