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Cochlear Implants for Children with Auditory Neuropathy Spectrum Disorder: What Are We Learning?

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1.  1) Audiological findings that characterize ANSD are: a) Normal outer hair cell function as measured by present otoacoustic emissions (OAEs) or the presence of a cochlear microphonic (CM). b) Abnormal auditory nerve response as observed by absent or markedly abnormal ABR c) Typically absent acoustic reflexes d) All the above e) There are no consistent electrophysiological patterns among patients

2.  2) Regarding ANSD, the following statements are true: a) Pure tone thresholds can range from normal to profound b) Speech recognition abilities tend to be disproportionately poorer for the degree of hearing loss c) Hearing aids or cochlear implants do not help patients with ANSD d) Some individuals with AN have little or no communication difficulties while others are functionally deaf e) A, B and D

3.  3) Certain pre and peri natal conditions are known to be strongly associated with ANSD, including: a) Prolonged NICU stay b) Hyperbilirubinemia c) Low birth weight d) Prematurity e) All the above

4.  4) Programming strategies that have proven to be helpful in some cases include a) Using a slower rate of stimulation b) Using a wider pulse width c) Measuring current thresholds and balancing comfort levels across channels d) Measuring pitch matching and eliminating channels with similar percepts e) All the above

5.  5) Audiological management of an infant with ANSD is challenging because: a) All children with ANSD will reject hearing aids b) If infant is identified early with ANSD it may be several months before child's detection thresholds can be adequately measured c) It is not possible to obtain threshold estimates with ABR d) Often, children have other co-morbid conditions that impact development and assessment e) B, C and D

6.  6) Cochlear implant candidacy for children with ANSD... a) Is clearly indicated for all children once identified b) Includes the recommendation for bilateral simultaneous implantation c) May be a process with the decision determined after monitoring progress over time d) Should be based on pure tone audiometric thresholds per FDA approved guidelines e) is always covered by commercial insurance, no questions asked!

7.  7) MRI is an important diagnostic tool of when determining cochlear implant candidacy because cochlear nerve deficiency appears to be the root cause of ANSD in a) 75% of known cases b) 50% of known cases c) 25% of known cases d) 5% of known cases e) it is not important

8.  8) Outcomes for children with ANSD tend to a) be similar for all patients b) may be predicted prior to surgery and device activation c) are affected by the presence of other medical and developmental concerns d) always result in improvements in speech perception performance e) be undifferentiated from children with SNHL

9.  9) Cochlear implant management of children with ANSD a) requires thorough counseling about possible outcomes b) should include discussion of the use of visual support for language development c) may require more frequent programming sessions d) should include monitoring using a standard speech perception test battery e) all the above

10.  10) A preliminary study at UNC of pediatric CI users with ANSD suggests that: a) electrically evoked auditory event-related potentials (eERPs)can be recorded from cochlear implant users who have ANSD b) Responses for patients with poorer speech perception performance is different from those who are better performers c) Waveform morphology from patients who were good performers was comparable to those from matched peers who have SNHL. d) Other electrically evoked auditory potentials hold promise as predictors of speech perception performance in these patients. e) All the above