Otologic Manifestations of Wegener's Granulomatosis

Abstract

In localized cases, biopsy specimens are often small, and it is frequently difficult to make a histologic diagnosis. The prognosis for hearing was poor when appropriate treatment was not given in the early stages of the disease. Therefore, WG should be included in the differential diagnosis in cases of atypical inflammatory states of the ear. Early diagnosis and appropriate treatment are important to prevent irreversible changes in the middle ear and inner ear.

Wegener granulomatosis in parotid fistula and bilateral nasal mass

“…Therefore, in such cases where histopathology is reported as atypical inflammatory disease, WG should remain a differential diagnosis. [13] As suggested in Fentonand O'Sullivan [14] and Moussa's and Abou-Elhmd [15] studies, since it is common in the head and neck region, a biopsy should be made from paranasal sinus or nasal mucosa. We also obtained biopsy samples from nasal mucosa in our case, revealing vasculitis and granulomatous inflammation.…”

Otologic Wegener's Granulomatosis

“…1,3 The ear is involved in 19%---61% of all cases but is unusual to be the first and only sign of disease in WG. 1,3 Facial nerve palsy has been reported during the course of the disease but is extremely rare as a presenting feature. 3 Pulmonary involvement is frequent, including pleural effusion.…”

“…4 In localized cases, it is frequently difficult to make a definite histologic diagnosis. 1,3,5 ANCA are detectable in nearly 100% of patients with active generalized disease WG, but only 60% in localized form. High-sensitive PR3-ANCA ELISA yields a sensitivity of 96% and a specificity of 98.5%.…”

“…1,2 Upper airway tract involvement is frequent over the course of the disease. 1,3 Cytoplasmatic pattern antineutrophil cytoplasmic antibodies (c-ANCA) are highly specific for WG in the active phase.…”

Bilateral cochlear ossification in a patient with Takayasu arteritis

“…TAK predominantly affects the aorta and/or its branches, and most of the major manifestations of TAK come from limb or organ ischemia resulting from the stenosis of the involved arteries [2]. Sensorineural hearing loss (SNHL) is a well-recognized manifestation of various systemic vasculitides, such as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides [3,4] and Cogan's syndrome [2]; however, in patients with TAK, only a few cases of SNHL have been reported in the English literature. Accordingly, the pathogenesis remains poorly understood, and there is no established treatment strategy for SNHL [5][6][7][8][9][10][11].…”