Mastoiditis and facial paralysis as initial manifestations of Wegener's Granulomatosis

Wegener's Granulomatosis (WG) is characterized by necrotizing granulomas and vasculitis. If left untreated, the prognosis is poor – a 90% mortality rate within 2 years. Several authors have described the otologic manifestations of WG; these authors, however, have not mentioned the stage of the...

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Bibliographic Details
Published in:Brazilian journal of otorhinolaryngology 2012-04, Vol.78 (2), p.80-86
Main Authors: de Albuquerque Maranhão, André Souza, Chen, Vitor Guo, Rossini, Bruno Almeida Antunes, Testa, José Ricardo Gurgel, de Oliveira Penido, Norma
Format: Article
Language:English
Subjects:
Adult
Audiometry
facial paralysis
Facial Paralysis - etiology
Female
Granulomatosis with Polyangiitis - complications
Hearing Loss, Sensorineural - etiology
Humans
Male
mastoiditis
Mastoiditis - etiology
Middle Aged
Original
Otitis Media with Effusion - etiology
wegener granulomatosis
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Summary:Wegener's Granulomatosis (WG) is characterized by necrotizing granulomas and vasculitis. If left untreated, the prognosis is poor – a 90% mortality rate within 2 years. Several authors have described the otologic manifestations of WG; these authors, however, have not mentioned the stage of the disease in which these findings present – whether as initial manifestations or subsequent to other findings. To describe three confirmed cases of WG with mastoiditis as the first manifestation, progressing to peripheral facial paralysis (PFP). A clinical series study. Patients diagnosed with WG that initially presented with otologic findings are described. The three cases presented with unilateral otalgia, otorrhea, and hearing loss associated with ipsilateral PFP. None recovered in spite of the treatment; an investigation of associated diseases was therefore undertaken. Positive ANCA-C titers where detected in all patients, confirming the diagnosis of WG. Clinical improvement was seen after treatment of WG; the PFP regressed and hearing thresholds improved partially. Complications of otitis media (mastoiditis and PFP) that do not respond to the usual treatment require an investigation of associated diseases; WG should be included for an early diagnosis to change the prognosis in these patients.
ISSN:1808-8694
1808-8686
DOI:10.1590/S1808-86942012000200013