The large vestibular aqueduct syndrome and sensorineural hearing loss in the pediatric population.

Sensorineural hearing loss, associated with a dilated vestibular aqueduct, is often described as progressive. Since 1982, computed tomography of the mastoids has become part of the routine investigation of deaf children at Sainte-Justine Hospital. Using clinical, audiometric and radiological material from 130 patients with sensorineural hearing loss, we identified 18 patients with enlarged vestibular aqueducts. The large vestibular aqueduct population was then compared to the normal vestibular aqueduct patients in regard to the degree and evolution of the sensorineural hearing loss. Progression of hearing loss was noted in 46% of patients in the presence of large vestibular aqueducts as compared to 35% in the absence of this anomaly. Normal radiographic findings did not exclude the possibility of further hearing loss.