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Related Concept Videos

Alterations in Muscle Tone lll01:11

Alterations in Muscle Tone lll

Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...
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The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...
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Related Experiment Video

Updated: May 30, 2026

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
09:39

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Published on: July 29, 2016

Early subclinical cochlear dysfunction in myotonic dystrophy type 1.

V Pisani1, A Tirabasso, S Mazzone

  • 1Department of Neurosciences, University of Rome "Tor Vergata", Italy. valerio.pisani@uniroma2.it

European Journal of Neurology
|July 23, 2011
PubMed
Summary
This summary is machine-generated.

Myotonic dystrophy type 1 patients show cochlear dysfunction, even without hearing loss. Transient-evoked otoacoustic emissions (TEOAE) can detect early signs of hearing impairment in DM1.

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Area of Science:

  • Otoacoustic emissions
  • Audiology
  • Genetics

Background:

  • Myotonic dystrophy type 1 (DM1) is an inherited disorder with varied symptoms.
  • Precocious presbyacusis (early hearing loss) is a known feature of DM1, but its cause is unclear.
  • Outer hair cell (OHC) function in the cochlea is vital for hearing and has not been studied in DM1.

Purpose of the Study:

  • To investigate outer hair cell (OHC) status in DM1 patients using transient-evoked otoacoustic emissions (TEOAE).
  • To assess cochlear function in DM1 patients, including those without audiometrically detected hearing loss.

Main Methods:

  • Recruited 25 DM1 patients and 28 age-matched controls.
  • Performed routine audiological evaluation and TEOAE recordings on all participants.

Main Results:

  • DM1 patients exhibited a high prevalence of sensorineural high-frequency hearing loss (HFHL).
  • TEOAE recordings revealed impaired cochlear function in DM1 patients, both with (HFHL+) and without (HFHL-) hearing loss.
  • Cochlear dysfunction extended to lower frequencies in DM1 patients with HFHL compared to those without.

Conclusions:

  • Cochlear impairment occurs in DM1, even before standard audiometry detects hearing loss.
  • TEOAE assessment is valuable for identifying early cochlear dysfunction in DM1 patients.
  • This suggests TEOAE can aid in managing precocious presbyacusis in DM1.