宾-西本曼(Bing-Siebenmann)型内耳畸形为先天遗传性内耳畸形中罕见类型,其特点是骨迷路发育良好,膜迷路及其感觉终器发育不全或畸形发育。现将我院收治1例报告如下。 患男,21岁,陕西汉中人,因先天性左外耳道闭锁、小耳廓畸形住院。父母系中均无类似病史,非近亲结婚,母亲孕期病史及服药史不详。体检除左小耳廓畸形、耳孔闭锁外,全身未见异常。X线 Bing-Siebenmann-type inner ear deformity is a rare type of congenital hereditary inner ear deformity characterized by good development of the bony labyrinth, labyrinth and sensory terminal hypoplasia or deformity. Now in our hospital admitted to a report as follows. Male, 21 years old, Hanzhong, Shaanxi, due to congenital left external ear canal atresia, small auricle deformity hospitalization. No similar parental history, non-relatives married, mother’s history and medication history is unknown. Physical examination except left auricle deformity, ear closure, the body no exception. X-ray