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CS&D 320 > lecture 15: disorders of the inner ear, nerve, & central pathway - exam 3 > Flashcards

lecture 15: disorders of the inner ear, nerve, & central pathway - exam 3 Flashcards

1
Q

prenatal

A

congenital

disorder occurs before birth

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2
Q

postnatal

A

acquired

after birth

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3
Q

prenatal causes

A

malformations

genetic –> syndromic & non-syndromic

environmental

most etiologies of prenatal hearing loss are unknown – 64%

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4
Q

michel / complete labyrinthine aplasia

A

complete failure of development of inner ear & auditory nerve –> NO COCHLEA

pretty rare

can be unilateral, bilateral, or one type on one side & another type on the other side

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5
Q

mondini dysplasia

A

incomplete development & malformation of the inner ear –> PARTIAL COCHLEA

1-1.5 turns of cochlea (should be 2.5)

more common than complete

can be unilateral, bilateral, or one type on one side & another type on the other side

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6
Q

treatment for complete aplasia

A

ASL

vibrotactile aid

auditory brainstem implant

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7
Q

treatment for mondini aplasia

A

varies depending on severity

hearing aids often sufficient

can require CI & sometimes split array CI

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8
Q

superior canal dehiscence

A

opening in the bone that covers the superior semicircular canal of the inner ear

disrupts vestibular system
- cant keep eyes straight while moving
- loud sounds cause dizziness too

SNHL

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9
Q

genetic causes of hearing loss

A

50-60% of children w hearing loss (known cause)

can be syndromic (30%) or nonsyndromic (70%)

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10
Q

congenital hearing loss branches

A

environment - 25%

genetic - 60%

other - 15%

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11
Q

syndromic

A

hearing loss associated w/ other symptoms

400 syndromes associated w/ hearing loss

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12
Q

non-syndromic

A

hearing loss not associated w/ any other symptoms

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13
Q

prelingual

A

occurs before the child has developed language

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14
Q

pendred syndrome

A

SNHL from mondini dysplasia
- SNHL typically progressive

can co-occur w/ conductive HL if additional ear malformations

goitre – neck swelling from thyroid dysfunction

autosomal recessive

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15
Q

waardenburg syndrome presentation

A

heterochromia or distinct blue eyes

white forelock of hair

prominent nasal root

SNHL

have a relative w/ waardenburg –> tends to get worse w/ each generation

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16
Q

waardenburg affects

A

formation of melanocytes

create melanin - important for inner ear function

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17
Q

waardenburg types

A

several

not all cause hearing loss but when it does it is present & birth & generally stable

most autosomal dominant

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18
Q

usher syndrome presentation

A

SNHL & retinitis pigmentosa

(night blindness, loss of peripheral vision)

vision loss can be progressive

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19
Q

what is the most common cause of deafblindness

A

usher syndrome

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20
Q

usher syndrome hearing loss

A

SNHL

profound at birth or less sever but progressive

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21
Q

brachio-oto-renal syndrome

A

preauricular pits/tags

branchial cleft cysts (neck masses)

abnormal kidney function

SNHL (& sometimes mixed)
stable

caused by EYA1 gene mutation

autosomal dominant

22
Q

Connexin 26

A

40-50% of non-syndromic genetic HL

mutation of GJB2 gene makes connexin
cells along BM have underdeveloped gap junctions

almost always congenital - severe to profound
late onset possible - mild & progressive

SNHL
stable

autosomal recessive

happens a lot w incest

23
Q

what is the most cause of genetic hearing loss

A

connexin 26

24
Q

vestibular aqueduct

A

canal between inner ear & skull that carries endolymph to sac

25
enlarged vestibular aqueduct
when sac is too large endolymph can flow back into the cochlea & mix w/ perilymph (kills hair cells) SNHL or mixed HL, progressive can look conductive can occur on its own or w/ other conditions (pendred, mondini, etc) autosomal recessive
26
postnatal / environmental causes
infections ototoxicity noise induced hearing loss Meniere's disease presbycusis
27
infection - meningitis
inflammation of the meninges many causes (viral or bacterial) can be fatal
28
meningitis HL
usually bilateral SNHL can be unilateral variable configuration & degree
29
ototoxicity
from antibiotics or cancer treatment extended high freq audiometry & OAEs can monitor HL & be an early indicator of HL
30
significant shift in high freq threshold due to ototoxicity is
>20 dB change at 1 freq >10 dB change at 2 adjacent freqs loss of response at 3 consecutive freqs where there were previously responses
31
noise induced HL
caused by exposure to loud sound high freqs, OAEs, ABR, & speech in noise perception may all be affected before HL detected on audiogram threshold change can be temporary or permanent, never fully returns to normal not always symmetrical
32
time/intensity tradeoff
semi loud sounds cause HL over a longer time of exposure very loud sounds can cause instant hearing loss
33
general recommendation for safe listening
85 dB or less for 8 hours a day 3 dB exchange rate = for every increase in sound level of 3 dB, safe time cut in half
34
Meniere's Disease symptoms
low freq often fluctuating (only cases of fluctuating SNHL) need multiple audiograms to diagnose SNHL usually unilateral but can be bilateral progressive roaring, low freq tinnitus vertigo (can be very severe) feeling of pressure in ears
35
meniere's cause
generally unknown thought to be over secretion or under absorption of endolymph in sac
36
meniere's treatment
low sodium diet historically - doesn't work full labyrinthectomy used for severe cases gives you full HL in that ear
37
presbycusis
HL due to aging SNHL sloping bilateral symmetrical slow progression
38
what is the most common type of HL
presbycusis
39
vestibular schwannoma
benign tumor caused by overgrowth of Schwann cells on CN VIII covers AN usually unilateral except this one disease that causes them all over your body
40
vestibular schwannoma symptoms
tinnitus SNHL absent ARTs poor speech perception despite normal thresholds (&/or rollover) vertigo & balance problems
41
auditory neuropathy spectrum disorder
thought to be lesion in the synapses between IHCs & CN VIII or on CN VIII itself difficult to diagnose, no gold standard present OAEs w/ abnormal ABR audiometry highly variable comorbid, less common on its own
42
ANSD symptoms
hearing loss poor speech perception difficulty hearing in noise not consistent w/ audiogram
43
ANSD treatment
controvertial may implant
44
central auditory processing disorder symptoms
general difficulty processing speech or auditory scene
45
CAPD signs
difficulty w/ supra threshold psychoacoustic processing including temporal & binaural integration & auditory discrimination
46
CAPD diagnosis
no gold standard or known cause difficult to conclude domain specificity --> ear, brain, both? audiograms & objective tests usually noral testing varies but usually involves a series of psychoacoustic tasks
47
non organic HL
faking usually for compensation (adults) or attention (teens)
48
cues for suspecting non organic HL
case history inconsistency on hearing tests hard to hold on to a specific loudness in your head disagreement between speech reception thresholds & PTA
49
what should an AuD if suspected faking
objective tests BC forehead test -- can't tell which ear they hear it in children - say yes if you heard it & no if you didn't
50
stenger test
can be used to detect non organic HL w/ difference of at least 20 dB HL between ears
51
stenger test stimulus
tones or spondees presented at same time in both ears
52
stenger test level
10 dB above threshold (of SRT) in better ear & 10 dB below threshold in worse ear think they are heading tone just in their worse ear because that one is louder if true unilateral HL - they will still respond bcs they are hearing the tone/spondees in better ear

CS&D 320 (19 decks)

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