Four patients (7 ears) with a large vestibular aqueduct (VA) were examined for history of vertigo and vestibular function. Vertigo was observed in all the 4 patients. The caloric responses were significantly poorer in ears with a large VA than in the controls. On a patient with a large VA who had several attacks of sudden hearing loss and vertigo following minor head trauma, long-time exposure to sunshine, common cold, and exercise, vestibular function tests were performed several times; the caloric responses were found to fluctuate and direction changing apogeotropic positional nystagmus was observed. These findings suggest that in patients with a large VA, not only hearing but also the vestibular function are generally impaired. We believe than direct transmission of intracranial pressure changes to the inner ear or subsequent inner ear fluid movement through the large endolymphatic sac and duct have an influence on the cochlea and vestibule.