Related Experiment Video
Updated: Jul 20, 2026
07:07
Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea
Published on: February 21, 2016
Cochlear developmental defect and background-dependent hearing thresholds in the Jackson circler (jc) mutant mouse
Alfredo Calderon1, Adam Derr, Barden B Stagner
1Section on Neurogenetics, Laboratory of Molecular Biology, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, 5 Research Court, Rockville, MD 20850, USA.
Hearing Research
|September 12, 2006
Summary
The Jackson circler (jc) mouse mutation causes circling and hearing loss due to cochlear development defects. Genetic mapping reveals its location on chromosome 10, homologous to human 6q21.
Area of Science:
- Genetics
- Neuroscience
- Developmental Biology
Background:
- The Jackson circler (jc) is a mouse mutation causing circling behavior and impaired acoustic startle response.
- Previous studies indicated potential inner ear defects, but detailed characterization was lacking.
Purpose of the Study:
- To refine phenotypic and genetic parameters of the original jc mutation.
- To characterize a new mutant allele, jc(2J).
- To investigate the genetic basis of circling behavior and hearing loss in jc mice.
Main Methods:
- Open-field behavior tests to assess ambulatory and circling behavior.
- Auditory-evoked brainstem response (ABR) and distortion-product otoacoustic emissions (DPOAEs) to evaluate hearing.
- Histological analysis of cochlear development.
- Genetic mapping using intrasubspecific intercross and genome-wide linkage scans.
Main Results:
- Homozygous jc mutants displayed circling behavior similar to inner ear mutants.
- jc/jc and jc(2J)/jc(2J) mice showed elevated ABR thresholds, indicating hearing impairment.
- Cochlear development was arrested at gestational day 13.5, with failure of apical turn formation.
- jc was mapped to chromosome 10 (25cM), homologous to human 6q21.
- Hearing thresholds varied significantly across different mouse genetic backgrounds.
Conclusions:
- The jc mutation disrupts cochlear development, leading to circling behavior and hearing loss.
- The gene responsible for jc is located on mouse chromosome 10.
- Genetic background significantly influences the manifestation of jc-related hearing defects.
Related Concept Videos
01:31Hearing
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.
01:13The Cochlea
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.