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CMSD5260 Hearing Disorders > Autoimmune and Neurological Disorders > Flashcards

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
How can you manage MD from medical therapy?
Antivertigo agents Steroids Diuretics Vasodilators
26
How do you manage HL from MD?
Amplification Difficult due to fluctuating nature Usually more effective in chronic stable disease Meniett device Local pressure applied Variable results Expensive
27
How can MD be managed by surgical therapy?
Remember Meniere’s disease can be very debilitating Surgical therapy Chemical labyrinthectomy Endolymphatic sac decompression Labyrinthectomy Vestibular nerve section
28
What is the surgical method to treat MD?
Chemical Labyrinthectomy
29
What is the surgical method to treat MD?
Endolymphatic Sac Decompression
30
What is the surgical method to treat MD?
Labyrinthectomy
31
What is the surgical method to treat MD?
Vestibular Nerve Section
32
How important is counseling to MD patients?
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
Explain the blood supply to the vestibular organ and cochlea.
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
What are the essential parts for the balance system?
Inner ear Semicircular canals, vestibule Vision Special sense Proprioception Somatosensory Information is integrated at the brainstem and cerebellum with cortical input
35
What is Nystagmus?
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
What causes Vertigo?
Infection: meningitis, encephalitis, labyrinthitis, neuronitis Central causes (eg strokes, tumors) Peripheral causes Meniere’s disease Benign paroxysmal positional vertigo
37
What is meningitis?
Meningitis-infection/inflammation of the meninges Encephalitis-infection/inflammation of the brain
38
What causes meningitis?
Caused by bacteria or virus Similar symptoms Bacterial meningitis -> antibiotics Viral meningitis -> antivirals
39
What are the clinical features of bacterial meningitis?
High fever Malaise Nausea/vomiting Photophobia/phonophobia Seizures Headaches Restricted neck movement
40
How does bacterial meningitis affect hearing?
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
In bacterial meningitis cases, cochlear implants are an option for bilateral profound SNHL but…
Ossification of labyrinth post-infection/inflammation SNHL Important to rule out when considering cochlear implantation
42
What is Vestibular Neuronitis?
Inflammation of the vestibular nerve Part of the CN8 Usually viral cause History of recent URI No hearing loss Vertigo, nausea, vomiting, nystagmus
43
What is Labyrinthitis?
Inflammation of the entire labyrinth Bacterial or viral Hearing loss and vertigo Nausea/vomiting May lead to labyrinthitis ossificans
44
What is BPPV?
Benign paroxysmal positional vertigo Usually follows head trauma Vertigo Lasts seconds* Induced by head movement Semicircular canal stones No hearing loss
45
How do you diagnose BPPV?
Diagnosis-Dix-Hallpike test Management-Epley maneuver
46
What are the symptoms of vertigo?
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
What are 2 CN7 weaknesses?
Paralysis No movement Paresis Some movement Symptom and NOT a diagnosis Underlying etiology
48
What are the CN7 Weakness-Etiology?
Idiopathic (Bell’s palsy) Infections (OM, Herpes) Congenital Trauma Tumors
49
What is Bell's Palsy?
Diagnosis of exclusion Rule out other causes The most common cause of facial nerve weakness (85%)
50
Explain the epidemiology of Bell's palsy.
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
How does Bell's Palsy affect hearing?
Hearing changes Stapedius muscle can be affected  and impairs speech discrimination in noise Viral neuropathy/inflammation may also affect CN8
52
What is the etiology of Ramsay Hunt Syndrome?
Herpes Zoster Oticus (shingles) Vesicular eruptions, facial palsy, impaired lacrimation 25% will develop SNHL and vestibular symptoms
53
How can brain lesions/tumors affect hearing?
* 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
What are Glomus Tumors?
Aka paragangliomas Rare tumors arising from glomus bodies Glomus bodies serve as baro-receptor cells (oxygen pressure sensor) Usually benign
55
What are the types of Glomus Tumors?
Glomus tympanicum (middle ear) Glomus jugulare (jugular bulb) Carotid body tumor (carotid artery) Glomus vagale (vagus nerve) Other parts of the body
56
What is Glomys Tympanicum?
Confined to middle ear space (can spread locally) Clinical presentation Pulsatile tinnitus, CHL, aural fullness Middle ear lesions may be seen on otoscopy
57
What is Glomus Jugulare?
Arises in the jugular bulb The confluence of veins in the brain Can compress the brain/brainstem SNHL, CHL, or mixed Pulsatile tinnitus
58
IAC = CPA =
IAC = internal auditory canal CPA = cerebellopontine angle
59
Give the types of IAC and CPA tumors. (7)
Acoustic neuroma (vestibular schwannoma)* Meningioma Epidermoid Arachnoid cyst Other neuromas Paragangliomas Metastasis
60
What is Acoustic Neuroma?
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
Explain the epidemiology of Acoustic Neuroma.
Epidemiology Onset between 30-50 years 60% are females 95% are unilateral Bilateral AN -> neurofibromatosis type II (von Recklinghausen disease)
62
What is the clinical presentation of Acoustic Neuroma?
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
What are the audiological features of acoustic neuromas?
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
What is Meningioma?
- 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
What are epidermoid tumors?
-> 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
What is Arachnoid Cyst?
Benign cystic lesion arising from the arachnoid Clinical presentation similar to AN and meningiomas
67
Explain 3 Central Auditory Processing Tasks.
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
What are hearing clinical findings of CAD?
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)

CMSD5260 Hearing Disorders (12 decks)

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