# Functional development of hair cells and neurons in the inner ear

> **NIH NIH R01** · BOSTON CHILDREN'S HOSPITAL · 2021 · $575,994

## Abstract

Project summary
 Hair cells of the inner ear are the primary receptors of the auditory system. They transduce mechanical
information, associated with sound waves, into electro-mechanical (outer hair cells) and electro-chemical (Inner
hair cells) signals, which lead to amplification of the initial signal and activation of afferent neuronal fibers,
respectively. While hair cells and neuronal fibers appear before birth in mice, development and maturation of
the hair cells, neurons and synapses proceeds until hearing onset, ~postnatal day 12. This process is believed to
be dynamic and modulated by hair cell activity. In particular, recent work has shown that lack of hair cell
transmission, due to absence of functional synapses or defective mechanosensation, leads to altered neuronal
maturation and specification. Successful outcomes for new therapies, including gene therapy, aimed at restoring
hair cell function after birth, may depend on restoration of auditory circuits, including mature and functional
hair cell synapses and neuronal fibers. Here we propose to assess how disruption or loss of sensory transduction
in several mouse models affects hair cell function, synaptic maturation and spiral ganglion specification.
Furthermore, we will determine if inner ear gene therapy is capable of reversing any of these observed changes
and identify the conditions for optimal recovery of auditory function. We will combine state-of-the-art
technologies to address these important questions, including high-resolution imaging, electrophysiology, single
cell RNA sequencing and localization of RNA transcripts.

## Key facts

- **NIH application ID:** 10120057
- **Project number:** 2R01DC008853-11A1
- **Recipient organization:** BOSTON CHILDREN'S HOSPITAL
- **Principal Investigator:** Gwenaelle S Geleoc
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2021
- **Award amount:** $575,994
- **Award type:** 2
- **Project period:** 2007-07-01 → 2025-11-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10120057

## Citation

> US National Institutes of Health, RePORTER application 10120057, Functional development of hair cells and neurons in the inner ear (2R01DC008853-11A1). Retrieved via AI Analytics 2026-05-23 from https://api.ai-analytics.org/grant/nih/10120057. Licensed CC0.

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