Mice as Models for Human Hereditary Deafness

Author(s):  
Karen P. Steel ◽  
Alexandra Erven ◽  
Amy E. Kiernan
Keyword(s):  
2021 ◽  
Author(s):  
Daniel R. Romano ◽  
Eri Hashino ◽  
Rick F. Nelson

AbstractSensorineural hearing loss (SNHL) is a major cause of functional disability in both the developed and developing world. While hearing aids and cochlear implants provide significant benefit to many with SNHL, neither targets the cellular and molecular dysfunction that ultimately underlies SNHL. The successful development of more targeted approaches, such as growth factor, stem cell, and gene therapies, will require a yet deeper understanding of the underlying molecular mechanisms of human hearing and deafness. Unfortunately, the human inner ear cannot be biopsied without causing significant, irreversible damage to the hearing or balance organ. Thus, much of our current understanding of the cellular and molecular biology of human deafness, and of the human auditory system more broadly, has been inferred from observational and experimental studies in animal models, each of which has its own advantages and limitations. In 2013, researchers described a protocol for the generation of inner ear organoids from pluripotent stem cells (PSCs), which could serve as scalable, high-fidelity alternatives to animal models. Here, we discuss the advantages and limitations of conventional models of the human auditory system, describe the generation and characteristics of PSC-derived inner ear organoids, and discuss several strategies and recent attempts to model hereditary deafness in vitro. Finally, we suggest and discuss several focus areas for the further, intensive characterization of inner ear organoids and discuss the translational applications of these novel models of the human inner ear.


ORL ◽  
1977 ◽  
Vol 39 (4) ◽  
pp. 241-246 ◽  
Author(s):  
Hans H. Elverland ◽  
Iain W.S. Mair ◽  
Olav Borud
Keyword(s):  

2014 ◽  
Vol 03 (02) ◽  
pp. 1-10
Author(s):  
Mahmoud Eltayeb Koko Musa ◽  
Yousuf Hasan Yousuf Bakhit ◽  
Ashraf Yahia Osman Mohamed ◽  
Asaad Tageldein Idris ◽  
Ashraf Ahmed Mohamed Ahmed Fadul ◽  
...  

2012 ◽  
Vol 48 (4) ◽  
pp. 455-462 ◽  
Author(s):  
R. A. Zinchenko ◽  
A. A. Osetrova ◽  
E. I. Sharonova

2015 ◽  
Vol 24 (13) ◽  
pp. 3651-3661 ◽  
Author(s):  
Takashi Iizuka ◽  
Kazusaku Kamiya ◽  
Satoru Gotoh ◽  
Yoshinobu Sugitani ◽  
Masaaki Suzuki ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document