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Related Concept Videos

Hair Cells01:22

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.
Auditory Pathway01:15

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...
The Cochlea01:13

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.
Anatomy of the Ear01:16

Anatomy of the Ear

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...
Unrenewable Cells00:50

Unrenewable Cells

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.
Photoreceptors
The retina is composed of several layers and contains specialized cells called photoreceptors. The photoreceptors (rods and cones) change their membrane potential when stimulated by light energy. There are two types of photoreceptors—rods and cones—which differ in the shape of their outer...

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Related Experiment Video

Updated: May 21, 2026

Isolation and Culture of Primary Cochlear Hair Cells from Neonatal Mice
06:07

Isolation and Culture of Primary Cochlear Hair Cells from Neonatal Mice

Published on: September 15, 2023

Retrocochlear function of the peripheral deafness gene Cacna1d.

Somisetty V Satheesh1, Katrin Kunert, Lukas Rüttiger

  • 1Department of Neurogenetics, Carl von Ossietzky University Oldenburg, 26111 Oldenburg, Germany.

Human Molecular Genetics
|June 9, 2012
PubMed
Summary

Genetic mutations in the deafness gene Cacna1d disrupt auditory brainstem processing. Ablating Ca(v)1.3 channels in mice caused significant volume and cell loss in the auditory brainstem, impacting hearing aid development.

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Last Updated: May 21, 2026

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Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea
07:07

Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea

Published on: February 21, 2016

Area of Science:

  • Neuroscience
  • Genetics
  • Auditory Neuroscience

Background:

  • Hearing impairment is a common sensory deficit, often linked to genetic mutations.
  • Current research primarily focuses on inner ear malfunctions, neglecting other auditory system components.
  • The peripheral deafness gene Cacna1d, encoding the Ca(v)1.3 channel, is implicated in hearing loss.

Purpose of the Study:

  • To investigate the role of the Cacna1d gene and its Ca(v)1.3 channel in the downstream processing of acoustic information within the auditory brainstem.
  • To determine if Ca(v)1.3 plays a role beyond the inner ear in auditory function.
  • To elucidate the impact of Ca(v)1.3 ablation on the structure and function of the superior olivary complex (SOC).

Main Methods:

  • Generation of a conditional Cacna1d-eGFP(flex) mouse allele.
  • Ca(v)1.3 ablation in the auditory brainstem using Egr2::Cre driver mice.
  • Structural assessment of the SOC, including volume and cell counting.
  • Auditory brainstem response (ABR) measurements to assess neural activity.
  • Immunohistochemical analysis to evaluate protein expression (K(v)1.2, K(v)1.1, KCC2, choline acetyltransferase).

Main Results:

  • Significant reduction in the volume (43-47%) and cell number (46-56%) of major SOC nuclei in knockout mice.
  • Abnormal lateral superior olive formation observed as early as postnatal day 4 (P4), indicating a perinatal role.
  • Decreased auditory nerve response amplitudes and increased central auditory processing amplitudes in knockout mice.
  • Reduced expression of K(v)1.2 linked to amplitude changes in the central auditory system.
  • No significant changes in K(v)1.1, KCC2, or choline acetyltransferase expression.

Conclusions:

  • Ca(v)1.3 plays a crucial retrocochlear role in auditory processing, affecting the auditory brainstem.
  • Mutations in deafness genes can impact both sensory cells and neurons within the auditory pathway.
  • The findings suggest that effective hearing aids must also address central auditory processing deficits.