Placode and neural crest origins of congenital deafness in mouse models of Waardenburg-Shah syndrome.

ABSTRACT: Mutations in the human genes encoding the endothelin ligand-receptor pair EDN3 and EDNRB cause Waardenburg-Shah syndrome (WS4), which includes congenital hearing impairment. The current explanation for auditory dysfunction is defective migration of neural crest-derived melanocytes to the inner ear. We explored the role of endothelin signaling in auditory development in mice using neural crest-specific and placode-specific Ednrb mutation plus related genetic resources. On an outbred strain background, we find a normal representation of melanocytes in hearing-impaired mutant mice. Instead, our results in neural crest-specific Ednrb mutants implicate a previously unrecognized role for glial support of synapse assembly between auditory neurons and cochlear hair cell

SUBMITTER: Tan J 

PROVIDER: S-EPMC11762213 | biostudies-literature | 2025 Jan

REPOSITORIES: biostudies-literature