Characteristics and Progression of Hearing Loss in Children with Down Syndrome.

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.