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Related Concept Videos

Local Anesthetics: Differential Sensitivity of Nerve Fibers01:24

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Local anesthetics (LAs) block the sodium channels of nerve trunks, sensory nerve endings, and neuromuscular junctions. Although LAs can block all kinds of nerves, the sensitivity of nerve fibers differs according to nerve types and structures. LAs are known to block myelinated fibers faster than unmyelinated ones. Also, they block pain or sensory neurons at low concentrations without affecting the motor neurons involved in muscle contractions. This helps relieve labor pain without affecting the...
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Peripheral thermosensation is the perception of external temperature. A change in temperature (on the surface of the skin and other tissues) is detected by a family of temperature-sensitive ion channels called Transient Receptor Potential, or TRP, receptors. These receptors are located on free nerve endings. Those detecting cold temperatures are closer to the surface of the skin than the nerve endings detecting warmth. These thermoTRP channels, while temperature selective, have relatively...
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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.
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Hair Cells01:22

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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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Hearing01:31

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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.
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Updated: Mar 27, 2026

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
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Temperature sensitive auditory neuropathy.

Qiujing Zhang1, Lan Lan1, Wei Shi1

  • 1Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China.

Hearing Research
|January 19, 2016
PubMed
Summary
This summary is machine-generated.

This study identifies otoferlin (OTOF) gene mutations as a cause of temperature-sensitive auditory neuropathy in children. Hearing loss associated with fever may improve with age or cochlear implants.

Keywords:
Auditory neuropathyCochlear implantOTOFOtoferlinTemperature-sensitive

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Area of Science:

  • Genetics
  • Otolaryngology
  • Pediatrics

Background:

  • Auditory neuropathy is a rare disorder affecting hearing.
  • Temperature-sensitive auditory neuropathy presents with fluctuating hearing loss during fever.

Observation:

  • Three children aged 2-6 experienced severe hearing loss with fever.
  • Hearing thresholds varied daily; two patients showed age-related improvement.
  • One patient benefited from a cochlear implant.

Findings:

  • Genetic analysis revealed otoferlin (OTOF) mutations in all patients.
  • Specific mutations identified include c.2975_2978delAG/c.4819C>T, c.4819C>T/c.4819C>T, and c.2382_2383delC/c.1621G>A.
  • OTOF mutations are strongly suggested as the cause of this condition.

Implications:

  • Otoferlin mutations may be the primary cause of temperature-sensitive auditory neuropathy.
  • Hearing loss in these patients may improve over time.
  • Cochlear implantation is a viable treatment option for severe cases.