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Strial dysfunction in mice with cochleo-saccular abnormalities.

K P Steel, C Barkway, G R Bock

Hearing Research

|January 1, 1987

Summary

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Dominant spotting (Wv/Wv) mutant mice exhibit cochlear degeneration and absent endocochlear potential (EP), leading to profound hearing loss. Some mice partially escaped these effects, showing intermediate hearing capabilities.

Area of Science:

Otopathology
Genetics of Hearing Loss
Auditory Neuroscience

Background:

Dominant spotting (Wv/Wv) mutation causes cochleo-saccular degeneration in mice.
Previous studies have linked Wv/Wv to strial abnormalities and hearing impairment.

Purpose of the Study:

Investigate the auditory phenotype of Wv/Wv mutant mice.
Characterize the relationship between strial dysfunction, endocochlear potential (EP), and hearing loss.

Main Methods:

Auditory brainstem response (ABR) testing to assess hearing thresholds.
Electrophysiological measurements of endocochlear potential (EP).
Histological examination of cochlear structures.

Main Results:

Related Experiment Videos

Wv/Wv mice displayed significantly elevated hearing thresholds (around 100 dB SPL) and near-zero EP.

Stria vascularis was structurally abnormal, correlating with outer hair cell degeneration in the basal cochlea.

Five mice showed partial escape from mutation effects, with intermediate EP and hearing thresholds.

Conclusions:

The Wv/Wv mutation primarily affects the stria vascularis, leading to secondary neuroepithelial degeneration and profound hearing loss.
This pathology differs from previously described neuroepithelial abnormalities causing hearing loss.
The findings highlight the critical role of the stria vascularis in maintaining cochlear function and hearing.