Major Criteria

·        Congenital sensoineural hearing loss

·        Pigmentary disturbances of iris: Complete heterochromia iridis, partial segmental heterochromia iridis, hypoplastic blue iridis

·        White forelock

·        Distropia canthorum

·       Affected first degree relative

Minor Criteria

·        Congenital leukoderma: several area of hypopigmented skin

·        Medial eyebrow flare (synophyrys)

·        Broad and high nasal root

·        Hypoplasia of alae nasi

·       Premature graying of hair