Autoimmune and Neurological Disorders Flashcards

1
Q

The body uses the immune system to fight
________________________________
_________________ are immune system derived proteins that recognize antigens
_____________are unique part of foreign target
____________ and ____________are integral part of immunity

A

The body uses the immune system to fight infections and diseases
Antibodies are immune system-derived proteins that recognize antigens
Antigens are a unique part of foreign target
T cells and B cells are integral parts of immunity

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2
Q

What are T cells?

A

Cell-mediated immunity
T cell receptor recognizes foreign material
Helper T cells
Cytotoxic T cells

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3
Q

What are B cells?

A

Humoral immunity
B cells produce antibodies against antigens

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4
Q

When do autoimmune diseases occur?

A

Occurs when the immune system attacks body’s own organs and cells
Autoantibodies recognize normal body cells and components as foreign antigens

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5
Q

Describe Autoimmune ear diseases (AIED):

A

Autoantibodies attack the inner ear
Inflammation of blood vessels (vasculitis)
SNHL (cochlea)
CHL (ossicles)
Vertigo/imbalance (vestibular organ)

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6
Q

What HL can autoimmune diseases cause?

A

Rapidly progressive or fluctuating SNHL
Rarely CHL
Usually bilateral

Tinnitus
Normal otoscopy

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7
Q

When can Autoimmune Inner Ear Diseases occur?

A

May occur alone or with other autoimmune diseases
Systemic lupus erythematosus (SLE)
Rheumatoid arthritis (RA)
Multiple sclerosis
HIV/AIDS
Meniere’s disease

Many others have been associated with SNHL

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8
Q

What is Lupus?

A

Inflammation of connective tissues and organs and is the most common autoimmune disease

Females&raquo_space; Males
Typically diagnosed in young adults
Much more common in Blacks and Asians

Severity ranges from mild to fatal
Direct attack on inner ear
20% have SNHL
Sudden, fluctuating

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9
Q

What are the features of Lupus?

A

Depends on which antibodies are formed
Skin, joints, kidneys, and lungs most affected
Hepatomegaly
May affect nervous system
Headache, tinnitus
High fevers and malaise
Flares up intermittently

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10
Q

How can we manage Lupus?

A

Refer to MD (rheumatology, ENT for SNHL)
Corticosteroids (potent anti-inflammatory)
Immunosuppressive meds
Non-steroidal anti-inflammatory meds (NSAIDs)
Amplification (rarely CIs)

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11
Q

What is Rheumatoid Arthritis?

A

Autoimmune disorder
Synovial joints attacked by autoantibodies
Synovitis can occur in any synovial joints

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12
Q

What is MS?

A

Autoimmune disease of the CNS
Autoantibodies damage the myelin sheath around axons of the brain and spinal cord

Myelin sheath:
Surrounds axons of neurons
Crucial in transmission

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13
Q

What is the epidemiology of MS?

A

Canada has one of the highest rates of MS in the world
About 133 out of every 100,000 Canadians affected
Most common neurological disease in young adults in Canada
Females&raquo_space; Males

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14
Q

What are the symptoms of MS?

A

Depends on the area of demyelination
Double vision
Loss of balance
Impaired speech
Extreme fatigue
Paresis/paralysis
SNHL

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15
Q

How do you manage MS?

A

Amplification (remember the varying course of disease)
Refer to MD
Corticosteroids
NSAIDs
Other immunosuppressive/biologics

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16
Q

What is HIV?

A

HIV-human immunodeficiency virus
Virus that infects humans

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17
Q

What is AIDS?

A

AIDS-acquired immunodeficiency syndrome
Disease that is caused by HIV
Not all people with HIV have AIDS
Patients with AIDS are immunosuppressed

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18
Q

What are the features of HIV and AIDS?

A

Up to 50% with HIV/AIDS have SNHL
No clear pathophysiology
HIV may directly affect CNS and peripheral nerves

SNHL can also be caused by ototoxic medications or opportunistic infections

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19
Q

What is Meniere’s Disease?

A

Idiopathic condition
Vertigo
Tinnitus
Aural fullness
SNHL

Cyclical in nature
Fluctuating signs and symptoms

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20
Q

What are the onset details of Menier’s Disease?

A

Average onset during the 40’s
Rare before this time

Slight female preponderance

Starts in one ear
Contralateral ear involved between 2-75%
About half have family history

Multi-factorial in origin:
Autoimmune
Genetic
Trauma
Infectious
Hormonal

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21
Q

What is the primary pathophysiology of MD?

A

Primary pathophysiology is endolymphatic hydrops
Too much endolymph causes dilation of vestibule and scala media
May lead to rupture and mixing of endolymph and perilymph

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22
Q

How can MD affect hearing?

A

Often low-frequency SNHL
Often unilateral (initially)
Mild to moderate loss of speech discrimination
Loudness recruitment

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23
Q

How do you manage MD?

A

Dietary
Medical
Surgical

Goal is to control inner ear fluid build up/pressure and improve circulation

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24
Q

How can you manage MD from your diet?

A

Low salt diet
Low cholesterol or triglyceride
Avoid caffeine and tobacco

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25
Q

How can you manage MD from medical therapy?

A

Antivertigo agents
Steroids
Diuretics
Vasodilators

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26
Q

How do you manage HL from MD?

A

Amplification
Difficult due to fluctuating nature
Usually more effective in chronic stable disease

Meniett device
Local pressure applied
Variable results
Expensive

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27
Q

How can MD be managed by surgical therapy?

A

Remember Meniere’s disease can be very debilitating

Surgical therapy
Chemical labyrinthectomy
Endolymphatic sac decompression
Labyrinthectomy
Vestibular nerve section

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28
Q

What is the surgical method to treat MD?

A

Chemical Labyrinthectomy

29
Q

What is the surgical method to treat MD?

A

Endolymphatic Sac Decompression

30
Q

What is the surgical method to treat MD?

A

Labyrinthectomy

31
Q

What is the surgical method to treat MD?

A

Vestibular Nerve Section

32
Q

How important is counseling to MD patients?

A

Patient counseling is very important:
Explain the course of the disease
Tinnitus retraining therapy
Some will recover spontaneously
Some develop chronic and debilitating disease

33
Q

Explain the blood supply to the vestibular organ and cochlea.

A

The vestibular organ and cochlea are supplied by the same blood supply
CN VIII (vestibulocochlear nerve) provides innervation
The damage leads to vertigo and HL

34
Q

What are the essential parts for the balance system?

A

Inner ear
Semicircular canals, vestibule

Vision
Special sense

Proprioception
Somatosensory

Information is integrated at the brainstem and cerebellum with cortical input

35
Q

What is Nystagmus?

A

Oscillatory movement of the eyes caused by brainstem/cerebral problems
Consists of fast and slow components
The direction of nystagmus (right or left) is noted by the fast component
Crucial indicator for the presence of vestibular dysfunction
ENG/VNG

36
Q

What causes Vertigo?

A

Infection: meningitis, encephalitis, labyrinthitis, neuronitis
Central causes (eg strokes, tumors)
Peripheral causes
Meniere’s disease
Benign paroxysmal positional vertigo

37
Q

What is meningitis?

A

Meningitis-infection/inflammation of the meninges

Encephalitis-infection/inflammation of the brain

38
Q

What causes meningitis?

A

Caused by bacteria or virus
Similar symptoms

Bacterial meningitis -> antibiotics
Viral meningitis -> antivirals

39
Q

What are the clinical features of bacterial meningitis?

A

High fever
Malaise
Nausea/vomiting
Photophobia/phonophobia
Seizures
Headaches
Restricted neck movement

40
Q

How does bacterial meningitis affect hearing?

A

Hearing loss
Up to 10% of children
Younger -> higher risk
Pneumococcal meningitis more common
Damages cochlea within few days of infection

All affected should be hearing tests
When stable and 6 months later

41
Q

In bacterial meningitis cases, cochlear implants are an option for bilateral profound SNHL but…

A

Ossification of labyrinth post-infection/inflammation
SNHL
Important to rule out when considering cochlear implantation

42
Q

What is Vestibular Neuronitis?

A

Inflammation of the vestibular nerve
Part of the CN8
Usually viral cause
History of recent URI
No hearing loss
Vertigo, nausea, vomiting, nystagmus

43
Q

What is Labyrinthitis?

A

Inflammation of the entire labyrinth
Bacterial or viral
Hearing loss and vertigo
Nausea/vomiting
May lead to labyrinthitis ossificans

44
Q

What is BPPV?

A

Benign paroxysmal positional vertigo
Usually follows head trauma
Vertigo
Lasts seconds*
Induced by head movement
Semicircular canal stones
No hearing loss

45
Q

How do you diagnose BPPV?

A

Diagnosis-Dix-Hallpike test

Management-Epley maneuver

46
Q

What are the symptoms of vertigo?

A

Symptoms may be severe
Patient may spend days/weeks in bed
Balance may be affected for months

Any reports of persistent vertigo should receive an audiogram and referred

47
Q

What are 2 CN7 weaknesses?

A

Paralysis
No movement

Paresis
Some movement

Symptom and NOT a diagnosis
Underlying etiology

48
Q

What are the CN7 Weakness-Etiology?

A

Idiopathic (Bell’s palsy)
Infections (OM, Herpes)
Congenital
Trauma
Tumors

49
Q

What is Bell’s Palsy?

A

Diagnosis of exclusion
Rule out other causes

The most common cause of facial nerve weakness (85%)

50
Q

Explain the epidemiology of Bell’s palsy.

A

Idiopathic LMN facial palsy
Epidemiology
Incidence of 15-20 per 100,000
Incidence increases with age
Female to male ratio is 1.2:1
Familial tendency
Recurrent cases in 5-10%

51
Q

How does Bell’s Palsy affect hearing?

A

Hearing changes
Stapedius muscle can be affected  and impairs speech discrimination in noise
Viral neuropathy/inflammation may also affect CN8

52
Q

What is the etiology of Ramsay Hunt Syndrome?

A

Herpes Zoster Oticus (shingles)
Vesicular eruptions, facial palsy, impaired lacrimation
25% will develop SNHL and vestibular symptoms

53
Q

How can brain lesions/tumors affect hearing?

A
  • Lesions that occur anywhere along the auditory pathway can cause hearing loss
    Masses/tumors, strokes/bleeds
  • Common tumors associated with hearing loss
    Glomus tumors
    Acoustic neuromas (vestibular schwannomas)
    Meningiomas
54
Q

What are Glomus Tumors?

A

Aka paragangliomas
Rare tumors arising from glomus bodies
Glomus bodies serve as baro-receptor cells (oxygen pressure sensor)
Usually benign

55
Q

What are the types of Glomus Tumors?

A

Glomus tympanicum (middle ear)
Glomus jugulare (jugular bulb)

Carotid body tumor (carotid artery)
Glomus vagale (vagus nerve)
Other parts of the body

56
Q

What is Glomys Tympanicum?

A

Confined to middle ear space (can spread locally)
Clinical presentation
Pulsatile tinnitus, CHL, aural fullness
Middle ear lesions may be seen on otoscopy

57
Q

What is Glomus Jugulare?

A

Arises in the jugular bulb
The confluence of veins in the brain
Can compress the brain/brainstem
SNHL, CHL, or mixed
Pulsatile tinnitus

58
Q

IAC =
CPA =

A

IAC = internal auditory canal
CPA = cerebellopontine angle

59
Q

Give the types of IAC and CPA tumors. (7)

A

Acoustic neuroma (vestibular schwannoma)*
Meningioma
Epidermoid
Arachnoid cyst
Other neuromas
Paragangliomas
Metastasis

60
Q

What is Acoustic Neuroma?

A

Arise from schwann cells of the 8th nerve (vestibular portion)
Schwann cells surrounds the nerve
Starts in IAC and extends into CPA
Benign but locally aggressive
Facial nerve paresis is a late sign

61
Q

Explain the epidemiology of Acoustic Neuroma.

A

Epidemiology
Onset between 30-50 years
60% are females
95% are unilateral

Bilateral AN -> neurofibromatosis type II (von Recklinghausen disease)

62
Q

What is the clinical presentation of Acoustic Neuroma?

A

Progressive symptoms due to slow growing nature of the tumor
Compression causes symptoms (direct effect vs disruption of blood supply)
Rarely, dizziness and other CN findings (late signs)

63
Q

What are the audiological features of acoustic neuromas?

A

Asymmetric SNHL
Abnormal adaptation-inability to sustain responses to pure tones (fatigue)
Impedance-elevated or absent acoustic reflexes
Speech discrimination-rollover effect observed
All asymmetric SNHL must be referred asap

Lawsuits in US

64
Q

What is Meningioma?

A
  • Benign tumor arising from meninges
    Meninges-covering of the CNS
  • Clinical presentation can be similar to AN
    Cochlear, vestibular, CN7, and cerebellar symptoms
    Can occur anywhere along the CNS (speech usually more affected than hearing)
65
Q

What are epidermoid tumors?

A

-> Benign tumors similar to cholesteatoma occurring at the CPA

-> Clinical presentation similar to AN and meningioma
More commonly involves the CN7 (facial twitching/paresis)

66
Q

What is Arachnoid Cyst?

A

Benign cystic lesion arising from the arachnoid

Clinical presentation similar to AN and meningiomas

67
Q

Explain 3 Central Auditory Processing Tasks.

A

Auditory discrimination
Unable to recognize one sound from another (pat-pet)

Auditory association
Unable to relate meaning to a sound

Auditory closure
Inability to complete the missing part of a verbal message (-uper –arket)

Auditory memory
Unable to recall things heard or an auditory sequence (phone numbers)

Auditory localization
Inability to find the source of sound

Auditory figure-ground perception
Inability to isolate sound in noise

68
Q

What are hearing clinical findings of CAD?

A

Standard hearing tests often normal
CAPD test battery
No specific diagnostic criteria

Hearing aids are not typically beneficial
Intensive learning and behavioural therapy centres in the US (Canada)