Gene therapy corrects stereocilia defects in the inner ears of mice with inherited deafness

Friday, October 23, 2015

For the first time, scientists have used gene therapy to correct defective structures in the inner ears of newborn mice, according to results of a new study by researchers from the National Institute on Deafness and Other Communication Disorders (NIDCD), part of the National Institutes of Health. The mice had a type of hereditary deafness also found in humans.

Hair cells are small sensory cells of the inner ear that transform sound vibrations into nerve impulses. After sound waves enter the inner ear’s cochlea, they produce waves in a fluid layer. The waves lift the overlying hair cells, and bend tiny hair-like structures on the hair cell surfaces—called stereocilia—by driving them into an overlying membrane. Bending the stereocilia triggers an electrical signal that is sent to the brain and interpreted as sound.