We discuss the rare case of a 68-year-old woman with Vogt-Koyanagi-Harada (VKH) syndrome and sensorineural hearing loss (SNHL) who was successfully treated with intratympanic corticosteroid injections. The patient had presented with bilaterally asymmetric (i.e., moderate and moderate to severe) SNHL, tinnitus, vertigo, and vitiligo. She received two intratympanic injections in her worse-hearing ear over the course of 1 month. Subsequent audiometry showed an immediate 5- to 10-dB improvement in her hearing across multiple frequencies, as well as a long-term improvement to near-normal thresholds. The hearing thresholds in her untreated ear remained stable. To the best of our knowledge, this is the first report of a patient with VKH syndrome who was successfully treated with intratympanic steroid application.