Inner ear anomalies in congenital aural atresia

Jeffrey T. Vrabec, Jerry W. Lin

Research output: Contribution to journalArticlepeer-review

28 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1421-1426
Number of pages6
JournalOtology and Neurotology
Issue number9
StatePublished - Dec 2010


  • Anomaly
  • Aural atresia
  • Conductive hearing loss
  • Facial nerve grading system
  • Facial paralysis
  • Inner ear malformation
  • Internal auditory canal
  • Posterior semicircular canal

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Sensory Systems
  • Clinical Neurology


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