Inflammatory / infectious / autoimmune / systemic disorders
Granulomatosis with polyangiitis

Topic Completed: 1 October 2013

Minor changes: 29 June 2020

Revised: 07 January 2019

Copyright: (c) 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Granulomatosis with polyangiitis [title] ear

Nat Pernick, M.D.
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Cite this page: Pernick N. Granulomatosis with polyangiitis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/earwegenersgran.html. Accessed August 10th, 2020.
Definition / general
  • Systemic necrotizing vasculitis that typically involves kidneys, lung, upper aerodigestive tract
  • Otologic involvement (otitis media, tympanic membrane perforation, sensorineural hearing loss, perforation of ear lobes, external otitis) as well as facial palsy occurs in 20 - 60% who have disease at traditional sites
  • Elevated serum ANCA
  • Corticosteroids, immunosuppressive drugs may cause long term remission and reverse hearing loss and facial palsy
Microscopic (histologic) description
  • Vasculitis, necrosis or granulomatous inflammation (few cases have all 3)
  • Extra levels / recuts increases sensitivity by 7% (Ann Pathol 2005;25:87)
Differential diagnosis
  • Polyarteritis nodosa: necrotizing vasculitis of small to medium sized muscular arteries
  • Rheumatoid arthritis: conductive hearing loss due to involvement of incus-maleus and incus-stapes articulations
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