Progress in Modeling and Targeting Inner Ear Disorders with Pluripotent Stem Cells

Pei Ciao Tang, Eri Hashino, Rick F. Nelson

Research output: Contribution to journalReview articlepeer-review

13 Scopus citations


Sensorineural hearing loss and vestibular dysfunction are caused by damage to neurons and mechanosensitive hair cells, which do not regenerate to any clinically relevant extent in humans. Several protocols have been devised to direct pluripotent stem cells (PSCs) into inner ear hair cells and neurons, which display many properties of their native counterparts. The efficiency, reproducibility, and scalability of these protocols are enhanced by incorporating knowledge of inner ear development. Modeling human diseases in vitro through genetic manipulation of PSCs is already feasible, thereby permitting the elucidation of mechanistic understandings of a wide array of disease etiologies. Early studies on transplantation of PSC-derived otic progenitors have been successful in certain animal models, yet restoration of function and long-term cell survival remain unrealized. Through further research, PSC-based approaches will continue to revolutionize our understanding of inner ear biology and contribute to the development of therapeutic treatments for inner ear disorders.

Original languageEnglish (US)
Pages (from-to)996-1008
Number of pages13
JournalStem Cell Reports
Issue number6
StatePublished - Jun 9 2020
Externally publishedYes


  • disease modeling
  • inner ear
  • organoid
  • stem cells
  • therapy

ASJC Scopus subject areas

  • Biochemistry
  • Genetics
  • Developmental Biology
  • Cell Biology


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