Waardenburg syndrome is a group of genetic conditions characterized by hearing loss and changes in coloring (pigmentation) of the hair, skin, and eyes. The hearing loss can occur in one or both ears. People with Waardenburg syndrome often have very pale blue eyes or different colored eyes (such as one blue eye and one brown eye). There may also be segments of two different colors in the same eye. Distinctive hair coloring (such as a patch of white hair or hair that prematurely turns gray) is another common sign of the condition. The features of Waardenburg syndrome vary among affected individuals, even among people in the same family. There are four known types of Waardenburg syndrome. The types are distinguished by their physical characteristics and sometimes by their genetic cause. Types I and II have very similar features, except people with type I almost always have eyes that appear widely spaced because the tear ducts are spread outward. People with type II do not have the appearance of widely spaced eyes. Type III (sometimes called Klein-Waardenburg syndrome) includes abnormalities of the upper limbs in addition to hearing loss and changes in pigmentation. Type IV (also known as Waardenburg-Shah syndrome) has signs and symptoms of both Waardenburg syndrome and Hirschsprung disease (a disorder that can lead to blockage of the large intestine).