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Advanced Management of Complex Cases

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

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

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

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

5.  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

6.  Valvassori and Clemis are credited with recognizing the clinical relationship between EVA and hearing loss in 1954 1978 1994 2005

7.  The vestibular aqueduct is considered mature when a child is 5 weeks gestation 3 months old 12 months old 3-4 years old

8.  When pure tone testing yields air bone gaps, a VEMP response is typically ______ and ipsilateral acoustic reflexes can be _____ with EVA. absent, present present, present absent, absent present, absent

9.  The combination of EVA, thyroid dysfunction and goiter is known as Usher's syndrome Jervell and Lange-Nielsen syndrome Pendred syndrome Osteogenesis imperfecta

10.  Aural rehabilitation for a patient with EVA would entail amplification cochlear implant FM system with or without amplification and / or cochlear implant Any of the above depending on the degree and impact of hearing loss

11.  Which of the following precludes a child's eligibility for cochlear implant candidacy? Auditory Neuropathy Spectrum Disorder Autism Visual Impairment Absent/aplastic auditory nerve

12.  What is the primary goal for typically developing children receiving cochlear implants? To hear some environmental sounds To enhance lipreading skills To improve hearing for learning auditory based language To learn to talk

13.  What is the most frequent infectious cause of hearing loss in children? Cytomegalovirus Bacterial Meningitis Rubella HIV

14.  What percentage of children with sensorineural hearing loss have additional medical conditions/disabilities? 10-15% 80-85% 30-40% 0-10%

15.  A consideration for a child with Cerebral Palsy who is using a wheelchair would be Can he place the processor on his ear unassisted Is his head position against the back of the wheelchair going to affect placement of the processor or retention of the headpiece Will he be able to swim while wearing the processor Will an electric wheelchair interfere with the processor