( iments performed by Purdy at the Advan...)Scientists identify molecular cause for one form of deafness

"As the ability of espin to cross-link F-actin is decreased by using genetically modified 'deafness' mutants with progressively more damaged actin binding sites, the structure changes from a well-ordered crystalline array of filaments to a nematic, liquid crystal-like state," said Wong, who also is a researcher at the Frederick Seitz Materials Research Laboratory on campus and at the university's Beckman Institute for Advanced Science and Technology.

In the liquid crystalline state, the bundles maintain their orientation order ?that is, they point roughly along the same direction ?but lose their positional order. These nematic liquid crystals are commonly used in watch displays and laptop displays.

Wong and his colleagues also found that a mixture of mutant espin and normal espin would prevent the structural transition from occurring. If gene expression could turn on the production of just a fraction of normal espin linkers, a kind of rescue attempt at restoring hearing could, in principle, be made.

"We have identified the underlying molecular cause for one form of deafness, and we have identified a mechanism to potentially 'rescue' this particular kind of pathology," Wong said. "Even so, this is really the first step. This work has relevance to not just human hearing, but also to artificial sensors."