Mastoidite e paralisia facial como manifestações iniciais de Granulomatose de Wegener

Abstract

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. Aim: To describe three confirmed cases of WG with mastoiditis as the first manifestation, progressing to peripheral facial paralysis (PFP). Material and Method: A clinical series study. Patients diagnosed with WG that initially presented with otologic findings are described. Results: 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. Conclusion: 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.

Granulomatosis with Polyangiitis with Bilateral Facial Palsy and Severe Mixed Hearing Loss

“…Morales-Angulo et al reported ENT manifestations in the course of the disease in 88% of patients, and in 28% of cases it was the first symptom of the disease. Several head and neck regions may be affected by the disease, with nose and paranasal sinuses being the most frequently involved sites (60–90% of cases) [13, 14]. The GPA lesions may also be located in the larynx, oral cavity, orbit, and parotid gland [13].…”

“…Destructive granulomatous masses may cause erosion of the ossicles or spread through the mastoid towards the petrous apex [18]. This process may be accompanied by effusion, mastoiditis, and/or facial palsy [14]. Osteomastoiditis accompanied by peripheral facial nerve palsy may occur in 8%–10% of cases [14, 16].…”

“…This process may be accompanied by effusion, mastoiditis, and/or facial palsy [14]. Osteomastoiditis accompanied by peripheral facial nerve palsy may occur in 8%–10% of cases [14, 16]. …”

“…Morales-Angulo et al [12] reported that in 50% of patients who underwent head and neck biopsy of suspicious lesions (mainly nasal) the result confirmed the GPA by showing typical histological criteria. Other authors confirm that often multiple biopsies are required, because the results are inconclusive in over 50% of cases [14]. Bradley [21] suggested that to make a diagnosis of GPA complete histopathological picture is not always essential.…”

“…Many authors recommend conservative treatment, as it usually results in nerve function recovery, whereas surgery may increase the risk of additional nerve injury [14, 19]. However, unusual manifestation at the onset of the disease may delay the correct diagnosis and the suspicion of GPA only appears in the case of failure of conventional therapy or when additional symptoms from other organs occur.…”