Otologic Manifestations and Progression in Patients with Wegener's granulomatosis: A Survey in 55 Patients.

Abstract

Granulomatosis with polyangiitis (GPA; also known as Wegener's granulomatosis) is a primary systemic vasculitis involving the ear, nose and throat system (ENT) and lower respiratory tract. Because of the lack of knowledge regarding the clinical findings of GPA due to the limited number of studies, the current study was designed to investigate the prevalence and nature of the otology manifestations in the disease course. In the current prospective study, patients with a diagnosis of GPA from 2012-2016 were included. A definitive diagnosis was made based on the history, physical examination (otomicroscopy, Rinne and Weber test), audiometry, tympanometry, cytoplasmic and perinuclear anti-neutrophil cytoplasmic antibody (C-ANCA and P-ANCA) investigations, and pathologic studies. Twenty-seven male and 28 female patients aged 41.6±15.3 years were enrolled. Ear involvement was found in 20 patients (36.3%), and the most prevalent symptom was loss of hearing followed by otalgia and tinnitus. Tinnitus improved in none of the patients. The most prevalent sign was otitis serous followed by mastoiditis and external otitis. The most important audiometry finding was sensorineural hearing loss. Pathological studies using pulmonary samples were more useful for diagnosis. Precise clinical examination is crucial for the early diagnosis of GPA. Otological manifestations are common, especially loss of hearing and otitis serous, and can be the first sign of this disease. Early diagnosis can lead to better treatment of Wegener's granulomatosis.