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Advanced Management of Complex Cases: Usher Syndrome

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1.  Type 1 Usher Syndrome is characterized by hearing loss not present at birth, but progressive in nature, vision and balance issues may be progressive stable, sloping, moderate-to-severe sensorineural hearing loss, no balance problems, vision problems tend to progress more slowly with the onset of Retinitis Pigmentosa often not apparent until the teens congenital, profound sensorineural hearing loss at birth, vestibular dysfunction and vision loss beginning at age 10 None of the above

2.  Which of the following is NOT a symptom of vestibular dysfunction? head lag W sitting torticollis late crawling

3.  Which of the following are symptoms of Retinitis Pigmentosa (RP)? night blindness tunnel vision blindness all of the above

4.  Ushers Syndrome is caused by autosomal dominant gene autosomal recessive gene all of the above none of the above

5.  What percentage of children with hearing loss have Usher Syndrome? 6% to 8% 3% to 6% 7% to 9% 10% - 12%

6.  How many clinical types of Usher Syndrome have been identified? 3 4 2 5

7.  An evaluation of vision includes which of the following tests? visual field test to measure a person's peripheral vision electroretinogram to measure the electrical response of the eye's light-sensitive cells retinal examination to observe the retina and other structures in the back of the eye all of the above

8.  Type 2 Usher Syndrome is characterized by hearing loss not present at birth, but progressive in nature, vision and balance issues may be progressive stable, moderate-to-severe sensorineural hearing loss, no balance problems, vision problems tend to progress more slowly with the onset of RP often not apparent until the teens. congenital, profound sensorineural hearing loss at birth, vestibular dysfunction and vision loss beginning at age 10 none of the above

9.  How many different genes have been identified for Usher Syndrome? 11 15 10 5

10.  Ushers Syndrome is a rare genetic disorder that is the leading genetic cause of deaf-blindness in North America only Europe only in Asia only in the world