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.
- 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.
- 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).
- 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).
- 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.
(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.