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Updated: Jun 26, 2026

Data Acquisition and Analysis In Brainstem Evoked Response Audiometry In Mice

Data Acquisition and Analysis In Brainstem Evoked Response Audiometry In Mice

Published on: May 10, 2019

Mouse models for human hereditary deafness.

Michel Leibovici¹, Saaid Safieddine, Christine Petit

¹Institut Pasteur, Unite de Genetique et Physiologie de l'Audition, Paris, France.

Current Topics in Developmental Biology

|February 3, 2009

Summary

This summary is machine-generated.

Mouse models have been instrumental in understanding genetic hearing loss mechanisms. They reveal cochlear defects, aiding research into causes of deafness and potential therapies for hearing impairment.

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Last Updated: Jun 26, 2026

Data Acquisition and Analysis In Brainstem Evoked Response Audiometry In Mice

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Published on: May 10, 2019

Surgical Method for Virally Mediated Gene Delivery to the Mouse Inner Ear through the Round Window Membrane

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Published on: March 16, 2015

Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea

Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea

Published on: February 21, 2016

Area of Science:

Genetics
Otolaryngology
Molecular Biology

Background:

Hearing impairment is a common human condition with numerous identified causative genes.
Understanding the precise pathogenic mechanisms of genetic deafness remains challenging through patient testing alone.

Purpose of the Study:

To highlight the utility of mouse models in dissecting the pathophysiological processes underlying genetic forms of deafness.
To illustrate diverse cochlear defects identified using these models.

Main Methods:

Utilizing genetically modified mouse models to study specific forms of inherited deafness.
Analyzing cochlear structures and functions in these models to elucidate disease mechanisms.

Main Results:

Mouse models have successfully revealed various structural and functional defects in the cochlea associated with genetic deafness.
Specific examples include otoferlin defects (DFNB9), hair bundle abnormalities (Usher syndrome), and connexin-26 mutations affecting gap junctions (DFNB1).

Conclusions:

Mouse models are crucial for understanding the molecular and cellular basis of genetic hearing loss.
These models provide insights into diverse mechanisms of cochlear dysfunction, paving the way for future therapeutic strategies.