Isolated inner ear decompression sickness (DCS) is recognized in deep diving involving breathing of helium-oxygen mixtures, particularly when breathing gas is switched to a nitrogen-rich mixture during decompression. The biophysical basis for this selective vulnerability of the inner ear to DCS has not been established. A compartmental model of inert gas kinetics in the human inner ear was cons...

The inner ear, one of the most complex organs, contains within its bony shell three sensory systems, the evolutionary oldest gravity receptor system, the three semicircular canals for the detection of angular acceleration, and the auditory system--unrivaled in sensitivity and frequency discrimination. All three systems are susceptible to a host of afflictions affecting the quality of life for a...

Rock 'n' roll sounds best turned up loud, but as hundreds of aging rockers (and millions of their aging fans) are discovering, the cost of all that glorious noise is hearing loss and tinnitus. And it's not just loud music—our ears are daily assaulted by the roar of the jet engine, the shriek of the power tool, the blare of the car horn, and the pounding of the jackhammer. In most cases, it seem...

As many as 40% of patients with congenital sensorineural hearing loss will have a structural inner ear malformation identified on a temporal bone imaging study (1). Anomalies of the internal auditory canal (IAC) are rare and have been reported to account for only 12% of all congenital temporal bone abnormalities. Internal auditory canal abnormalities may be associated with hypoplasia or aplasia...

Rieger syndrome is a dysembryogenetic disease in which labyrinthic damage can be associated with other genetic anomalies. The case presented here is of a patient who has bilateral dysgenesis of the iris, with bulbar atrophy and dyscoria. The patient does not present any malformation of the craniofacial structures, of the periumbilical skin, or of the skeletal bones. The case is, therefore, a va...

her daughter. Most of the descriptions have appeared in the Continental and American literature*. As far as can be ascertained this is the first description of the fully developed syndrome in an African Negro. The salient features of this type of dysostosis are v 1) paipeorai nssures siopmg aownwaras ana laterally('antimongoloid') with a coloboma in the outer portion of the lower lids and, more...

The Notch signaling pathway is thought to regulate multiple stages of inner ear development. Mutations in the Notch signaling pathway cause disruptions in the number and arrangement of hair cells and supporting cells in sensory regions of the ear. In this study we identify an insertional mutation in the mouse Sfswap gene, a putative splicing factor, that results in mice with vestibular and coch...

BACKGROUND The zona pellucida (ZP) domain is part of many extracellular proteins with diverse functions from structural components to receptors. The mammalian β-tectorin is a protein of 336 amino acid residues containing a single ZP domain and a putative signal peptide at the N-terminus of the protein. It is 1 component of a gel-like structure called the tectorial membrane which is involved in ...

The Kresge Hearing Research Institute-3 (KHRI-3) antibody binds to a guinea pig inner ear supporting cell antigen (IESCA) and causes hearing loss. To gain insight into the mechanism of antibody-induced hearing loss, we used antibody immunoaffinity purification to isolate the IESCA, which was then sequenced by mass spectroscopy, revealing 10 guinea pig peptides identical to sequences in human ch...

Classical studies have postulated the action of an endomesodermal signal initiating inner ear induction, subsequently followed by a neural tube-derived signal to complete the process of otic placode formation in the surface ectoderm. Members of the Fibroblast growth factor (FGF) gene family have been implicated in these processes. In this review, expression analysis and recent experimental evid...