Clinical course of pediatric congenital inner ear malformations

Objective: To determine any factors that could improve the early detection and management of congenital inner ear malformations, Study Design: A retro spective review was performed of all patients with a diagnosis of inner ear malformation at Loyola University Medical Center (LUMC) and the Hospital f or Sick Children (HSC) between 1987 and 1995, Clinical records and audiomet ric data were accumulated. One neuroradiologist reviewed every temporal bon e computed tomography (CT) scan. Methods: Forty-six pediatric patients with congenital inner ear anomalies evaluated at two tertiary care hospitals. R esults: The average patient age at initial assessment was 25.7 months. A fa mily history of hearing loss was noted in only dye patients (12.8%). A majo r nonotological deformity was seen in 41% of patients. The average hearing threshold was 88 dB, All three patients with sudden hearing loss had vestib ular aqueduct enlargement, Two of the three patients with common cavity ano malies had a history of recurrent meningitis. Twenty-seven patients had a v estibular aqueduct deformity, the most frequent radiographic abnormality in the series. Conclusions: Because inner ear malformation was diagnosed afte r 24 months of age in a significant percentage of patients, we recommend in creased parental education and vigilance by primary care practitioners. Uni versal newborn screening may be the key to earlier detection of these infan ts. For children with idiopathic sensorineural hearing loss, we recommend a temporal bone CT scan. Patients with vestibular aqueduct enlargement must be counseled about the risk of progressive sensorineural hearing loss, meni ngitis, and the need to avoid contact sports. Patients with common cavity a bnormalities should be considered for exploratory tympanotomy and also educ ated about the risk for meningitis.