title: Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness.
abstract: Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab.
authors.affiliation: Department of Otorhinolaryngology, School of Medicine, Kyung Hee University, Seoul, Korea.
authors.forename: Sang Hoon
authors.initials: SH
authors.lastname: Kim
authors.affiliation: Department of Otorhinolaryngology, School of Medicine, Kyung Hee University, Seoul, Korea.
authors.forename: A Ra
authors.initials: AR
authors.lastname: Jung
authors.affiliation: Department of Otorhinolaryngology, School of Medicine, Kyung Hee University, Seoul, Korea.
authors.forename: Su Il
authors.initials: SI
authors.lastname: Kim
authors.affiliation: Department of Otorhinolaryngology, School of Medicine, Kyung Hee University, Seoul, Korea.
authors.forename: Seung Geun
authors.initials: SG
authors.lastname: Yeo
keywords: Bilateral hearing loss; Facial paralysis; Granulomatosis with polyangiitisis; Rituximab