Research Article
The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome
Table 1
Classifications of Waardenburg syndrome.
| | Clinical manifestations | The incidence rate of SNHL | Genetic mutation |
| Type 1 | Dystopia canthorum, white forelock, white eyelashes, leukoderma, heterochromia iridis | 60% | PAX3 |
| Type 2 | The absence of dystopia canthorum | 90% | MITF, WS2B, WS3B EDNRB, EDN3, SOX10 SNAI2 |
| Type 3 | Type 1 + upper limb abnormalities | 60% | PAX3 |
| Type 4 | Type 2 + Hirschsprung’s disease | 90% | EDNRB, EDN3, SOX10 |
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