PROJECT SUMMARY
This application seeks partial support for “Modeling Hearing and Balance Disorders in Mice: The HEar@JAX
Workshop”, to be held at The Jackson Laboratory (JAX) in Bar Harbor, ME, on September 15-20, 2024. This
recurring biennial workshop is one of a kind as it 1) specifically focuses on the use of the mouse model to
investigate fundamental and translational questions about hearing and balance, and 2) has a compact 5-day
format with morning lectures and afternoon laboratory time. The course is an intensive training opportunity for
20 graduate or medical students, postdocs or investigators who are either new to the inner ear field, or new to
the mouse model. Six organizers and eight invited speakers are ideally qualified to give lectures and laboratory
training in their own fields to broadly cover inner ear research, in particular the periphery. The lectures will address
human and mouse genetics-based gene discovery, mouse models of hearing loss and balance disorders, development
and regeneration of the inner ear, mechanotransduction, afferent and efferent innervation, brainstem function, noise-
and age-related hearing loss, ototoxicity and synaptopathy. Modern techniques including single cell approaches and
viral and CRISPR/Cas9-based gene therapy will be discussed. An otolaryngologist’s perspective on patients with
hearing loss will also be presented. Laboratory time will include cochlear dissections for immunolabeling or for organ
culture. Live cultures will be used for calcium imaging, antibiotic damage and to test transduction channel function.
Confocal microscopy of fixed or live samples representing normal and mutant tissues will enable participants to observe
defects resulting in hearing loss and vestibular disorders. Other activities will include inner ear gene transfer,
paint-fill, as well as practical and interactive tutorials for physiological testing (e.g., ABR, DPOAE) and to navigate the
gEAR database. All lecture slides and protocols introduced during the course will be made available to
participants to maximize the benefits of attending the course. A hallmark of this workshop is the close interaction
between participants and faculty at the Highseas Conference Center, which also serves as the residence and
dining facility. Sharing meals and other social activities encourages network development and fosters
collaboration. To enhance their visibility, participants will have the opportunity to present a poster about their
own research.
Course organizers: Matt Kelley (NIH/NIDCD; Chair), Guy Richardson (U of Sussex), Ronna Hertzano (U of
Maryland, NIH/NIDCD), Cat Weisz (NIH/NIDCD), Mike Bowl (U College London), Basile Tarchini (The Jackson
Laboratory, R13 PI). Invited speakers: Radha Kalluri (U of Southern California), Hela Azaiez (U of Iowa), Gwenaelle
Geleoc (Harvard U), Ulrich Mueller (Johns Hopkins U), Lisa Olson (Columbia U), Betsy Driver (NIH/NIDCD),
Vidhya Munnamalai (The Jackson Laboratory), Ksenia Gnedeva (U of Southern California).