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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...
The Auditory Ossicles01:11

The Auditory Ossicles

The auditory ossicles of the middle ear transmit sounds from the air as vibrations to the fluid-filled cochlea. The auditory ossicles consist of two malleus (hammer) bones, two incus (anvil) bones, and two stapes (stirrups), one on each side. These bones develop during the fetal stage and are the ones to ossify first. They are fully mature at birth and do not grow afterward.
The aptly named stapes look very much like a stirrup. The three ossicles are unique to mammals, and each plays a role in...
Hearing01:31

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.
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.

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Related Experiment Video

Updated: May 23, 2026

Quantitative Assessment of Cortical Auditory-tactile Processing in Children with Disabilities
09:38

Quantitative Assessment of Cortical Auditory-tactile Processing in Children with Disabilities

Published on: January 29, 2014

Auditory function in children with Charcot-Marie-Tooth disease.

Gary Rance1, Monique M Ryan, Kristen Bayliss

  • 1Department of Audiology and Speech Pathology, The University of Melbourne, 550 Swanston Street, Parkville 3010, Australia. grance@unimelb.edu.au

Brain : a Journal of Neurology
|April 24, 2012
PubMed
Summary

Children with inherited neuropathies often have auditory neuropathy, impacting speech processing and everyday listening. This study highlights the need for early detection and intervention for hearing difficulties in these patients.

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Area of Science:

  • Neurology
  • Audiology
  • Genetics

Background:

  • Inherited neuropathies primarily affect peripheral nerves, with cranial nerve involvement, particularly auditory pathways, being less understood.
  • Hearing loss is noted in some children with these conditions but lacks systematic study.
  • Auditory impairment can significantly affect speech development, social interaction, and education.

Purpose of the Study:

  • To investigate auditory pathway function in school-aged children with inherited neuropathies.
  • To assess speech perception and real-world listening abilities in affected children.
  • To determine the prevalence and characteristics of auditory difficulties in this population.

Main Methods:

  • Twenty-six children with genetically confirmed Charcot-Marie-Tooth disease (CMT) were studied.
  • Participants included 18 with demyelinating (CMT1) and 8 with axonal (CMT2) forms.
  • Auditory brainstem responses (ABRs), speech perception, and everyday listening skills were evaluated.

Main Results:

  • All subjects had normal or near-normal sound detection thresholds.
  • Electrophysiological tests revealed auditory neuropathy, characterized by delayed or low-amplitude ABRs in both CMT groups.
  • >60% of CMT1 and >85% of CMT2 patients exhibited impaired auditory temporal processing and/or abnormal speech understanding.

Conclusions:

  • Children with Charcot-Marie-Tooth disease frequently experience auditory neuropathy, even with normal hearing thresholds.
  • Auditory pathway dysfunction significantly impacts speech processing and communication in affected children.
  • Systematic audiological assessment is crucial for early identification and management of hearing deficits in inherited neuropathies.