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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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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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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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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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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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Anatomy of the Ear01:16

Anatomy of the Ear

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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: Mar 8, 2026

Simple Surgical Induction of Conductive Hearing Loss with Verification Using Otoscope Visualization and Behavioral Clap Startle Response in Rat
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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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Congenital hearing loss.

Anna M H Korver1, Richard J H Smith2, Guy Van Camp3

  • 1Department of Pediatrics, St Antonius Hospital, PO BOX 2500, 3430 EM Nieuwegein, The Netherlands.

Nature Reviews. Disease Primers
|January 13, 2017
PubMed
Summary
This summary is machine-generated.

Congenital hearing loss is common in children, often diagnosed through newborn screening. Early intervention and understanding genetic causes improve speech, social development, and quality of life.

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Area of Science:

  • Pediatrics
  • Genetics
  • Otolaryngology

Background:

  • Congenital hearing loss is a prevalent childhood condition.
  • Early detection via neonatal screening is crucial for development.
  • Causes include environmental, prenatal, and genetic factors.

Purpose of the Study:

  • To review the causes and management of congenital hearing loss.
  • To highlight the importance of early diagnosis and intervention.
  • To discuss the role of genetic factors and testing.

Main Methods:

  • Literature review of congenital hearing loss.
  • Analysis of etiological factors (environmental, infectious, genetic).
  • Overview of diagnostic and management strategies.

Main Results:

  • Genetic factors are the primary cause in developed countries.
  • Inner ear homeostasis and mechano-electrical transduction are key affected areas.
  • Cytomegalovirus infection is a significant risk factor.

Conclusions:

  • Understanding the etiology guides therapeutic decisions and genetic counseling.
  • Advances in genetic testing are vital for developing new strategies.
  • Improved pathophysiology knowledge will enhance screening and treatment.