Inner ear anomalies in congenital aural atresia.
OBJECTIVE
To define the prevalence of inner ear anomalies in aural atresia patients and to recognize patterns of developmental anomalies in aural atresia patients.


METHODS
Retrospective review.


METHODS
Academic medical center.


METHODS
Physical exam, audiometry, and temporal bone CT in selected patients.


METHODS
Pediatric patients with aural atresia.


METHODS
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.


CONCLUSIONS
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.
Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Texas 77030, USA. jvrabec@bcm.edu
Jeffrey T
JT
Vrabec
Jerry W
JW
Lin
D002648:Child; D003051:Cochlea / Q000002:abnormalities; D004427:Ear Diseases / Q000151:congenital* / Q000473:pathology*; D007758:Ear, Inner / Q000002:abnormalities* / Q000473:pathology*; D005146:Facial Asymmetry / Q000473:pathology; D005155:Facial Nerve Diseases / Q000151:congenital / Q000209:etiology; D007839:Functional Laterality / Q000502:physiology; D034381:Hearing Loss / Q000151:congenital / Q000209:etiology; D006314:Hearing Loss, Conductive / Q000175:diagnosis / Q000209:etiology; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014722:Vestibule, Labyrinth / Q000002:abnormalities