The Relationship Between Vestibular, Hearing, and Balance Outcomes in Individuals With Down Syndrome.

OBJECTIVES: The purpose of this study was to determine the prevalence of vestibular dysfunction in individuals with Down syndrome (DS) and its relationship with audiometric and balance outcomes.

DESIGN: Participants were 27 individuals with DS (mean age: 23.3 yrs; range: 7 to 38 yrs) and 20 neurotypical controls (mean age: 23.3 yrs; range: 7 to 47 yrs). All participants completed otoscopy, audiometric testing, 226 Hz tympanometry, wideband acoustic immittance (absorbance), air and bone conduction cervical and ocular vestibular evoked myogenic potential (VEMP) testing, and the video head impulse test. Balance and gait testing included gait speed, Timed Up and Go, and the Single Leg Stance.

RESULTS: In the participants with DS, 35.2% of ears demonstrated some degree of vestibular dysfunction (i.e., absent cervical VEMP, absent ocular VEMP, or abnormal video head impulse test). A large degree of variability in both the severity (normal to profound) and type (sensorineural, conductive, and mixed) of hearing loss was found in participants with DS. VEMP response rates tended to be higher when using bone-conducted compared with air-conducted stimuli. Overall, vestibular loss was more prevalent in those with sensorineural hearing loss. Compared with participants in the neurotypical control group, participants with DS had significantly longer Timed Up and Go scores, slower gait speed in the fast condition, and poorer balance in the Single Leg Stance eyes open and eyes closed conditions.

CONCLUSIONS: Findings from the present study confirm a large degree of heterogeneity in the severity and type of both hearing loss and vestibular dysfunction in individuals with DS. These findings represent, for the first time, a comprehensive vestibular assessment in individuals with DS. Approximately 1/3 of individuals with DS have some degree of vestibular involvement. Those with sensorineural hearing loss had higher rates of vestibular involvement. The presence of conductive hearing loss was also a factor when completing VEMP testing, and the use of bone conduction stimuli is recommended.