Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Acquired Auditory Vestibular Disorders > Autoimmune and Systemic Disorders affecting the auditory system > Flashcards

Autoimmune and Systemic Disorders affecting the auditory system Flashcards

Lectures 12 to 13 (104 cards)

Start Studying
1
Q

what does the immune system protect the body from?

A

Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

a healthy immune system has remarkable ability to distinguish between?

A

bodys own cells (self)
foreign cells (non self)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

When does autoimmunity occur?

A

when bodies powerful immune system that protects the body from invading microorganisms goes away and attacks the body itself

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Instead of protecting the body from external pathogens the immune system produce

A

autoantibodies or T lymphocytes reacting with the hosts own antigens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Cause of autoimmunity possibly interactions of various

A

immunologic, hormonal, genetic and viral factors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Autoimmune diseases can be cause spectrum of human illnesses and affect: gender?

A

Women

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Autoimmune responses can be limited to a single organ

A

Thyroid in Hashimotos disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Autoimmune diseases can cause systemic damage

A

systemic lupus
multiple sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

The endothelial cells of the stria are connected by tight junctions to establish the blood labyrinth barrier that controls movement of

A

circulating inflammatory and other cells/proteins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is an important determinant for the immune response of the inner ear?

A

blood labyrinth barrier

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Immune responses can occur in the inner ear activated by several immunologic mechanisms making the inner ear

A

susceptible to autoimmune conditions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Inner ear vasculature: What do blood vessels carry?

A

Immune cells, inflammatory cells, and hormones that affect inner ear function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is inner ear vasculature responsible for ?

A

delivery of systemic drugs and steroids for inner ear treatment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

The inner ear has extremely high metabolic demands requiring ?

A

A fully functional vascular system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What has unique homeostatic function that requires uncompromised blood flow?

A

stria vascularis and spiral ligament

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

blood labyrinth barrier allows for the endolymph to maintain:

A

a high K+ level required for production and maintenance of the endocochlear potential and normal cochlear function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Disruption of the blood labyrinth barrier due to vascular leakage can lead to ?

A

Immediate hearing loss

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Normal vascular reactions to inflammatory factors that would be harmless for most organs can be be quite harmful for the inner ear and lead to:

A

breakdown of strial integrity
decreased endolymph production
reduced EP levels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Vascular functions in the ear disease(systemic)

  • vasculature carries hormones responsible for
A

normal organ function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Vascular functions in the ear disease (systemic)

  • vasculature carries immune factors from
A

systemic infections and inflammatory disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Vascular functions in the ear disease (systemic)

  • endothelial cells are active participants in tissue response to
A

circulating inflammatory

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Vascular functions in the ear disease (systemic)

  • endothelial cell tight junctions are opened for
A

extracapillary movement of serum factors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Vascular functions in the ear disease (inner ear)

  • vasculature is the connection between
A

the body and ear

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Vascular functions in the ear disease (inner ear)

  • vascular endothelial cells are the
A

gatekeepers to the ear

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Vascular functions in the ear disease (inner ear) - nothing enters the ear without passing through
endothelial cells
26
Vascular functions in the ear disease (inner ear) - what is commonly seen in numerous hearing disorders?
Serum inflammatory factors
27
Vascular functions in the ear disease (inner ear) - breakdown of the BBB is the
first vascular reaction to inflammation
28
Vascular functions in the ear disease (inner ear) - steroids cause BBB restoration by
regulating tight junction genes
29
What is the first line of treatment in autoimmune, inflammatory and infectious conditions of the inner ear?
Steroids (glucocorticoids)
30
steroids can suppress the immune system and can suppress
production of inflammatory cells that cause apoptosis (cell death) of existing cells
31
Pathologies that affect the general metabolic system or biochemical balance of body can affect
the auditory system
32
diseases/disorders that can involve the auditory system include
autoimmune disorders - ms/SSNHL Vascular disorders - wegner granulomatosis Metabolic disorders - diabetes/thyroid
33
What is autoimmune inner ear disease characterized by?
Progressive bilateral SNHL responsive to immunosuppressive agents (corticosteroids)
34
AIED is one of the few cases of SNHL that is
reversible
35
Primary autoimmune inner ear disease
diseases restricted to the ear
36
Secondary autoimmune inner ear disease
multisystemic autoimmune diseases that also involve inner ear EX: Cogan syndrome or WG
37
Autoimmune inner ear disease epidemiology
female preponderance symptoms 25-50
38
Clinical presentation of AIED is
bilateral SNHL aural fullness tinniutus vestibular symptoms
39
Diagnosis of AIED
normal physical examination manifestations of systemic autoimmune disease such as: - effusion in ME and EAC and cough (Wegner) - skin lesions - visual loss (cogans)
40
Treatment of AIED is
corticosteroids
41
how many weeks of corticosteroids and irreversiable damage how many months of onset
~4 weeks ~3 months
42
differential diagnosis of AIED
SSNHL Menieres CN VIII schwannoma MS
43
AIED is typically ____ & _______ While SSNHL is ____ & ____
AIED = progressive and bilateral SSHL = unilateral and not progressive
44
SSHL is defined as
>30 dB HL SNHL occurring in at least three contiguous frequencies within <3 days
45
SSHL is typically unilateral or bilateral ?
UNILATERAL if bi lateral look for different cause
46
For SSNHL if one ear has been affected the other being affected during lifetime increases
4 to 17%
47
SSNHL no gender difference typically in what decade of life?
5th to 6th
48
Etiology of SSNHL
idiopathic hearing loss viral infections tumor ototoxic drugs trauma autoimmune disease meineres drug abuse perilymphatic fistula
49
Histopathologic findings of SSNHL
atrophy of - organ of corti - stria vascularis - tectorial membrane damage at cochlear basal turn
50
Audiologic findings of SSNHL
- no specific audiometric configuration or degree of loss high frequency or flat mild to profound SNHL POOR WRS
51
Vestibular findings of SSNHL
diziness or vertigo
52
SSNHL Immittance
normal tympanogram ARTS consistent with degree of HL
53
Evaluation of SSNHL
case history/ audiologic evaluation
54
for SSNHL MRI is useful to rule out
trauma tumors multiple sclerosis
55
Treatment of SSNHL
Oral corticosteroid therapy for 4 weeks
56
What are consequences of long term corticosteroid therapy
increased appetite and weight gain susceptibility to infection organ damage (blurred vision/upset stomach) bone loss increased hyperglycemia fluid retention/increased blood pressure
57
Prognosis of SSNHL
variable because multifactorial SSNHL can be permenant pts improve with no treatment
58
Indications for good Prognosis of SSNHL
Mild to low frequency SNHL Symptoms persisting for shorter time WRS may not recover
59
Indications for BAD prognosis of SSNHL
Age; very old/young high frequency or flat SNHL vertigo diabetes
60
demyelinating disease =
disease results in damage of of myelin sheath that surrounds nerve fibers in brain and spinal cord
61
demyelinating disease result in
impaired conduction of signals and neurological problems in affected nerves such as impaired - sensation - movement -cognition
62
Some causes of demyelination include
genetics infectous agents autoimmune disease idiopathic
63
Multiple sclerosis is a progressive
neurological autoimmune disorder
64
MS affects the ___ matter pathways
white matter pathways of CNS
65
What enters the brain, spinal cord, or optic and other nerves causing multiple focal areas of inflammation, demyelination, and nerve damage?
Immune cells form blood vessels
66
The focal damaged areas of MS are called
Plaques
67
In MS where are plaques more typical
white matter sparing axons
68
In MS where do plaques show preference ?
optic nerve and optic chiasm
69
Etiology of MS
unknown result of intrinsic (genetics) and extrinsic (environmental factors
70
Epidemiology of MS
common in Caucasians in the northern hemisphere - 10 to 60 years - females
71
Signs and Symptoms of MS
- visual system - paralysis of eye muscles - fatigue and malaise SPINAL CORD AFFECTED 1st - abnormal reflexes and poor coordination - exhibits heat factor -dysarthric of speech and ataxia - HL and vestibular problems
72
Peripheral audiologic signs and symptoms of MS
- high frequency SNHL - bi lateral - low frequency rising SNHL FLUCTUATING HL - in young adults should raise suspicion
73
Central audiologic signs and symptoms in MS
poorer WRS in speech in noise auditory processing deficits - abnormal ABR
74
what is the largest band of white matter in CNS
Corpus Callosum
75
Diagnosis of MS
History high levels of immunoglobulin Imaging - >2 lesions (plaques) in white matter pathways w/ history and clinical presentation
76
Treatment of MS
not cureable - immunosuppressive agents / oral corticosteroids
77
Differential diagnosis
Autoimmune diseases : 1)Susacs syndrome 2) Schilders disease Diabetes Stroke SSNHL
78
Susacs syndrome Characterized by:
self limiting syndrome - cerebral disturbances - visual problems - asymetirc fluctuating SNHL
79
Susacs syndrome often misdiagnosed as MS because of presence of
white matter defects in corpus callosum
80
what are the white matter defects ? Infarcts :
localzied dead tissue due to lack of blood supply
81
Susacs resembles MS because
affetcs women 20 to 40 fluctuating disease progression asymetric SNHL low frequency vertigo and diziness
82
Schilder Disease is (considered a childhood variant of MS)
Progressive degenerative demyelinating disorder of CNS
83
in Schilders disease what is affected
both adrenal gland as well as myelinated nerves and their axons
84
Clinical presentation of Schilder
- personality changes/ poor attention - irreversiable, progressive loss of intellectual function - vision and hearing impairment - balance - headaches/ vommiting/ seizures muscle weakness - atrophy of adrenal glands
85
Diagnosis of Schilder
on MRI to >2 large often bilateral plaques
86
treatment of Schilder
corticosteroids symptomatic treatment
87
Cogans Syndrome is a rare
chronic autoimmune inflammatory disorder
88
Cogan Syndrome epidemiology
young adults 3rd decade of life median age 22 no gender or racial predominance
89
Pathology of Cogans
lymphocyte and plasma cell infiltration of spiral ligament - endolymphatic hydrops - degernative changes organ of corti - new bone formation in the inner ear - demyelination and atrophy of vestibualr and cochlear branches
90
Cogan Syndrome clinical presentation
vestibulocochlear dysfunction and interstitial keratitis
91
Cogan Syndrome vestibular symptoms (generally sudden onset)
Imbalance/vertigo nausea and vommiting ataxia oscillopsia (perception of objects jiggling back and forth after turning head to one side)
92
Cogan Syndrome cochlear symptoms
tinnitus unilateral/bilateral high frequency sloping SNHL
93
Cogan Syndrome Occular
Interstitial Keratitis (red/painful/ light sensitive eyes) - blood vessels grow into cornea blurred and decreased vision
94
non specific systemic Cogan Syndrome
musculoskeletal symtpoms fever fatigue weight loss cardiovascular concerns
95
Cogan Syndrome Diagnosis
inflammatory eye disease and vestibulocochlear dysfunction
96
Cogan syndrome treatment
collabrative medical approach corticosteroids hearing aids cochlear implant vestibular rehabilitation
97
Cogan Syndrome Differential diagnosis
menieres disease 1) eye symptoms absent 2) CS imbalance an ataxia vs menieres objective vertigo 3) systemic manifestations in CS not in menieres Labrinthitis/neuronitits Wegners granulomatosis
98
Wegeners granulomatosis
autoimmune vasculitis affects upper/lower respiratory tracts/ears/ and kidneys
99
Wegeners Granulomatosis audiologic manifestations
swelling of pinna (polychondritits) conductive HL (otitis media and obstruction of nasopharynx) patients w/ SSNHL, wegner should be part of differential diagnosis
100
treatment of wegners granulomatosis
corticosteroids cytotoxic drugs can be fatal w/o treatment
101
Other multisystemic conditions
diabetes hyper tension cigarette smoking chronic alcoholism
102
Diabetes chronic (carbohydrate metabolism) results in blood
hyperglycemia
103
Diabetes manifestation of vascualr changes may affect
stria vascularis spiral ganglion
104
Hypertension blood pressure is
equal or above 140/90 mm HG

Acquired Auditory Vestibular Disorders (3 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions