先天性双侧外耳道闭锁合并有全身其他处异常的综合征,文献中曾有多种报道。作者们曾见到一个家族三代20人中间,有6例有先天性异常,认为系一种新的综合征。包括有以下特征: (1)先天性双侧对称性或孤立的次完全性外耳道闭锁;(2)双侧先天性垂直距骨(或其他足部畸形);(3)眼间距增宽,但不是过距症(hyperteloism),其他一些特征如第五手指较短,双侧手掌单一横皱摺,幽门狭窄及先天性眼外科等,但不是经常出现。根据家族成员的遗传学调查,作者们认为本病很象是有变异的常染色体显性遗传,未发现有已知的隐性遗传的特征。在家系中第二代有一个 Congenital bilateral external auditory canal obstruction with other systemic abnormalities syndrome, there have been many reports in the literature. The authors have seen a family of three generations of 20 people in the middle, there are 6 cases of congenital anomalies, that is a new syndrome. Including the following features: (1) congenital bilateral symmetry or isolated subtotal external auditory canal atresia; (2) bilateral congenital vertical talus (or other foot deformities); (3) Hypertension (hyperteloism), other features such as the fifth finger shorter, a single lateral fold palms, pyloric stenosis and congenital eye surgery, but not often appear. According to genetic studies of family members, the authors think the disease is much like an autosomal dominant inheritance with no known recessive inheritance. There is a second generation in the family