A 27-year-old woman suddenly developed persistent rotatory
dizziness with unsteadiness on standing and walking, associated with symptoms relating to the autonomic nervous system, all signs and symptoms disappearing without treatment in 3 days. Ten days before this episode she had noticed progressive bilateral impairment of hearing accompanied by
tinnitus. Caloric and audiometric tests confirmed bilateral impairment of the audiovestibular organ. A week later she also developed bilateral
iritis and
papillitis. The constellation of ocular and audiovestibular signs suggested
Cogan's syndrome. Under high-dosage
glucocorticoid treatment (initially 1,000 mg/d
prednisolone intravenously for 3 days, then 100 mg/d orally in decreasing doses down to 10 mg daily) the ocular signs improved, but the bilateral
hearing impairment persisted. A recurrence occurred after 5 months, while on a
prednisolone dosage of 10 mg daily, together for the first time with
arthralgias, suggesting systemic involvement. Although the symptoms quickly subsided when dosage was increased to 100 mg daily, repeated attempts at
dose reduction brought about renewed exacerbation at 70 mg daily. As the necessary high
steroid dosage led to severe side effects, an immunosuppressive
drug was added (100 mg
cyclophosphamide and 20 mg
prednisolone, both daily; later 5 mg
methotrexate weekly and 4 mg
prednisolone every other day). The symptoms had not recurred when re-examined 7 months later.