title: Deafness and cochlear fibrocyte alterations in mice deficient for the inner ear protein otospiralin.
abstract: In the cochlea, the mammalian auditory organ, fibrocytes of the mesenchymal nonsensory regions play important roles in cochlear physiology, including the maintenance of ionic and hydric components in the endolymph. Occurrence of human deafness in fibrocyte alterations underlines their critical roles in auditory function. We recently described a novel gene, Otos, which encodes otospiralin, a small protein of unknown function that is produced by the fibrocytes of the cochlea and vestibule. We now have generated mice with deletion of Otos and found that they show moderate deafness, with no frequency predominance. Histopathology revealed a degeneration of type II and IV fibrocytes, while hair cells and stria vascularis appeared normal. Together, these findings suggest that impairment of fibrocytes caused by the loss in otospiralin leads to abnormal cochlear physiology and auditory function. This moderate dysfunction may predispose to age-related hearing loss.
authors.affiliation: INSERM U.583, Physiopathologie et Thérapie des Déficits Sensoriels et Moteurs, Institut des Neurosciences de Montpellier, Hôpital Saint-Eloi, BP 74103, 80, rue Augustin Fliche, 34295 Montpellier cedex 05, France. delprat@pasteur.fr
authors.forename: Benjamin
authors.initials: B
authors.lastname: Delprat
authors.forename: Jérôme
authors.initials: J
authors.lastname: Ruel
authors.forename: Matthieu J
authors.initials: MJ
authors.lastname: Guitton
authors.forename: Ghyslaine
authors.initials: G
authors.lastname: Hamard
authors.forename: Marc
authors.initials: M
authors.lastname: Lenoir
authors.forename: Rémy
authors.initials: R
authors.lastname: Pujol
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Puel
authors.forename: Philippe
authors.initials: P
authors.lastname: Brabet
authors.forename: Christian P
authors.initials: CP
authors.lastname: Hamel
mesh_terms: D000818:Animals; D001309:Auditory Threshold; D003051:Cochlea / Q000166:cytology* / Q000473:pathology / Q000502:physiology* / Q000503:physiopathology; D003638:Deafness / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D004594:Electrophysiology; D018390:Gene Targeting; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D011506:Proteins / Q000235:genetics / Q000378:metabolism*
chemical_list: C503045:OTOS protein, human; C503046:Otos protein, mouse; D011506:Proteins