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Related Concept Videos

The Cochlea01:13

The Cochlea

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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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Nuclear Overhauser Enhancement (NOE)01:07

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Irradiation of a spin-active nucleus causes an increase or decrease in the signal intensity of neighboring nuclei that are not necessarily chemically bonded or involved in J-coupling.  This phenomenon, called the Nuclear Overhauser Enhancement (NOE), results from through-space interactions between the nuclear spins. The NOE effect decreases with increasing internuclear distance and is generally not observed beyond 4 angstroms. In NOE, dipole-dipole interactions between neighboring...
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Auditory Pathway01:15

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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.
When viewed cross-sectionally, the cochlea reveals the scala vestibuli and scala tympani flanking...
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Anatomy of the Ear01:16

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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...
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Related Experiment Video

Updated: Jun 6, 2025

In Vivo Morphometric Analysis of Human Cranial Nerves Using Magnetic Resonance Imaging in Menière's Disease Ears and Normal Hearing Ears
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Abnormal cochlear enhancement in Norrie disease.

Emil Jernstedt Barkovich1, Suely Fazio Ferraciolli1, Camilo Jaimes1

  • 1Department of Radiology, Massachusetts General Hospital, USA.

The Neuroradiology Journal
|November 24, 2024
PubMed
Summary

Norrie disease (ND) can cause hearing loss. Brain MRI revealed cochlear enhancement in a child with ND, suggesting this may precede hearing changes and aid in early detection.

Keywords:
Norrie diseaseX-linked blindnesscochlear enhancementgenetic hearing losspediatric sensorineural hearing lossstria vascularis

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Area of Science:

  • Ophthalmology
  • Genetics
  • Neurology

Background:

  • Norrie disease (ND) is a rare X-linked disorder.
  • It is characterized by retinal and cochlear vascular abnormalities.
  • Clinical manifestations include congenital blindness and progressive hearing loss.

Purpose of the Study:

  • To report a novel finding of bilateral cochlear enhancement on MRI in a child with ND.
  • To investigate the potential of cochlear enhancement as an early indicator of hearing changes in ND.
  • To explore the utility of cochlear enhancement in the context of emerging ND gene therapies.

Main Methods:

  • Case presentation of a 3-year-old child with ND.
  • Brain MRI with gadolinium contrast was performed.
  • Review of ND mouse models exhibiting stria vascularis degeneration.

Main Results:

  • Bilateral cochlear enhancement was observed on brain MRI in the patient.
  • This finding was present in a patient with normal hearing.
  • ND mouse models demonstrate progressive degeneration of the stria vascularis (SV).

Conclusions:

  • Cochlear enhancement on MRI may precede hearing loss in Norrie disease.
  • Temporal bone/internal auditory canal MRI should be considered in ND evaluation.
  • This finding could assist in candidate selection and assessing treatment effects for ND gene therapy.