Tauroursodeoxycholic acid prevents hearing loss and hair cell death in Cdh23(erl/erl) mice

Neuroscience. 2016 Mar 1:316:311-20. doi: 10.1016/j.neuroscience.2015.12.050. Epub 2015 Dec 31.


Sensorineural hearing loss has long been the subject of experimental and clinical research for many years. The recently identified novel mutation of the Cadherin23 (Cdh23) gene, Cdh23(erl/erl), was proven to be a mouse model of human autosomal recessive nonsyndromic deafness (DFNB12). Tauroursodeoxycholic acid (TUDCA), a taurine-conjugated bile acid, has been used in experimental research and clinical applications related to liver disease, diabetes, neurodegenerative diseases, and other diseases associated with apoptosis. Because hair cell apoptosis was implied to be the cellular mechanism leading to hearing loss in Cdh23(erl/erl) mice (erl mice), this study investigated TUDCA's otoprotective effects in erl mice: preventing hearing impairment and protecting against hair cell death. Our results showed that systemic treatment with TUDCA significantly alleviated hearing loss and suppressed hair cell death in erl mice. Additionally, TUDCA inhibited apoptotic genes and caspase-3 activation in erl mouse cochleae. The data suggest that TUDCA could be a potential therapeutic agent for human DFNB12.

Keywords: Cdh23; apoptosis; hearing loss; otoprotection; tauroursodeoxycholic acid (TUDCA).

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Analysis of Variance
  • Animals
  • Cadherins / genetics*
  • Caspases / genetics
  • Caspases / metabolism
  • Cell Count
  • Cell Death / drug effects
  • Cell Death / genetics
  • Cholagogues and Choleretics / pharmacology
  • Cholagogues and Choleretics / therapeutic use
  • Disease Models, Animal
  • Evoked Potentials, Auditory, Brain Stem / drug effects
  • Hair Cells, Auditory / drug effects
  • Hair Cells, Auditory / pathology*
  • Hearing Loss* / drug therapy
  • Hearing Loss* / genetics
  • Hearing Loss* / pathology
  • Hearing Loss* / prevention & control
  • In Situ Nick-End Labeling
  • Mice
  • Mice, Transgenic
  • Mutation / genetics*
  • Otoacoustic Emissions, Spontaneous
  • RNA, Messenger / metabolism
  • Taurochenodeoxycholic Acid / pharmacology
  • Taurochenodeoxycholic Acid / therapeutic use*


  • Cadherins
  • Cdh23 protein, mouse
  • Cholagogues and Choleretics
  • RNA, Messenger
  • Taurochenodeoxycholic Acid
  • ursodoxicoltaurine
  • Caspases