What are Some Symptoms of Usher Syndrome?

The following are some behavioral symptoms that may indicate Usher syndrome and can be used for initial screening purposes.

Hearing Loss

  • Born with profound sensorineural hearing loss for Type I Usher.
  • Have a moderate to severe, sloping sensorineural hearing loss for Type II Usher.
  • Has progressive hearing loss for Type III Usher.

Night Blindness

  • Has difficulty seeing when coming in from bright sunlight.
  • Trips over things when light changes or when light is dim.
  • Stays near light in a dark room or at night.
  • Positions self so light falls on the face of a speaker.
  • Has difficulty seeing seats of people in a darkened room (e.g., movie theater).

Glare Sensitivity

  • Squints and shades eyes in bright lights or fluorescent lighting.
  • Likes to wear sunglasses even in a building, but especially in bright light.
  • May appear awkward when exiting from a building (when faced with bright lights).

Needs Contrast

  • Has difficulty reading light copies or ditto copies.
  • Can't see stars at night.
  • Often spills when pouring liquids.

Restricted Visual Field

  • Stumbles on stairs and curbs.
  • Bumps into people, tables, and chairs, etc.
  • Has accidents at mealtime with objects placed to the side (e.g., spilled glasses).
  • Startles easily, "jumpy".
  • Seems to hold eyes in different directions when looking at some things.
  • Turns head while reading across a page.
  • Uses fingers to mark place while reading.
  • Has difficulty finding small objects that have been dropped.
  • Fails to glance at another person's hand wave from the side.
  • Is quiet or edges to one side when in a large group.
  • Frequently misses or fails to understand group instructions.

Problems with Acuity

  • Holds book close to eyes or bends forward to read.
  • Sits close to blackboard.

Balance Problems

(Especially important for Usher syndrome, Type I)

  • Late learning to walk (past 15 months).
  • Is considered clumsy.
  • Loses balance easily in dark.
  • Could not learn to ride a bicycle or required a long time to learn.
  • May have vestibular loss resulting in balance problems.


  • Is frequently last in completing group activities.
  • Exhibits anxiety in new areas.
  • Often last to enter a room.
  • May have repetitive behavior or routines at particular times.
  • May fail to participate fully in group activities associated with new situations in dark or dimly lit areas (e.g., parties, dances, outdoor games).
  • Frequently hesitates at the top or bottom of the stairs.
  • Avoids walking or running in unfamiliar areas, especially in bright sunlight or darkened areas.
  • Constantly appears to be visually scanning a group.