Ménière's disease

Question 1

Define Meniere’s disease.

Answer 1

An auditory disease characterised by an episodic sudden onset of vertigo, low-frequency hearing loss (in the early stages of the disorder), low-frequency roaring tinnitus, and sensation of fullness in the affected ear.

Disease = if idiopathic

Syndrome = if secondary to a number of known inner-ear disorders

Question 2

How common is Meniere’s disease?

Answer 2

• Primarily a disease of adulthood - usually in 40s
• 50% have a family history
• Slightly more common in females 1.1 : 1 ratio

Question 3

What is the aetiology of Meniere’s disease?

Answer 3

underlying response remains unknown but suggested:

• allergic responses
• congenital or acquired syphilis
• Lyme disease
• hypothyroidism
• stenosis or internal auditory canal
• acoustic or physical trauma
• viral infection
• hereditary factors

Question 4

What is the pathophysiology of Meniere’s disease?

Answer 4

• Endolymphatic hydrops probably due to over-production or imapired absorption of endolymph due to a variety of factors
• Excess endolymphatic fluid pressure causes distension and rupture of Reissener’s membrane –> potassium-rich endolymph is released into the perilymphatic space –> injury to sensory and neural elements of inner ear –> sudden hearing loss, tinnitus and vertigo.
• Between attacks, Reissner’s membrane may reattach itself, chemical balance restored and symptoms remit.
• Immune-mediated mechanisms likely to be implicated in the pathophysiology

Question 5

What are the clinical features of Meniere’s syndrome?

Answer 5

• Recurrent attacks of vertigo lasting >20min - spinning sensation
• Fluctuating (or permanent) sensorineural hearing loss
• Tinnitus ( +/- falling to one side)- “roaring tinnitus”
• Sense of aural fullness

Other:

• Drop attacks - without loss of consciousness or other neurological symptoms. Occur in late stages.
• Nystagmus (uncommon) - horizontal +/- rotatory

Question 6

What investigations would you do for Meniere’s disease?

Answer 6

Examination:

• Tandem walk - inability to walk in straight line heel-to-toe
• Postive Romberg’s test
• Fukuda’s stepping test - falling to one side when asked to march in place with eyes closed

Bloods:

• FBC, ESR, thyroid function, syphilis screen, fasting glucose, renal function, lipids - exclude systemic illness

Tests:

• Audiometry - helps diagnose Meniere’s if sensorineural hearing loss is found. But hearing loss can be transient so serial audiograms may be needed. Other:
  
  • Video nystagmography or electronystagmographic testing with bithermal caloric evaluation.
  • Electrocochleography.
  • Brainstem auditory evoked potentials

Imaging:

• MRI brain - advised for unilateral cases to exclude other causes e.g. acoustic neuroma.
• Mastoid radiograph - can aid MD diagnosis by forward location of the sigmoid sinus.

Question 7

How do you manage Meniere’s? (not on Sofia)

Answer 7

Bed rest and reassurance in acute attacks

Low salf diet and diuretics - e.g. hydrochlorothiazide

Vestibular suppressants, anti-emetics or corticosteroids - e.g. meclozine

Intratympanic injections - or corticosteroids or gentamicin

Antihistamine (e.g. cinnarizine) if prolonged, or buccal prochlorperazine if severe for up to 7days.

Question 8

What are the risk factors for Meniere’s?

Answer 8

• Recent viral infection
• Genetic predisposition
• Autoimmune disease e.g. vasculitis, RA, lupus