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Related Concept Videos

Hearing01:31

Hearing

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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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Auditory Pathway01:15

Auditory Pathway

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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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Unrenewable Cells00:50

Unrenewable Cells

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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...
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Pleiotropy01:33

Pleiotropy

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Pleiotropy is the phenomenon in which a single gene impacts multiple, seemingly unrelated phenotypic traits. For example, defects in the SOX10 gene cause Waardenburg Syndrome Type 4, or WS4, which can cause defects in pigmentation, hearing impairments, and an absence of intestinal contractions necessary for elimination. This diversity of phenotypes results from the expression pattern of SOX10 in early embryonic and fetal development. SOX10 is found in neural crest cells that form melanocytes,...
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The Auditory Ossicles01:11

The Auditory Ossicles

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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...
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Heart Sounds01:15

Heart Sounds

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Heart sounds are generated by the turbulence in blood flow due to the closing of heart valves. These sounds are best perceived slightly away from the valves, where the blood flow disseminates the sound.
Auscultation is the process of listening to these internal body sounds using a stethoscope. The heart produces four types of sounds, but only two—S1 and S2—can usually be heard with a stethoscope.
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Related Experiment Video

Updated: Jul 15, 2025

Behavioral Assessment of Hearing in 2 to 4 Year-old Children: A Two-interval, Observer-based Procedure Using Conditioned Play-based Responses
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Behavioral Assessment of Hearing in 2 to 4 Year-old Children: A Two-interval, Observer-based Procedure Using Conditioned Play-based Responses

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Syndromic Hearing Loss in Children.

Martin Lewis1, Caroline D Robson2, Felice D'Arco1

  • 1Department of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond St. London, London, WC1N3JH, UK.

Neuroimaging Clinics of North America
|September 23, 2023
PubMed
Summary

Recognizing specific temporal bone imaging patterns linked to other organ issues aids in diagnosing syndromic hearing loss. This review guides radiologists in identifying these radiological phenotypes for accurate diagnosis.

Keywords:
Branchiootorenal syndromeCHARGEDeafnessPendred syndromeSensorineural hearing loss (SNHL)Syndromic hearing loss

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Simple Surgical Induction of Conductive Hearing Loss with Verification Using Otoscope Visualization and Behavioral Clap Startle Response in Rat
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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

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Related Experiment Videos

Last Updated: Jul 15, 2025

Behavioral Assessment of Hearing in 2 to 4 Year-old Children: A Two-interval, Observer-based Procedure Using Conditioned Play-based Responses
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Simple Surgical Induction of Conductive Hearing Loss with Verification Using Otoscope Visualization and Behavioral Clap Startle Response in Rat
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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

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Area of Science:

  • Radiology
  • Genetics
  • Otolaryngology

Background:

  • Syndromic hearing loss diagnosis relies on identifying patterns across multiple organ systems.
  • Specific radiological appearances in the temporal bone are increasingly linked to genetic and syndromic diagnoses.

Purpose of the Study:

  • To provide a practical guide for radiologists on recognizing temporal bone phenotypes associated with syndromic hearing loss.
  • To consolidate current knowledge on radiological patterns aiding syndromic diagnosis.

Main Methods:

  • Literature review of recent publications on temporal bone imaging and syndromic hearing loss.
  • Analysis of radiological phenotypes and their association with genetic and clinical findings.

Main Results:

  • Specific temporal bone imaging findings can indicate syndromic causes of hearing loss.
  • Radiological pattern recognition facilitates precise genetic and syndromic diagnosis.

Conclusions:

  • Radiologists play a crucial role in diagnosing syndromic hearing loss through pattern recognition.
  • Understanding these radiological phenotypes is essential for effective patient management.