In recent years studies on isolated hair cells have suggested that there is an inherent tuning of hair cells determined by their mechanical and electrical properties. However, tuning for mammalian cochleas appears to be much more complicated since there are typically two types of receptor cells (inner and outer hair cells) imbedded in a highly organized framework of supporting cells, membranes and fluids. The major neural output of the cochlea can be monitored by recording the activity of myelinated axons of spiral ganglion cells, not only under normal conditions, but also when the discharge patterns are altered by ototoxic drugs, acoustic trauma or olivocochlear bundle stimulation. A model system with two excitatory influences, one sharply tuned and highly sensitive, and a second, broadly tuned and relatively insensitive, can account for much of the existing data. Results from single-neuron marking studies support the notion that these two influences probably involve interactions between inner and outer hair cells. More global influences such as the endocochlear potential also can act on auditory-nerve fibers through the hair-cell systems. Thus, the inherent frequency selectivity of the receptor cell is only one of many factors that determine the tuning of mammalian auditory-nerve fibers.
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http://dx.doi.org/10.1016/0378-5955(86)90093-6 | DOI Listing |
Bio Protoc
January 2025
ENT Institute and Department of Otorhinolaryngology, Eye & ENT Hospital, Fudan University, Shanghai, China.
The organ of Corti, located in the inner ear, is the primary organ responsible for animal hearing. Each hair cell has a V-shaped or U-shaped hair bundle composed of actin-filled stereocilia and a kinocilium supported by true transport microtubules. Damage to these structures due to noise exposure, drug toxicity, aging, or environmental factors can lead to hearing loss and other disorders.
View Article and Find Full Text PDFAdv Sci (Weinh)
January 2025
Department of Otolaryngology, Case Western Reserve University, Cleveland, OH, 44106, USA.
Usher syndrome type 1C (USH1C) is a genetic disorder caused by mutations in the USH1C gene, which encodes harmonin, a key component of the mechanoelectrical transduction complex in auditory and vestibular hair cells. USH1C leads to deafness and vestibular dysfunction in humans. An Ush1c knockout (KO) mouse model displaying these characteristic deficits is generated in our laboratory.
View Article and Find Full Text PDFPlant Cell Physiol
January 2025
Institute for Chemical Research, Kyoto University, Gokasho, Uji, 611-0011 Kyoto, Japan.
Lotus japonicus-ROOT HAIR LESS1-LIKE1 (LRL1) of Arabidopsis thaliana encodes a basic helix-loop-helix (bHLH) transcription factor (TF) involved in root hair development. Root hair development is regulated by an elaborate transcriptional network, in which GLABRA2 (GL2), a key negative regulator, directly represses bHLH TF genes, including LRL1 and ROOT HAIR DEFECTIVE6 (RHD6). Although RHD6 and its paralogous TFs have been shown to connect downstream to genes involved in cell morphological events such as endomembrane and cell wall modification, the network downstream of LRL1 remains elusive.
View Article and Find Full Text PDFiScience
January 2025
Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC 29425, USA.
Mutations in the human genes encoding the endothelin ligand-receptor pair and cause Waardenburg-Shah syndrome (WS4), which includes congenital hearing impairment. The current explanation for auditory dysfunction is defective migration of neural crest-derived melanocytes to the inner ear. We explored the role of endothelin signaling in auditory development in mice using neural crest-specific and placode-specific mutation plus related genetic resources.
View Article and Find Full Text PDFAge-related hearing loss affects one-third of the population over 65 years. However, the diverse pathologies underlying these heterogenous phenotypes complicate genetic studies. To overcome challenges associated with accurate phenotyping for older adults with hearing loss, we applied computational phenotyping approaches based on audiometrically measured hearing loss.
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