Advances in inner ear gene therapy: exploring cochlear protection and regeneration.
OBJECTIVE
To review the application of gene therapy in the inner ear. Gene delivery to the inner ear was first reported in 1996. Since then the field has progressed on multiple fronts.


BACKGROUND
More diverse and sophisticated vectors are improving the efficiency of delivery to the inner ear. Research is transitioning from the delivery of marker genes to the delivery of therapeutic genes in animal models of inner ear disease. Three distinct areas of research are developing: (1) delivery of genes for protection of spiral ganglion neurons with potential application in cochlear implantation, (2) delivery of genes for protection of hair cells and hearing preservation in degenerative diseases and cochlear insults and (3) the use of gene therapy to transform cells from one phenotype to another and replace lost cells, potentially restoring lost function.


CONCLUSIONS
Currently, no specific drugs are targeted at inner ear disease. The use of gene therapy in the inner ear is being applied in animal models of ototoxicity and ischemia reperfusion injury. Gene therapy can protect the inner ear from damage and even restore function through the regeneration of hair cells.
Maiorana
Carrie R
CR
Department of Otolaryngology, University of Kansas, Kansas City, KS 66160, USA.
Staecker
Hinrich
H
D000818:Animals; D003051:Cochlea / Q000502:physiology*; D007758:Ear, Inner / Q000502:physiology*; D015316:Genetic Therapy / Q000379:methods*; D006801:Humans; D007759:Labyrinth Diseases / Q000628:therapy