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Related Experiment Video

Updated: May 17, 2026

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells

Published on: July 29, 2016

Inner ear dysfunction in myotonic dystrophy type 1.

D G Balatsouras¹, D Felekis, M Panas

¹ENT Department, Tzanion General Hospital, Piraeus, Greece. dbalats@hotmail.com

Acta Neurologica Scandinavica

|November 6, 2012

Summary

This summary is machine-generated.

Myotonic dystrophy type 1 frequently causes hearing and vestibular issues. Subclinical cochlear damage, detected by otoacoustic emissions, is a universal finding in patients with this condition.

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In Vivo Morphometric Analysis of Human Cranial Nerves Using Magnetic Resonance Imaging in Menière's Disease Ears and Normal Hearing Ears

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Published on: February 21, 2018

Area of Science:

Neurology
Otolaryngology
Genetics

Background:

Myotonic dystrophy type 1 (DM1) is linked to diverse neurological deficits, including auditory and vestibular dysfunction.
Previous auditory investigations in DM1 primarily relied on subjective pure-tone audiometry.
Objective audiological and vestibular assessments are crucial for a comprehensive understanding of DM1's impact.

Purpose of the Study:

To conduct a detailed audiological and vestibular evaluation in patients with DM1.
To investigate the utility of objective methods like transiently evoked otoacoustic emissions (TEOAEs) in detecting subclinical cochlear damage.
To determine the prevalence of auditory and vestibular abnormalities in DM1.

Main Methods:

Studied 24 genetically diagnosed DM1 patients and 21 controls.
Performed audiological tests: pure-tone audiometry, tympanometry, auditory brainstem responses (ABRs), TEOAEs.
Conducted vestibular assessments using electronystagmography.

Main Results:

Hearing impairment was observed in 62.5% of DM1 patients; ABR abnormalities in 37.5%.
All DM1 patients exhibited subclinical cochlear damage, indicated by absent or reduced TEOAEs.
Vestibular hypesthesia occurred in 37.5% of patients, with spontaneous nystagmus in 15.6%.

Conclusions:

Auditory and vestibular abnormalities are common in DM1.
Subclinical cochlear damage is a consistent finding in DM1 patients.
Objective audiological testing, including TEOAEs, is vital for early detection of cochlear dysfunction in DM1.