Autoimmune and Neurological Disorders Flashcards
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
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
What are T cells?
Cell-mediated immunity
T cell receptor recognizes foreign material
Helper T cells
Cytotoxic T cells
What are B cells?
Humoral immunity
B cells produce antibodies against antigens
When do autoimmune diseases occur?
Occurs when the immune system attacks body’s own organs and cells
Autoantibodies recognize normal body cells and components as foreign antigens
Describe Autoimmune ear diseases (AIED):
Autoantibodies attack the inner ear
Inflammation of blood vessels (vasculitis)
SNHL (cochlea)
CHL (ossicles)
Vertigo/imbalance (vestibular organ)
What HL can autoimmune diseases cause?
Rapidly progressive or fluctuating SNHL
Rarely CHL
Usually bilateral
Tinnitus
Normal otoscopy
When can Autoimmune Inner Ear Diseases occur?
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
What is Lupus?
Inflammation of connective tissues and organs and is the most common autoimmune disease
Females»_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
What are the features of Lupus?
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
How can we manage Lupus?
Refer to MD (rheumatology, ENT for SNHL)
Corticosteroids (potent anti-inflammatory)
Immunosuppressive meds
Non-steroidal anti-inflammatory meds (NSAIDs)
Amplification (rarely CIs)
What is Rheumatoid Arthritis?
Autoimmune disorder
Synovial joints attacked by autoantibodies
Synovitis can occur in any synovial joints
What is MS?
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
What is the epidemiology of MS?
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»_space; Males
What are the symptoms of MS?
Depends on the area of demyelination
Double vision
Loss of balance
Impaired speech
Extreme fatigue
Paresis/paralysis
SNHL
How do you manage MS?
Amplification (remember the varying course of disease)
Refer to MD
Corticosteroids
NSAIDs
Other immunosuppressive/biologics
What is HIV?
HIV-human immunodeficiency virus
Virus that infects humans
What is AIDS?
AIDS-acquired immunodeficiency syndrome
Disease that is caused by HIV
Not all people with HIV have AIDS
Patients with AIDS are immunosuppressed
What are the features of HIV and AIDS?
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
What is Meniere’s Disease?
Idiopathic condition
Vertigo
Tinnitus
Aural fullness
SNHL
Cyclical in nature
Fluctuating signs and symptoms
What are the onset details of Menier’s Disease?
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
What is the primary pathophysiology of MD?
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
How can MD affect hearing?
Often low-frequency SNHL
Often unilateral (initially)
Mild to moderate loss of speech discrimination
Loudness recruitment
How do you manage MD?
Dietary
Medical
Surgical
Goal is to control inner ear fluid build up/pressure and improve circulation
How can you manage MD from your diet?
Low salt diet
Low cholesterol or triglyceride
Avoid caffeine and tobacco
How can you manage MD from medical therapy?
Antivertigo agents
Steroids
Diuretics
Vasodilators
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
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
What is the surgical method to treat MD?
Chemical Labyrinthectomy
What is the surgical method to treat MD?
Endolymphatic Sac Decompression
What is the surgical method to treat MD?
Labyrinthectomy
What is the surgical method to treat MD?
Vestibular Nerve Section
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
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
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
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
What causes Vertigo?
Infection: meningitis, encephalitis, labyrinthitis, neuronitis
Central causes (eg strokes, tumors)
Peripheral causes
Meniere’s disease
Benign paroxysmal positional vertigo
What is meningitis?
Meningitis-infection/inflammation of the meninges
Encephalitis-infection/inflammation of the brain
What causes meningitis?
Caused by bacteria or virus
Similar symptoms
Bacterial meningitis -> antibiotics
Viral meningitis -> antivirals
What are the clinical features of bacterial meningitis?
High fever
Malaise
Nausea/vomiting
Photophobia/phonophobia
Seizures
Headaches
Restricted neck movement
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
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
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
What is Labyrinthitis?
Inflammation of the entire labyrinth
Bacterial or viral
Hearing loss and vertigo
Nausea/vomiting
May lead to labyrinthitis ossificans
What is BPPV?
Benign paroxysmal positional vertigo
Usually follows head trauma
Vertigo
Lasts seconds*
Induced by head movement
Semicircular canal stones
No hearing loss
How do you diagnose BPPV?
Diagnosis-Dix-Hallpike test
Management-Epley maneuver
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
What are 2 CN7 weaknesses?
Paralysis
No movement
Paresis
Some movement
Symptom and NOT a diagnosis
Underlying etiology
What are the CN7 Weakness-Etiology?
Idiopathic (Bell’s palsy)
Infections (OM, Herpes)
Congenital
Trauma
Tumors
What is Bell’s Palsy?
Diagnosis of exclusion
Rule out other causes
The most common cause of facial nerve weakness (85%)
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%
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
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
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
What are Glomus Tumors?
Aka paragangliomas
Rare tumors arising from glomus bodies
Glomus bodies serve as baro-receptor cells (oxygen pressure sensor)
Usually benign
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
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
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
IAC =
CPA =
IAC = internal auditory canal
CPA = cerebellopontine angle
Give the types of IAC and CPA tumors. (7)
Acoustic neuroma (vestibular schwannoma)*
Meningioma
Epidermoid
Arachnoid cyst
Other neuromas
Paragangliomas
Metastasis
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
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)
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)
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
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)
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)
What is Arachnoid Cyst?
Benign cystic lesion arising from the arachnoid
Clinical presentation similar to AN and meningiomas
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
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)
