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5. Mechanics of Vision and Hearing Loss

Gene mutations in Usher syndrome affect the light-sensing rods and cones in the retinas at the back of the eye, triggering retinitis pigmentosa (RP). In RP, the rods and cones slowly begin to fail. This manifests as a loss of peripheral vision first, with central vision loss soon following. Gene mutations also affect the cochlea, which is a spiral structure in the inner ear that transmits sound. The mutations inhibit the cochlea’s function, causing hearing loss.

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