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Abstract: SA-PO046

Otological Manifestations in ANCA-Negative Pauci-Immune Vasculitis: A Compelling Clue When Serologies Are Negative

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical‚ Outcomes‚ and Trials


  • Baker, Megan Leila, Yale School of Medicine, New Haven, Connecticut, United States
  • Turner, Jeffrey M., Yale School of Medicine, New Haven, Connecticut, United States

Otological manifestations are often observed in ANCA-associated pauci-immune vasculitis(AAV), and include otalgia, hearing loss, vertigo, and tinnitus. The diagnosis of AAV in a patient presenting with otological manifestations can be challenging when otological symptoms are the predominant presenting feature or when ANCA testing results negative. A delayed diagnosis in these settings may result in more advanced hearing loss or recurrent AAV flares.

Case Description

A 66-year-old woman with CKDIIIa and hypertension presented with polyarthralgias, AKI, severe bilateral sensorineural hearing loss, vertigo, oral mucositis, scleritis, and hand joint pain with rash. Laboratory testing revealed leukocytosis, anemia, thrombocytopenia, negative anti-MPO and anti-PR3 titers, positive ANA(1:320), normal complement levels, and otherwise negative serologic workup. MRI brain showed left middle ear cavity scarring. She was given steroids for diagnosis of vestibular neuritis, labyrinthitis, and reactive arthritis with persistent hearing loss but recovery of AKI and other symptoms. Several months later she developed altered mental status, fevers, and AKI, and was found to have a UTI. After initial improvement with antibiotics, steroids, and IV fluids, she developed recurrent polyarthralgias and AKI. Kidney biopsy demonstrated pauci-immune necrotizing glomerulonephritis. Pulse dose steroids and rituxumab for ANCA-negative pauci-immune glomerulonephritis resulted in improvement in Cr. With non-recovery of her hearing loss, she ultimately underwent cochlear implant surgery.


Otological symptoms such as sensorineural hearing loss incurred in this case along with AKI should prompt an evaluation for AAV, as otitis media is a relatively common manifestation of disease. This case demonstrates the specific diagnostic challenge presented by an ANCA-negative pauci-immune vasculitis with otological involvement. In comparison to cases with positive ANCA serologies, cases of MPO- and PR3-negative pauci-immune vasculitis present a significant diagnostic challenge, being less common and less readily recognized. The co-occurrence of otological symptoms and AKI should prompt a higher clinical suspicion for AAV even in the absence of positive ANCA serologies, as these cases are estimated to represent a small fraction(~13%) of cases of otitis media associated with AAV.