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Open Access Publications from the University of California

A Case Series of Granulomatosis With Polyangiitis Primarily Diagnosed by Otological Manifestations.

  • Author(s): Sahyouni, Ronald
  • Moshtaghi, Omid
  • Abouzari, Mehdi
  • Le, Phuonganh
  • Birkenbeuel, Jack
  • Cheung, Dillon
  • Lin, Harrison W
  • Djalilian, Hamid R
  • et al.

OBJECTIVE:To describe a case series of previously undiagnosed granulomatosis with polyangiitis (GPA) patients who presented primarily with otological manifestations. METHOD:We report a series of patients visited at a neurotology clinic who were eventually diagnosed with GPA based on their otologic complaints and had no prior knowledge of having this condition. RESULTS:In this series, 10 (91%) patients presented with hearing loss (HL), more than half of which were bilateral (60%). Upon audiometric examination, all but 1 patient had mixed, conductive, or sensorineural HL. All patients presented with eustachian tube dysfunction (ETD), otitis media with effusion (OME), or both. Nasal endoscopy showed intranasal pathology in 3 (27%) patients. Otologic symptoms were improved in all patients after treatment with an average of 4 in-office follow-up appointments. CONCLUSION:GPA should be included in the differential diagnosis of adults with unexplained mixed hearing loss, new onset serous effusion, or acute otitis media in the absence of a previous history of ETD. Laboratory tests (ie, anti-neutrophil cytoplasmic autoantibody, erythrocyte sedimentation rate, and C-reactive protein) along with a urinalysis can aid in screening these patients. In cases in which the index of suspicion is high, repeated testing could reduce the risk of false negative findings.

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