Table 4 Diagnostic criteria for initial onset VKH Disease

1. No ocular trauma or surgery preceding onset of disease*

2. Bilateral involvement (verified with ICGA and/or EDI-OCT) *

3. Exclusion of other infectious, inflammatory or masquerading entities, in particular other stromal choroiditis entities (i.e. tuberculosis, sarcoidosis or syphilis) *

4. Diffuse choroiditis evidenced by ICGA and/or EDI-OCT *

5. Signs and symptoms of less than 4 weeks’ duration*

6. Absence of clinical findings compatible with chronic disease (i.e. sunset glow fundus or integumentary signs (vitiligo, alopecia & poliosis) *

7. Exudative retinal detachments (evidenced by pooling and pinpoint leaking points on FA and ICGA) (very helpful criterion when present)

8. Disc hyperfluorescence (helpful criterion)

9. Neurological / auditory findings (meningismus, tinnitus, acute hearing loss) (helpful criterion)