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Related Experiment Video

Updated: Jun 15, 2025

Modified Experimental Conditions for Noise-Induced Hearing Loss in Mice and Assessment of Hearing Function and Outer Hair Cell Damage
07:13

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Hidden hearing loss in a Charcot-Marie-Tooth type 1A mouse model.

Luis R Cassinotti, Lingchao Ji, M Caroline Yuk

    JCI Insight
    |August 23, 2024
    PubMed
    Summary

    Hidden hearing loss (HHL) can be caused by auditory nerve demyelination, not just inner hair cell issues. Charcot-Marie-Tooth type 1A mice show HHL, suggesting peripheral neuropathy patients may experience hearing difficulties.

    Keywords:
    Demyelinating disordersNeuroscienceOtology

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    Area of Science:

    • Neuroscience
    • Auditory Neuroscience
    • Otolaryngology

    Background:

    • Hidden hearing loss (HHL) is characterized by normal hearing thresholds but impaired auditory nerve function.
    • While inner hair cell (IHC) synaptopathy is a known cause, auditory nerve (AN) demyelination is an emerging factor.
    • Charcot-Marie-Tooth type 1A (CMT1A) is a prevalent hereditary peripheral neuropathy.

    Purpose of the Study:

    • To investigate the role of peripheral myelinopathy in causing HHL using a CMT1A mouse model.
    • To examine the functional and structural changes in the auditory nerve associated with CMT1A.
    • To explore the potential for CMT1A patients to serve as a model for studying HHL.

    Main Methods:

    • Utilized a mouse model of Charcot-Marie-Tooth type 1A (CMT1A).
    • Assessed auditory function through electrophysiological recordings, including sound-evoked cochlear compound action potentials.
    • Examined the structure of the auditory nerve, focusing on myelination and heminodes near inner hair cells.

    Main Results:

    • CMT1A mice displayed functional characteristics of HHL, including reduced auditory nerve responses.
    • Observed disorganization of auditory nerve heminodes and minor fiber loss near inner hair cells.
    • These findings support demyelination as a cause of HHL and link heminodal defects to altered auditory potentials.

    Conclusions:

    • Mild disruptions in auditory nerve myelination, as seen in CMT1A, can lead to hidden hearing loss.
    • Patients with CMT1A and similar peripheral neuropathies are likely to experience HHL.
    • Studying hearing in CMT1A patients may aid in developing clinical diagnostic tests for HHL.