BACKGROUND A considerable amount of research has been published about genetic hearing impairment. Fifty to sixty percent of hearing loss is thought to have a genetic cause. Genes may also play a significant role in acquired hearing loss due to aging, noise exposure, or ototoxic medications. Between 1995 and 2012, over 100 causative genes have been identified for syndromic and nonsyndromic forms...

INTRODUCTION Congenital abnormalities of the inner ear is the most common cause of neurosensory hearing loss. Michel inner ear deformity is a rare developmental anomaly refers to the total aplasia of the inner ear. It is caused by developmental arrest of otic placode early during the third week of gestational age. CASE REPORT We have discussed here that three year old girl diagnosed Michel ap...

Meniere's disease, also know as endolymphatic hydrops, is a disorder of the inner ear of unknown cause. It is speculated that Meniere’s disease may be an immune-mediated disorder. Symptoms include tinnitus, vertigo, heightened sensitivity to loud sounds, fluctuating loss of hearing, headache, and aural fullness. In acute phases, nausea, vomiting, and disabling dizziness may occur. Usually, atta...

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...

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...