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In addition, 7 gene hits have been directly implicated in human and/or mouse hearing loss (Table 1), and 7 others are related to gene families associated with inner ear abnormalities (Table 2) [22] [24].
The mouse hearing loss correlated with an alteration in the fate of supporting cells (Deiters'-to-pillar cells) along the entire length of the cochlear duct, with the most extreme abnormalities found at the apical or low-frequency end.
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We examined Cloth-ears mice for hearing loss by ABR analysis.
No basal vacuolization was found in temporal bones of mice without hearing loss.
However, no mutant mice with hearing loss and paroxysmal tremor have been reported.
TEM was performed on four temporal bones of septic mice with hearing loss and two sham animals without hearing loss.
These pathological alterations could be found exclusively in temporal bones of mice with hearing loss (supplementary material Fig. S3A D).
Hair cells of mice with hearing loss of 15 20 dB showed a basal vacuolization ranging from the basal pole of the inner hair cell to the basal membrane.
Cmah-null mice with hearing loss are closely associated with neurological disease (Alzheimer's disease, Parkinson's disease and Huntington's disease), psychological disorders, skeletal and muscular disorders, infectious disease, and hereditary disorder.
On the other hand, it was reported in a study that, in a senescence-accelerated mouse, prone 8 (SAMP8) strain mouse shows premature hearing loss and cochlear degeneration repeating the processes noticed in human presbycusis (i.e., strial, sensory, and neural degeneration) [ 8].
Based on studies in the Slc26a4−/− mouse model, fluctuating hearing loss may be due to oscillations of a negative feedback system that oscillates and generates fluctuations in the endocochlear potential and thereby in the driving force on which hearing depends.
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