Related Experiment Video
Updated: Oct 18, 2025
06:07
Author Spotlight: Advancements in Cultivating Mouse Hair Cells for Auditory Research
Published on: September 15, 2023
2.9K
Inner hair cell dysfunction in Klhl18 mutant mice leads to low frequency progressive hearing loss
Neil J Ingham1, Navid Banafshe1, Clarisse Panganiban1
1Wolfson Centre for Age-Related Diseases, King's College London, London, United Kingdom.
Plos One
|October 1, 2021
Summary
Two mouse models with Klhl18 gene mutations exhibit progressive, low-frequency hearing loss. This impairment stems from abnormal inner hair cell stereocilia, impacting low-frequency sound perception.
Area of Science:
- Genetics
- Neuroscience
- Otolaryngology
Background:
- Age-related hearing loss (presbycusis) typically affects high frequencies first.
- Low-frequency hearing pathologies are less common but significantly impact auditory function.
Purpose of the Study:
- To investigate the genetic basis of a progressive, low-frequency hearing impairment.
- To characterize the auditory phenotype and underlying cellular mechanisms in mutant mouse models.
Main Methods:
- Auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) testing.
- Electrophysiological recordings (compound action potentials, cochlear microphonics, summating potentials).
- Scanning electron microscopy of cochlear hair cells.
Main Results:
- Mutant mice showed progressive increases in ABR thresholds from 4 weeks, indicating neural and brainstem dysfunction.
- DPOAEs remained normal, suggesting intact outer hair cell function.
- Inner hair cell stereocilia in the apical cochlea abnormally tapered, while synapses were normal.
Conclusions:
- The Klhl18 gene is crucial for maintaining the structure of inner hair cell stereocilia.
- Klhl18 dysfunction leads to impaired low-frequency hearing by affecting inner hair cell function.
Related Concept Videos
01:22Hair Cells
42.3K
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.
42.3K
00:50Unrenewable Cells
2.5K
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...
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...
2.5K
01:13The Cochlea
47.5K
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.
47.5K