Mitochondrial Mutations and Hearing Loss

  • Nathan Fischel-Ghodsian


Mitochondrial-related hearing loss is not uncommon, can be caused by a number of different mutations, and because of the susceptibility of individuals with the 1555 mutation and their maternal relatives to aminoglycosides is important to diagnose. The role of acquired mitochondrial mutations in presbycusis is suggested at this time, but requires further study for confirmation that the that relationship exists is causal. Lastly, the pathophysiological pathways between the mitochondrial mutations and the hearing impairment are not well understood, and experimental approaches using animal models and a dissection of mitochondrial RNA processing and translation are applied to eventually elucidate these pathways.


Hearing Loss Sensorineural Hearing Loss Sensorineural Deafness Mitochondrial Mutation Deaf Individual 
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Copyright information

© Kluwer Academic/Plenum Publishers, New York 2000

Authors and Affiliations

  • Nathan Fischel-Ghodsian
    • 1
  1. 1.Ahmanson Department of Pediatrics, Steven Spielberg Pediatric Research CenterCedars-Sinai Medical Center and UCLA School of MedicineLos AngelesUSA

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