lec 6 Flashcards

1
Q

50% of hearing loss is genetic. what are the causes of the other 50%? (2)

A
  • non-genetic factors (examples: oxygen deprivation, syphillis)
  • idiopathic causes
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2
Q

out of the genetic cases of hearing loss, what percentage are syndromic?

A

30%

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3
Q

T or F: most genetic hearing loss is due to dominant genes

A
  • false! 70% recessive, 15% dominant, 15% other genetic
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4
Q

half of the cases of hearing loss in developed countries are from issues with the ___ gene.

A

GJB2

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5
Q

treacher collins:
a) is hearing loss associated with TC syndromic or non-syndromic?
b) list some symptoms of TC

A

a) syndromic
b) microtia/melotia/anotia, micrognathia, maxillary hypoplasia, undersized cheek bones, eye anomalies

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6
Q

T or F: auditory evoked potentials (AEPs) and otoacoustic emissions (OAEs) are behavioural tests

A
  • false! they are non-behavioural (do not require active response from person)
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7
Q

what are AEPs? (2)

A
  • auditory evoked potentials measure change in electrical activity of brain in response to sound.
  • response is small, so it is amplified and stored.
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8
Q

AEPs: frequency specific results are more accurate in the ___ frequencies (near field).

A

higher

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9
Q

what are the 4 AEPs we discussed? which is the main one SLPs see?

A
  1. auditory brainstem response (ABR) (main one SLPs see)
  2. auditory middle latency response (AMLR)
  3. late/cortical
  4. event-related potentials (ERP)
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10
Q

a) how fast are ABRs?
b) which part of the brain are they associated with?
c) what is the key measure of ABR?
d) which frequencies is ABR more accurate for?

A

a) 10-15 msec
b) 8th nerve-midbrain
c) wave V = threshold for what person is hearing (although slightly worse than true threshold)
d) higher Hz

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11
Q

why is ABR ideal for babies?

A
  • because it is unaffected by state of consciousness
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12
Q

a) how fast are middle AEPs?
b) which part of the brain are they associated with?
c) what are middle AEPs useful for?
d) how do myogenic responses relate?

A

a) 15-60 msec
b) auditory cortex
c) diagnostic markers for learning issues, depth of anesthesia, autism… (care about Pa measure)
d) myogenic response occurs in same time period

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13
Q

are middle AEPs useful for babies? why or why not?

A
  • not useful for babies because person must be alert
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14
Q

a) how fast are late/cortical AEPs?
b) which part of the brain are they associated with?
c) what are late/cortical AEPs useful for?
d) what are they measuring?

A

a) 70-250 msec
b) primary auditory cortex
c) biomarkers for CAPD, attention, perception, SLP treatment efficacy…
d) measuring Hz-specific thresholds (care about P2 amplitude)

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15
Q

P300: diff bw P3a and P3b response?

A
  • P3a: response to rare event
  • P3b: onset of rare, task-relevant stimulus
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16
Q

N400: do correct or incorrect responses invoke greater responses?

A
  • incorrect (implying person understands it was incorrect)
17
Q

P600: how is this response elicited? what is it called?

A
  • by hearing/reading grammatical/syntactical errors
  • called syntactic positive shift (SPS)
18
Q

what is ASSR? is it more or less accurate than ABR?

A
  • auditory steady-state response
  • presents 500, 1000, 2000, and 4000 Hz bilaterally
  • more accurate than ABR
19
Q

what causes spontaneous OAEs? are these electrical events?

A
  • caused by movement of outer hair cells
  • acoustic events
20
Q

what causes evoked OAEs? what are the physiological necessities for evoked OAEs (2)?

A
  • caused by introduction of a sound
  • require normal OHC function + clear outer and middle ear
21
Q

if OAEs are present, we can assume 3 things, what are they?

A
  1. suggests OHC responding region of the cochlea is normal (no info about other structures)
  2. suggests no more than a mild hearing loss
  3. suggests little or no conductive loss
22
Q

pros (3) vs cons (2) of OAEs?

A
  • pros: fast, infant-friendly, diff bw sensory and neural lesions.
  • cons: potential mild loss, misses auditory neuropathy spectrum disorder.
23
Q

what is ANSD?

A
  • inner ear receives sounds but info leaving cochlea is disrupted in some way.
  • abnormal temporal encoding and neural asynchrony.
24
Q

T or F: someone born without an auditory nerve will fail an OAE test

A
  • false they would actually pass
25
Q

what are 3 risk factors for ANSD?

A
  1. hyperbilirubinemia at birth
  2. oxygen deprivation during/before birth
  3. shaken baby syndrome
26
Q

how does ANSD present? (3)

A
  • bilateral hearing loss from mild to profound
  • fluctuating hearing is possible
  • delayed or no speech development
27
Q

what do these findings suggest:
1. OAEs are present and typical.
2. ABR waves are absent or abnormal.
3. Cannot generate audiogram using ASSR.
4. inverted ABR waveform.
5. present cochlear microphonic

A

ANSD!

28
Q

what is the one “treatment” that is accessible for ALL children w/ ANSD?

A

manual language

29
Q

what are recommendations for children w/ ANSD?

A
  • regular assessments within first year of life
  • MRI to determine presence of auditory nerve
  • consultation with neurologist + eye specialist
  • ongoing speech and language therapy