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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
Q

enlarged vestibular aqueduct

A

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
Q

postnatal / environmental causes

A

infections

ototoxicity

noise induced hearing loss

Meniere’s disease

presbycusis

27
Q

infection - meningitis

A

inflammation of the meninges

many causes (viral or bacterial)

can be fatal

28
Q

meningitis HL

A

usually bilateral SNHL

can be unilateral

variable configuration & degree

29
Q

ototoxicity

A

from antibiotics or cancer treatment

extended high freq audiometry & OAEs can monitor HL & be an early indicator of HL

30
Q

significant shift in high freq threshold due to ototoxicity is

A

> 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
Q

noise induced HL

A

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
Q

time/intensity tradeoff

A

semi loud sounds cause HL over a longer time of exposure

very loud sounds can cause instant hearing loss

33
Q

general recommendation for safe listening

A

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
Q

Meniere’s Disease symptoms

A

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
Q

meniere’s cause

A

generally unknown

thought to be over secretion or under absorption of endolymph in sac

36
Q

meniere’s treatment

A

low sodium diet historically - doesn’t work

full labyrinthectomy used for severe cases
gives you full HL in that ear

37
Q

presbycusis

A

HL due to aging

SNHL

sloping

bilateral

symmetrical

slow progression

38
Q

what is the most common type of HL

A

presbycusis

39
Q

vestibular schwannoma

A

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
Q

vestibular schwannoma symptoms

A

tinnitus

SNHL

absent ARTs

poor speech perception despite normal thresholds (&/or rollover)

vertigo & balance problems

41
Q

auditory neuropathy spectrum disorder

A

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
Q

ANSD symptoms

A

hearing loss

poor speech perception

difficulty hearing in noise

not consistent w/ audiogram

43
Q

ANSD treatment

A

controvertial

may implant

44
Q

central auditory processing disorder symptoms

A

general difficulty processing speech or auditory scene

45
Q

CAPD signs

A

difficulty w/ supra threshold psychoacoustic processing

including temporal & binaural integration & auditory discrimination

46
Q

CAPD diagnosis

A

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
Q

non organic HL

A

faking

usually for compensation (adults) or attention (teens)

48
Q

cues for suspecting non organic HL

A

case history

inconsistency on hearing tests
hard to hold on to a specific loudness in your head

disagreement between speech reception thresholds & PTA

49
Q

what should an AuD if suspected faking

A

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
Q

stenger test

A

can be used to detect non organic HL w/ difference of at least 20 dB HL between ears

51
Q

stenger test stimulus

A

tones or spondees presented at same time in both ears

52
Q

stenger test level

A

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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