BACKGROUND The vestibular apparatus of the vertebrate inner ear uses three fluid-filled semicircular canals to sense angular acceleration of the head. Malformation of these canals disrupts the sense of balance and frequently causes circling behavior in mice. The Epistatic circler (Ecl) is a complex mutant derived from wildtype SWR/J and C57L/J mice. Ecl circling has been shown to result from th...

Many children have benefited from cochlear implant device including those with congenital malformation of the inner ear. The results reported in children with malformed cochlea are very encouraging. We describe 2 cases of Mondini's malformation with severe sensorineural hearing loss. Cochlear implantation was performed and both of them underwent post-implantation speech rehabilitation. Post-imp...

recent developments in medicine have given us a better insight into a group of disorders known as autoimmune diseases. in particular, advances have occurred in our understanding of the autoimmune inner ear disease (aied). in this article, the authors review the different postulated theories in the pathogenesis of this disease. the clinical presentation, the available para-clinical diagnostic to...

Anomalies of the course of the facial nerve have been reported in association with middle and inner ear malformations. Bifurcation of its intratemporal portion is a rare malformation in which focal splitting of one or more facial nerve segments occurs. We describe the CT appearance of this anomaly and discuss its possible embryology. Facial nerve bifurcation is important to recognize in patient...

Klippel-Feil syndrome is defined as the fusion of cervical vertebra with associated congenital anomalies but was rarely reported to be associated with Mondini Malformation. We report a newborn girl with severe neck extension, computed tomography (CT) of the neck after birth showed fusion of the fifth, sixth, and seventh cervical vertebrae, compatible with Klippel-Feil Syndrome and CT temporal b...

OBJECTIVE To explore the clinical experience of multi-channel cochlear implantation in patients with bilateral inner ear malformations. METHOD Multi-channel cochlear implantation was conducted among 180 patients from 1997 to 2001. Twenty-five of them, being with inner ear malformations, were given hearing and speech rehabilitation including sound detection, word discrimination, auditory compr...