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Otol Neurotol. 2010 Dec;31(9):1421-6.

Inner ear anomalies in congenital aural atresia.

Author information

1
Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Texas 77030, USA. jvrabec@bcm.edu

Abstract

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.

PMID:
21113986
[Indexed for MEDLINE]

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