The eye symptoms may be accompanied by a varying constellation of systemic symptoms, such as auditory (tinnitus,[6] vertigo,[6] and hypoacusis), neurological (meningismus, with malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back, or a combination of these factors;[6] meningitis,[4] CSF pleocytosis, cranial nerve palsies, hemiparesis, transverse myelitis and ciliary ganglionitis[6]), and cutaneous manifestations, including poliosis, vitiligo, and alopecia.

[6] The sequence of clinical events in VKH is divided into four phases - prodromal, acute uveitic, convalescent, and chronic recurrent.

[6] Fever, headache, nausea, meningismus, dysacusia (discomfort caused by loud noises or a distortion in the quality of the sounds being heard), tinnitus, and/or vertigo may occur.

[6] In 70% of VKH cases, the onset of visual blurring is bilaterally contemporaneous; if initially unilateral, the other eye is involved within several days.

[7] Although sometimes a viral infection, or skin or eye trauma precedes an outbreak,[6] the exact underlying initiator of VKH disease remains unknown.

For example, it has been associated with human leukocyte antigens (HLA) HLA-DR4 and DRB1/DQA1,[10] copy-number variations of complement component 4,[10] a variant IL-23R locus[10] and with various other non-HLA genes.

[2] Diagnostic confirmation and an estimation of disease severity may involve imaging tests such as retinography, fluorescein or indocyanine green angiography, optical coherence tomography and ultrasound.

[2][5][9][7] For example, indocyanine green angiography may detect continuing choroidal inflammation in the eyes without clinical symptoms or signs.

[9] In refractory situations, other immunosuppressives such as cyclosporine,[2][3] or tacrolimus,[9] antimetabolites (azathioprine, mycophenolate mofetil or methotrexate[9]), or biological agents such as intravenous immunoglobulins (IVIG) or infliximab may be needed.

[6] VKH syndrome is named for ophthalmologists Alfred Vogt from Switzerland and Yoshizo Koyanagi and Einosuke Harada from Japan.