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.

Key words:

Question mark ear, Butterfly-shape ear, Auriculocondylar syndrome, Surgical methods