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Related Concept Videos

Hair Cells01:22

Hair Cells

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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.
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Unrenewable Cells00:50

Unrenewable Cells

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In humans, the photoreceptor cells of the eye and sensory hair cells of the ear lack stem cells. These cells are thus unrenewable and cannot be replaced when they are damaged or destroyed.
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The Cochlea01:13

The Cochlea

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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.
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Hedgehog Signaling Pathway02:33

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The Hedgehog gene (Hh) was first discovered due to its control of the growth of disorganized, hair-like bristles phenotype in Drosophila, much like hedgehog spines. Hh plays a crucial role in the development of organs and the maintenance of homeostasis in both invertebrates and vertebrates. However, while Drosophila has only one Hh protein, mammals have multiple functional Hedgehog proteins - Sonic (Shh), Desert (Dhh), and Indian Hedgehog (Ihh). All of these homologous proteins have adapted to...
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Auditory Pathway01:15

Auditory Pathway

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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...
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Auditory Hair Cell Mechanotransduction Channels Dynamically Shape the Mechanical Properties of Their Membrane Environment.

Advanced science (Weinheim, Baden-Wurttemberg, Germany)·2025
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Infrared light stimulates the cochlea through a mechanical displacement detected and amplified by hair cells.

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Author Correction: LOXHD1 is indispensable for maintaining TMC1 auditory mechanosensitive channels at the site of force transmission.

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SUB-immunogold-SEM reveals nanoscale distribution of submembranous epitopes.

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LOXHD1 is indispensable for maintaining TMC1 auditory mechanosensitive channels at the site of force transmission.

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Related Experiment Video

Updated: Nov 14, 2025

Dextran Labeling and Uptake in Live and Functional Murine Cochlear Hair Cells
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Loxhd1 Mutations Cause Mechanotransduction Defects in Cochlear Hair Cells.

Alix Trouillet1, Katharine K Miller1, Shefin Sam George1

  • 1Department of Otolaryngology-Head and Neck Surgery, School of Medicine, Stanford University, Stanford, California 94305.

The Journal of Neuroscience : the Official Journal of the Society for Neuroscience
|March 12, 2021
PubMed
Summary

The hearing loss gene LOXHD1 is crucial for mechanotransduction in cochlear hair cells after the first week of life. Mutations disrupt sound detection by affecting hair bundle function, not structure.

Keywords:
LOXHD1PLAT domainhair cellmechanotransductionnonsense-associated altered splicingstereocilia

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Area of Science:

  • Otoacoustic Emissions and Auditory Neuroscience
  • Molecular and Cellular Biology of Hearing
  • Genetics of Hearing Loss

Background:

  • Sound detection in the inner ear relies on the mechanical deflection of cochlear hair cell bundles.
  • Tip links (TLs) connect stereocilia in the hair bundle, gating mechanotransduction channels.
  • Mutations in the LOXHD1 gene are associated with human hearing loss (DFNB77).

Purpose of the Study:

  • To investigate the role of the LOXHD1 gene in the auditory mechanotransduction process.
  • To determine the temporal onset and molecular basis of mechanotransduction defects in LOXHD1-mutant mice.

Main Methods:

  • Generation and analysis of two LOXHD1 mouse models with mutations in the PLAT repeat.
  • Electrophysiological recordings of mechanotransduction currents in inner hair cells (IHCs).
  • Immunolocalization studies to assess the localization of LOXHD1 and key TL proteins (Harmonin, LHFPL5).

Main Results:

  • LOXHD1 mutations caused severe mechanotransduction defects in IHCs starting around postnatal day 11.
  • The defect onset correlated with the developmental expression pattern of LOXHD1 in the hair bundle.
  • Hair bundle morphology and TL protein complex integrity (Harmonin, LHFPL5) were preserved in mutants.

Conclusions:

  • LOXHD1 is essential for a critical step in hair bundle development required for mature mechanotransduction.
  • The identified LOXHD1-dependent step is vital for normal hearing function in mice and humans.
  • Mechanotransduction machinery is present but not activatable in LOXHD1-mutant hair cells, indicating a novel functional requirement.