定量观察了出生后１３个月的先天缺失ＳＯＤ１基因小鼠的耳蜗毛细胞以及螺旋神经节和疆孔内的神经纤维，并与同龄野生型和出生后２个月的野生型小鼠进行了比较。发现毛细胞和螺旋神经节的缺损约为５０％，神经纤维的减少则超过５０％，提示由于与铜和锌相结合的过氧化物歧化酶的缺失导致了耳蜗神经与终器的严重退变 Quantitative observation of cochlear hair cells and neurofibrils in the splenic ganglia and villus of SOD1 gene-deficient mice at 13 months after birth was compared with wild-type mice of the same age and wild-type mice at 2 months after birth . Defects in hair cells and spiral ganglia were found to be about 50% and nerve fiber loss was more than 50%, suggesting a severe degeneration of the cochlear nerve and terminator due to the loss of the superoxide dismutase bound to copper and zinc