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 13-18, 2020. This
workshop was held 8 times previously and is one of a kind. It offers lectures and laboratory time in a compact
format with a special interest on the auditory and vestibular periphery and mouse genetics. It is an intensive
training opportunity for 20 graduate or medical students, postdocs or investigators wishing to gain experience in
the fundamental biology and the translational potential of the mouse inner ear. The course is generally aimed at
beginners in the field and is structured with classroom lectures in the morning and hands-on laboratory sessions
in the afternoon. Seven organizers and nine invited speakers are ideally qualified to give lectures and
laboratory training in their own fields. 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, optogenetics and viral and
Crispr/Cas9-based gene therapy will be discussed. An otolaryngologist's perspective on patients with hearing loss and
a primer on the culture of deafness will also be presented. Laboratory time will include cochlear and vestibular
dissection for culture or immunolabeling, transduction channel testing and calcium imaging. Confocal microscopy of
fixed or live samples representing normal and mutant tissues will enable participants to observe defects resulting in
hearing loss and circling behavior. Other activities will demonstrate inner ear gene transfer, paint-fill, and
multiple physiological assessments. All lecture slides and a protocol book of all methods introduced 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: Karen Avraham (Tel-Aviv U), Matt Kelley (NIDCD/NIH), Guy Richardson (U of Sussex),
Ronna Hertzano (U of Maryland), Cat Weisz (NIDCD/NIH), Mike Bowl (MRC Harwell), Basile Tarchini
(JAX). Invited speakers: Uli Muller (Johns Hopkins U), Ruth Anne Eatock (U of Chicago), Angelica
Doetzlhofer (Johns Hopkins U), Kevin Ohlemiller (Washington U), Wade Chien (NIDCD/NIH), Mark
Warchol (Washington U), Betsy Driver (NIDCD/NIH), Vidhya Munnamalai (JAX), Leona Gagnon (JAX).