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- W1863631 abstract "espanolEl sindrome de Waardenburg tipo IV (WS4), tambien conocido como sindrome de Shah�Waardenburg o enfermedad de Waardenburg�Hirschsprung es una alteracion congenita poco frecuente caracterizada por la asociacion de hipoacusia neurosensorial, anormalidades pigmentarias de la piel, los ojos y el cabello, y aganglionosis colonica. Se han descrito cuatro tipos de sindrome de Waardenburg (WS) siendo el WS4 el mas infrecuente con cincuenta casos descritos en la literatura. Las publicaciones sobre los resultados del implante coclear (IC), en el tratamiento de la hipoacusia en este sindrome, son discordantes, aunque en la mayoria de casos la evolucion postimplante es muy satisfactoria. Presentamos el caso de un paciente con WS4 con hipoacusia neurosensorial tratado con un implante coclear a la edad de 25 meses con un rendimiento postimplante superior a la media de ninos implantados a la misma edad. EnglishWaardenburg 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." @default.
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- W1863631 date "2011-01-01" @default.
- W1863631 modified "2023-09-22" @default.
- W1863631 title "El implante coclear en el síndrome de Waardenburg tipo IV" @default.
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