This is a case report of a 29-year-old man, who presented with progressive confusion, memory problems and personality changes during 1.5 months. Later, he developed bilateral hearing impairment but had no visual symptoms. A brain MRI showed numerous small hyperintensities in both hemispheres, a retinal fluorescence angiography revealed multiple hyperfluorescent arterial occlusions, and an audiogram showed bilateral hearing impairment. The patient was treated for Susac syndrome with high-dose corticosteroids initially, followed by intravenous immunoglobulin and cyclophosphamide.