Lecture 8- Meniere Disease

Question 1

What is Meneiere Disease?

Answer 1

Multifactorial disorder probably initiated by a combination of genetic and environmental factors

Associated with accumulation of endolymph in the cochlear duct and vestibular organs

Question 2

What is Meneiere Disease characterized by?

Answer 2

• Episodic, spontaneous vertigo, usually accompanied by fluctuating SNHL, tinnitus, and aural fullness
• Cochlear symptoms may occur between episodes of vertigo
• Vertigo crises are more frequent during early ears of disease

Question 3

What are the diagnostic criteria for Meneiere Disease?

Answer 3

• 2 or more episodes of spontaneous vertigo lasting between 20 minutes and 12 hours
• Low frequency SNHL in 1 ear, defined at least one occasion before, during, or after one of the episodes of vertigo
• Fluctuating hearing symptoms (HL, tinnitus, aural fullness) in affected ear
• There is no other diagnosis that better explains the vestibular symptoms

Question 4

What is vertigo?

Answer 4

Sensation of movement in the absence of movement, or an altered sensation of movement during normal movement of the head

Question 5

What are some triggers for vertigo attacks?

Answer 5

Dietary: caffeine, sodium

Sound: Tullio phenomenon

Pressure: Hennebert symptom/sign

Question 6

What is a vertiginous episode?

Answer 6

Defined by the time the patient has to be immobile and cannot move

• Can be <20 minutes or >12 hours, but these are not common findings; other vestibular disorders should be considered

Question 7

How is the affected ear determined?

Answer 7

Thresholds shift to at least 35 dB HL at 2 consecutive frequencies below 2 kHz (or 30 dB shift compared to contralateral ear)

Question 8

Do SNHL and vertigo have to occur at the same time?

Answer 8

No

• SNHL may precede the onset of vertigo by months or years
• Recurrent vertigo may occur before the SNHL in weeks or months, but tinnitus or aural fullness are usually associated with the first episode of vertigo

Question 9

When is Meniere Disease considered likely?

Answer 9

• 2 or more episodes of vertigo or dizziness, lasting between 20 minutes and 24 hours each
• Fluctuating hearing symptoms (SNHL, tinnitus, or aural fullness) in the affected ear
• There is no other diagnosis that better explains vestibular symptoms

Question 10

What is the differential diagnosis for Meniere Disease?

Answer 10

• DFNA9, COCH mutation
• Autoimmune inner ear disease
• Stroke
• Cogan syndrome
• ELST
• CPA mass
• Susac syndrome
• Infectious causes (e.g., otosyphilis)
• 3rd window syndromes
• Vestibular migraine
• Vestibular schwannoma
• Vogt-Koyanagi-Harada syndrome

Question 11

When should Familial Meniere Disease be considered?

Answer 11

• At least one first or second relative has definitive or probably Meniere
• May occur in 8-9% of cases of MD in European populations, but has been described in nearly all ethnic groups
• Typically fits AD inheritance, although mitochondrial recessive has been described
• DTNA, FAM136A