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Granulomatosis With Polyangiitis Presenting as Acute Bilateral Facial Nerve Paralysis
Nicholas Toomey1, Aaron J Done1, Doron Sagiv1
1Department of Otolaryngology-Head and Neck Surgery, University of California, Davis, USA.
The Annals of Otology, Rhinology, and Laryngology
|September 27, 2025
Summary
Bilateral facial palsy is a rare symptom of Granulomatosis with polyangiitis (GPA). This case highlights GPA diagnosis even without systemic involvement, emphasizing middle ear biopsies for accurate diagnosis.
Area of Science:
- Otorhinolaryngology
- Rheumatology
- Neurology
Background:
- Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis affecting small vessels.
- Facial palsy is an uncommon head and neck manifestation of GPA.
- Bilateral facial palsy is an exceedingly rare presentation of GPA.
Purpose of the Study:
- To report a rare case of bilateral facial paralysis as the initial presentation of GPA.
- To increase awareness of this unusual manifestation of GPA.
Main Methods:
- Retrospective chart review of a patient with bilateral facial paralysis.
- Diagnostic workup included imaging (CT, MRI), laboratory tests (PR3-ANCA), and middle ear biopsy.
- Surgical intervention involved mastoidectomy with facial nerve decompression.
Main Results:
- A 52-year-old female presented with sequential bilateral facial paralysis, hearing loss, and otorrhea.
- Imaging revealed opacified mastoids without cholesteatoma.
- Diagnosis of GPA was confirmed by positive PR3-ANCA and middle ear biopsy showing vasculitis.
- Patient showed significant improvement in facial nerve function after treatment with rituximab and prednisone.
Conclusions:
- This case represents the first reported instance of bilateral facial palsy due to GPA without other systemic involvement.
- The absence of systemic signs and a negative initial autoimmune workup should not rule out GPA.
- Re-evaluation for systemic involvement and biopsies of affected tissues are critical for diagnosing GPA in such cases.
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