OBJECTIVES: To define the prevalence of definite Ménière's disease (MD) among patients presenting with characteristic symptoms and examine the utility of published diagnostic guidelines. STUDY DESIGN AND SETTING: Retrospective review in an academic referral practice. RESULTS: The prevalence of definite MD in these 295 individuals was 64%. The next-largest group (23%) consisted of patients with only cochlear symptoms. Those initially classified as probable are usually reclassified as definite with extended follow-up. Of those with definite MD, the mean duration of disease at last follow-up was 7.6 years, 56% were female, 19% had bilateral disease, and 34% required surgical management for vertigo. CONCLUSIONS: The 1995 AAO-HNS guidelines are useful for classification of MD according to certainty of diagnosis and severity of disease, though some modifications could be considered. SIGNIFICANCE: Application of consistent diagnostic criteria is essential for epidemiological, genetic, or outcomes studies of Ménière's disease.