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  • Title: [Isolated unilateral otitis with facial nerve paralysis as initial symptom of Wegener granulomatosis. An unusual clinical course].
    Author: Hofmann T, Kainz J, Koc C, Smolle KH, Brunner G.
    Journal: Laryngorhinootologie; 1998 Jun; 77(6):352-4. PubMed ID: 9701761.
    Abstract:
    BACKGROUND: Wegener's granulomatosis is an immunepathogenic disease of unknown origin. The histopathological picture shows granulomatous inflammation with epitheloid cells, granulomas, and general vasculitis. The diagnosis of Wegener's granulomatosis is made on the basis of the clinical picture, serum cANCA, and histologic examination of biopsies. PATIENT: We present the case of a 57-year-old white male patient who was admitted to our ENT Hospital with a six weeks' history of otalgia and incomplete ipsilateral facial palsy since the day before admission. The patient had clinical features of acute otitis media without signs of mastoiditis. Despite a ten days' course of intravenous antibiotic treatment, the intensity of facial palsy progressed and the general condition of the patient worsened. A mastoidectomy and decompression of the facial nerve were performed, demonstrating sclerosis of the mastoid cells. Three weeks after release from the hospital, the patient was admitted again with recurrent fever, cephalea, loss of weight, and arthritic pain. There were no signs of recurrent otitis media or mastoiditis, and sigmoid sinus thrombosis was ruled out. Even under aggressive, intravenous antibiotic treatment the general physical condition continued to worsen; septic temperatures and signs of beginning renal failure occurred. The patient was transferred to the ICU with the diagnosis of sepsis of unknown origin. There bloodtests were positive for cANCA, which is highly specific for Wegener's granulomatosis. Under therapy with cyclophosphamide and i.v. corticosteroid, the patient recovered with 14 days. CONCLUSION: The lack of symptoms in the upper respiratory tract in our patient was unusual, indicating that in patients with recurrent otitis media, facial palsy, mastoiditis, or external otitis Wegener's granulomatosis should be ruled out as differential diagnosis.
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