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Related Concept Videos

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...
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...
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.
ATP Synthase: Mechanism01:48

ATP Synthase: Mechanism

In animals, the mitochondrial F1F0 ATP synthase is the key protein that synthesizes ATP molecules through a complex catalytic mechanism. While the nuclear genome encodes the majority of ATP synthase subunits, the mitochondrial genome encodes some of the enzyme's most critical components. The formation of this multi-subunit enzyme is a complex multi-step process regulated at the level of transcription, translation, and assembly. Defects in one or more of these steps can result in decreased ATP...
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.
Animal Mitochondrial Genetics02:59

Animal Mitochondrial Genetics

Among all the organelles in an animal cell, only mitochondria have their own independent genomes. Animal mitochondrial DNA is a double-stranded, closed-circular molecule with around 20,000 base pairs. Mitochondrial DNA is unique in that one of its two strands, the heavy, or H, -strand is guanine rich, whereas the complementary strand is cytosine rich and called the light, or L, -strand. Compared to nuclear DNA, mitochondrial DNA has a very low percentage of non-coding regions and is marked by...

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Related Experiment Video

Updated: Jun 5, 2026

Assessing Mitochondrial Function in Sciatic Nerve by High-Resolution Respirometry
08:19

Assessing Mitochondrial Function in Sciatic Nerve by High-Resolution Respirometry

Published on: May 5, 2022

The mitochondrial connection in auditory neuropathy.

Anthony T Cacace1, Joaquim M B Pinheiro

  • 1Department of Communication Sciences and Disorders, Wayne State University, Detroit, Mich 48202, USA. cacacea @ wayne.edu

Audiology & Neuro-Otology
|January 27, 2011
PubMed
Summary

Auditory neuropathy (AN), a condition affecting the auditory nerve, is frequently linked to mitochondrial dysfunction. Research indicates AN

Area of Science:

  • Neuroscience
  • Genetics
  • Ophthalmology

Background:

  • Auditory neuropathy (AN) is characterized by the degeneration of the auditory nerve.
  • Mitochondrial dysfunction is increasingly recognized as a contributing factor in neurological disorders.

Purpose of the Study:

  • To explore the direct and indirect links between mitochondrial dysfunction and auditory neuropathy.
  • To consolidate evidence from various studies implicating mitochondrial health in auditory nerve function.

Main Methods:

  • Review of known mitochondrial-based neurological diseases associated with AN.
  • Analysis of conditions affecting mitochondrial dynamics and axonal transport.
  • Examination of neonatal conditions toxic to mitochondria and their impact on auditory mechanisms.

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Morphological and Functional Evaluation of Ribbon Synapses at Specific Frequency Regions of the Mouse Cochlea
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Morphological and Functional Evaluation of Ribbon Synapses at Specific Frequency Regions of the Mouse Cochlea

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Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers
10:31

Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers

Published on: September 29, 2017

Related Experiment Videos

Last Updated: Jun 5, 2026

Assessing Mitochondrial Function in Sciatic Nerve by High-Resolution Respirometry
08:19

Assessing Mitochondrial Function in Sciatic Nerve by High-Resolution Respirometry

Published on: May 5, 2022

Morphological and Functional Evaluation of Ribbon Synapses at Specific Frequency Regions of the Mouse Cochlea
09:54

Morphological and Functional Evaluation of Ribbon Synapses at Specific Frequency Regions of the Mouse Cochlea

Published on: May 10, 2019

Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers
10:31

Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers

Published on: September 29, 2017

  • Integration of data from genetic, temporal bone, biochemical, molecular, behavioral, electroacoustic, and electrophysiological studies.
  • Main Results:

    • Auditory neuropathy is observed in genetic disorders with known mitochondrial defects.
    • Impaired mitochondrial dynamics and axonal transport are associated with AN.
    • Neonatal hyperbilirubinemia, toxic to mitochondria, is linked to AN.
    • Deficiencies in the respiratory chain and oxidative phosphorylation impact peripheral auditory function.

    Conclusions:

    • Mitochondrial dysfunction is a significant factor in the pathogenesis of auditory neuropathy.
    • A comprehensive body of evidence supports the connection between mitochondrial health and auditory nerve integrity.