BACKGROUND: Langerhans' cell histiocytosis (LCH) is a rare disease with variable clinical appearance. The etiology of LCH remains unclear to date. It is currently believed that clonal accumulation and proliferation of CD1a-positive Langerhans' cells are causative. METHODS: A 2-year-old boy presented with hypacusis and disturbance of balance. Auditory brainstem-evoked responses (ABRs) revealed severe bilateral labyrinthine hearing loss. Magnetic resonance imaging (MRI) showed inflammatory changes with bone erosion in both temporal bones including labyrinthine systems and mastoid processes. RESULTS: During bilateral surgical exploration, fragile slightly yellowish tissue with necrotic areas was found that turned out to be LCH on histology. Chemotherapy with vinblastine and prednisone was subsequently initiated, leading to continuing complete remission. CONCLUSIONS: The initial presentation of LCH with bilateral temporal bone involvement is a very rare condition. The signs and symptoms of otologic histiocytosis can mimic those of acute and chronic infectious ear disease. Only a surgically obtained biopsy leads to definitive diagnosis and appropriate therapy.
BACKGROUND: Langerhans' cell histiocytosis (LCH) is a rare disease with variable clinical appearance. The etiology of LCH remains unclear to date. It is currently believed that clonal accumulation and proliferation of CD1a-positive Langerhans' cells are causative. METHODS: A 2-year-old boy presented with hypacusis and disturbance of balance. Auditory brainstem-evoked responses (ABRs) revealed severe bilateral labyrinthine hearing loss. Magnetic resonance imaging (MRI) showed inflammatory changes with bone erosion in both temporal bones including labyrinthine systems and mastoid processes. RESULTS: During bilateral surgical exploration, fragile slightly yellowish tissue with necrotic areas was found that turned out to be LCH on histology. Chemotherapy with vinblastine and prednisone was subsequently initiated, leading to continuing complete remission. CONCLUSIONS: The initial presentation of LCH with bilateral temporal bone involvement is a very rare condition. The signs and symptoms of otologic histiocytosis can mimic those of acute and chronic infectious ear disease. Only a surgically obtained biopsy leads to definitive diagnosis and appropriate therapy.