Genetic and pharmacological intervention for treatment / prevention of hearing loss

Cotanche DA Laboratory of Cellular and Molecular Hearing Research Department of Otolaryngology Children’s Hospital Boston Massachusetts, USA 
Department of Otology & Laryngology Harvard Medical School Massachusetts, USA Division of Health Sciences and Technology Harvard-MIT Boston-Cambridge Massachusetts, USA  


Twenty years ago it was first demon-strated that birds could regenerate their cochlear hair cells following noise damage or aminoglycoside treatment. 

An understanding of how this structural and functional regeneration occurred might lead to the development of thera-pies for treatment of sensorineural hear-ing loss in humans. 

Recent experiments have demonstrated that noise exposure and aminoglycoside treatment lead to apoptosis of the hair cells. 

In birds, this programmed cell death induces the adja-cent supporting cells to undergo regeneration to replace the lost hair cells. 

Although hair cells in the mammalian cochlea undergo apoptosis in response to noise damage and ototoxic drug treatment, the supporting cells do not possess the ability to undergo regeneration. 

However, current experiments on genetic manipulation, gene therapy, and stem cell transplantation suggest that regeneration in the mammalian cochlea may eventually be possible and may one day provide a therapeutic tool for hear-ing loss in humans.  

Learning outcomes: The reader should be able to: 

(1) Describe the anatomy of the avian and mammalian cochlea, identify the individual cell types in the organ of Corti, and distinguish major features that participate in hearing function, 
(2) Demonstrate a knowledge of how sound damage and aminoglycoside poisoning induce apoptosis of hair cells in the cochlea, 
(3) Define how hair cell loss in the avian cochlea leads to regeneration of new hair cells and distinguish this from the mammalian cochlea where there is no regeneration fol-lowing damage, and 
(4) Interpret the potential for new approaches, such as genetic manipulation, gene therapy and stem cell transplantation, could provide a therapeutic approach to hair cell loss in the mammalian cochlea.

 Fuente J Commun Disord. 2008; 41(5): 421-443