Objective: Accessory auricular anomaly is a small elevation of skin containing a bar of elastic cartilage localized most commonly just anterior to the tragus or ascending crus of the helix. The anomaly may exist isolated or may be associated with other congenital anomalies of the first arch. The purpose in this study is to detect prevalence of accessory auricle in Turkey and find out whether it is associated with other craniofacial anomalies or hearing loss. Methods: The study was performed on 850 children from the age of 7 to 9 during a screening program in primary schools. Complete otolaryngologic examination and acoustic reflectometry measurements were performed on all the children. Full physical examination, tympanometric and audiometric evaluation and EEG measurements were added to the cases with accessory auricle anomaly. Results: Among 850 children examined, four had accessory auricle anomaly and prevalence of the anomaly was calculated as 0.47% (95% confidence interval (CI), 0.13-1.20%). Children were developmentally normal, and no other congenital craniofacial or systemic anomaly was detected in any of the cases. Further, tympanometric, audiometric evaluations and EEG tests were in normal limits. Conclusions: In this study, the prevalence rate of the condition was calculated as 0.47%. Although one study from China reported this prevalence as 0.22%, the difference between the reported prevalances was not statistically significant. Further, although external ear anomalies may present together with cranifacial anomalies and neurologic disorders like epilepsy, neuromotor retardation and EEG disorders, in our cases, mental and motor development was normal and epilepsy history or abnormal EEG patterns do not exist. On the other hand, no hearing loss was found to be associated with accessory auricles. (C) 2002 Elsevier Science Ireland Ltd. All rights reserved.