29 January 2014

Mechanism of inherited hearing loss uncovered

A better understanding of the inner ear may aid future treatment

A team of researchers have made an important discovery. The findings of this discovery could better explain some inherited forms of hearing loss in humans.

A group of proteins in the hair cells of the inner ear were discovered to be crucial for the ear's ability to detect sounds. Furthermore, researchers discovered that mutations in these proteins are associated with inherited forms of hearing loss in humans.

The findings provide researchers with a better understanding of the mechanism of the inner ear and may aid future treatments in relation to inherited hearing loss.

”?Stereocilia' and V-shaped brushes

For a human to hear, sound-induced vibrations are transformed into electrical impulses by hair cells in the inner ear. Microscopic hair-like projections, known as ”?stereocilia' exist on the surface of these hair cells that detect vibrations.

The stereocilia consist of proteins that control the cell division in the hair cell and have the shape of a V-brush. The proteins of the stereocilia define the exact placement of the stereocilia and enable the creation of the V-shaped brushes. Mutations of these proteins are associated with inherited forms of hearing loss in humans.

About the study

The study was carried out by researchers from IRCM in Montréal (Institut de recherches cliniques de Montréal) and was published online in the scientific journal Development Cell.

Researchers have discovered that the proteins of the stereocilia in the inner ear play a central role in the auditory system, which provides a better understanding of inherited hearing loss.



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