The most noticeable symptom is a rapid loss of vision. There may also be neurological signs such as severe headache, vertigo, nausea, and drowsiness. Loss of hearing, and loss of hair (alopecia) and skin color may occur along, with whitening (loss of pigmentation) of the hair and eyelashes (poliosis).

Annual incidence is estimated at 1/400,000. The condition predominantly affects young women and individuals with darker pigmentation (those of Asian, Hispanic, or Native American origin). Children may be affected, but the mean age of onset is about 30 years of age (ranging from 10 to 52 years).

The American Uveitis Society in 1978 recommended the following diagnostic criteria: (1) the absence of any history of ocular trauma or surgery; and (2) the presence of at least three of the following four signs: (a) bilateral chronic iridocyclitis; (b) posterior uveitis, including exudative retinal detachment, forme ...

There are four specific phases of VKH – prodromal, acute uveitic, chronic uveitic, and recurrent chronic phase. The acute uveitic phase consists of blurry vision and photophobia secondary to chorioretinal inflammation.