OBJECTIVE To study a family with inner ear malformations and sensorineural hearing loss. DESIGN Clinical, radiological, and genetic study of the members of a family with different degrees of sensorineural hearing loss. RESULTS The males in the family manifested profound congenital hearing loss with severe inner ear malformations, while the only affected female had progressive hearing loss t...

In the early 19th century Flourens had already proved by experiment that by stimulating the semi-circular canals of the inner ear certain rhythmical eye movements (called nystagmus) could be caused and Purkinje showed that in human beings vertigo was induced by rotation. Ménière was the first to prove that diseases of the inner ear can produce giddiness. Later, a number of physiologists, especi...

The inner ear is structurally complex. A molecular description of its architecture is now emerging from the use of contemporary methods of cell and molecular biology, and from studies of ontogenetic development. With the application of clinical and molecular genetics, it has now become possible to identify genes associated with inherited, non-syndromic deafness and balance dysfunction in humans...

Genetically engineered strains of mice, modified by gene targeting (knockouts), are increasingly being employed as alternative effective research tools in elucidating the genetic basis of human deafness. An impressive array of auditory and vestibular mouse knockouts is already available as a valuable resource for studying the ontogenesis, morphogenesis and function of the mammalian inner ear. T...

Patients with autoimmune inner ear disease develop rapidly progressive sensorineural hearing loss over a period of several weeks or months, often accompanied by vestibular loss. This disease can occur as a distinct clinical entity or in association with an underlying autoimmune disorder. Treatment comprises immunosuppression by corticosteroids, cytostatic drugs or tumor necrosis factor-α antago...