Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology · 2010
Inner ear anomalies in congenital aural atresia.
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Vrabec Jeffrey T, Lin Jerry W
Аннотация
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.
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.