What is tietz sendromu?

Tietz Syndrome is a very rare genetic condition that affects the skin and hearing. This syndrome is caused by mutations in the GJB2 gene, which provides instructions for making a protein called connexin 26. The main features of Tietz Syndrome are congenital sensorineural hearing loss which is usually severe or profound, along with abnormal skin pigmentation. People with Tietz Syndrome typically have pale skin and white forelock, which is a distinctive feature. Some individuals may also experience vision problems or other neurological symptoms. Tietz Syndrome is inherited in an autosomal dominant pattern, meaning that an affected person has a 50% chance of passing the condition on to each of their children. There is no known cure for Tietz Syndrome, but there are treatment options available to manage the symptoms.