The clinical features of patients with congenital microtia mainly involve auricle, external auditory canal and middle ear, while the inner ear is often not involved. According to the degree of deformity, the most commonly used clinical classification is three types:

Grade I: the size and shape of auricle have changed, but the important surface mark structure of auricle exists, and the external auditory canal is narrow, and in severe cases, the external auditory canal appears atresia;

ⅱ degree: the most typical, only vertical helix, sausage-shaped, closed external auditory canal;

ⅲ degree: Only the mass consisting of skin and cartilage is left, and in severe cases, there is no ear.

There are more than ten types of middle ear developmental disorders in patients with congenital microtia, mainly the development of ossicles, tympanic muscles and facial nerves, which are closely related to the severity of external ear malformation. Congenital external ear malformation can be manifested as one of the clinical symptoms of sequence sign when it is serious, such as oto-otovertebral spectrum (OAVS). In addition to microtia, patients with microtia also suffer from hemifacial deformity (hypoplasia of temporal bone, maxilla or mandible), soft tissue deformity (preauricular vegetation or giant mouth), eyelid defect (eyelid defect, eyelid conjunctival epithelial cyst), spinal deformity and congenital kidney and heart defects.