Atypical presentation of granulomatosis with polyangiitis: a case report and review of the literature

Background: Granulomatosis with polyangiitis (GPA) is a systemic autoimmune disease characterized by necrotizing granulomatous vasculitis of the small- and medium-sized vessels. Classical GPA affects the upper and lower airways and kidneys. It commonly affects the nose and paranasal sinuses, middle and inner ear, and subglottic region of the larynx. Therefore, otolaryngological involvement is common in GPA and can sometimes be the initial presentation. In rare cases, otologic signs and symptoms can be the initial manifestations of this disease, including recurrent otitis media, otitis media with effusion, and sensorineural hearing loss. Case Description: In the present study, we describe an atypical case of GPA of a 22-year-old female. The patient presented with severe and complicated otitis media with hearing loss and polyneuropathy (facial nerve paralysis and trigeminal nerve impairment), in addition to nasopharyngeal and parotid infiltration, in the absence of other otolaryngologic and systemic manifestations of GPA. Conclusions: The diagnosis of GPA can be challenging due to its wide range of clinical manifestations. Otologic signs and symptoms are rare in this disease and can sometimes be the initial manifestations. Early diagnosis and treatment are important to prevent serious and permanent complications of the disease. Otolaryngologists should have high index of suspicion to systemic diseases such as GPA.