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Related Experiment Videos

Cochlear dysfunction in patients with mitochondrial myopathy.

Stavros Korres1, Dimitrios Balatsouras, Panagiota Manta

  • 1ENT Clinic of Athens National University, Ippokration Hospital, 23 Achaion Street, Agia Paraskevi, GR-18536 Athens, Greece. dbalats@hotmail.com

ORL; Journal for Oto-Rhino-Laryngology and Its Related Specialties
|November 6, 2002
PubMed
Summary

Mitochondrial myopathy patients often show subclinical cochlear dysfunction, detected by absent otoacoustic emissions (OAE) despite normal hearing. OAEs may aid in monitoring cochlear health in these individuals.

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Area of Science:

  • Otolaryngology
  • Neurology
  • Genetics

Background:

  • Mitochondrial myopathy is a group of inherited disorders affecting muscle energy production.
  • Subclinical cochlear involvement in mitochondrial myopathy is not well understood.
  • Otoacoustic emissions (OAEs) are objective measures of outer hair cell function in the cochlea.

Purpose of the Study:

  • To investigate cochlear function in patients with mitochondrial myopathy.
  • To assess the utility of otoacoustic emissions in detecting subclinical cochlear dysfunction.

Main Methods:

  • Audiological evaluation including pure tone audiometry and transiently evoked otoacoustic emissions (TEOAEs).
  • Neurological and ENT examinations.
  • Histological confirmation of diagnosis in most patients.

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Main Results:

  • Most patients with mitochondrial myopathy had absent TEOAEs despite normal pure tone hearing thresholds.
  • This indicates subclinical cochlear dysfunction.
  • Control subjects exhibited normal TEOAE responses.

Conclusions:

  • Subclinical cochlear involvement is common in mitochondrial myopathy.
  • The cochlea's high metabolic rate may predispose it to damage.
  • Otoacoustic emissions are a valuable tool for clinical assessment and monitoring of cochlear function in mitochondrial myopathy patients.