Inner ear anomalies in congenital aural atresia

OBJECTIVES: To define the prevalence of inner ear anomalies in aural atresia patients and to recognize patterns of developmental anomalies in aural atresia patients. STUDY DESIGN: Retrospective review. SETTING: Academic medical center. INTERVENTION: Physical exam, audiometry, and temporal bone CT in selected patients. PATIENTS: Pediatric patients with aural atresia. MAIN OUTCOME MEASURE: Prevalence of inner ear anomalies and coexisting facial paralysis or sensorineural hearing loss. RESULTS: In this series of 118 patients with aural atresia, associated facial palsy was seen in 13%, whereas inner ear anomalies were present in 22%, including all patients with facial palsy. Interestingly, the inner ear anomalies often did not display a significant sensorineural hearing loss. Bilateral inner ear anomalies were frequently encountered despite unilateral atresia. Most anomalies involved the semicircular canals including several uncommon variants of posterior semicircular canal anatomy. CONCLUSION: Inner ear anomalies are common in the presence of aural atresia, especially when there is concurrent congenital facial palsy. The presence of inner ear anomalies should be recognized as a common feature of craniofacial microsomia.