Menière’s disease

Menière’s disease

What is Menière’s disease?

Menière’s disease  (MD) is a disorder of the inner ear characterised by sudden vertigo attacks, fluctuating hearing loss, tinnitus, and a feeling of fullness or pressure in the ear. French physician Prosper Menière described it in 1861 (1).

People believe that Menière’s disease affected Julius Caesar, Martin Luther, Vincent van Gogh and, more recently, Alan Shepard (American astronaut) and Huey Lewis of the rock band Huey Lewis and the News (2).

The term Meniere’s syndrome is  used to describe symptoms of Meniere’s disease  when the cause is known.  Meniere’s disease  refers explicitly to cases where the cause is unknown. 

Menière’s disease is a debilitating condition but fortunately, not life-threatening.

Vincent van Gogh

Relevant anatomy

The inner ear processes sound and movement by converting them into neural signals transmitted to the brain through the cochleovestibular nerve. It comprises two main components: the cochlea, responsible for hearing, and the vestibular apparatus, which controls balance and stabilises images during head movement. 

Located within the temporal bone on the side of the skull, the inner ear’s outer portion is known as the otic capsule, a dense, bony structure. Inside this capsule lies a delicate membranous system consisting of ducts, surrounded by perilymph and endolymph fluids. These fluids are essential for the inner ear’s proper function, with endolymph produced by the stria vascularis and dark cells. 

The endolymphatic sac

The circulation and drainage of endolymph through the endolymphatic duct and absorption by the endolymphatic sac are vital components of this dynamic process. Maintaining the distinction between peri- and endolymph is critical for the inner ear to function effectively, and any mixing can impair its ability to work correctly.

Endolymphatic sac and endolymphatic hydrops

The endolymphatic sac is responsible for regulating the volume and pressure of the fluid within the inner ear, known as endolymph. It also plays a role in maintaining the balance of potassium ions in the inner ear, which is essential for proper hearing and balance function.

Endolymphatic hydrops is  characterized by increased pressure due to the accumulation of endolymph fluid in the inner ear. (3).  It is widely believed to be the underlying mechanism responsible for the symptoms of Menière’s disease. However, there is some debate on whether it is the cause or merely a result of an unknown underlying cause. The rise in pressure and build-up of endolymph can be attributed to overproduction, circulation issues, and reduced absorption in the endolymphatic sac. During a Menière’s attack, the symptoms are primarily linked to endolymphatic hydrops, with relief occurring as the pressure subsides.

The endolymphatic sac is also known for its immunological properties, which allow it to mount a defence mechanism known as an immune response. This involves the recruitment of immunocompetent cells and the secretion of protective chemicals. Unfortunately, this immune response can sometimes inadvertently damage the inner ear, resulting in symptoms such as hearing loss and dizziness, characteristic of Menière’s disease (4).

Normal endolymphatic pressure
Endolymphatic hydrops

Polyganglionitis episodica (PGE)​

Polyganglionitis episodica (PGE) is a condition characterized by recurring inflammation in multiple ganglia of the cranial nerves. Many experts believe that the herpes simplex type 1 virus (HSV-1) may be the primary cause of Menière’s disease, as it is known to affect nerve cell bodies, particularly those of the cranial nerves implicated in Menière’s symptoms (5).

HSV-1 is a common virus that remains in the nerve cells for life once a person is infected. While most individuals infected with HSV-1 do not experience symptoms, some may experience recurrent episodes of inflammation due to factors such as stress or illness compromising the body’s immune response. This can lead to the virus becoming active and attacking the nerves it inhabits, potentially causing symptoms such as hearing loss, tinnitus, dizziness, and loss of balance.

Middle ear muscles and Meniere's Disease

According to this theory, Menière’s disease is caused by the abnormal and sustained contraction of the tensor tympani muscle in the middle ear (6). Two of the smallest muscles in the human body are found in the middle ear. The stapedial muscle connects to the stapes (stirrup), and contraction with tilting of the stapes occurs reflexively to loud sound, protecting the inner ear from noise-induced damage.

On the other hand, the tensor tympani muscle reacts to many different triggers, such as the patient’s voice, chewing and swallowing, and aims to protect the inner ear from sound. Momentarily, it protects the inner ear by dampening the sound. 

However, abnormal sustained contraction of the tensor tympani muscle with a sustained inward displacement of the stapes, unfortunately, causes sustained increased inner ear pressure with compression of the outer hair cells and the generation of the symptoms of Menière’s disease. Why this abnormal and often sustained muscle contraction (dystonia) occurs is not known. Stress may play a role. muscle activation. The surgical section of the tendon of the tensor tympani muscle may relieve symptoms but can cause other problems, such as hypersensitivity to body-generated sounds as described.

Who gets Menière's disease?

Menière’s disease is a relatively uncommon condition, with a prevalence of about 200 per 100,000, representing approximately 0.2% of the population. (7). There may be a genetic component, as some individuals have a familial predisposition to the disease. However, it is often misdiagnosed, as demonstrated by the Framingham study in the US, where 2% of patients believed they had Menière’s disease, which is ten times higher than expected (8).

The disease is more common in female patients, with a female-to-male ratio of approximately 3:2. It is typically seen in patients between the ages of 30 and 70, with an increasing incidence with each decade. While rare in children, it has been reported in individuals of various ethnic backgrounds. Social standing may also play a role in presenting Menière’s disease.

Experts have no consensus regarding the risk of developing bilateral Menière’s disease, with estimates varying widely in the literature (15). In the author’s experience the bilateral Menière’s disease incidence is approximately 15% to 20%. The longer a patient has had the disease in one ear, the lower the risk of developing it in the second ear. According to Silverstein, 75% of patients who develop Menière’s disease in the second ear do so within five years of the first ear (16).

What causes Menière's disease?

The precise cause of this condition remains unknown, but research suggests that a combination of genetic predisposition, inner ear abnormalities, and environmental factors may contribute to its development (10, 11,12)

Possibilities include:

  • Virus infections (especially herpes simplex type 1 or HSV-1)
  • Injuries
  • Blood circulation problems
  • Allergies and food intolerance
  • Immune reactions
Allergies may cause Menière’s disease

Causes of Menière’s syndrome

The following conditions cause Menière’s  syndrome:

  • Vestibular migraine (14)
  • Recurrent labyrinthitis
  • Perilymph fistula (PLF), enlarged vestibular aqueduct (EVA) and superior canal dehiscence (SCD)
  • Acoustic neuroma/vestibular schwannoma
  • Multiple sclerosis
  • Syphilis
  • Congenital ear malformations
  • Impaired blood supply in the distribution of the anterior inferior cerebellar artery, transient ischaemic attacks (TIA) and stroke.
  • Autoimmune inner ear disease
  • Cogan syndrome
  • Endolymphatic sac tumour
  • Vestibular paroxysmia
  • Autosomal dominant sensorineural hearing loss
  • Susac syndrome

The link between Menière’s disease and other conditions

Research has revealed a significant correlation between Menière’s disease and various other conditions, including migraine, benign paroxysmal positional vertigo (BPPV), motion sickness, hypothyroidism, autoimmune disease, allergy, and diabetes. Studies have shown that these conditions are more prevalent among individuals with Menière’s disease than the general population. For instance, while migraine affects roughly 14% of the population, it is 50 times more likely to be present in individuals with Menière’s disease, with half of these patients also experiencing migraines.(13, 14)

There is a link between Menière’s disease and migraine

The diagnosis of Menière’s disease

The following criteria are used to diagnose Menière’s disease (17):

  1. Two or more episodes of vertigo or dizziness, each lasting 20 minutes to 24 hours
  2. Audiometrically documented low- to medium-frequency sensorineural hearing loss in one ear, defining the affected ear on at least one occasion before, during or after one of the episodes of vertigo. (Definite MD)
  3. Fluctuating aural symptoms (hearing, tinnitus or fullness) in the affected ear. (Probable MD)
  4. Not better accounted for by another vestibular diagnosis. (also MRI)

Other symptoms

The following symptoms, although not diagnostic, are often reported by patients(18):

  • Sensitivity to visual stimuli and bright light
  • Sound distortion and sensitivity in the affected ear
  • Headaches
  • Anxiety and fear of falling
  • Diarrhoea, sweating, nausea, vomiting and increased heart rate during the attacks
  • Loss of balance and disequilibrium
  • Exhaustion after an attack and sleepiness

One issue with diagnosing Menière’s disease is its rarity and tendency to occur outside regular business hours, when specialists may be unavailable. Emergency doctors are typically the ones who encounter patients during an attack. Still, due to the overlap in symptoms with life-threatening conditions like heart attacks and strokes, it can be challenging to differentiate and prioritize care. As a result, Menière’s disease may be overlooked or its management delayed.

Drop attacks

Drop attacks, also known as Otolithic Tumarkin’s crisis, are a unique manifestation of Menière’s disease in which individuals experience sudden and unpredictable falls while remaining fully conscious. These episodes are linked to a rapid mechanical distortion of the otolith organs, specifically the utricle and saccule, triggering an immediate activation of vestibular reflexes. In some cases, patients describe a sensation of the entire world suddenly flipping upside down (19).

Drop attacks: “Being pushed by the invisible hand

Lermoyez syndrome

In 1919, the French otologist Dr Marcel Lermoyez discovered a unique syndrome in which a sudden onset of vertigo restores hearing and alleviates tinnitus and ear fullness. This rare condition is now considered a variant of Meniere’s disease (20).

Dr Marcel Lermoyez

Triggers of attacks

Some  patients can pinpoint specific triggers that may precipitate an attack of symptoms. These triggers can range from stress, overwork, fatigue, seasonal changes, emotional distress, other preexisting medical conditions, changes in altitude or season, consumption of certain foods, and excessive salt intake. 

Caffeine has the potential to worsen tinnitus, while alcohol consistently exacerbates poor balance.

Does salt trigger Menière’s disease?

The natural course of Menière's disease

Unpredictable patterns of occurrence characterize Menière’s disease. It can manifest as isolated attacks, with long periods of remission in between, or as frequent clusters of attacks. Some patients may experience a sudden cessation of symptoms that never return, while others may develop symptoms over time. For example, patients with initial hearing loss in one ear may later develop vertigo and other characteristic symptoms (21, 22).

This progression is known as delayed endolymphatic hydrops. Alternatively, some patients may initially present with vertigo and later experience hearing fluctuations and loss. Over time, recurrent attacks can lead to permanent hearing loss and diminishing vertigo episodes, resulting in persistent imbalance, disequilibrium, and tinnitus. This is a result of the ongoing damage within the inner ear.

Patients may also experience nonspecific symptoms like neck stiffness, motion sickness, nausea, vision disturbance, and concentration issues. In the early stages, fluctuating low-frequency sensorineural hearing loss is common, and it can evolve into a stable or peaked pattern over time. While Menière’s disease rarely progresses to total deafness, hearing loss can often be managed with hearing aids or cochlear implants.

Clinical examination

A clinical examination may not provide definitive results, particularly if a patient is not experiencing symptoms. Ideally, a specialist would assess the patient during an attack, but this may not always be feasible. During an attack of Menière’s disease, patients often have impaired balance, leading to a specific side-to-side walking pattern and potential falls. They may also exhibit paleness, nausea, vomiting, sweating, and diarrhoea symptoms. In emergencies, heart attack and stroke are typically ruled out. 

Nystagmus, abnormal eye movements, and sensorineural hearing loss may be present and can be evaluated with tuning fork testing or a formal hearing test (23). Other neurological abnormalities are typically not found until later stages of the disease, when inner ear damage occurs. In these cases, abnormalities in the vestibular system may also be detected along with hearing loss, even without an attack.

Special tests in Menierè’s disease

The following special tests may help to manage patients with MD(24):

  • Hearing tests, including word recognition
  • Immittance and reflex testing of the ear
  • Auditory brainstem response audiometry (ABR)
  • Balance and vestibular testing (VNG, DVA, VHIT, and caloric testing)
  • Vestibular evoked myogenic potentials (VEMP)
  • Computed tomography (CT scan)
  • Magnetic resonance imaging (MRI) with gadolinium
  • Blood tests
A caloric test

The psychological impact of Menière’s disease

In my years of practice, I have worked with numerous patients diagnosed with Menière’s disease and have observed a significant correlation between the physical symptoms of the condition and the development of psychological issues. It is not uncommon for individuals to experience feelings of depression, anxiety, fear, anger, and avoidance behaviour as they navigate the challenges presented by this chronic illness (25, 26).

Many of my patients have shared how their lives have been drastically impacted by Menière’s disease, leading to changes in lifestyle, avoidance of social activities such as shopping or dining out, and even a fear of driving. A sense of uncertainty and an inability to plan for the future often plague these individuals, causing emotional distress and helplessness. Witnessing adult men break down in tears due to the overwhelming burden of this condition is a poignant reminder of the toll it takes on one’s mental well-being.

Furthermore, the strain of Menière’s disease extends beyond the individual afflicted with the condition, affecting their relationships and those closest to them. Spouses, in particular, may struggle to understand their partners’ challenges, leading to a lack of empathy and support. It is essential to recognise that when Menière’s disease impacts one partner, the other also bears the weight of its repercussions, creating a ripple effect that can strain the foundation of a relationship.

Menière’s disease is a stressfull condition

Menière’s disease management

Striving for the patient to achieve spontaneous remission is the ideal outcome. It is essential to never give up hope by stating that no treatment is available, as we are continuously working towards managing symptoms and preventing further damage to the inner ear structures until the exact cause of Meniere’s disease is known. Treatments claiming a success rate of 60% should be cautiously approached, as this is not significantly different from a placebo effect. While it is beneficial to help a portion of patients, it is important to consider individual variations in treatment outcomes (27).

The management of Meniere’s disease can be addressed through various approaches, including lifestyle modifications, medication, devices, surgery, vestibular rehabilitation, psychological support, and addressing hearing loss and tinnitus. Customizing treatment plans based on each patient’s unique needs and responses is essential, as no single approach has proven to be universally effective across all cases.

Lifestyle modification

This usually does not work, but it is advised for the minimal benefit to a few. It includes:

  • Salt restriction
  • Caffeine restriction
  • Stress relief
  • Alcohol restriction
Alcohol is not good if you suffer from Menière’s disease

Medical treatment

Medical treatment is divided into (28):

  • Medication to stop the attacks and reduce the symptoms.
  • Medication to prevent the attacks.
  • Medication to destroy the inner ear function.
Betahistine is used in Menière’s disease

Intratympanic treatment for Menière’s disease

Intratympanic treatment for Menière’s  disease involves the direct injection of either gentamicin or steroids into the middle ear through the eardrum (33,34). Gentamicin is an antibiotic that is used to destroy the dark cells in the inner ear that produce the endolymph and even the hair cells that may be causing vertigo and hearing loss in patients with Menière’s  disease. On the other hand, steroids are anti-inflammatory medications that can help reduce inflammation in the inner ear and alleviate symptoms of Meniere’s disease such as vertigo and hearing loss.

This type of treatment is considered when conservative methods, such as medication and dietary changes, have not provided relief for the patient. The injections are typically done in a doctor’s office and may require multiple sessions over a period of time. While both treatments have shown promising results in reducing symptoms of Menière’s disease, the choice between gentamicin and steroids will depend on the individual patient’s needs and preferences.

Intratympanic injections for Menière’s disease

Surgery for Menière's disease

Historically, several surgical procedures were described to treat Menière’s disease, but many are not performed anymore (29). Patients should not receive surgery unless their vestibular system has been adequately assessed and the function documented. The most common procedures and operations performed nowadays include:

  • Myringotomy with placement of a grommet (ventilation tube)
  • Endolymphatic sac surgery
  • Labyrinthectomy
    • Transcanal (TC) approach
    • Transmastoid (TM) approach
  • Vestibular neurectomy
    • Middle cranial fossa (MCF) approach
    • Retrosigmoid (RS) approach
    • Retrolabyrinthine (RL) approach
Endolymphatic sac decompression and opened sac
Labyrinthectomy with vestibular nerve section. Vestibular nerve (V), Facial nerve (F).
Vestibular neurectomy. Vestibular division (V) and Cochlear division (C)

Vestibular rehabilitation

Vestibular rehabilitation is a beneficial treatment approach for managing the symptoms of Menière’s disease, but it is important to note that it will not cure the condition (30). This specialised therapy involves targeted exercises designed to help the brain adapt and compensate for the inner ear dysfunction associated with Menière’s disease.

While resources are available online for vestibular rehabilitation exercises, it is strongly recommended that individuals seek the guidance of a trained specialist, such as a physiotherapist or audiologist with expertise in vestibular rehabilitation, to ensure optimal results. These professionals can tailor a rehabilitation program to suit the individual’s needs and monitor progress over time.

In addition to exercises, vestibular rehabilitation includes education on lifestyle modifications and strategies to minimize the risk of falls and injuries in everyday situations. By working with a specialist and committing to a rehabilitation program, individuals with Menière’s disease can improve their balance, reduce symptoms, and enhance their overall quality of life.

Hearing loss and tinnitus

Patients with Menière’s disease can benefit from various hearing amplification options at any stage. Hearing aids, bone conduction devices, and cochlear implantation (CI) are all available options. It is recommended to work with a trained audiologist when obtaining hearing aids, as they can provide guidance and support throughout the process. A trial period should always be offered to ensure the chosen hearing aid is the best fit for the patient, who should have the option of choosing from more than one device (31, 32).

Tinnitus, a common symptom of Menière’s disease, can be managed effectively. Tinnitus counselling and retraining therapy provided by trained audiologists can be highly beneficial. In some cases, hearing aids may also help improve tinnitus symptoms. Patients must know that treatments are available and should not simply be told to live with their tinnitus. Patients should be cautious of medicines, devices, and medications claiming to cure tinnitus. They should always consult an ENT surgeon or audiologist before trying new treatments.

implantable hearing solutions - cochlear implants
A cochlear implant system

Psychological support

Menière’s disease is a physical condition that can often lead to the development of psychological issues in patients. Those suffering from Menière’s disease need to seek support from a psychologist, who can provide appropriate therapy and coping strategies. In more severe cases, a psychiatrist may need to be consulted to prescribe medication. Additionally, the partners of individuals with Menière’s disease may also benefit from psychological support to better understand and navigate the challenges of living with the condition.

Dr Hofmeyr's experience

Dr. Hofmeyr specializes in treating patients with Menière’s disease. He has a proven track record of successfully managing and controlling attacks in many of his patients. With extensive experience in performing intratympanic injections and endolymphatic sac surgeries, Dr. Hofmeyr has operated on over 200 cases.

Dr Hofmeyr is proficient in a variety of surgical approaches, including the middle cranial fossa (MCF), transmastoid (TM), translabyrinthine (TL), transcanal (TC), and retrosigmoid (RS) methods.

He is also experienced in bone conduction hearing aids and cochlear implants (CI) for patients with Menière’s disease and hearing loss.


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