Abstract
Langerhans cell histiocytosis is a group of diseases characterized by an inflammatory neoplasia of myeloid precursor cells driven by mutations in the mitogen-activated protein kinase pathway leading to the proliferation and accumulation of Langerhans cells [1,2]. Clinical manifestations range from isolated disease to multifocal lesions and systemic involvement with a worse prognosis [2,3]. The otologic features are rare and may include otorrhea, otalgia, subjective hearing loss and vertigo misleading the diagnosis to an inflammatory process [3]. The CT scan shows destruction of the skull with characteristic “punched out” lytic lesions that can be multiple and multifocal. Tissue biopsy confirms the diagnosis [3].
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