Inner ear anomalies and conductive hearing loss in children with Apert syndrome: an overlooked otologic aspect.

摘要: Objective To identify the occurrence of inner ear structural anomalies and conductive hearing loss (CHL) in children with Apert syndrome. Study design Retrospective review. Setting Pediatric tertiary referral center. Patients Twenty pediatric patients syndrome were found; all (38/40 ears) had anomalies. Intervention(s) Computerized tomography head/temporal bone, pure-tone (including air bone conduction) audiometry, tympanometry. Main outcome measure(s) Imaging demonstrating anomalies, including malformations cochlea, dilated vestibule, and/or semicircular canal; audiologic findings air-bone gap(s). Results Hearing was found 90% syndrome, 80% them CHL. Air-bone gaps at frequencies, larger low frequencies. Fifty percent (20/40) ears better than 0 dB level conduction thresholds 250 500 Hz. Normal middle pressure mobility intact eardrum. Inner patients, bilateral involvement. Most frequently observed malformed lateral canal, cochlear dysplasia. Conclusion Children may present significant CHL that cannot be explained by minor pathologies alone. This be, least partially, attributed to anomalies; however, these are usually not recognized patients. Failure close gap after surgical intervention raise suspicion computed tomographic scan temporal can provide definitive proof.