【摘要】 问号耳是一种以耳廓中下部形态异常为主的先天性畸形，典型表现为耳轮下缘和耳垂之间的自然延续中断，存在不同程度的切迹或完全分离，还可以伴随耳廓上部的明显外突，所以又称之为蝶形耳。问号耳可以以散发性、家族性和耳髁突综合征特征之一等三种形式存在。该畸形的病因目前尚未明确，多认为与咽弓发育和下颌骨分化相关的EDN1-EDNRA-DLX信号通路异常有关。手术是矫正问号耳的根本方法。本文对问号耳的病因以及手术方式的研究进展进行归纳总结。更多还原

【Abstract】 Question mark ear is a congenital malformation that is characterized by an abnormal shape of the lower middle part of the auricle. It typically presents as an interruption of the natural continuation between the posterior helix and the lobule, with varying degrees of incision or complete separation. It also can be accompanied by a significant prominence of the upper auricle, so it is also called butterfly-shape ear in our country. Question mark ear can exist in three forms: Sporadic, familial, and one of the features of the Auriculocondylar syndrome. The etiology is not yet clear, but most studies suggest that the EDN1-EDNRA-DLX signaling pathway associated with pharyngeal arch development and mandibular differentiation may be an important cause. Surgery is the fundamental method to correct this malformation. In this article, the research progress on the etiology and surgical methods of question mark ear was reviewed.更多还原