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Otology/Neurotology

(219) Granulomatosis with Polyangiitis Presenting as Acute Bilateral Facial Nerve Paralysis

Monday, October 2, 2023
2:45 PM – 3:45 PM East Coast USA Time
Has Audio
Disclosure(s):
Aaron John Done, MD: No relevant relationships to disclose.
Marjan Alidoost, MD: No relevant relationships to disclose.


Introduction: Granulomatosis with polyangiitis (GPA) is an auto-immune disease characterized by granuloma formation with necrotizing vasculitis that typically presents with respiratory or renal involvement. Rarely it may present with facial nerve (FN) involvement.

Methods: We present a case of a 52-year-old female who presented initially with right-sided FN palsy at another hospital that was treated as Bell’s palsy. Two months later, the patient presented in our medical center with bilateral otalgia, hearing loss, and a new onset left sided FN paralysis. Physical exam confirmed bilateral House-Brackman 6. Binocular otomicroscopy demonstrated granulation tissue filling the entirety of bilateral external auditory canals with purulent drainage. Nasal endoscopy revealed normal mucosa. MRI temporal bone demonstrated extensive soft tissue involvement extending from external auditory canal into inner ear structures, and fallopian canal, with no restriction on DWI sequence. Serology panel for autoimmune diseases revealed positive serine proteinase 3 but negative cytoplasmic antineutrophil autoantibody (cANCA), with no granulomatosis within a biopsy taken from the left ear canal polyp. Patient ultimately underwent left mastoidectomy with facial nerve decompression and was diagnosed with GPA based on soft tissue biopsies from mastoid cavity and the serology. She was started on rituximab and prolonged treatment of steroids.

Results: Two-months after the surgery and starting rituximab and steroids patient progressed to House-Brackman 3 on left, with slight movement on the right. Patient is scheduled to undergo right facial nerve decompression.

Conclusions: To the best of our knowledge, we are the first to report GPA presenting as bilateral acute FN paralysis. Isolated bilateral facial paralysis is a rare initial presentation of GPA. Our patient demonstrated significant improvement in facial nerve function after surgical decompression and initiation of rituximab and high dose steroid therapy.