Accelerated Cognitive Decline Associated With Hearing Loss and Bilateral Vestibulopathy: Insights From a Prospective Cross-Sectional Study Using the Repeatable Battery for the Assessment of Neuropsychological Status Adjusted for the Hearing Impaired in the DFNA9 Population.
Hanne Gommeren, Joyce Bosmans, Julie Moyaert and 9 others
PMID 36607747WHAT IT FOUND
DFNA9 patients over 55 scored significantly lower on cognitive tests than matched controls, particularly in attention and memory.
Under 55, differences were not significant after correction. Six older patients scored low enough to be flagged for mild cognitive impairment risk.
Key findings
01DFNA9 patients aged 55 and older had significantly lower scores on the Attention subscale and Total Scale compared to controls, even after correcting for multiple tests.
02DFNA9 patients younger than 55 did not show statistically significant differences in cognitive scores compared to controls after correction.
03Six DFNA9 patients, all aged 55 or older, had index scores indicating a risk for mild cognitive impairment.
STILL TO COME
How it was doneWhat they foundWhat it means for PTsWhat it means for OTsWhat it means for SLPs
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What it does not show
The study is cross-sectional, so it cannot determine if hearing/vestibular loss caused cognitive decline or if both are symptoms of aging or the genetic condition. Controls were matched for age, sex, and education but not for hearing or vestibular function, meaning differences in cognition could be due to sensory deprivation rather than the genetic mutation itself. Vestibular function in controls was only assessed with vHIT, not the full battery (caloric/rotatory chair) used in patients, potentially missing subtle vestibular deficits in the control group. Hearing aid use was not recorded, so the cognitive impact of treated vs. untreated hearing loss could not be analyzed. Only one patient was under 35, so presymptomatic carriers were underrepresented.
Declared interests
The study was supported by non-U.S. government funding. No specific commercial conflicts of interest were declared in the provided text, though the study was part of larger research efforts (DFNA9-evolution, GECkO, ERP-Norm) approved by the Antwerp University Hospital ethics committee.
The easy way to misread this
Do not assume that the genetic mutation itself causes cognitive decline. The study did not match controls for hearing and vestibular function, so the observed cognitive differences in older patients may be due to the sensory deprivation (hearing and balance loss) rather than the DFNA9 gene directly.