Research NewsHuman Genetics

The Architecture of Hearing

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Science  14 Nov 1997:
Vol. 278, Issue 5341, pp. 1223-1224
DOI: 10.1126/science.278.5341.1223

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In this issue (p. 1315), geneticists report cloning the mutated gene responsible for causing deafness without any other symptoms in many members of a Costa Rican family, bringing the total number of such genes pinpointed in the past year to three. All three appear to be involved in the operation of the sound-sensitive hair cells in the inner ear, or cochlea. Two of them code for proteins that apparently help organize actin, a structural protein that stiffens the microscopic projections that crown these cells; the third helps build an electrical channel that may enable hair cells to reset themselves after they are exposed to sound. All three could offer glimpses into how hair cells normally develop and function, and point to treatments to keep hearing sharp.