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That will let us link specific mutations to specific disruptions in the ear that cause hearing loss. The long-term goal is to try to manipulate or replace those genes in order to overcome those problems. Individual mutations might disrupt the work of a cascade of genes that cooperate to construct certain nerves or sections of the inner ear that sense sound; that stimulation is then turned into electrical impulses that go to the hearing center of the brain. Understanding how a specific mutation disrupts that cascade might lead to ways to repair the damage, Zuo said. For example, one type of damage that occurs in children whose hearing is damaged by chemotherapy is loss of hair cells in the inner part of the ear.
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These cells sprout extremely fine hairs that move in response to sound waves and set up the electrical impulse that goes into the brain. These are the same cells damaged in adults by prolonged exposure to loud noise. In the future we'd like to be able to prevent the loss of these hair cells or trigger their re-growth with some form of pharmacological or gene therapy intervention, Zuo said. Other authors of this study include Mohammad Habiby Kermany, Lisan Parker and Yun-Kai Guo (St. Jude); Darla Miller (Oak Ridge National Laboratory; Oak Ridge, Tenn. ); and Douglas Swanson, Tai-June Yoo and Dan Goldowitz (University of Tennessee Health Science Center, Memphis).
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