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Cases with facial nerve palsy and masked mastoiditis were thoroughly analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The mean age of patients was 37\u00a0years. All manifested unilateral facial nerve palsy and hearing loss, while only 2 reported aural complaints suggesting inflammatory cause of the disease. All cases were qualified for surgical intervention. Intraoperative findings were similar: granulation tissue in tympanic cavity and\/or pneumatic spaces of the mastoid process. Only 50% of histopathological results suggested vasculitis. In all cases, elevated levels of antineutrophil cytoplasmic antibodies (ANCA) against peroxidase 3 (PR3-ANCA) were determined. Two patients presented rapid progression of the disease and died within 1\u00a0week and 2\u00a0months, respectively. Four other patients manifested gradual improvement of hearing and facial nerve function after treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>GPA should be included into differential diagnosis in all cases of persistent facial nerve palsy especially when otological symptoms coexist. Even localized GPA could be very aggressive, revelating generalized form of the disease. Rapid systemic treatment of GPA can protect hearing and facial nerve from permanent severe dysfunction. The ACR\/EULAR 2017 provisional classification criteria for GPA seem to be valuable tool in diagnosing ENT patients with localized otological form of the disease.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s00405-021-07166-w","type":"journal-article","created":{"date-parts":[[2021,11,19]],"date-time":"2021-11-19T10:02:40Z","timestamp":1637316160000},"page":"4271-4278","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Facial nerve paresis in the course of masked mastoiditis as a revelator of GPA"],"prefix":"10.1007","volume":"279","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9946-2851","authenticated-orcid":false,"given":"Joanna","family":"Marsza\u0142","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7862-1411","authenticated-orcid":false,"given":"Anna","family":"Bartochowska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7349-1996","authenticated-orcid":false,"given":"Randy","family":"Yu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0006-6352","authenticated-orcid":false,"given":"Ma\u0142gorzata","family":"Wierzbicka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,11,19]]},"reference":[{"issue":"1","key":"7166_CR1","doi-asserted-by":"publisher","first-page":"345","DOI":"10.1007\/s00405-012-2110-8","volume":"270","author":"M Martinez Del Pero","year":"2013","unstructured":"Martinez Del Pero M, Rasmussen N, Chaudhry A, Jani P, Jayne D (2013) Structured clinical assessment of the ear, nose and throat in patients with granulomatosis with polyangiitis (Wegener\u2019s). 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