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AUDE 5410 Aud-Vestib Disorders > ME Disorders (Otosclerosis) > Flashcards

ME Disorders (Otosclerosis) Flashcards

1
Q

what is otosclerosis

A

focal disease, unique to the human temporal bone
insidious and progressive condition

There is ankylosis (fixation) of the stapes footplate to the oval window due to abnormal bony growth

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2
Q

what does it affect

A

otic capsule from where inner ear develops

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3
Q

what is the etiology

A

exact etiology is unknown but it is primarily an active remodeling process of the endochondral (results in cartilage development) layer of the temporal bone

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4
Q

only humans have it

A

true

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5
Q

ALL IS PROGRESSIVE UNTIL YOU DO SURGERY

A

TRUE

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6
Q

~ 70% cases are unilateral but often one ear is affected first

A

false, bilateral

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7
Q

is there normally remodeling of the otic capsule

A

normally once embryo period ends, no remodeling of the otic capsule and what you have is what you hav
no new bone is layed down after birth

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8
Q

can you have fixxation of other ossicles in otosclerosis

A

NO
WHEN FOOTPLATE IS FIXED INTO THE OVAL WINDOW

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9
Q

what is the main site of fixation

A

Fissula ante fenestram

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10
Q

what is teh Fissula ante fenestram

A

A minute slit like passage in the otic labyrinthine wall anterior to the oval window
anterior to oval window, when bone remodeling happens the footplate is ossified into the window

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11
Q

Active remodeling of this bone

A

otosclerosis cause

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12
Q

The degree of the footplate involvement in otosclerosis is

A

highly variable

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13
Q

what is the mode of transmission

A

ad
vertical transmission
goes from generation to generation

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14
Q

In the majority of cases, involvement is limited to

A

anterior portion of the footplate

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15
Q

audio we see originally

A

LF CHL is the result (slowly rising)

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16
Q

when bony fixation of the entire footplate what is audio

A

more flat conductive HL is observed

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17
Q

can you see a flat configuration?”

A

YES

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18
Q

what is obliterative otosclerosis

A

the bony growth may overgrow the footplate

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19
Q

It is not possible by audiologic testing alone to differentiate between otosclerosis of the footplate and obliterative otosclerosis

A

true

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20
Q

why is the difference between them important?

A

management
oto - can put in fake footplate
obl oto - chl can turn into permanent snhl

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21
Q

what are the sites

A

Obliterative otosclerosis of the round window

Cochlear otosclerosis

Histologic otosclerosis

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22
Q

Rare cases of ostosclerosis occurring on the round window

A

Obliterative otosclerosis of the round window

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23
Q

In rare cases, the bony growth spreads to the cochlea, the result is a progressive irreversible SNHL worse in the high frequencies
The bony growth is believed to affect the spiral ligament
The spiral ligament fibrocytes function in conjunction with the stria vascularis to mediate cochlear ion homeostasis

A

Cochlear otosclerosis

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24
Q

10 times more common than large clinical otosclerotic lesions
In this case, lesions do not encroach on stapes footplate or cochlea
They remain small and asymptomatic, discovered only incidentally on histologic examination

A

Histologic otosclerosis

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25
Q

what is the differential diagnosis of otosclerosis

A

osteogenesis imperfecta

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26
Q

what are the stages

A

initial - otospongeosis
intermediate
final inactive - bone stops growing and gets mineralized

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27
Q

primarily a disturbance of physiologic factors that normally serve to inhibit remodeling of the otic capsule

A

otosclerosis

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28
Q

Fixation of other ossicles to each other is NOT otosclerosis, instead it is called

A

ossicular ossification

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29
Q

what is mode of transmission

A

ad

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30
Q

progressive how

A

severity worse over time until it reaches inactive stage

usually starts as unilateral and moves bilateral

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31
Q

traits of ad

A

present in every generation
vertical transmission
complete penetrance (have the genetic predisposition but do not manifest the phenotype) & expressivity (range of severity)

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32
Q

how long do adults wait until getting help with their hearing

A

10-15 years

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33
Q

what could otosclerosis may be related to that is persistent in the otic capsule

A

measles virus

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34
Q

what is some evidence that supports the measles hypothesis

A

Measles viral-like particles found in the osteoblasts and pre-osteoblasts in active otosclerotic lesions

Measles antigen and measles virus genes have been discovered within actively growing otosclerotic lesions

Measles is a disease of humans and closely connected primates; otosclerosis occurs only in humans

A significant decline in otosclerosis was observed with the measles vaccination program

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35
Q

gene associated with type 1 osteogenesis imperfecta

A

COL1A1 gene

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36
Q

Type 1 osteogenesis imperfecta shares some clinical and histological similarities with

A

otosclerosis

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37
Q

_____ of all patients with type 1 osteogenesis imperfecta develop a hearing loss indistinguishable from otosclerosis

A

~ 50%

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38
Q

Some patients with ostosclerosis have _______, a feature found in almost all cases of type 1 osteogenesis imperfecta

A

blue sclera

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39
Q

what are the similarities of osteogenesis imperfecta and otosclerosis

A

share some clinical and histological similarities
around 50% of pts with type 1 develop HL indistinguishable from otosclerosis
blue sclera
histopathology of temporal bones is identical
both inherited as ad

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40
Q

Single most common cause of hearing loss in young adulthood

A

age

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41
Q

In 90% of cases, the age of onset is between

A

15 to 45 yrs

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42
Q

About _____ cases are bilateral

A

70%

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43
Q

2:1 ___ to ____ ratio

A

female, male

44
Q

otosclerosis is most common in

A

white females
rare in asians and blacks

45
Q

n 50% of females, initial awareness/rapid acceleration of the hearing loss occurs

A

during/immediately after pregnancy

46
Q

what is seen on the audio for these

A

bilateral chl or mixed hl with rising configuration
can present unilaterally initially

47
Q

50 to 60% of patients present with __________ which may indicate sensorineural involvement

A

roaring, hissing, or pulsatile tinnitus,

48
Q

what can fluctuationsin tinnitus be caused by

A

related to metabolic or hormonal changes

49
Q

what is paracusis willis

A

People with a conductive hearing loss hear better in noise than normal hearing individuals
This finding may be explained by the fact that in noise generally the intensity of conversation is louder, which makes it easy to hear

50
Q

what is schwartze sign

A

In some cases especially younger adults, increased vascularity of the actively growing bone near the oval window is reflected through the TM as a reddish blush discoloration or glow
reddish glow to the ™

51
Q

what we would see pure tone in early stag3e

A

Normal or mild conductive hearing loss with rising configuration

52
Q

mild stage pure tone results

A

Conductive/mixed hearing loss with rising or flat configuration

53
Q

late stage pure tone results

A

Flattening of the previously rising conductive or mixed hearing loss
It is not uncommon to see a mixed hearing loss especially in older adults, due to presbycusis now accompanying the long standing otosclerosis

54
Q

why do we see flattening out of the rising loss in older patients?

A

because presbycusis is a hf sloping loss

55
Q

what do you run into with bilateral chl

A

masking dilemma

56
Q

what is carhart’s notch

A

BONE ONLY

at 2000 Hz, bc can be 15-20db worse than any f the other frequencies
disappearance or absent abg here

Possibly due to mechanical effects of the disease itself on the auditory system, i.e., the effect of the stapes fixation on the ME resonance

57
Q

do all pt show carhart’s notch

A

no
only <40% of PTs

58
Q

is carhart’s unique to otosclerosis

A

no
only present in <40%
just veause you dont have notch doesnt mean they cant have it
also see in other ossification & osteogenesis imperfecta
otosclerosis is more common than these other ones

59
Q

ONLY me condition that can give you normal tymps

A

otosclerosis

60
Q

what would immittance show

A

Generally normal (Jerger Type A) or As tympanogram, with low admittance and narrow gradient
Abnormal acoustic reflexes in most cases
Reduced stapes mobility, which attenuates stimulus intensity and makes acoustic reflex production difficult
Acoustic reflex decay often cannot be performed due to absence of acoustic reflexes
Tone decay is usually negative (test of retrocochlear pathology)

61
Q

what would speech reveal

A

SRT in agreement with pta
wrs is excellent/good at suprathreshold levels

62
Q

what is the clinical reason to do decay? what are you suspecting?

A

if you think it is a retro lesion
ALWAYS DO CONTRA DECAY - ipsi is not sensitive

63
Q

what are surgical indications for otosclerosis

A

pre operative bone is target
need conductive componenet of at least 25dB HL bw 250-1000 hz on audiogram

64
Q

The bigger the ABG gap the better the prognosis for restored hearing after surgery

A

true

65
Q

what is the steps for surgery with bilateral otosclerosis

A

The poorer ear is operated on first
The second ear is operated on at least one year later if the operated ear remains stable

66
Q

mri or ct for otosclerosis

A

ct is better for this becaues it can see the small bones

67
Q

what is a negative rinne test

A

need - before they proceed with surgery
suggesting a conductive or mixed hearing loss (bone is better than air air)
Positive Rinne test, suggesting normal hearing or SNHL

68
Q

what is surgical indication

A

condition that indicates you need a surgery
before surgery

69
Q

what is a surgical complication

A

follows surgery
surgical complications

70
Q

what are surgical indications

A

SNHL in the contralateral ear is not a contraindication to stapedectomy but does require thoughtful consideration

Cases of advanced otosclerosis are an indication for surgery
Such patients may show dramatic improvement in their speech discrimination abilities following surgery

71
Q

what are conraindications to surgery

A

dead contralateral ear
active OM or OE or TM perf
large exostosis that can affect access to ME

72
Q

what is the abs contraindication of surgery

A

otosclerosis may involve the endolymphatic duct resulting in S/S of Meniere’s disease

73
Q

careful considerations prior to surgery to consider

A

Patients for whom vestibular function is critical for employment
Otologic problems in contralateral ear that may threaten hearing over time
Superior semicircular canal dehiscence (SSCD) syndrome

74
Q

what is a stapedotomy

A

A small hole made in the stapes footplate during surgery

75
Q

what is half stapedectomy

A

Half removal of the stapes footplate during surgery

76
Q

what is total stapedectomy

A

Total removal of stapes footplate during surgery

77
Q

A stainless steel, titanium, platinum, or teflon piston to replace the stapes footplate

A

prosthesis or implant used in stapes surgery

78
Q

No difference in the success rate/outcome between stapedotomy vs. stapedectomy procedures

A

true

79
Q

will you ding at air port checkpoints with this prosthesis?

A

no because the titanium and teflon wont make you ding

80
Q

will mri effect prosthesis?

A

depends on the material
teflon no problem
titanium usually no problem

81
Q

can be performed under local anesthesia with sedation or under general anesthesia

A

Stapedotomy and stapedecetomy

82
Q

how long does Stapedotomy and stapedecetomy take

A

about 30-45 mins
laser surgery is routinely used to vaporize parts of the stapes
remainder of stapes is removed with an instrument

83
Q

tCurrent prostheses are safe with lower power MRI scanners

A

true </= 1.5 tesla

84
Q

prostheses that are compatible with MRI scanners of all strengths

A

Titanium, platinum, and plastic

85
Q

Failure rate of surgery is about ______ %

A

1 to 3% (can result in a profound SNHL)

86
Q

complications of surgery

A

WRS can worsen (up to 30%) if hearing had cochlear involvement
oval window otosclerosis
round window otosclerosis

87
Q

WRS sometimes worsens (by up to 30%) if there was cochlear involvement

A

Stapedectomy can change a the flat mixed hearing loss to a sloping SNHL with poorer WRS
Decreased hearing at 4000 Hz is often observed post surgery

88
Q

what is window otosclerosis

A

Otosclerosis that obliterates the oval window cannot easily be managed or removed with a laser; Other cutting instruments used
Surgery takes longer and it may be difficult to accurately assess the length of the prosthesis needed

89
Q

what is round window otosclerosis

A

Can cause permanent conductive hearing loss
Surgical removal of otosclerosis from a completely obliterated round window universally results in SNHL and should not be attempted

90
Q

what is hyperacusis

A

increased sensitivity to sounds; often temporary

91
Q

Due to VII nerve damage during surgery – rare complication
If the facial nerve is completely filling the oval window niche, surgery may have to be aborted

A

facial paralysis/weakness

92
Q

may have to be sacrificed due to its location

A

chorda tympani

93
Q

what happens if damage to chorda tympani occures

A

Temporary decreased taste/sensation for 3 to 6 months till compensation occurs by the opposite nerve and other taste/sense nerves and mechanisms

94
Q

what is perilymphatic fistual

A

pathologic communication between inner ear and ME

occurs at either round or oval window

can occur during early or late postoperative period

95
Q

what is the result of perilymphatic fistula

A

Fluctuating, sudden, or progressive SNHL
Vertigo
Other symptoms include
tinnitus
disequilibrium
aural fullness

96
Q

what is labyrinthitis

A

Vertigo during or immediately after surgery is indicative of labyrinthine insult
rare but serious

97
Q

what is labyrinthitis caused by

A

Air or blood entering the vestibule
Mechanical trauma to the utricle, which lies in close proximity to the oval window

98
Q

attributed to surgical trauma in approx 1% of cases

A

snhl

99
Q

what can cause immediate chl during complications of surgery

A

Malfunction of prosthesis
Failure to recognize malleus fixation
Round window obliteration
ME effusion
Superior semicircular canal dehiscence (SSCD) syndrome

100
Q

what is SSCD

A

The roof of the superior semicircular canal is missing
A conductive hearing loss similar to otosclerosis can be found in some patients with SSCD

101
Q

Delayed-onset conductive hearing loss
duriing surgery complications

A

Occurs in ~ 5% of successful stapedectomies
Most common cause is erosion of long process of incus with displacement of the prosthesis

102
Q

Patients with otosclerosis do well with amplification as the hearing aid provides the amplification the ME system cannot

A

true

103
Q

what is differential diagnosis for otosclerosis

A

meniere’s disease
osteogenesis imperfecta
SSCD syndrom

104
Q

meniere’s disease

A

Dizziness/vertigo (more common and of much longer duration in Meniere’s disease)
Tinnitus, which can be roaring like otosclerosis
Low frequency hearing loss, which is sensorineural in Meniere’s

105
Q

SSCD syndrome

A

Thinning/absence of part of the bone of the semicircular canal is thought to predispose patients to this syndrome
Low frequency conductive hearing loss (250 to 1000 Hz)

106
Q

how can SSCD be ruled out from being differential for otosclerosis

A

temporal bone CT scan

107
Q

who would surgery not be an option for? they want it but they cannot?

A

age - older - 70-80’s, you do not recover as quickly and have other medical issues and may have chronic issues with age

health issues - dead ear on the opposite side, if you have significant autoimmune conditions, if you already have a chronic infection etc.

PT refuses surgery for any reason

AUDE 5410 Aud-Vestib Disorders (18 decks)

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