What are Some Symptoms of Usher Syndome?

The following are some behavioral symptoms that may indicate Usher syndrome and can be used for initial screening purposes. Use the Behavioral Observations form along with this section.

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.

Other

  • 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.