BACKGROUND Congenital cytomegalovirus (CMV) infection is a leading cause of sensorineural hearing loss (SNHL). The mechanisms of pathogenesis of CMV-related SNHL are still unclear. The aim is to study congenital CMV-related damage in the fetal inner ear, in order to better understand the underlying pathophysiology behind CMV-SNHL. RESULTS We studied inner ears and brains of 20 human fetuses, ...

Spontaneous cerebrospinal fluid (CSF) fistula in congenital malformations of the inner ear is rare, and it is found most commonly in infants and children with impaired hearing. Spontaneous CSF otorrhea in adults is often due to defects in the tegmen and the bone overlying the posterior fossa in an otherwise normal ear. In the literature, there are few reports of adultonset spontaneous CSF otorr...

(Edmonds C. Inner ear barotrauma: a retrospective clinical series of 50 cases. SPUMS J. 2004; 34: 11-4.) A retrospective series of 50 cases of inner ear barotrauma is reviewed, as regards the diving and otological history, the clinical manifestations, basic audiometric investigations and treatments. This disorder may be predicted to some degree by a previous history of otological barotraumas, o...

The elongation and contraction of outer hair cells (OHCs) greatly contribute to the high sensitivity and frequency selectivity of the mammalian inner ear. The motility of OHCs is believed to be based on the conformational changes of the motor protein prestin in the plasma membrane of OHCs. In the present study, four lines of research on the inner ear were conducted. First, the mechanism by whic...

Autoimmunity is thought to be one cause of sensorineural hearing loss (SNHL). Sera from patients with rapidly progressive hearing loss have been shown to contain antibodies to a 68-kD protein in heterologous inner-ear tissue. Using guinea pig inner-ear tissue as the antigenic substrate and either Western blot or immunofluorescence (IF) or both, we tested sera from 74 patients suspected to have ...

In accordance with longstanding convention, the adult ear is here described in terms of its three portions, namely the outer, the middle and the inner ear. The inner ear, comprising the bony and membranous labyrinth with its central connections, arises from a set of structures quite distinct from those which give rise to the outer and middle ears. The development of the inner ear, which is the ...

In vertebrates, the inner ear arises from the otic placode, a thickened swathe of ectoderm that invaginates to form the otic vesicle. We report that histone demethylase KDM4B is dynamically expressed during early stages of chick inner ear formation. A loss of KDM4B results in defective invagination and striking morphological changes in the otic epithelium, characterized by abnormal localization...

In this review the authors discuss the physiopathologic and electrophysiologic changes of the inner ear in Meniere's disease, otosclerosis, ototoxicity, acoustic neuroma, presbycusis, noise-induced hearing loss, metabolic hearing loss, perilymphatic fistula, syphilis, autoimmune inner ear disease, congenital genetic hearing loss, and viral labyrinthitis. The etiology and pathogenesis of hearing...

Gfi1 was first identified as causing interleukin 2-independent growth in T cells and lymphomagenesis in mice. Much work has shown that Gfi1 and Gfi1b, a second mouse homolog, play pivotal roles in blood cell lineage differentiation. However, neither Gfi1 nor Gfi1b has been implicated in nervous system development, even though their invertebrate homologues, senseless in Drosophila and pag-3 in C...

Malformations of bony inner ear are rare anomalies occurring in approximately 20% of patients with congenital sensorineural hearing loss. Conductive hearing loss is usually associated with abnormalities of the external and middle ear. Recent reports of patients with lateral semicircular canal malformations indicate inner ear malformations to be associated with sensorineural or conductive hearin...