Ehlers-Danlos Syndrome

Ehlers-Danlos Syndrome is a rare syndrome that can cause a defect in the sound conducting mechanism of the middle ear, and which may result in hearing loss.

Normally when sound waves enter the ear they are conducted from the middle ear to the inner ear by three tiny bone joints called the hammer, the anvil and the stirrup. But due to the syndrome the joints may become hypermobile. This may cause the sound not to be conducted effectively from the middle to the inner ear. Patients suffering from hearing loss caused by Ehlers-Danlos Syndrome may find that hearing aids are the most effective remedy. In addition, Ehlers-Danlos Syndrome may cause other symptoms such as skin or heart problems. Basically, the syndrome is a defect in the connection tissue in the body. The defect is caused by a mutation in the genes which control the formation of albumen (protein) from which the connective tissue is built. The syndrome is hereditary.