title: The quest for restoring hearing: Understanding ear development more completely.
abstract: Neurosensory hearing loss is a growing problem of super-aged societies. Cochlear implants can restore some hearing, but rebuilding a lost hearing organ would be superior. Research has discovered many cellular and molecular steps to develop a hearing organ but translating those insights into hearing organ restoration remains unclear. For example, we cannot make various hair cell types and arrange them into their specific patterns surrounded by the right type of supporting cells in the right numbers. Our overview of the topologically highly organized and functionally diversified cellular mosaic of the mammalian hearing organ highlights what is known and unknown about its development. Following this analysis, we suggest critical steps to guide future attempts toward restoration of a functional organ of Corti. We argue that generating mutant mouse lines that mimic human pathology to fine-tune attempts toward long-term functional restoration are needed to go beyond the hope generated by restoring single hair cells in postnatal sensory epithelia.
authors.affiliation: Department of Biology, CLAS, University of Iowa, Iowa City, IA, USA.
authors.forename: Israt
authors.initials: I
authors.lastname: Jahan
authors.affiliation: Department of Biology, CLAS, University of Iowa, Iowa City, IA, USA.
authors.forename: Ning
authors.initials: N
authors.lastname: Pan
authors.affiliation: Department of Biology, CLAS, University of Iowa, Iowa City, IA, USA.
authors.forename: Karen L
authors.initials: KL
authors.lastname: Elliott
authors.affiliation: Department of Biology, CLAS, University of Iowa, Iowa City, IA, USA.
authors.forename: Bernd
authors.initials: B
authors.lastname: Fritzsch
mesh_terms: D000818:Animals; D051792:Basic Helix-Loop-Helix Proteins / Q000378:metabolism; D004423:Ear / Q000254:growth & development* / Q000502:physiology*; D053263:Gene Regulatory Networks; D006198:Hair Cells, Auditory / Q000502:physiology; D006309:Hearing / Q000502:physiology*; D006801:Humans; D012038:Regeneration
chemical_list: D051792:Basic Helix-Loop-Helix Proteins
keywords: Atoh1; cochlea; development; diffusible factors; expression regulation; lateral inhibition; organ of Corti; patterning