The quest for restoring hearing: Understanding ear development more completely

Bioessays. 2015 Sep;37(9):1016-27. doi: 10.1002/bies.201500044. Epub 2015 Jul 24.

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.

Keywords: Atoh1; cochlea; development; diffusible factors; expression regulation; lateral inhibition; organ of Corti; patterning.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Review

MeSH terms

  • Animals
  • Basic Helix-Loop-Helix Transcription Factors / metabolism
  • Ear / growth & development*
  • Ear / physiology*
  • Gene Regulatory Networks
  • Hair Cells, Auditory / physiology
  • Hearing / physiology*
  • Humans
  • Regeneration

Substances

  • Basic Helix-Loop-Helix Transcription Factors