Two cases of unsuspected intralabyrinthine schwannoma are reported and the literature is reviewed. The mean age of the patients was 56 years, with the most common symptoms being hearing loss, dizziness, and tinnitus. All patients demonstrated a profound sensorineural hearing loss with low discrimination scores, and recruitment was elicited in two patients. A radiographic evaluation was negative in all but one case. Five of the six patients had a preoperative diagnosis of Ménière's disease. Transcanal labyrinthectomy was employed to remove tumor in all patients. This review suggests that a patient, presenting with symptoms of atypical Ménière's disease and negative radiographic evaluation, should alert the surgeon to the possibility of an intralabyrinthine schwannoma.