Igarashi M, MacRae D, O-Uchi T, Alford B R
ORL J Otorhinolaryngol Relat Spec. 1981;43(1):4-16. doi: 10.1159/000275520.
This is a report of cochleo-saccular degeneration found in temporal bones from a patient who had suffered from slowly progressive and total sensorineural deafness which had an inherited origin. At age 8, this patient began to complain of hearing loss, and by age 10 she was totally deaf. The patient was 1 of 3 female siblings who have suffered from an exactly identical progressive disease: deafness, absent gastric motility, small bowel diverticulitis and ulceration, and sensory neuropathy. The temporal bone pathology found in this case was the degenerative change in the cochlear duct and sacculus. No pathology was found in the utriculus and semicircular canals.
这是一份关于在一位患有遗传性缓慢进行性全感音神经性耳聋患者颞骨中发现的蜗球囊变性的报告。该患者8岁时开始诉说听力损失，到10岁时完全失聪。该患者是3名女性兄弟姐妹之一，他们都患有完全相同的进行性疾病：耳聋、胃动力缺乏、小肠憩室炎和溃疡以及感觉神经病变。该病例中发现的颞骨病理变化是蜗管和球囊的退行性改变。椭圆囊和半规管未发现病理变化。
