A GRAVER1, S KUO1, S YEW1, G KIRKLAND1
1Royal Hobart Hospital, Tasmania
Background: Otitis media (OM) with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) is an increasingly recognised entity in Japan. Typical clinical features include prominent otological symptoms, ANCA-positivity with predominantly myeloperoxidase (MPO)-ANCA, peripheral facial nerve palsy, hypertrophic pachymeningitis and systemic involvement with lung and kidney lesions. Delayed diagnosis and treatment can lead to irreversible sensorineural hearing loss.
Case Report: A 49-year-old male presented to his general practitioner unwell with sequential, bilateral otitis media (OM), manifesting symptomatically with discomfort, hyper-acuity and hearing impairment. Treatment involved local application of auricular drops, with near-resolution of symptoms; right-sided hearing impairment persisted. There was no history of ocular or sinus disease.
The patient remained non-specifically unwell. Routine pathology testing identified significant renal impairment (creatinine 293 µmol/L), elevated inflammatory markers (erythrocyte sedimentation rate 34 mm/hr, C-reactive protein 24 mg/L), hypoalbuminaemia (30 g/L) and nephrotic range proteinuria (3.04 g/day).
A renal biopsy was performed, and demonstrated features of pauci-immune crescentic necrotising glomerulonephritis. Subsequent testing identified positive antinuclear antibody (titre 1280, centromere pattern) and positive MPO-ANCA, at 29.1 U/mL.
Treatment for ANCA-associated vasculitis (AAV) was initiated, with slow improvement in renal function. Radiologically, there was no vasculitis involvement of the lower respiratory tract or sinuses.
The clinical features of this case are consistent with OMAAV. Investigation for hypertrophic pachymeningitis was not undertaken with gadolinium-enhanced magnetic resonance imaging, due to the degree of renal impairment. There was no clinical evidence of facial palsy.
Conclusions: This case demonstrates AAV presenting with renal impairment, preceded by OM. OMAAV should be considered in patients presenting with intractable OM with hearing impairment, as delay in treatment can lead to irreversible hearing loss and progressive systemic disease.