Because Usher syndrome affects hearing, balance, and vision, diagnosis of the disorder usually includes the evaluation of all three senses. Evaluation of the eyes may include a visual field test to measure a person’s peripheral vision, an electroretinogram (ERG) to measure the electrical response of the eye’s light-sensitive cells, and a retinal examination to observe the retina and other structures in the back of the eye. A hearing (audiologic) evaluation measures how loud sounds at a range of frequencies need to be before a person can hear them.

Vestibular function tests such as a videonystagmography (VNG) measures involuntary eye movements that help identify a balance  problem.

Early diagnosis of Usher syndrome is very important. The earlier that parents know if their child has Usher syndrome, the sooner that child can begin special educational training programs to manage the loss of hearing and vision.