Content area
Full Text
Wegener's Granulomatosis (WG) is an autoimmune disease. The lungs, kidneys and upper respiratory organs are major sites of organ involvement. Although nose, ear and throat involvement can be observed in most of cases during the natural course, their individual involvement is quite rare. A case with bilateral refractory chronic otitis media and spontaneous CSF fistula that was diagnosed as WG is presented.
Submitted : 16 May 2011 Accepted : 15 July 2011
Introduction
Wegener's Granulomatosis (WG) is an immune mediated disease which is characterized by necrotizing vasculitis of small arteries and veins. The specific organization of necrotized vessels ultimately results with necrotizing granuloma formation in the affected organs [1]. The upper respiratory tract involvement is usually presented by nasal congestion, epistaxis, discharge, sinusitis, cough, stridor, otitis media, hearing deficit and oral ulceration[2]. The other organs which are commonly involved are lungs (45%-70% at presentation and 85% throughout the disease course), kidneys (77% of patients), heart, nervous system and muscles and joints[3].
The nose and paranasal sinuses are mainly affected organs concerning the upper respiratory tractus. They are involved in %85 of cases during disease progress and the ear involvement is seen approximately in 35% of all cases with WG4,[5]. The patients are commonly presented with nonspecific symptoms including upper respiratory tract infections, sinusitis, fever, fatique, myalgias in its early stages. These nonspecific findings may delay the early diagnosis of the disease so that the profound organ involvements are seen later in disease progress. The otologic manifestations occasionally may be the only presenting feature in WG. There are only a few cases reported; chronic otitis media, mastoiditis, facial nerve paralysis, sensorineural hearing loss were the only presenting symptoms[6-9].
The certain diagnosis of WG can be made with the presence of circulating pattern antineutrophil cytoplasmic antibodies (C-ANCA) and anti-PR3 antibodies which are specific to WG in the active phase. Also histological examination of biopsy specimens of specific organs involved may be helpful in certain diagnosis making[10].
This case report presents a patient with WG whose initial only presenting feature was bilateral otitis media with effusion which rapidly progressed to refractory otitis media, sensorineural hearing loss and spontaneous CSF fistula.
Case report
A 50-year-old woman was admitted at the Department of Otolaryngology of Uludag University with...