Sudden onset sensorineural hearing loss is a well recognized entity frequently encountered in otolaryngological practice. However, the combination of such deafness as part of a wider systemic disorder is fortunately rare. Almost 100 years after the syndrome was classified, we describe a case of Vogt–Koyanagi–Harada syndrome occurring unusually in a Caucasian woman and characterized by sudden hearing loss. A brief review of this rare condition is presented.