Early intervention in localized Wegener's granulomatosis with sensorineural hearing loss preserves hearing.

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.