J Audiol Otol.  2016 Apr;20(1):55-58. 10.7874/jao.2016.20.1.55.

Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness

• Kim SH
• Jung AR
• Kim SI
• Yeo SG

• KMID: 2167672
• DOI: http://doi.org/10.7874/jao.2016.20.1.55

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.

Keyword

Granulomatosis with polyangiitisis; Bilateral hearing loss; Facial paralysis; Rituximab

MeSH Terms

Deafness
Facial Paralysis*
Granuloma
Hearing Loss, Bilateral
Hearing Loss, Sensorineural
Hearing Loss, Sudden*
Humans
Respiratory System
Rituximab
Vasculitis
Vertigo