ENLARGED VESTIBULAR AQUEDUCT AND SENSORINEURAL HEARING-LOSS IN CHILDHOOD

Objective: To determine if all children with enlarged vestibular aqued ucts (EVAs) have development of uniform progressive sensorineural hear ing loss (SNHL). To determine whether the size of the EVA correlates w ith severity, frequencies involved, and stability of SNHL. To determin e if the audiologic pattern of SNHL correlates with likelihood of prog ression of SNHL. Design: Retrospective study. Setting: Children's Nati onal Medical Center, Washington, DC, a tertiary care center with a lar ge otologic practice. Patients: Fifteen children (26 ears) with EVA on computed tomographic scan. Methods: History, physical examination, co mputed tomographic scans, and serial audiograms were reviewed. Factors analyzed included age at diagnosis, audiometric configuration (high t one, midtone, low tone, flat), degree of hearing loss at presentation, length of follow-up, and presence of associated inner ear anomalies. Results: Nine ears had progressive SNHL, 16 ears had stable SNHL, and 1 ear had profound SNHL. The predominant audiologic configuration was flat. The audiogram configuration does not correlate with progression of SNHL. The size of the vestibular aqueduct does not correlate with t he level, type, or progression of SNHL. Conclusion: Our study failed t o uncover factors that might be predictive of progression of hearing l oss. We conclude that until a better understanding of the natural hist ory and pathophysiologic condition of EVAs is achieved, there is no su rgical or other intervention that can be demonstrated as being efficac ious.