Waardenburg's Syndrome and Heterochromia Iridum in a Deaf School Population

Waardenburg''s syndrome consists of lateral displacement of the inner canthi of the eyes (dystopia canthorum), a broad nasal root and confluent eyebrows, heterochromia iridum, a white forelock and congenital deafness. The syndrome is inherited as a dominant, but affected individuals do not necessarily have all of the characteristics cited.Five hundred and fourteen pupils at a school for the deaf were screened for features of this syndrome. Three cases were discovered. Eleven other deaf children were found to have heterochromia iridum and two more had white forelocks. The interocular dimensions of the remaining children were recorded as standards by which to judge the presence of dystopia canthorum. The results of chromosomal analysis in two cases with Waardenburg''s syndrome were normal.The findings provide further evidence that Waardenburg''s syndrome is a distinct entity and call in question Mackenzie''s concept of a comprehensive “first arch syndrome”.