Conditions / Syndrome

Waardenburg syndrome type 3

info · Syndrome · ICD-10: E70.3

A Waardenburg syndrome characterized by upper limb anomalies, congenital hearing loss, dystopia canthorum and pigmentation anomalies of eyes, hair, and skin that has_material_basis_in heterozygous or homozygous mutation in the PAX3 gene on chromosome 2q36.

Signs and symptoms

  • Short palpebral fissure
  • Prominent nasal tip
  • Delayed fine motor development
  • Flexion contracture of finger
  • Bilateral wrist flexion contracture
  • Blue irides
  • Partial albinism
  • Epicanthus inversus
  • Premature graying of hair
  • Posteriorly rotated ears

Also known as: Klein-Waardenburg syndrome; WS3; Waardenburg syndrome type III; Waardenburg syndrome with upper limb anomalies