ABSTRACT
OBJECTIVES: To evaluate hearing impairment in children with Down syndrome, and to describe the factors that influence the severity of hearing loss or changes in hearing over time. STUDY DESIGN: Using the Audiological and Genetic Database (AudGenDB), audiograms of children with Down syndrome were analyzed retrospectively for type, severity, and laterality of hearing loss, as well as changes over time. Medical charts and imaging results were reviewed to identify factors influencing hearing loss. RESULTS: Among the 1088 patients with Down syndrome included in the study, 921 had hearing loss in at least 1 ear, 91.1% had bilateral hearing loss, and 8.9% had unilateral hearing loss (1760 total ears with hearing loss). Of the ears with hearing loss, 18.8% (n = 180) had moderate or worse hearing loss. "Undefined" hearing loss and pure conductive hearing loss (CHL) were the most common types, followed by mixed hearing loss and sensorineural hearing loss (SNHL). Three-quarters (75.4%) of the children had experienced chronic otitis media or more than 2 episodes of acute otitis media. Patients with bilateral, mixed hearing loss or a history of seizures were at risk for more severe hearing loss. CHL, absence of cholesteatoma, and placement of first ear tubes before age 2 years were associated with greater improvement in hearing over time, whereas SNHL and mixed hearing loss were associated with progressive decline. CONCLUSION: Children with Down syndrome who have bilateral, mixed hearing loss or a history of seizures are at risk for more severe hearing loss. SNHL and mixed hearing loss should not be overlooked in patients with CHL. All patients with Down syndrome will benefit from serial audiograms, especially those children with SNHL or mixed hearing loss, which is likely to worsen over time.
Subject(s)
Down Syndrome/complications , Hearing Loss/etiology , Child , Child, Preschool , Databases, Factual , Disease Progression , Female , Hearing Loss/diagnosis , Hearing Loss/epidemiology , Hearing Tests/methods , Humans , Male , Retrospective Studies , Risk FactorsABSTRACT
Introducción. Algunos usuarios de la prótesis coclear Nucleus 22 que usan la estrategia de estimulación SPEAK podrían beneficiar su comprensión del habla con el uso de la estrategia CIS incluso con poco entrenamiento. Sin embargo verificar esta hipótesis es difícil porque la estrategia CIS no ha sido implementada en los procesadores de habla para la Nucleus 22 que están disponibles comercialmente. Métodos. Tres adultos con implantes Nucleus 22, usuarios experientes de la estrategia SPEAK, participaron en este estudio. Su identificación de consonantes fue evaluada con la strategia SPEAK (usando los procesadores de los mismos pacientes y la estrategia CIS (usando una interface especial que desarrollamos para este propósito). Un usuario del implante de tallo cerebral (ITC) Nucleus también fue estimulado con la estrategia CIS y la interface especial. Resultados: Aunque solo tuvieron unos minutos de entrenamiento con la estrategia CIS los pacientes con implantes cocleares obtuvieron niveles de percepción de consonantes cercanos a los obtenidos con la estrategia SPEAK. El usuario del ITC también fue estimulado exitosamente con la estrategia ClS. Conclusiones. Algunos usuarios del implante Nucleus 22 podrian obtener un beneficio significativo de la estrategia CIS si esta estuviera disponible comercialmente. Implementar la estrategia CIS es también factible para los usuarios del ITC Nucleus.