Deafness and Cochlear Fibrocyte Alterations in Mice Deficient for the Inner Ear Protein Otospiralin

• DOI: 10.1128/mcb.25.2.847-853.2005
• Publication Date: 2005-01-04T04:56:02Z
• Authors: Benjamin Delprat; Jérôme Ruel; Matthieu J. Guitton; Ghyslaine Hamard; Marc Lenoir; Rémy Pujol; Jean-Luc Puel; Philippe Brabet; Christian P. Hamel
• Keywords: Fibrocyte; Saccule; Auditory neuropathy

AbstractIn the cochlea, mammalian auditory organ, fibrocytes of mesenchymal nonsensory regions play important roles in cochlear physiology, including maintenance ionic and hydric components endolymph. Occurrence human deafness fibrocyte alterations underlines their critical function. We recently described a novel gene, Otos, which encodes otospiralin, small protein unknown function that is produced by cochlea vestibule. now have generated mice with deletion Otos found they show moderate deafness, no frequency predominance. Histopathology revealed degeneration type II IV fibrocytes, while hair cells stria vascularis appeared normal. Together, these findings suggest impairment caused loss otospiralin leads to abnormal physiology This dysfunction may predispose age-related hearing loss. ACKNOWLEDGMENTSWe thank Harold Cremer (CNRS UMR 6156, IBDM, Marseille, France) for providing ES Marie-Christine Simmler (INSERM U. 587, Paris, giving us LacZneo cassette. Blastocyste injection chimeras were obtained at Plateforme de Recombinaison Homologue Cochin Institute (INSERM). Animals bred Mireille Gallego. SEM TEM performed CRIC (Centre Régional d'Imagerie Cellulaire) technical support Chantal Cazevieille Florence Tribillac. Jean-Louis Pasquier artwork.This study was supported grants from Institut la Santé et Recherche Médicale B.D. has fellowship Caisse d'Epargne, Montpellier, Région Languedoc-Roussillon, France.