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Related Concept Videos

Auditory Pathway01:15

Auditory Pathway

6.8K
Auditory pathways constitute the complex neural circuits responsible for transmitting and interpreting auditory information from the peripheral auditory system to the brain. Sound waves are initially captured by the outer ear, funneled through the ear canal, and reach the tympanic membrane (eardrum). These vibrations are transmitted via the middle ear's ossicles to the inner ear's cochlea.
When viewed cross-sectionally, the cochlea reveals the scala vestibuli and scala tympani flanking...
6.8K
The Cochlea01:13

The Cochlea

49.9K
The cochlea is a coiled structure in the inner ear that contains hair cells—the sensory receptors of the auditory system. Sound waves are transmitted to the cochlea by small bones attached to the eardrum called the ossicles, which vibrate the oval window that leads to the inner ear. This causes fluid in the chambers of the cochlea to move, vibrating the basilar membrane.
49.9K
Hair Cells01:22

Hair Cells

44.0K
Hair cells are the sensory receptors of the auditory system—they transduce mechanical sound waves into electrical energy that the nervous system can understand. Hair cells are located in the organ of Corti within the cochlea of the inner ear, between the basilar and tectorial membranes. The actual sensory receptors are called inner hair cells. The outer hair cells serve other functions, such as sound amplification in the cochlea, and are not discussed in detail here.
44.0K
Hearing01:31

Hearing

56.2K
When we hear a sound, our nervous system is detecting sound waves—pressure waves of mechanical energy traveling through a medium. The frequency of the wave is perceived as pitch, while the amplitude is perceived as loudness.
56.2K
Anatomy of the Ear01:16

Anatomy of the Ear

10.8K
Auditory sensation, commonly called hearing, involves the transformation of sonic waves into neural impulses facilitated by the structures of the auditory organ. The prominent, flesh-like structure on the side of the head, called the auricle, directs sound waves towards the auditory canal. The auricle is often mislabeled as the pinna, a term more aligned with mobile structures like a feline's external ear. The auditory canal penetrates the cranium via the external auditory meatus of the...
10.8K
Auditory Perception01:17

Auditory Perception

903
The auditory system is essential for sound perception, utilizing various critical structures. When sound waves enter the outer ear, they travel through the ear canal and cause the eardrum to vibrate. These vibrations are then transmitted to the middle ear, where three tiny bones – the malleus, incus, and stapes – amplify the sound. This amplification is crucial, as it ensures that the sound vibrations are strong enough to be conveyed to the inner ear. These vibrations then reach the...
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Genetic studies identify known and novel variants for recessively inherited moderate to severe hearing loss in consanguineous families from Pakistan.

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Lifelong dynamic maintenance of stereocilia bundles in mammalian auditory hair cells.

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Single-molecule fluorescence microscopy reveals regulatory mechanisms of MYO7A-driven cargo transport in stereocilia of live inner ear hair cells.

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Related Experiment Video

Updated: Dec 20, 2025

Dextran Labeling and Uptake in Live and Functional Murine Cochlear Hair Cells
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Dextran Labeling and Uptake in Live and Functional Murine Cochlear Hair Cells

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Myosins and Hearing.

Thomas B Friedman1, Inna A Belyantseva2, Gregory I Frolenkov3

  • 1Laboratory of Molecular Genetics, National Institute on Deafness and Other Communication Disorders, Porter Neuroscience Research Center, National Institutes of Health, Bethesda, MD, USA. friedman@nidcd.nih.gov.

Advances in Experimental Medicine and Biology
|May 27, 2020
PubMed
Summary
This summary is machine-generated.

Genetic variants in six myosin genes are linked to hereditary hearing loss. Research confirms their role in inner ear function and deafness, supported by animal models and cellular studies.

Keywords:
DeafnessHair cellsHarmoninHearingHelio gene-gunMYH14MYH9MYO15AMYO3AMYO6MYO7ARetinitis pigmentosaShaker 2 mouseStereociliaUsher syndromeWhirler mouse

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Author Spotlight: Unraveling the Role of Myosin-7a and Usher Proteins in Hearing and Human Disease
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Author Spotlight: Unraveling the Role of Myosin-7a and Usher Proteins in Hearing and Human Disease

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Dissection of the Auditory Bulla in Postnatal Mice: Isolation of the Middle Ear Bones and Histological Analysis
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Last Updated: Dec 20, 2025

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Author Spotlight: Unraveling the Role of Myosin-7a and Usher Proteins in Hearing and Human Disease
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Dissection of the Auditory Bulla in Postnatal Mice: Isolation of the Middle Ear Bones and Histological Analysis
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Area of Science:

  • Genetics
  • Neuroscience
  • Otolaryngology

Background:

  • Hearing loss is a common neurosensory disorder with diverse genetic causes.
  • Over a hundred genes are implicated in hereditary deafness, with a significant portion encoding myosin proteins.

Purpose of the Study:

  • To review the evidence linking specific myosin genes to hearing loss.
  • To focus on six key myosin genes with substantial evidence for a causative role in deafness.

Main Methods:

  • Review of genetic, clinical, and animal model data.
  • Analysis of biochemical, physiological, and cell biological studies on inner ear function.

Main Results:

  • Pathogenic variants in MYO3A, MYO6, MYO7A, MYO15A, MYH14, and MYH9 are definitively or moderately associated with human deafness.
  • Animal models (mouse, zebrafish) with mutations in these genes exhibit hearing loss.
  • Studies demonstrate the crucial roles of these myosins in inner ear development and function.

Conclusions:

  • Six specific myosin genes (MYO3A, MYO6, MYO7A, MYO15A, MYH14, MYH9) are substantial contributors to hereditary hearing loss.
  • The collective evidence from human genetics, animal models, and cellular studies strongly supports their role in normal hearing.