Research Article

The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome

Table 1

Classifications of Waardenburg syndrome.

Clinical manifestationsThe incidence rate of SNHLGenetic mutation

Type 1Dystopia canthorum, white forelock, white eyelashes, leukoderma, heterochromia iridis60%PAX3

Type 2The absence of dystopia canthorum90%MITF, WS2B, WS3B  
EDNRB, EDN3, SOX10  
SNAI2

Type 3Type 1 + upper limb abnormalities60%PAX3

Type 4Type 2 + Hirschsprung’s disease90%EDNRB, EDN3, SOX10