A review of cochlear implantation in mitochondrial sensorineural hearing loss.

@article{Sinnathuray2003ARO,
  title={A review of cochlear implantation in mitochondrial sensorineural hearing loss.},
  author={Arasa Raj Sinnathuray and Vikram Raut and Aziz Awa and Adam Magee and Joseph G Toner},
  journal={Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology},
  year={2003},
  volume={24 3},
  pages={418-26}
}
OBJECTIVE Mitochondrial sensorineural hearing loss (SNHL) may be nonsyndromic (occurring in isolation), associated with the A1555G mutation in the MTRNR1 gene. Mitochondrial SNHL may also be syndromic, associated with the A3243G point mutation in the MTTL1 gene. In syndromic cases-mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS), maternally inherited diabetes and deafness, Kearns-Sayre syndrome, and chronic progressive external ophthalmoplegia-the SNHL compounds… CONTINUE READING

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