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.

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.