How inner-ear hair cells build and keep their sound-sensing bundles
Hair Cell Polarization and Sensory Bundle Development
['FUNDING_R01'] · JACKSON LABORATORY · NIH-11235144
This research is learning how tiny hair bundles in inner-ear sensory cells form and stay the right shape to help people born deaf or who lose hearing later in life.
Quick facts
| Phase | ['FUNDING_R01'] |
|---|---|
| Study type | Nih_funding |
| Sex | All |
| Sponsor | JACKSON LABORATORY (nih funded) |
| Locations | 1 site (BAR HARBOR, UNITED STATES) |
| Trial ID | NIH-11235144 on ClinicalTrials.gov |
What this research studies
From a patient's point of view, researchers are using lab models to understand how the hair bundle — the tiny, actin-based structure that senses sound — is built and maintained in inner-ear sensory cells. They focus on proteins known to be involved in congenital deafness, including the GPSM2–Gαi complex and the motor protein Myosin-15A, to see how these factors are positioned and moved within hair cells. Most experiments use well-established mouse models and cellular assays to observe stereocilia placement, length control, and molecular interactions. The goal is to map the molecular steps that could be targeted to prevent or repair hair-bundle defects that cause hearing loss.
Who could benefit from this research
Good fit: People with congenital sensorineural hearing loss (for example due to GPSM2 mutations like in Chudley-McCullough syndrome) or adults with progressive hair-cell–related hearing loss would be the most relevant candidates for future related studies.
Not a fit: Patients whose hearing loss is due to middle-ear (conductive) problems, auditory nerve degeneration beyond hair cells, or causes unrelated to hair-bundle or hair-cell biology are unlikely to benefit directly from this work.
Why it matters
Potential benefit: If successful, this work could reveal molecular targets to prevent or reverse hair-bundle defects that underlie some forms of congenital and acquired sensorineural hearing loss.
How similar studies have performed: Previous basic research in mice has shown GPSM2, Gαi, and Myosin-15A affect hair-bundle shape and can cause genetic deafness, but translating these findings into human therapies is still new and unproven.
Where this research is happening
BAR HARBOR, UNITED STATES
- JACKSON LABORATORY — BAR HARBOR, UNITED STATES (ACTIVE)
Researchers
- Principal investigator: TARCHINI, BASILE ROBIN — JACKSON LABORATORY
- Study coordinator: TARCHINI, BASILE ROBIN
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.