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.

AKA endolymphatic hydrops

Question 2

Aetiology of Ménière’s disease

Answer 2

Ménière’s disease = idiopathic
Ménière’s syndrome = secondary to inner ear disorder
- Allergic response (esp. to food)
- Congenital or acquired syphilis
- Lyme disease
- Hypothyroidism
- Stenosis of the internal auditory canal
- Acoustic or physical trauma

Over-production or impaired absorption of endolymph.
Attack: excessive endolymphatic fluid pressure → distension and rupture of Reissner’s membrane → release of potassium-rich endolymph into the perilympatic space + 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 3

Risk factors for Ménière’s disease

Answer 3

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

Question 4

Symptoms of Ménière’s disease

Answer 4

One episode hearing loss (sensorineural)
- Usually unilateral
- Initially in low frequencies
Vertigo
- Recurrent episodes - clustered attacks (<12 hours) over weeks
- “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 and can resume normal activities immediately after

Question 5

Differentials for Ménière’s disease

Answer 5

Acoustic neuroma
Vestibular neuronitis
Vestibular migraine
Viral labyrinthitis
BPPV
Vertebrobasilar insufficiency

Question 6

Signs of Ménière’s disease on examination

Answer 6

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 7

Criteria for diagnosis of Ménière’s disease

Answer 7

Requires all of:
- Two or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours.
- Audiometrically documented low-to-medium frequency sensorineural hearing loss in one ear, defining and locating to the affected ear on at least one occasion before, during, or after an episode of vertigo.
- Fluctuating aural symptoms (hearing loss, tinnitus, or fullness) in the affected ear.
- Not better accounted for by an alternative vestibular diagnosis.

Question 8

Investigations for Ménière’s disease

Answer 8

TFTs/lyme/syphilis serology/ANA/ANCA/Rheumatoid factor

Pure tone air and bone conduction: unilateral sensorineural hearing loss, initially ↓freq, then ↑freq
Audiometry: low-to-medium frequency sensorineural hearing loss in one ear, defining and locating to the affected ear on at least one occasion before, during, or after an episode of vertigo.
Speech audiometry: no discrepancies on speech recognition threshold
Tympanometry/immitance/stapedial reflex levels: normal
Otoacoustic emission: absence of measurable OAE

Question 9

Management for Ménière’s disease

Answer 9

MDT: ENT, physiotherapy, hearing therapist, audiologist, counsellor, psychologist

Conservative
- Restrict salt intake to 1500-2300mg/day
- Limit caffeine intake
- Reduce alcohol consumption
- Stop smoking
- Manage stress
- Tinnitus maskers, retraining therapy, hearing aids, medication, biofeedback, neuromonics
- Hearing aids or assistive listening devices
- people with ‘liability to sudden and unprovoked or unprecipitated episodes of disabling dizziness’ should stop driving and inform the DVLA

Medical
- Anti-vertigo (prevent future attacks): betahistine
- Treat emesis: prochlorperazine, cyclizine, promethazine
- Treat acute attacks: diazepam, steroids PO/intratympanic injection
- Diuretics (reduces endolymph volume) e.g. hydrochlorothiazide

Surgical
Gentamicin instillation via grommets
Saccus decompression

Question 10

Complications of Ménière’s disease

Answer 10

Falls
Profound hearing loss (1-6%)

Question 11

Prognosis for Ménière’s disease

Answer 11

Acute attacks usually settle within 24h
Most patients start with hearing loss and tinnitus, then may or may not develop complete clinical profile
Symptoms tend to get worse over time regardless of medical intervention
May go into periods of remission