Hearing and sound‑localization problems in Fragile X syndrome
Official title: Myelination Deficits Underlying Auditory Issues in Fragile X Syndrome
Funded at Oklahoma State University Stillwater · NIH-11125625 · Data updated 2026-11-12
This work looks at whether reduced myelin (nerve insulation) causes trouble hearing and locating sounds in people with Fragile X syndrome.
Quick facts
| Grant type | R15 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Oklahoma State University Stillwater NIH-funded |
| Lab location | 1 site (Stillwater, United States) |
| Project start | September 9, 2021 |
| Project end | July 31, 2028 |
| Funding year | 2025 |
| Award (this year) | $444,238 |
| NIH project number | 2R15HD105231-02A1 |
| Project ID | NIH-11125625 on NIH RePORTER |
Who could benefit from this research
✓Could help
People with Fragile X syndrome and caregivers concerned about listening or sound‑sensitivity problems would be most relevant to this line of research and any future human studies.
✕Less likely to help
People whose hearing difficulties stem from ear‑level damage (for example chronic middle‑ear disease or cochlear injury) rather than brainstem myelination are less likely to benefit directly.
What this research studies
Researchers will use mice that model Fragile X to study whether the insulation (myelin) around hearing nerve fibers in the brainstem is thinner or altered. They will use genetic methods plus microscopy to examine myelin structure, record auditory brainstem responses (ABRs) to measure signal timing, and run startle‑response tests to gauge hearing behavior. The team aims to connect anatomical myelin defects with slowed or changed sound processing that could explain difficulties in noisy environments. Though this project is lab‑based in animals, the findings are intended to guide future human screening or treatment approaches for Fragile X and related autism‑spectrum auditory problems.
Why it matters
Potential benefit: Could identify a measurable brain change behind hearing problems in Fragile X and point toward new screening or treatment strategies.
How similar studies have gone: Earlier animal work, including the team's prior R15, found thinner myelin and altered auditory brainstem responses in Fragile X mice, but translating these results to human patients has not yet been demonstrated.
Where this research is happening
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Oklahoma State University Stillwater
Stillwater, United States Active
Researchers
- Principal investigator: Mccullagh, Elizabeth Anne — Oklahoma State University Stillwater
- Study coordinator: Mccullagh, Elizabeth Anne
Related research
Other NIH-funded projects on the same topics.
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Understanding Fragile X Syndrome: How it progresses and affects daily life
A long-term study using existing Fragile X data to map how the condition changes over time in children and young adults, including how autism, race, and location influence outcomes.
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Sleep and mood links in Fragile X Syndrome
A short, patient-friendly study to understand how sleep problems relate to mood and depressive symptoms in people with Fragile X–linked autism.
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Connecting 3D genome misfolding to gene silencing in Fragile X syndrome
A study in people with Fragile X syndrome focuses on how 3D genome folding errors may turn off important genes, contributing to the condition’s features.
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Cortical circuit problems in Fragile X mice and people
Researchers are studying how brain circuits go awry in Fragile X syndrome by comparing mice and people to understand why certain brain rhythms and responses are altered.
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Brain biology in fragile X syndrome: how brain signals unfold and vary in patients
This grant supports research to understand brain signals and why symptoms vary in people with fragile X syndrome, aiming to translate findings into better care.
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Brain research on Fragile X Syndrome to improve therapies
A multi-year program studies brain function and differences in Fragile X Syndrome to help develop better treatments for people with FXS.
About this research
- This is an active NIH-funded research project — typically early-stage science, not a clinical trial accepting patient enrollment.
- Some NIH-funded labs run parallel clinical studies or seek volunteers for related work. To check, contact the principal investigator or institution listed above.
- For full project details, budget, and progress reports, visit the official NIH RePORTER page.