Wegener's granulomatosis is an idiopathic vasculitis of small arteries, arterioles, and capillaries primarily affecting the upper aerodigestive tract, lungs, and kidneys. Typical presentation is in middle-aged patients with a slight male predominance. Otologic manifestations are common and range from 19% to 45% of cases (103). They include conductive hearing loss, sensorineural hearing loss, otalgia, otorrhea, and serous otitis media. Neurologic involvement is frequent with nearly half of patients demonstrating either peripheral or central neuropathy (104). Cranial nerve involvement was reported in 6.5% of patients (105). When the ear is affected by the disorder, facial nerve involvement has been estimated at 5% (103,106). Injury to the facial nerve may be from destructive granulomatous lesions involving the skull base, necrotizing vasculitis, or compressive effect due to granuloma in the middle ear (107). Pathologically, the disorder is characterized by noncaseating granulomas with necrotizing vasculitis. Diagnosis can be facilitated using a sensitive serum marker, c-ANCA.
Treatment consists of immunosuppressive therapy, including methotrexate, cyclopho-sphamide, and prednisone; Septra may help keep patients in remission. Immunosuppressive therapy now offers a 70% to 85% remission rate, which is remarkable, considering the invariably fatal outcome within one year without treatment. Several studies have demonstrated almost universal resolution of neuropathy with institution of systemic immunosuppressive therapy (108). In rare cases where facial nerve involvement persists after systemic therapy has been instituted, many have advocated against facial nerve decompression, citing concerns of increased risk of nerve injury due to granulation tissue surrounding the nerve (103,109,110). Dagum, in a literature review, reported six cases of facial paralysis in Wegener's granulomatosis, all of whom had resolution of the paralysis with treatment of the disease (107).
Chapter 8 discusses Wegener' s granulomatosis is further detail.
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