Meniere’s Disease

Introduction

Meniere’s disease is a relatively common cause of peripheral vertigo. It presents with the triad of vertigo, tinnitus and hearing loss.

The incidence of Meniere’s disease is estimated to be between 100 and 150 in every 100,000 people.

Aetiology

The exact aetiology and pathophysiology of Meniere’s disease are not fully understood.

However, a significant number of patients who have Meniere’s disease have endolymphatic hydrops. This is effectively where endolymph builds up in the membranous labyrinth.¹ For more information on anatomy, see the Geeky Medics guide to the anatomy of the ear.

Risk factors

The main risk factor for Meniere’s disease is a family history of the disease.

Clinical features

History

Typical symptoms of Meniere’s disease include:

• Recurrent spontaneous vertigo (lasting between 20 mins and several hours)
• Progressive or fluctuating hearing loss (sensorineural, initially affecting the low frequencies)
• Tinnitus
• Aural fullness

Other important areas to cover in the history include:

• Nausea/vomiting: this may suggest labyrinthitis
• Family history of Meniere’s disease

Clinical examination

All patients with vertigo and hearing loss require a comprehensive ENT examination, particularly the ears. 

Ear infections can cause the same symptoms as Meniere’s. Therefore it’s important to exclude a treatable cause such as acute otitis media.

Patients may present with ongoing horizontal nystagmus. They may also present with sensorineural hearing loss. 

A neurological examination must be performed in any patient with acute onset vertigo, as a cerebellar stroke can cause vertigo.²

Differential diagnoses

Differential diagnoses to consider in the context of suspected Meniere’s disease include:

• Vestibular migraine: history of migraines, dizziness can persist for up to 72 hours
• Labyrinthitis: tends to be preceded by a viral illness or another underlying infectious cause like otitis media
• Benign paroxysmal positional vertigo (BPPV): hearing will be normal
• Stroke
• Vestibular schwannoma (acoustic neuroma)

Investigations

Audiometry

Although no specific investigations are required to diagnose Meniere’s disease, most patients receive a formal audiology assessment. 

The most common audiometric pattern early in the disease is either a low-frequency sensorineural hearing loss or combined low- and high-frequency sensorineural hearing loss with normal hearing in the mid-frequencies. 

Later in the disease, the hearing loss tends to affect all frequencies. 

Imaging

MRI imaging is used to exclude abnormalities in the cerebellopontine angle (e.g. vestibular schwannoma or meningioma) rather than diagnose Meniere’s disease.³

Diagnosis

The below criteria must be met for a definite diagnosis of Meniere’s disease:

• Two or more spontaneous episodes of vertigo lasting, with each episode lasting between 20 minutes and 12 hours.
• Audiological assessment demonstrating low to moderate sensorineural hearing loss on the affected side.
• Aural fullness

Patients without hearing loss may still be diagnosed with Meniere’s disease.⁴ Some patients do not meet all the above criteria and are diagnosed with probable Meniere’s disease.

Patients can develop bilateral Meniere’s disease.

Management

Medical management

The mainstay of management are lifestyle and dietary changes, such as minimising salt and caffeine intake. However, the quality of evidence is low.⁵

Betahistine, a histamine analogue, is commonly used, despite increasing evidence suggesting more of a placebo effect. Vestibular sedatives (e.g. prochlorperazine) can be useful when patients have acute episodes of vertigo.⁶

Vestibular rehabilitation can also benefit patients.

Surgical management

Surgical management involves the injection of medication intratympanically, either dexamethasone/methylprednisolone or gentamicin. Although effective at reducing the amount of vertiginous episodes patients suffer from, gentamicin is ototoxic and can cause hearing loss in the injected ear.⁷

There is currently increasing evidence supporting the use of intratympanic steroids.

More advanced surgical options such as labyrinthectomy and vestibular neurotomy are used in a few selected patients where other management options have been exhausted. 

Complications

Complications of Meniere’s disease include:

• Intermittent flares of Meniere’s disease
• Long-term intermittent tinnitus, which can have a significant impact on well-being and quality of life
• Long-term hearing loss.

Prognosis

Meniere’s disease tends only to affect one ear but can affect both in around 30% of patients.⁸ Although most patients can continue with their daily lives, some patients will have long-term issues with balance and hearing loss.

Key points

• Meniere’s disease presents a triad of peripheral vertigo, tinnitus and hearing loss
• The exact cause is unknown, but many patients have endolymphatic hydrops
• Important investigations include audiological assessment and imaging (MRI) to exclude other causes of vertigo
• Management involves dietary and lifestyle advice, with refractory cases considered for intratympanic injections of steroids or gentamicin
• Complications include long-term intermittent tinnitus and hearing loss on the affected side

Reviewer

Mr Georgios Kontorinis MD, MSc, FRCS (ORL)

Honorary Clinical Associate Professor

School of Medicine, Dentistry & Nursing

University of Glasgow

Editor

Dr Chris Jefferies

References

Text references

1. John C Watkinson, Raymond W Clarke, Chrstopher P Aldren, et al. Scott-Brown’s Otorhinolaryngology and Head and Neck Surgery. Vol Volume 2: Paediatrics, The Ear, and Skull Base Surgery. 8th ed. Taylor & Francis Group; 2018.
2. Choi KD, Lee H, Kim JS. Vertigo in brainstem and cerebellar strokes. Current Opinion in Neurology. 2013;26(1):90-95.
3. Singh K, Singh MP, Thukral CL, Rao K, Singh K, Singh A. Role of Magnetic Resonance Imaging in Evaluation of Cerebellopontine Angle Schwannomas. Indian J Otolaryngol Head Neck Surg. 2015;67(1):21-27. 
4. Basura GJ, Adams ME, Monfared A, et al. Clinical Practice Guideline: Ménière’s Disease Executive Summary. Otolaryngol Head Neck Surg. 2020;162(4):415-434. 
5. Hussain K, Murdin L, Schilder AG. Restriction of salt, caffeine and alcohol intake for the treatment of Ménière’s disease or syndrome. Cochrane Database of Systematic Reviews. 2018;(12).
6. Lacour M, van de Heyning PH, Novotny M, Tighilet B. Betahistine in the treatment of Ménière’s disease. Neuropsychiatr Dis Treat. 2007;3(4):429-440.
7. Yetişer S. Intratympanic Gentamicin for Intractable Ménière’s Disease – A Review and Analysis of Audiovestibular Impact. International Archives of Otorhinolaryngology. 2018;22(2):190.
8. Harcourt J, Barraclough K, Bronstein AM. Meniere’s disease. BMJ. 2014;349:g6544.

Image references

• Figure 1. Blausen.com staff. Internal ear anatomy demonstrating bony and membranous aspects of the labyrinth aspect of the inner ear. License: [CC BY]
• Figure 2. Ijustam. Menieres hearing loss. License: [Public domain]