Waardenburg syndromes are deafness syndromes associated with pigmentary disturbances. Their incidence is 1/270,000 births/year. They are distinguished by their autosomal dominant transmission and their irregular depigmentation. Waardenburg syndrome type II (type IIA when linked to locus 3p13; type IIB when not linked to this locus) is a group of heterogeneous entities distinguished from Waardenburg syndrome type I by the absence of dystopia canthorum. The presence of a family history of congenital deafness or pigmentation anomalies is of importance for the diagnosis. Hearing aids to counter deafness and management of the associated malformations are recommended. Skin and eyes photoprotection is highly recommended.
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