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.