[Histiocytosis X of the temporal bone]

Histiocytosis X (HX) is a rare disease. There are many questions and debates in its etiology, pathology, clinical type and therapy. To enhance the recognition of otolaryngologists in HX of the ear and temporal bone and the level of diagnosis and therapy, 30 years' results of 21 patients with HX of the ear and temporal bone proved by histiopathology were reviewed, 18 patients were cured (85%), 6 cases were residual of diabete insipidus and dwarfism (28%), 3 cases died (14%). The clinical classification system should be based on the extention of diseases. Surgical curettage, radiation therapy are mainstays of treatment; chemotherapy should be used in patients with aggressive and disseminate diseases and etiology of histiocytosis should be studied on immune system that potentially aid in therapy.