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 w ho had otological manifestations in the later stages of CSS. Methods: The h istory, treatment, and outcome were evaluated using office and hospital cha rt data in these five cases. Results. In all cases, otological manifestatio ns occurred during an advanced stage of CSS and was characterized by the pr esence of dense aural discharge, granulomatous eosinophilic infiltration in to 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 rapi d, complete cessation of aural discharge and reversal or stabilization of h earing loss. Otological manifestations have been stable over a range of fol low-up from 1 to 20 years on a maintenance regimen of steroids. Conclusions . Temporal bone involvement in CSS is rare. Surgical intervention of the te mporal bone is relegated to complications that may include infectious masto iditis 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 compl ications.