Histiocytosis X: otologic presentations

Histiocytosis X is a relatively rare disease presenting as 3 clinical syndromes: eosinophilic granuloma, Hand-Schuller-Christian disease, and Abt-Letterer-Siwe disease. Although the clinical expressions of the disease differ, the underlying histopathology appears to be the same. Since the disease frequently involves and not infrequently presents in the head and neck region, it is particularly relevant to the otolaryngologist. We have recently treated two young children presenting with bilateral otorrhea. Although histiocytosis X was entertained early in the evaluation of these patients, the diagnosis was difficult and prolonged in both cases. One case ultimately required electron microscopic study to confirm the diagnosis. Important aspects of the diagnosis are reviewed. Treatment of histiocytosis X includes surgery, radiation therapy and chemotherapy. These treatment strategies are discussed, with particular emphasis on the management of the ear involvement.