SUMMARY

Waardenburg syndrome type IV (WS4), also knownas Shah–Waardenburg syndrome or Waardenburg–Irschsprung disease, is a rare congenital disorder characterized by the association of sensorineural deafness,pigmentary abnormalities of the skin, eyes, and hairand colonic aganglionosis. Four types of Waardenburg syndrome have been described. Type IV (WS4) is the least common, with only fifty cases reported in the literature. Published data on the results of the use of cochlear implants for the treatment of hearing loss in this syndrome are conflictive. However, most studies have reported favourable outcomes. We report a case of a WS4 patient with sensorineural hearing loss treated with a cochlear implant at 25 months of age with a postimplantation performance well above the average for children implanted at the same age.