Clinical Focus  |   December 2012
Early Intervention in Localized Wegener’s Granulomatosis With Sensorineural Hearing Loss Preserves Hearing
Author Affiliations & Notes
  • Vasuky Sriskandarajah
    Epsom and St. Helier University Hospitals National Health Service Trust, Carshalton, England
  • Rhea A. Bansal
    Epsom and St. Helier University Hospitals National Health Service Trust, Carshalton, England
  • Robin Yeoh
    Epsom and St. Helier University Hospitals National Health Service Trust, Carshalton, England
  • Amolak S. Bansal
    Epsom and St. Helier University Hospitals National Health Service Trust, Carshalton, England
  • Correspondence to Amolak S. Bansal: Amolak.Bansal@esth.nhs.uk
  • Editor and Associate Editor: Larry Humes
    Editor and Associate Editor: Larry Humes×
Hearing Disorders / Clinical Focus
Clinical Focus   |   December 2012
Early Intervention in Localized Wegener’s Granulomatosis With Sensorineural Hearing Loss Preserves Hearing
American Journal of Audiology December 2012, Vol.21, 121-126. doi:10.1044/1059-0889(2012/12-0003)
History: Accepted 20 May 2012 , Received 16 Jan 2012 , Revised 16 May 2012
American Journal of Audiology December 2012, Vol.21, 121-126. doi:10.1044/1059-0889(2012/12-0003)
History: Accepted 20 May 2012 , Received 16 Jan 2012 , Revised 16 May 2012

Purpose: Wegener’s granulomatosis has been renamed granulomatosis with polyangiitis (GPA). In this article, the authors refer to WG as “WG/GPA” to indicate the old and new names of this condition. WG/GPA is a systemic necrotizing granulomatous vasculitis that can affect, in particular, the lungs, sinuses, and kidneys. The authors report 2 cases with antineutrophil cytoplasmic antibodies (c-ANCA) positive WG/GPA whose initial presentations were in the form of both conductive and sensorineural hearing loss without systemic features. The authors contrast the reversal of hearing loss and prevention of disease progression with early recognition and treatment.

Method: The authors present 2 contrasting cases of WG/GPA. Changes in hearing were measured through use of a GSI 61 audiometer as well as guidelines from the British Society of Audiology. Serum ANCA were detected by indirect immunofluorescence and formalin fixed neutrophils. Proteinase 3 (PR3) and myeloperoxidase antibodies were measured through use of a fluoroenzyme immunoassay.

Results: Persistent deafness and systemic disease are more likely, and more aggressive therapy was required when the diagnosis of WG/GPA was delayed.

Conclusion: WG/GPA should be considered in acute or subacute deafness presenting over days to weeks and even in the absence of systemic symptoms. A negative or weak ANCA with absent antibodies to serine PR3 and myeloperoxidase should not exclude the possible diagnosis of WG/GPA, and a high index of suspicion should be maintained.

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