Systemic Langerhans Cell Histiocytosis with Bilateral Temporal Bone Involvement in an Adult

Langerhance Cell Histiocytosis is a granulomatous disorder, with a variable clinical expression, usually diagnosed in children. Its systemic form carries a poor prognosis. We present a 45 years old male with vertigo and otorrhea lasting three months, with history revealing former resection of eosinophilic granuloma from the right tibia and diabetes insipidus. The patient underwent biopsy from the left mastoid and the histopathological examination was consistent with Langerhance Cell Histiocytosis. Due to evidence of lung involvement the patient was treated with several chemotherapy and steroids. There were two incidences of recurrence; both treated with radiation and additional ARA C protocols. Since then all his vestibular and neuropathic symptoms resolved and his lung lesions subsided. Conclusion: Langerhance Cell Histiocytosis is a rare cause of temporal bone disease in an adult especially in the systemic form. Suspicion is warranted in cases of unresponsive ear disease. Histopathological diagnosis is essential to ensure prompt local treatment and for early recognition of potential multifocal and systemic involvement.