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.
Prominent auditory and cutaneous pigmentary abnormalities coexist as clinical features in several diseases. Vogt-Koyanagi-Harada (VKH) syndrome is an autoimmune condition characterized by ocular, neurologic, auditory, and cutaneous manifestations. Otologic complaints may include sensorineural hearing loss (SNHL), tinnitus, and/or vertigo.
Previous presentation: The information in this article has been updated from its original presentation as a poster at the Eastern Section meeting of the Triological Society; Jan. 23-25, 2009; Boston.