患者,女,14岁。因双耳自幼听力差,左耳间断流脓4～5年,经门诊拍摄X线片见左耳乳突巨大胆脂瘤,于1985年6月入院。自幼双耳听力差,右耳尤甚,说话靠看口形,无眩晕。追问病史:自出世即颈短,右眼外斜,颈后枕部有囊性膨出。家族中无此病史。母孕五胎,第一胎健在;第二、三胎因畸形生后死亡(何种畸形不详);该患儿为第四 Patient, female, 14 years old. Due to poor hearing of both ears since childhood, left ear intermittent purulent 4 to 5 years, the clinic X-ray film see the left ear mastoid giant cholesteatoma, admitted in June 1985. Earle ear hearing poor, especially the right ear, talking by looking at the mouth shape, no dizziness. Question history: Since the birth of the neck is short, right eye diagonal, posterior occipital cystic bulging. No such history in the family. Mother twins, the first child alive; the second and third child died of deformity (what deformity is unknown); the fourth child was fourth