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4 results for “progressive hearing loss”
Inner hair cell dysfunction in Klhl18 mutant mice leads to low frequency progressive hearing loss
<p>Age-related hearing loss in humans (presbycusis) typically involves impairment of high frequency sensitivity before becoming progressively more severe at lower frequencies. Pathologies initially affecting lower frequency regions of hearing are less common. Here we describe a progressive, predominantly low-frequency recessive hearing impairment in two mutant mouse lines carrying different mutant alleles of the <i>Klhl18</i> gene: a spontaneous missense mutation (<i>Klhl18<sup>lowf</sup></i>) and a targeted mutation (<i>Klhl18<sup>tm1a(KOMP)Wtsi</sup></i>). Both males and females were studied, and the two mutant lines showed similar phenotypes. Threshold for auditory brainstem responses (ABR; a measure of auditory nerve and brainstem neural activity) were normal at 3 weeks old but showed progressive increases from 4 weeks onwards. In contrast, distortion product otoacoustic emission (DPOAE) sensitivity and amplitudes (a reflection of cochlear outer hair cell function) remained normal in mutants. Electrophysiological recordings from the round window of <i>Klhl18<sup>lowf</sup></i> mutants at 6 weeks old revealed 1) raised compound action potential thresholds that were similar to ABR thresholds, 2) cochlear microphonic potentials that were normal compared with wildtype and heterozygous control mice and 3) summating potentials that were reduced in amplitude compared to control mice. Scanning electron microscopy showed that <i>Klhl18<sup>lowf</sup></i> mutant mice had abnormally tapering of the tips of inner hair cell stereocilia in the apical half of the cochlea while their synapses appeared normal. These results suggest that Klhl18 is necessary to maintain inner hair cell stereocilia and normal inner hair cell function at low frequencies.</p>
Inner hair cell dysfunction in Klhl18 mutant mice leads to low frequency progressive hearing loss
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Data from: Deficit of mitogen-activated protein kinase phosphatase 1 (DUSP1) accelerates progressive hearing loss
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Long-term NAD+ supplementation prevents the progression of age-related hearing loss in mice
GEO Series GSE213994. Mus musculus. 10 samples. Type: Expression profiling by high throughput sequencing.
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International Brain Laboratory public data
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