[Show abstract][Hide abstract] 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.
Full-text · Article · Feb 2005 · Molecular and Cellular Biology