OBJECTIVE To explore the clinical and audiological characteristics of children with congenital malformations of the external ear. METHODS One hundred and ten patients with congenital malformations of the external ear ranged from 2002 to 2006 were involved in this study. The mean age was one year and four months. The children were divided into three groups according to the appearance of the external ear. The first group consisted of 94 patients with auricle malformation and atresia of the external auditory meatus. The second group consisted of 8 patients with abnormal external auditory meatus (no atresia) and auricle malformation. The other 8 patients only with auricle malformation were included in the third group. All children underwent ABR tests, while some of these children accepted DPOAE and Acoustic-immittance measurements. RESULTS (1) Severe abnormal results of ABR were observed in 79 abnormal ears (78.22%) in children of first group, while moderate or severe abnormal results of ABR were observed only in 8 opposite side ears (normal ears). (2) Sixty-two and a half percent (5 ears) of ears of the second group (8 ears) had severely abnormal ABR results. (3) Severe abnormal ABR results occurred in 44.44 percent (4 ears) of ears of the third group (9 ears). Moderate or severe abnormal results of ABR were not observed in the opposite side ears (normal ears) in second and third groups. CONCLUSIONS Congenital malformations of the external ear is an important factor affecting children's hearing. Auditory nerve impairment can be observed in children with congenital malformations of the external ear.