Tietz SyndromeSymptoms, Doctors, Treatments, Advances & More
Learn About Tietz Syndrome
Tietz syndrome is a disorder that is characterized by profound hearing loss and unusually light-colored skin and hair (hypopigmentation). The hearing loss in affected individuals is caused by abnormalities of the inner ear (sensorineural hearing loss) and is present from birth. Although people with Tietz syndrome are born with white hair and very pale skin, their hair color often changes over time to blond or light red. Affected individuals sunburn very easily, although they may tan slightly or develop reddish freckles with limited sun exposure. Their skin and hair color will remain lighter than those of other members of their family.
Genetic changes that cause disease are called pathogenic variants. Pathogenic variants in the MITF gene cause Tietz syndrome. The MITF gene provides instructions for making a protein that plays a role in the development, survival, and function of certain types of cells. MITF proteins attach to each other or to other proteins, creating a two-protein unit (dimer). The dimer attaches to specific areas of DNA and helps control the activity of particular genes. Proteins that are involved in controlling gene activity are called transcription factors.
Tietz syndrome is a very rare disorder, although its exact prevalence is unknown. Only a few affected families have been described in the scientific literature.
This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In most cases, a person with Tietz syndrome has one parent who also has the condition.
Texas Health Family Care
Dr. William Drake has been serving the primary care needs of North Texas families since 1995. He is focused on providing quality preventative medicine as the first line of defense for patients of all ages. Dr. Drake is dedicated to serving his patients by providing personalized health and wellness services through exemplary care, education and research. He is especially passionate about research and treatments in the areas of juvenile and adult diabetes.Dr. Drake earned his medical degree from the University of Michigan in East Lansing. He completed his family medicine residency at The University of Texas Health Science Center at San Antonio. Dr. Drake is a Fellow of the American Academy of Family Physicians and a member of the American Diabetes Association, Juvenile Diabetes Research Foundation, Tarrant County Medical Society, Texas Academy of Family Physicians and Texas Medical Association. Dr. Drake is rated as an Advanced provider by MediFind in the treatment of Tietz Syndrome. He is also highly rated in 47 other conditions, according to our data. His clinical expertise encompasses Cirrhosis, Xanthoma, Familial Combined Hyperlipidemia, and High Cholesterol.
Texas Health Family Care
Shane Ashford is a primary care provider, practicing in Family Medicine in Denton, Texas. Dr. Ashford is rated as an Experienced provider by MediFind in the treatment of Tietz Syndrome. He is also highly rated in 29 other conditions, according to our data. His clinical expertise encompasses Cirrhosis, Xanthoma, Familial Combined Hyperlipidemia, and Familial Hypertension.
Texas Health Adult Care
Sandip Mehta is a primary care provider, practicing in Internal Medicine in Hurst, Texas. Dr. Mehta is rated as an Experienced provider by MediFind in the treatment of Tietz Syndrome. He is also highly rated in 7 other conditions, according to our data. His clinical expertise encompasses Vitamin D Deficiency, High Cholesterol, Hypothyroidism, and Hypertensive Heart Disease.
Published Date: June 25, 2026
Published By: National Institutes of Health