Below is an instruction from Human. Write a response.
    ### Instruction:
    What is (are) Tietz syndrome ?
    ### Response:
    Tietz syndrome is a disorder characterized by profound hearing loss from birth, fair skin, and light-colored hair. 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 darkens over time to blond or red.
    