Otological manifestations of Churg-Strauss syndrome.

OBJECTIVES/HYPOTHESIS: To describe the clinical features, pathogenesis, and management of the otological complications in Churg-Strauss syndrome (CSS).

STUDY DESIGN

Retrospective review of five patients with documented CSS who had otological manifestations in the later stages of CSS.

METHODS

The history, treatment, and outcome were evaluated using office and hospital chart data in these five cases.

RESULTS

In all cases, otological manifestations occurred during an advanced stage of CSS and was characterized by the presence of dense aural discharge, granulomatous eosinophilic infiltration into mastoid and middle ear, and subacute severe to profound hearing loss. In all cases, the recurrent aural discharge and granulomatous infiltrate was unresponsive to conventional treatment including repeated myringotomies and aggressive antibiotics. Systemic steroid administration resulted in a rapid, complete cessation of aural discharge and reversal or stabilization of hearing loss. Otological manifestations have been stable over a range of follow-up from 1 to 20 years on a maintenance regimen of steroids.

CONCLUSIONS

Temporal bone involvement in CSS is rare. Surgical intervention of the temporal bone is relegated to complications that may include infectious mastoiditis and intracranial involvement. Recognition of the association between CSS and otological disease is important because it is highly responsive to systemic steroids, and early steroid treatment may prevent progression to irreversible hearing loss, infectious otomastoiditis, or intracranial complications.