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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

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Preparativo Vestibular e ENEM flashcards
1
Q

what does the immune system protect the body from?

A

Disease

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2
Q

a healthy immune system has remarkable ability to distinguish between?

A

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

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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

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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

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5
Q

Cause of autoimmunity possibly interactions of various

A

immunologic, hormonal, genetic and viral factors

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6
Q

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

A

Women

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7
Q

Autoimmune responses can be limited to a single organ

A

Thyroid in Hashimotos disease

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8
Q

Autoimmune diseases can cause systemic damage

A

systemic lupus
multiple sclerosis

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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

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10
Q

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

A

blood labyrinth barrier

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11
Q

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

A

susceptible to autoimmune conditions

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12
Q

Inner ear vasculature: What do blood vessels carry?

A

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

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13
Q

What is inner ear vasculature responsible for ?

A

delivery of systemic drugs and steroids for inner ear treatment

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14
Q

The inner ear has extremely high metabolic demands requiring ?

A

A fully functional vascular system

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15
Q

What has unique homeostatic function that requires uncompromised blood flow?

A

stria vascularis and spiral ligament

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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

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17
Q

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

A

Immediate hearing loss

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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

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19
Q

Vascular functions in the ear disease(systemic)

  • vasculature carries hormones responsible for
A

normal organ function

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20
Q

Vascular functions in the ear disease (systemic)

  • vasculature carries immune factors from
A

systemic infections and inflammatory disease

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21
Q

Vascular functions in the ear disease (systemic)

  • endothelial cells are active participants in tissue response to
A

circulating inflammatory

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22
Q

Vascular functions in the ear disease (systemic)

  • endothelial cell tight junctions are opened for
A

extracapillary movement of serum factors

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23
Q

Vascular functions in the ear disease (inner ear)

  • vasculature is the connection between
A

the body and ear

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24
Q

Vascular functions in the ear disease (inner ear)

  • vascular endothelial cells are the
A

gatekeepers to the ear

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25
Q

Vascular functions in the ear disease (inner ear)

  • nothing enters the ear without passing through
A

endothelial cells

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26
Q

Vascular functions in the ear disease (inner ear)

  • what is commonly seen in numerous hearing disorders?
A

Serum inflammatory factors

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27
Q

Vascular functions in the ear disease (inner ear)

  • breakdown of the BBB is the
A

first vascular reaction to inflammation

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28
Q

Vascular functions in the ear disease (inner ear)

  • steroids cause BBB restoration by
A

regulating tight junction genes

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29
Q

What is the first line of treatment in autoimmune, inflammatory and infectious conditions of the inner ear?

A

Steroids (glucocorticoids)

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30
Q

steroids can suppress the immune system and can suppress

A

production of inflammatory cells that cause apoptosis (cell death) of existing cells

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31
Q

Pathologies that affect the general metabolic system or biochemical balance of body can affect

A

the auditory system

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32
Q

diseases/disorders that can involve the auditory system include

A

autoimmune disorders
- ms/SSNHL

Vascular disorders
- wegner granulomatosis

Metabolic disorders
- diabetes/thyroid

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33
Q

What is autoimmune inner ear disease characterized by?

A

Progressive bilateral SNHL
responsive to immunosuppressive agents (corticosteroids)

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34
Q

AIED is one of the few cases of SNHL that is

A

reversible

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35
Q

Primary autoimmune inner ear disease

A

diseases restricted to the ear

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36
Q

Secondary autoimmune inner ear disease

A

multisystemic autoimmune diseases that also involve inner ear
EX: Cogan syndrome or WG

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37
Q

Autoimmune inner ear disease epidemiology

A

female preponderance
symptoms 25-50

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38
Q

Clinical presentation of AIED is

A

bilateral SNHL
aural fullness
tinniutus
vestibular symptoms

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39
Q

Diagnosis of AIED

A

normal physical examination
manifestations of systemic autoimmune disease such as:
- effusion in ME and EAC and cough (Wegner)
- skin lesions
- visual loss (cogans)

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40
Q

Treatment of AIED is

A

corticosteroids

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41
Q

how many weeks of corticosteroids and irreversiable damage how many months of onset

A

~4 weeks
~3 months

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42
Q

differential diagnosis of AIED

A

SSNHL
Menieres
CN VIII schwannoma
MS

43
Q

AIED is typically ____ & _______
While SSNHL is ____ & ____

A

AIED = progressive and bilateral
SSHL = unilateral and not progressive

44
Q

SSHL is defined as

A

> 30 dB HL SNHL occurring in at least three contiguous frequencies within <3 days

45
Q

SSHL is typically unilateral or bilateral ?

A

UNILATERAL
if bi lateral look for different cause

46
Q

For SSNHL if one ear has been affected the other being affected during lifetime increases

A

4 to 17%

47
Q

SSNHL no gender difference typically in what decade of life?

A

5th to 6th

48
Q

Etiology of SSNHL

A

idiopathic hearing loss
viral infections
tumor
ototoxic drugs
trauma
autoimmune disease
meineres
drug abuse
perilymphatic fistula

49
Q

Histopathologic findings of SSNHL

A

atrophy of
- organ of corti
- stria vascularis
- tectorial membrane
damage at cochlear basal turn

50
Q

Audiologic findings of SSNHL

A
  • no specific audiometric configuration or degree of loss

high frequency or flat mild to profound SNHL

POOR WRS

51
Q

Vestibular findings of SSNHL

A

diziness or vertigo

52
Q

SSNHL Immittance

A

normal tympanogram
ARTS consistent with degree of HL

53
Q

Evaluation of SSNHL

A

case history/ audiologic evaluation

54
Q

for SSNHL MRI is useful to rule out

A

trauma
tumors
multiple sclerosis

55
Q

Treatment of SSNHL

A

Oral corticosteroid therapy for 4 weeks

56
Q

What are consequences of long term corticosteroid therapy

A

increased appetite and weight gain
susceptibility to infection
organ damage (blurred vision/upset stomach)
bone loss
increased hyperglycemia
fluid retention/increased blood pressure

57
Q

Prognosis of SSNHL

A

variable because multifactorial
SSNHL can be permenant
pts improve with no treatment

58
Q

Indications for good Prognosis of SSNHL

A

Mild to low frequency SNHL
Symptoms persisting for shorter time
WRS may not recover

59
Q

Indications for BAD prognosis of SSNHL

A

Age; very old/young
high frequency or flat SNHL
vertigo
diabetes

60
Q

demyelinating disease =

A

disease results in damage of of myelin sheath that surrounds nerve fibers in brain and spinal cord

61
Q

demyelinating disease result in

A

impaired conduction of signals and neurological problems in affected nerves such as impaired
- sensation
- movement
-cognition

62
Q

Some causes of demyelination include

A

genetics
infectous agents
autoimmune disease
idiopathic

63
Q

Multiple sclerosis is a progressive

A

neurological autoimmune disorder

64
Q

MS affects the ___ matter pathways

A

white matter pathways of CNS

65
Q

What enters the brain, spinal cord, or optic and other nerves causing multiple focal areas of inflammation, demyelination, and nerve damage?

A

Immune cells form blood vessels

66
Q

The focal damaged areas of MS are called

A

Plaques

67
Q

In MS where are plaques more typical

A

white matter sparing axons

68
Q

In MS where do plaques show preference ?

A

optic nerve and optic chiasm

69
Q

Etiology of MS

A

unknown
result of intrinsic (genetics) and extrinsic (environmental factors

70
Q

Epidemiology of MS

A

common in Caucasians in the northern hemisphere
- 10 to 60 years
- females

71
Q

Signs and Symptoms of MS

A
  • 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
Q

Peripheral audiologic signs and symptoms of MS

A
  • high frequency SNHL
  • bi lateral
  • low frequency rising SNHL
    FLUCTUATING HL - in young adults should raise suspicion
73
Q

Central audiologic signs and symptoms in MS

A

poorer WRS in speech in noise
auditory processing deficits
- abnormal ABR

74
Q

what is the largest band of white matter in CNS

A

Corpus Callosum

75
Q

Diagnosis of MS

A

History
high levels of immunoglobulin
Imaging
- >2 lesions (plaques) in white matter pathways w/ history and clinical presentation

76
Q

Treatment of MS

A

not cureable
- immunosuppressive agents / oral corticosteroids

77
Q

Differential diagnosis

A

Autoimmune diseases :
1)Susacs syndrome
2) Schilders disease
Diabetes
Stroke
SSNHL

78
Q

Susacs syndrome
Characterized by:

A

self limiting syndrome
- cerebral disturbances
- visual problems
- asymetirc fluctuating SNHL

79
Q

Susacs syndrome often misdiagnosed as MS because of presence of

A

white matter defects in corpus callosum

80
Q

what are the white matter defects ? Infarcts :

A

localzied dead tissue due to lack of blood supply

81
Q

Susacs resembles MS because

A

affetcs women 20 to 40
fluctuating disease progression
asymetric SNHL low frequency
vertigo and diziness

82
Q

Schilder Disease is
(considered a childhood variant of MS)

A

Progressive degenerative demyelinating disorder of CNS

83
Q

in Schilders disease what is affected

A

both adrenal gland as well as myelinated nerves and their axons

84
Q

Clinical presentation of Schilder

A
  • 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
Q

Diagnosis of Schilder

A

on MRI to >2 large often bilateral plaques

86
Q

treatment of Schilder

A

corticosteroids
symptomatic treatment

87
Q

Cogans Syndrome is a rare

A

chronic autoimmune inflammatory disorder

88
Q

Cogan Syndrome epidemiology

A

young adults
3rd decade of life
median age 22
no gender or racial predominance

89
Q

Pathology of Cogans

A

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
Q

Cogan Syndrome clinical presentation

A

vestibulocochlear dysfunction and interstitial keratitis

91
Q

Cogan Syndrome vestibular symptoms
(generally sudden onset)

A

Imbalance/vertigo
nausea and vommiting
ataxia
oscillopsia (perception of objects jiggling back and forth after turning head to one side)

92
Q

Cogan Syndrome cochlear symptoms

A

tinnitus
unilateral/bilateral high frequency sloping SNHL

93
Q

Cogan Syndrome Occular

A

Interstitial Keratitis (red/painful/ light sensitive eyes)
- blood vessels grow into cornea
blurred and decreased vision

94
Q

non specific systemic Cogan Syndrome

A

musculoskeletal symtpoms
fever
fatigue
weight loss
cardiovascular concerns

95
Q

Cogan Syndrome Diagnosis

A

inflammatory eye disease and vestibulocochlear dysfunction

96
Q

Cogan syndrome treatment

A

collabrative medical approach
corticosteroids
hearing aids
cochlear implant
vestibular rehabilitation

97
Q

Cogan Syndrome Differential diagnosis

A

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
Q

Wegeners granulomatosis

A

autoimmune vasculitis affects upper/lower respiratory tracts/ears/ and kidneys

99
Q

Wegeners Granulomatosis
audiologic manifestations

A

swelling of pinna (polychondritits)
conductive HL (otitis media and obstruction of nasopharynx)

patients w/ SSNHL, wegner should be part of differential diagnosis

100
Q

treatment of wegners granulomatosis

A

corticosteroids
cytotoxic drugs
can be fatal w/o treatment

101
Q

Other multisystemic conditions

A

diabetes
hyper tension
cigarette smoking
chronic alcoholism

102
Q

Diabetes chronic (carbohydrate metabolism) results in blood

A

hyperglycemia

103
Q

Diabetes manifestation of vascualr changes may affect

A

stria vascularis
spiral ganglion

104
Q

Hypertension blood pressure is

A

equal or above 140/90 mm HG

Acquired Auditory Vestibular Disorders (3 decks)

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