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.

Keywords: Bilateral hearing loss; Facial paralysis; Granulomatosis with polyangiitisis; Rituximab.

Fig. 1. Right (A) and left (B) tympanic membrane showing bulging.

Fig. 2. Pure tone audiometry of the patient at first out patient department visit (A) and after steroid tapering therapy and intratympanic steroid injection (B).

Fig. 3. Axial (A) and coronal (B) views showing fluid density in both middle ear cavities and external auditory canals, with mastoid air cells (black arrows). No abnormality was observed in the facial nerve canal or ossicular chain. Both maxillary and ethmoid sphenoid sinusitis are present (white arrows).

Fig. 4. A, B: Axial views of the patient at admission, showing multiple variable sized cavitary and noncavitary nodules and bronchial wall thickening in both lungs. C, D: Axial views at follow up showing increased size and extent of multiple variable sized cavitary and non-cavitary nodules and bronchial wall thickening with newly developed centrilobular nodules in both lungs.

Fig. 5. Lung biopsy showing characteristics of Wegener's granulomatosis. Hematoxylin and eosin staining at (A) 20× and (B) 40× original magnification. White arrows indicate a necrotizing granulomatous inflammation with giant cells, palisading histiocytes and nuclear debris. The black arrow indicates hemorrhagic fibrinous exudates fillingthe bronchoalveolar spaces.