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Deciphering Auditory Hyperexcitability in Otogl Mutant Mice Unravels an Auditory Neuropathy Mechanism
https://orcid.org/0009-0001-0399-4667
https://orcid.org/0000-0002-3801-2416
https://orcid.org/0000-0003-0105-2198
https://orcid.org/0000-0003-3226-3828
https://orcid.org/0000-0002-6911-7552
https://orcid.org/0000-0001-5325-1370
https://orcid.org/0000-0001-9932-6438
https://orcid.org/0000-0002-5305-9095
https://orcid.org/0000-0002-5269-0854
https://orcid.org/0000-0001-8283-804X
https://orcid.org/0000-0002-9695-9050
https://orcid.org/0000-0001-8608-0723
https://orcid.org/0000-0002-2441-9274
https://orcid.org/0000-0003-2692-4984
https://orcid.org/0000-0001-9211-1960
https://orcid.org/0000-0003-1604-7308
https://orcid.org/0000-0003-2092-5097
https://orcid.org/0000-0001-7816-7503
https://orcid.org/0000-0002-9069-002X
https://orcid.org/0000-0001-6742-8739
https://orcid.org/0000-0003-1054-1479
https://orcid.org/0000-0002-1287-2709
AbstractAuditory neuropathies affect the spiral ganglion neurons of the auditory nerve or their synapses with the sensory hair cells, distorting the sound information transmitted from the ear to the brain. Deciphering the underlying pathophysiological mechanisms remains challenging owing to the diversity of spiral ganglion neuron subtypes and associated central auditory circuits. An auditory neuropathy mechanism is unraveled by investigating the origin of auditory hyperexcitability in a mouse model for hereditary congenital deafness. Otogl encodes the large Otogelin‐like protein, which is related to secreted epithelial mucins and is implicated in the mechanical stimulation of cochlear outer hair cells. Heterozygous Otogl+/− mutant mice display auditory hyperexcitability, highlighted by their susceptibility to audiogenic seizures induced by loud sounds. It is shown that Otogl is transiently expressed in a subpopulation of spiral ganglion neurons during cochlear development. Despite their apparently normal hearing, Otogl+/− mice display poor activation of the spiral ganglion neurons processing loud sounds and an elevation of the activation threshold of the middle the ear muscle reflex that attenuates loud sounds. The findings reveal how a neuropathy affecting spiral ganglion neurons specialized in loud sound processing and associated with the middle the ear muscle reflex can manifest itself as auditory hyperexcitability.
Deciphering Auditory Hyperexcitability in Otogl Mutant Mice Unravels an Auditory Neuropathy Mechanism
https://orcid.org/0009-0001-0399-4667
https://orcid.org/0000-0002-3801-2416
https://orcid.org/0000-0003-0105-2198
https://orcid.org/0000-0003-3226-3828
https://orcid.org/0000-0002-6911-7552
https://orcid.org/0000-0001-5325-1370
https://orcid.org/0000-0001-9932-6438
https://orcid.org/0000-0002-5305-9095
https://orcid.org/0000-0002-5269-0854
https://orcid.org/0000-0001-8283-804X
https://orcid.org/0000-0002-9695-9050
https://orcid.org/0000-0001-8608-0723
https://orcid.org/0000-0002-2441-9274
https://orcid.org/0000-0003-2692-4984
https://orcid.org/0000-0001-9211-1960
https://orcid.org/0000-0003-1604-7308
https://orcid.org/0000-0003-2092-5097
https://orcid.org/0000-0001-7816-7503
https://orcid.org/0000-0002-9069-002X
https://orcid.org/0000-0001-6742-8739
https://orcid.org/0000-0003-1054-1479
https://orcid.org/0000-0002-1287-2709
AbstractAuditory neuropathies affect the spiral ganglion neurons of the auditory nerve or their synapses with the sensory hair cells, distorting the sound information transmitted from the ear to the brain. Deciphering the underlying pathophysiological mechanisms remains challenging owing to the diversity of spiral ganglion neuron subtypes and associated central auditory circuits. An auditory neuropathy mechanism is unraveled by investigating the origin of auditory hyperexcitability in a mouse model for hereditary congenital deafness. Otogl encodes the large Otogelin‐like protein, which is related to secreted epithelial mucins and is implicated in the mechanical stimulation of cochlear outer hair cells. Heterozygous Otogl+/− mutant mice display auditory hyperexcitability, highlighted by their susceptibility to audiogenic seizures induced by loud sounds. It is shown that Otogl is transiently expressed in a subpopulation of spiral ganglion neurons during cochlear development. Despite their apparently normal hearing, Otogl+/− mice display poor activation of the spiral ganglion neurons processing loud sounds and an elevation of the activation threshold of the middle the ear muscle reflex that attenuates loud sounds. The findings reveal how a neuropathy affecting spiral ganglion neurons specialized in loud sound processing and associated with the middle the ear muscle reflex can manifest itself as auditory hyperexcitability.
Deciphering Auditory Hyperexcitability in Otogl Mutant Mice Unravels an Auditory Neuropathy Mechanism
https://orcid.org/0009-0001-0399-4667
https://orcid.org/0000-0002-3801-2416
https://orcid.org/0000-0003-0105-2198
https://orcid.org/0000-0003-3226-3828
https://orcid.org/0000-0002-6911-7552
https://orcid.org/0000-0001-5325-1370
https://orcid.org/0000-0001-9932-6438
https://orcid.org/0000-0002-5305-9095
https://orcid.org/0000-0002-5269-0854
https://orcid.org/0000-0001-8283-804X
https://orcid.org/0000-0002-9695-9050
https://orcid.org/0000-0001-8608-0723
https://orcid.org/0000-0002-2441-9274
https://orcid.org/0000-0003-2692-4984
https://orcid.org/0000-0001-9211-1960
https://orcid.org/0000-0003-1604-7308
https://orcid.org/0000-0003-2092-5097
https://orcid.org/0000-0001-7816-7503
https://orcid.org/0000-0002-9069-002X
https://orcid.org/0000-0001-6742-8739
https://orcid.org/0000-0003-1054-1479
https://orcid.org/0000-0002-1287-2709
AbstractAuditory neuropathies affect the spiral ganglion neurons of the auditory nerve or their synapses with the sensory hair cells, distorting the sound information transmitted from the ear to the brain. Deciphering the underlying pathophysiological mechanisms remains challenging owing to the diversity of spiral ganglion neuron subtypes and associated central auditory circuits. An auditory neuropathy mechanism is unraveled by investigating the origin of auditory hyperexcitability in a mouse model for hereditary congenital deafness. Otogl encodes the large Otogelin‐like protein, which is related to secreted epithelial mucins and is implicated in the mechanical stimulation of cochlear outer hair cells. Heterozygous Otogl+/− mutant mice display auditory hyperexcitability, highlighted by their susceptibility to audiogenic seizures induced by loud sounds. It is shown that Otogl is transiently expressed in a subpopulation of spiral ganglion neurons during cochlear development. Despite their apparently normal hearing, Otogl+/− mice display poor activation of the spiral ganglion neurons processing loud sounds and an elevation of the activation threshold of the middle the ear muscle reflex that attenuates loud sounds. The findings reveal how a neuropathy affecting spiral ganglion neurons specialized in loud sound processing and associated with the middle the ear muscle reflex can manifest itself as auditory hyperexcitability.
Deciphering Auditory Hyperexcitability in Otogl Mutant Mice Unravels an Auditory Neuropathy Mechanism
Advanced Science
Gagliardini, Mathilde (author) / Mechaussier, Sabrina (author) / Campos Pina, Carolina (author) / Morais, Monica (author) / Postal, Olivier (author) / Jean, Philippe (author) / Dupont, Typhaine (author) / Singh‐Estivalet, Amrit (author) / Udugampolage, Shéhanie (author) / Scandola, Cyril (author)
2025-02-18
Article (Journal)
Electronic Resource
English
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