Otosclerosis occurring with other pathologies has received little attention in the literature although these concomitant occurrences can be clinically relevant. We studied the clinical and histopathological characteristics of 182 cases of otosclerosis from our human temporal bone collection, and found 81 (44%) to have associated pathologies. Clinical pathological findings included vestibular symptoms and findings (e.g. Ménière's syndrome), otitis media in various forms, and to a lesser extent labyrinthine anomalies, tumors and other associated pathologies. Whether these coexisting pathologies are coincidental (usually) or causative as in the case of Ménière's syndrome with extensive otosclerosis, appropriate diagnosis and treatment of the patient with otosclerosis requires recognition of these potential clinical pathological relationships.