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The deletions range from 1,000 to 10,000 nucleotides, and the most common deletion is 4,997 nucleotides.Kearns-Sayre syndrome primarily affects the eyes, causing weakness of the eye muscles (ophthalmoplegia) and breakdown of the light-sensing tissue at the back of the eye (retinopathy).Mitochondria are structures within cells that convert the energy from food into a form that cells can use.Each cell contains hundreds to thousands of mitochondria, which are located in the fluid that surrounds the nucleus (the cytoplasm).It typically affects both ears and worsens gradually over time, making it difficult to understand speech and hear other sounds.
Researchers believe that impaired oxidative phosphorylation can lead to cell death in tissues that require large amounts of energy, such as the brain, muscles, and heart.
Changes in mitochondrial DNA are among the best-studied genetic factors associated with age-related hearing loss.
This form of hearing loss develops with age and can begin as early as a person's thirties or forties.
As people age, mitochondrial DNA accumulates damaging mutations, including deletions and other changes.
This damage results from a buildup of harmful molecules called reactive oxygen species, which are byproducts of energy production in mitochondria.