RESUMEN

Criteria for cochlear implants are expanding and now include children with disabilities in addition to hearing loss, such as those with Kabuki syndrome (KS). This case report describes language outcomes and changes in the quality of life of a female child with KS after cochlear implantation. The subject had a profound progressive sensorineural hearing loss, cognitive impairments, and other disabilities and communicated using vocalized sounds and the Dutch Sign Language. After cochlear implantation at an age of nine years and three months, the patient displayed no progress in speech production and minimal progress in receptive language development, but she had an increased awareness of the world and an increase in the quality of life.

Asunto(s)

Anomalías Múltiples/rehabilitación , Implantación Coclear/métodos , Sordera/rehabilitación , Cara/anomalías , Enfermedades Hematológicas/rehabilitación , Enfermedades Vestibulares/rehabilitación , Anomalías Múltiples/diagnóstico , Audiometría de Tonos Puros , Umbral Auditivo , Niño , Sordera/diagnóstico , Femenino , Estudios de Seguimiento , Enfermedades Hematológicas/diagnóstico , Humanos , Trastornos del Desarrollo del Lenguaje/diagnóstico , Trastornos del Desarrollo del Lenguaje/rehabilitación , Pruebas del Lenguaje , Imagen por Resonancia Magnética , Lengua de Signos , Medición de la Producción del Habla , Tomografía Computarizada por Rayos X , Enfermedades Vestibulares/diagnóstico