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Hearing

Hearing

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.

Gap Junctions

Gap Junctions

Multicellular organisms employ a variety of ways for cells to communicate with each other. Gap junctions are specialized proteins that form pores between neighboring cells in animals, connecting the cytoplasm between the two, and allowing for the exchange of molecules and ions. They are found in a wide range of invertebrate and vertebrate species, mediate numerous functions including cell differentiation and development, and are associated with numerous human diseases, including cardiac and...

Gap Junctions

Gap Junctions

The cytoplasm of adjacent animal cells can exchange small molecules, ions, and secondary messengers via the communication channels which form the gap junctions. These junctions comprise a few hundred to thousands of molecular channels, each made of two halves, called the connexon hemichannel. A connexon is a hexamer of six transmembrane connexin proteins, which assemble radially, thus forming a pore or channel in the center. One connexon hemichannel docks with a corresponding connexon on the...

Auditory Pathway

Auditory Pathway

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 the...

Hair Cells

Hair Cells

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.

The Cochlea

The Cochlea

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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Related Experiment Video

Updated: May 15, 2026

Mechanical Stimulation-induced Calcium Wave Propagation in Cell Monolayers: The Example of Bovine Corneal Endothelial Cells

Mechanical Stimulation-induced Calcium Wave Propagation in Cell Monolayers: The Example of Bovine Corneal Endothelial Cells

Published on: July 16, 2013

Hearing is normal without connexin30.

Anne-Cécile Boulay¹, Francisco J del Castillo, Fabrice Giraudet

¹Collège de France, Centre Interdisciplinaire de Recherche en Biologie (CIRB)/Centre National de la Recherche Scientifique (CNRS), Unité Mixte de Recherche (UMR) 7241/Institut National de la Santé et de la Recherche Médicale (Inserm), U1050, 75231 Paris cedex 05, France.

The Journal of Neuroscience : the Official Journal of the Society for Neuroscience

|January 11, 2013

Summary

This summary is machine-generated.

Connexin30 (Cx30) is not essential for hearing. Studies show connexin26 (Cx26) downregulation, not Cx30 absence, causes deafness linked to GJB6 deletions.

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Last Updated: May 15, 2026

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Area of Science:

Genetics
Otolaryngology
Molecular Biology

Background:

GJB2 and GJB6 genes encode connexin26 (Cx26) and connexin30 (Cx30), crucial for inner ear function.
Mutations in these genes are linked to DFNB1, a common form of hereditary deafness.
The precise role of Cx30 in hearing remains unclear due to its regulation of Cx26.

Purpose of the Study:

To investigate the independent role of Cx30 in auditory function.
To develop a Cx30 knock-out mouse model preserving partial Cx26 expression.

Main Methods:

Generation of a novel Cx30 knock-out mouse model (Cx30(Δ/Δ)).
Assessment of hearing in Cx30(Δ/Δ) mutants.
Analysis of Cx26 expression levels in relation to Cx30.

Main Results:

Cx30 and Cx26 expression are coordinately regulated, influenced by genomic spacing.
Cx30(Δ/Δ) mice exhibited normal hearing.
Cx26 expression was partially preserved in the Cx30(Δ/Δ) model.

Conclusions:

Cx30 is dispensable for normal cochlear function.
Defective Cx26 expression, rather than Cx30 deficiency, is the primary cause of deafness in GJB6-related DFNB1.
This challenges previous assumptions about Cx30's necessity in hearing.