Autoimmune/Systemic Disorders

Question 1

Autoimmune Inner Ear Disease (AIED)

Answer 1

Divided into two categories:
Primary (disease in the ear)
Secondary (disease that involves the ear)
Typically presents with bilateral SNHL that may be REVERSIBLE with corticosteroids
Characterized by
- progressive bilateral SNHL
- aural fullness
- tinnitus
- vestibular symptoms
Example secondary AIED include:
- Cogan’s syndrome
- Wegener granulomatosis
Differential Dx:
- Sudden SNHL
- Meniere’s disease
- Vestibular schwannoma
- MS
- otosyphilis

Question 2

Sudden Sensorineural Hearing Loss (SSHL)

Answer 2

A sudden (within 3 days) and SNHL of at least 3 contiguous freqs of >/= 30 dB HL
It is typically unilateral
Etiology includes many factors, most commonly idiopathic, from a viral infection, or vascular disorder
Treatment includes corticosteroids or intratympanic steroids

Question 3

Multiple Sclerosis (MS)

Answer 3

Progressive neurological autoimmune disorder. Demyelination of the myelin sheaths in the CNS causing impairment of sensation, movement, cognition, and any other nerves affected.
May be caused by either genetics (intrinsic), infectious agents (extrinsic), autoimmune disease, or idiopathic.
Plaques (scars) are a hallmark of MS and affect the white matter pathways of the CNS.
S/S include:
- variable SNHL (some present low freq rising to high)
- variable ARTs
- vestibular problems
- poor WRS from cochlear nuclei and central neural pathways being affected
- abnormal ABRs
- paralysis of eye muscles
- fatigue
- poor reflexes
- numbness, tingling, paresthesia
- 1 degree C above normal body temp
- dysarthric speech
- ataxia
Cannot be cures, treat the symptoms
Differential Dx:
- Susac’s syndrome
- Schilder’s disease
- Stroke
- SSHL

Question 4

Susac Syndrome

Question 5

Schlider’s Disease

Question 6

Cogan Syndrome

Question 7

Wegener’s Granulomatosis

Question 8

Diabetes

Question 9

Hypertension

Question 10

Cigarette Smoking/Alcoholism