Histiocytosis X of the Temporal Bone

Abstract

EDITOR'S NOTE: Each of the rare lesions reported in this group of case reports initially presented as a common otologic problem: middle ear effusion. (apparent) Meniere's disease. recurrent acute otitis media. and otitis extema. Today a diagnosis of necrotizing ("malignant") otitis extema would immediately be suspected in an elderly diabetic with granulation tissueon the ear canal floor. However. the other cases would stillfool most of us, at least on the firstor second visit. The solution isnot to order excessive testsand x-ray films on every patient.A 73-year-old woman was admitted to the hospital for evaluation and treatment of paralysis of the left face. She had undergone bilateral stapedectomies several years prior to admission. Three months prior to admission she developed bilateral acute otitis media and was given antibiotic therapy. The otitis media failed to resolve, and bilateral myringotomies with placement of rympanostorny tubes were carried out. There was still no improvement. so the tubes were removed and antibiotics continued. Subsequently, left facial paralysis developed along with mild vertigo and tinnitus.Pertinent physical findings included an amber effusion in the right ear. The myringotomy on the left was still patent and allowed passage of amber fluid. Tuning fork tests were consistent with a bilateral conductive hearing loss. There was complete paralysis of the entire left face.Laboratory studies included a white blood cell count of 1O,7oo/mm", with a shift to the left. Blood glucose was 178 mg/dl. Temporal bone tomograms and computerized tomography of the head revealed opacification of the mastoid air cells without bone destruction. There was mucosal thickening in the left sphenoid sinus. Intravenous cefazolin was begun, and subsequent cultures grew a bacillus species sensitive to clindamycin, which was added to the regimen. Audiologic assessment revealed profound hearing loss bilaterally. HowFrom the Departmentof Otorhinolaryngology and Human Communication, University of Pennsylvania School of Medicine.