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Updated: May 8, 2026

Isolation and Culture of Primary Cochlear Hair Cells from Neonatal Mice

Isolation and Culture of Primary Cochlear Hair Cells from Neonatal Mice

Published on: September 15, 2023

Genetic deafness of central origin.

M S Deol¹, M P Frank, K P Steel

¹Department of Genetics and Biometry, University College London, Wolfson House, 4 Stephenson Way, London, NW1 2HE, UK.

|September 10, 2013

Summary

The quivering gene in mice causes genetic deafness originating in the central nervous system, not the cochlea. This study identifies a novel form of retrocochlear hearing loss.

Area of Science:

Genetics
Neuroscience
Auditory Science

Background:

Deafness can arise from various genetic factors affecting the auditory system.
Understanding the origin of hearing loss is crucial for developing targeted therapies.

Purpose of the Study:

To investigate the cause of deafness in mice with the autosomal recessive quivering gene.
To determine if the hearing impairment is cochlear or retrocochlear.

Main Methods:

Histological examination of cochleae in mutant and normal mice.
Measurement of auditory brainstem responses (ABRs) at the round window.
Assessment of auditory evoked potentials in the inferior colliculus.

Main Results:

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Cochlear structures were histologically normal in quivering mice.

Auditory thresholds at the round window were comparable between mutant and normal mice.

Significantly elevated thresholds for inferior colliculus potentials were observed in mutant mice, indicating retrocochlear dysfunction.

Conclusions:

The quivering mutation in mice leads to deafness of central nervous system origin.
This represents the first documented case of genetically induced hearing loss originating centrally.