# Auditory function, cognition, language and brain structure in Down Syndrome

> **NIH NIH R01** · UNIVERSITY OF WISCONSIN-MADISON · 2022 · $2,281,845

## Abstract

ABSTRACT
 Down syndrome (DS) is a leading known cause of intellectual disability and a highly recognized genetic
syndrome that involves multiple medical co-morbidities. Hearing deficits in DS are estimated to occur up to rates
of 80-90% and are thought to be caused by a combination of structural and functional abnormalities in the
external, middle and/or inner ear. This project aims to tackle a timely and significant question regarding the role
of hearing loss in DS on auditory function, cognition, language, and structural integrity of brain regions that are
important for hearing. Despite the known pervasive nature of hearing deficits in DS, to date, research has yet to
identify specific consequences of hearing deficits associated with trisomy 21. Aim 1 will assess hearing status,
auditory integrity and maturation using objective measures of auditory processing in subcortical auditory
brainstem responses (ABR) and cortical auditory evoked potentials (CAEPs), and functional processing
behavioral measures of speech identification and sound localization. The goal is to understand how hearing loss
impacts auditory processing and auditory maturation in DS is after compensating for hearing loss. Aim 2 will
investigate the associations between hearing/auditory deficits and overall intellectual functioning as well as
memory, attention, and executive function, as these cognitive domains have been shown to be influenced in
critical ways by hearing status and auditory impairments in non-DS populations. Aim 2 will also examine the
associations between hearing and auditory function and receptive and expressive vocabulary, obtained via
standardized measures and language samples. Aim 3 will investigate the associations between hearing/auditory
deficits and imaging measures of structure and microstructure in brain regions involved in auditory processes,
focusing on regional morphometry, relaxometry, and microstructural diffusion measures. We aim to understand
whether abnormalities are domain specific within auditory processes, global to all auditory brain regions, or
generalized/global to DS brains but not auditory-specific, i.e., related to issues from generic pathophysiology in
DS. This work meets the programmatic objectives of INvestigation of Co-occurring conditions across the Lifespan
to Understand Down syndrome (INCLUDE) and will advance understanding of the consequences of hearing loss
in DS for functional auditory, cognitive and language outcomes and brain integrity. One immediate benefit is that
results will inform clinical assessment and intervention of hearing loss catered specifically to the pathophysiology
in DS. Longer-term impact is the identification of measures to evaluate effectiveness of future intervention of
hearing loss in DS.

## Key facts

- **NIH application ID:** 10391010
- **Project number:** 1R01DC019511-01A1
- **Recipient organization:** UNIVERSITY OF WISCONSIN-MADISON
- **Principal Investigator:** ANDREW L ALEXANDER
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2022
- **Award amount:** $2,281,845
- **Award type:** 1
- **Project period:** 2022-01-15 → 2024-12-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10391010

## Citation

> US National Institutes of Health, RePORTER application 10391010, Auditory function, cognition, language and brain structure in Down Syndrome (1R01DC019511-01A1). Retrieved via AI Analytics 2026-05-25 from https://api.ai-analytics.org/grant/nih/10391010. Licensed CC0.

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