Otological manifestations of Churg-Strauss syndrome.
OBJECTIVE
To describe the clinical features, pathogenesis, and management of the otological complications in Churg-Strauss syndrome (CSS).


METHODS
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.
Ishiyama
A
A
Division of Head and Neck Surgery, University of California At Los Angeles School of Medicine, Los Angeles, California 90095, USA. Ishiyama@ucla.edu
Canalis
R F
RF
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002908:Chronic Disease; D015267:Churg-Strauss Syndrome / Q000145:classification / Q000150:complications* / Q000175:diagnosis; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D006311:Hearing Disorders / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008417:Mastoiditis / Q000209:etiology; D008875:Middle Aged; D008876:Middle Ear Ventilation; D010033:Otitis Media / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D012008:Recurrence; D012189:Retrospective Studies; D013256:Steroids; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D000900:Anti-Bacterial Agents; D000893:Anti-Inflammatory Agents; D013256:Steroids