Fixing the tiny hair-like structures in the inner ear that help hearing
Investigating the mechanisms of stereocilia length regulation and innovative strategies for restoring hearing
['FUNDING_R01'] · UNIVERSITY OF CALIFORNIA, SAN DIEGO · NIH-11371358
This project explores AAV gene-delivery approaches to rebuild and lengthen inner-ear hair bundles to help adults with inherited sensorineural hearing loss.
Quick facts
| Phase | ['FUNDING_R01'] |
|---|---|
| Study type | Nih_funding |
| Sex | All |
| Sponsor | UNIVERSITY OF CALIFORNIA, SAN DIEGO (nih funded) |
| Locations | 1 site (LA JOLLA, UNITED STATES) |
| Trial ID | NIH-11371358 on ClinicalTrials.gov |
What this research studies
Researchers are focusing on the tiny hair-like stereocilia in inner-ear hair cells and how actin-related proteins control their length and connections. They will use AAV-based gene delivery and laboratory models to test ways to restore stereocilia height and the link to the tectorial membrane. The team targets proteins known to cause inherited deafness (like Eps8, MyoXVa, whirlin, and stereocilin) and will examine molecular and structural changes after treatment. The overall aim is to develop approaches that could be translated into therapies to recover hearing.
Who could benefit from this research
Good fit: Adults (21+) with genetic forms of sensorineural hearing loss linked to defects in stereocilia proteins (for example Eps8, MyoXVa, whirlin, or stereocilin) would be the most relevant candidates.
Not a fit: People whose hearing loss is due to non-stereocilia causes (such as cochlear nerve damage, middle-ear disease, or broadly diffuse hair-cell loss not tied to these proteins) may not benefit from these approaches.
Why it matters
Potential benefit: If successful, this work could restore hair-cell structure and improve or recover hearing for people with stereocilia-related inherited hearing loss.
How similar studies have performed: Prior animal work has shown that restoring certain stereocilia proteins or using AAV delivery can rescue hair-cell structure and hearing in mice, but human benefit has not yet been proven.
Where this research is happening
LA JOLLA, UNITED STATES
- UNIVERSITY OF CALIFORNIA, SAN DIEGO — LA JOLLA, UNITED STATES (ACTIVE)
Researchers
- Principal investigator: MANOR, URI — UNIVERSITY OF CALIFORNIA, SAN DIEGO
- Study coordinator: MANOR, URI
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 below.