Introduction FMF is a monogenic autoinflammatory disease with recurring episodes of fever and serositis attacks. FMF is associated with mutations in pyrin. On the other hand mutations in a molecule in the same pathway, cryopyrin, is characterized by inflammatory features involving the inner ear as well. A study has suggested the involvement of cochlea in Behçet disease, which is a polygenic aut...

Retino-cochleo-cerebral angiopathy or Susac syndrome is a rare autoimmune disease that selectively affects the vessels of retina, inner ear and central nervous system. Differentiation multiple sclerosis presents difficulties due to similarity MRI semiotics these two diseases. This article clinical cases patients with who were diagnosed at onset disease. Based on analysis our own observations li...

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...

The purpose of this study was to prove the hypothesis that caloric response in Ménière's disease (MD) is reduced by hydropic expansion of the vestibular labyrinth, not by vestibular hypofunction, by evaluating the correlation morphologically using an intravenous Gadolinium (IV-Gd) inner ear MRI. In study I, the prevalence of abnormal video Head Impulse Test (vHIT) results among the patients wit...

Current thinking is that Ménière’s disease (or, more accurately, Ménière’s syndrome) is a phenotype of unstable or erratic inner ear hearing and vestibular function that arises from failure of one or more of the many inner ear homoeostatic systems that regulate endolymph and perilymph, aff erent and eff erent nerve signalling, and blood fl ow. Clinically, the fl uctuating and progressive sensor...