Atypical Cogan's Syndrome Is Common among Japanese Patients: A Case Report and Literature Review in Japan.

We report the case of a 55-year-old Japanese woman who presented with fever, headache, vertigo, sensorineural hearing loss, and scleritis, leading to a diagnosis of Cogan's syndrome (CS). Her hearing acuity recovered to previous levels after high-dose glucocorticoid treatment, despite seven weeks having passed since the onset. We reviewed 30 adult cases of Japanese CS using PubMed and Ichushi-Web. We identified several clinical features of Japanese patients with CS, including an older age of onset, less interstitial keratitis, more frequent fever and headache, elevated inflammatory marker levels, and a higher prevalence of large-vessel vasculitis, compared with studies on Caucasians.