Ménière's disease

Question 1

Define Ménière’s disease

Answer 1

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.

Question 2

What is the difference between Ménière’s disease and Ménière’s syndrome

Answer 2

Ménière’s disease = idiopathic

Ménière’s syndrome = secondary to inner ear disorder

Question 3

Aetiology of Ménière’s disease

Answer 3

Underlying cause unknown

Suggested aetiological agents:

• Allergic response (esp. to food)
• Congenital or acquired syphilis
• Lyme disease
• Hypothyroidism
• Stenosis of the internal auditory canal
• Acoustic or physical trauma

Question 4

Pathophysiology of Ménière’s disease

Answer 4

Over-production or impaired absorption of endolymph
During an attack the excessive endolymphatic fluid pressure causes distension and rupture of Reissner’s membrane, resulting in the release of potassium-rich endolymph into the perilympatic space and causes injury to the sensory and neural elements of the inner ear.
Between attacks Reissner’s membrane may reattach itself and chemical balance is restored

Question 5

Risk factors for Ménière’s disease

Answer 5

Family history
Recent viral infection
Autoimmune disease e.g. vasculitis, rheumatoid arthritis, lupus

Question 6

Symptoms of Ménière’s disease

Answer 6

One episode hearing loss
Vertigo (Recurrent episodes | “spinning sensation” | Minutes to hours | Associated Nausea and vomiting)
Tinnitus (Described as “roaring” | Usually unilateral
Sensation of fullness in one ear
May increase prior to an attack

Drop attacks - sudden loss of balance without loss of consciousness or other autonomic/neurological symptoms

Question 7

Signs of Ménière’s disease on examination

Answer 7

Positive Romberg’s test (Swaying or falling when asked to stand with feet together and eyes closed)
Fukuda’s stepping test (Turning towards the affected side when asked to march in place with eyes closed)
Nystagmus (Horizontal and/or rotatory nystagmus that can be suppressed by visual fixation | Seen in acute attacks)
Tandem walk
Inability to walk (heel-to-toe) in a straight line.

Question 8

Investigations for Ménière’s disease

Answer 8

Pure-tone air and bone conduction with masking: unilateral sensorineural hearing loss, initially ↓freq, then ↑freq
Speech audiometry: No discrepancies on speech recognition threshold (SRT), Absence of positive roll-over index
Tympanometry/immitance/stapedial reflex levels: normal
Oto-acoustic emissions (OAE): Absence of measurable OAE in frequency range affected by MD
Electrocochleography: Abnormally large summating potential amplitude relative to AP amplitude
Electronystagmography: Unilateral ↓ vestibular response in affected ear suggest peripheral aetiology (e.g. vestibular neuronitis)
Rotary chair test: ↓ gain, abnormal phase and asymmetry in response