Worldwide distribution of Waardenburg syndrome

Chetan S. Nayak, Glenn Isaacson

Research output: Contribution to journalReview articlepeer-review

63 Scopus citations


To clarify the multiracial occurrence of Waardenburg syndrome, we present a case series and literature review. A computerized review of the English-language literature was conducted to assess the distribution of reported occurrences of Waardenburg syndrome in populations around the world. We detail the clinical features of 2 family cohorts: one of Western European origin and the other from South Asia. A computerized literature review found sporadic cases of the syndrome in many ethnic groups, including Japanese, Taiwanese, and Middle Eastern families. The highest reported incidence is among Kenyan Africans. Waardenburg syndrome accounts for between 2% and 5% of cases of congenital deafness. It was first described in Northern European cohorts and is widely identified in fair-skinned populations. We hope to raise awareness of the worldwide distribution of this important cause of hearing loss.

Original languageEnglish (US)
Pages (from-to)817-820
Number of pages4
JournalAnnals of Otology, Rhinology and Laryngology
Issue number9 I
StatePublished - Sep 2003


  • Congenital deafness
  • Dystopia canthorum
  • Genetic deafness
  • Heterochromia iridis
  • Waardenburg syndrome

ASJC Scopus subject areas

  • Otorhinolaryngology


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