Meniere’s Disease is a progressive disease of your inner ear that causes spontaneous episodes of vertigo, fluctuating hearing loss, tinnitus and aural fullness. It was described and named after Prosper Menier, a French physician, in 1860, who studied different forms of hearing loss. Meniere’s Disease causes episodes or attacks of severe vertigo, fluctuating hearing loss and tinnitus. Meniere’s treatment includes a multidisciplinary approach from your ENT, audiologist and physiotherapist to manage your symptoms and attacks.
Meniere’s Disease affects 190 per 100 000 people and is usually diagnosed in adults between the ages of 30 – 50 years, but the incidence increases with age. 33% of patients diagnosed with Meniere’s Disease will develop the disease on the other side after 10 years.
What is my vestibular apparatus?
Your vestibular apparatus is like your body’s gyroscope. You have one on each side that communicates with your brain to enable balanced, smooth, coordinated movement by interpreting head and body position in space and changes in velocity. Your vestibular apparatus are snugly embedded in your temporal bones. Your temporal bones are part of your skull, in your hairline above and around your ear. You may have heard the phrase “temporal headache” to describe pain in this area. Deeper within the bone, tucked into its special compartment or vestibule, sits your vestibular apparatus.
The hard bony labyrinth protects the soft membranous labyrinth, in the same way as the skull protects the brain. The bony labyrinth is filled with perilymphatic fluid that has a similar composition to cerebrospinal fluid. In contrast, the membranous labyrinth is filled with endolymph, with a similar composition to intracellular fluid. These different recipes of fluid result in different viscosities, which is important for the functioning of your gyroscopes.
You have five organs that sense movement and body position on each side, three semicircular canals (SCC) and two otoliths. These organs communicate with your brain via your vestibulocochlear nerve, CN VIII.
How do my vestibular gyroscopes work?
Balance is dependent on your eyes, body and ears, like a tripod. How the brain interprets the sensory information it receives from sight, proprioception and the vestibular apparatus determines if you can pirouette, land steady from a back flip or navigate the supermarket aisles without bumping into other customers.
Your semicircular canals detect changes in velocity. These three canals are at right angles to one another and communicate with the opposing SCC of the opposite side. That means your right posterior SCC communicates with your left anterior SCC. They can be described as the x,y and z axes of a 3D graph.
The otoliths detect changes in linear acceleration and gravity. Your utricle relays the change of linear speed, like the movement in a car. While your saccule detects gravitational change in an elevator.
Nerve impulses are generated when hair cells, stereocilia, at the base of the semicircular canal or otolith, the ampulla, are deflected because of the movement of endolymph, almost like seaweed in a current. This movement of the endolymph bends or deflects the stereocilia. This movement causes mechano-sensitive ion channels to open in the hair cell membrane. Potassium, from the potassium-rich endolymph, flows through these channels and changes the electrical potential. When the membrane’s electric potential changes the firing rate of the nerve cells also changes. This means more or fewer messages are sent to your brain to interpret the change in orientation or velocity.
You have experienced this in the real world: once you have reached a steady speed, in a car, plane or elevator, the deflection of hair cells stops, therefor you are not aware of the movement anymore, only when the speed of travel changes again will you notice it.
What happens with Meniere’s Disease?
Endolymphatic hydrops is the pathological change responsible for the symptoms of Meniere’s Disease. This is the abnormal increase in the volume of endolymph within the membranous labyrinth. If fluid volume is increased within the same container, pressure rises. This can be caused by mechanical obstruction of the duct that should drain the fluid from the temporal bone or issues with the anatomy of the duct itself. Mechanical pressure can cause chemical changes.
Meniere’s Disease symptoms are then caused by the physical increase in pressure on the cochlear nerve, explaining the hearing loss, aural fullness and tinnitus. The otolithic organs become distended, affecting the movement of stereocilia, explaining the vertigo. Chemically, the stereocilia membrane can rupture due to the increase in pressure, causing a potassium palsy of the vestibular nerve fibres, explaining the nystagmus and vertigo. Once the pressure is decreased, the symptoms will calm.
Causes of Meniere’s Disease
The exact pathophysiology as why Meniere’s Disease develops is still unclear. The following triggers can lead to a flare up of symptoms as it directly influence the hydraulic pressure of endolymph within the inner ear.
- Allergies
Allergies to pollen, certain foods, medication or other environmental contacts can worsen symptoms as they increase inflammation and pressure.
- Migraine
A history of migraine can contribute to Meniere’s Disease symptom severity.
- Trauma
Head injuries increase the risk of developing Meniere’s Disease.
- Family history
People who have direct family members with Meniere’s Disease are at greater risk because of genetics.
- Existing autoimmune disease
Rheumatoid arthritis, lupus and ankylosing spondylitis have been linked to a higher prevalence of Meniere’s Disease.
Do I have Meniere’s Disease?
Between Meniere’s Disease attacks, you may by symptom free. The following questions may indicate that Meniere’s Disease is causing your vertigo symptoms.
- Have you experienced spontaneous attacks of vertigo? That means no change in position, illness or trauma causes your vertigo?
- Are your vertigo attacks between 20 minutes to 12 hours long?
- Has a family member commented on your eye movement during your vertigo attacks?
- Do you experience a sensation of fullness in your ear during your vertigo attacks?
- Have you noticed any hearing loss on one side?
- Do you experienced a ringing sound in your ear?
If you answered yes to two of these questions, your vertigo may be caused by Meniere’s Disease.
How severe is my Meniere’s Disease?
Meniere’s Disease diagnosis can be definite or probable. A definite Meniere’s Disease requires confirmed sensorineural hearing loss of low and medium frequency with audiometry. In the early stages your hearing loss may fluctuate and therefor not show on hearing tests, and you’ll be diagnosed with probable Meniere’s Disease. The intensity of the vertigo attacks, duration of attacks and hearing loss, tinnitus and aural fullness are similar for both diagnosis, while the bounce back or recovery may differ once the diagnosis is confirmed.
Atypical Meniere’s Disease can be divided into:
- Cochlear Meniere’s Disease, where you only experience tinnitus, aural fullness and hearing loss, but no vertigo
OR
- Vestibular Meniere’s Disease, where you experience vertigo but no tinnitus, aural fullness or hearing loss.
Meniere’s Disease generally affects only one ear (Unilateral Meniere’s Disease), but may progress to affect the other ear later in life (Bolateral Meniere’s Disease). Patients with bilateral disease experience a greater disease burden and poorer quality of life because of more intens symptoms. Vertigo attacks may be more frequent if both ears are affected.
Tumarkin otolithic crises are sudden drop attacks or falls without warning that can happen as Meniere’s Disease progresses.
It should be investigated to rule out any other causes.
Diagnosis of Meniere’s Disease
Physiotherapy diagnosis
Our physiotherapists can identify if any central pathology is causing your symptoms, with visual and balance screening. Or is your vertigo caused by a different structure within the vestibular apparatus?
We make use of a bedside evaluation, which means all tests are done in the consultation room by your therapist or yourself.
Very often, you will have a completely normal physical evaluation between Meniere’s attacks. This is when the experience of your physiotherapist is important and your history, vertigo type, duration of attacks, hearing loss, tinnitus and aural fullness will lead us to suspect possible Meniere’s Disease.
We understand the physiological stages you’ll go through, and custom-fit your treatment program. Our physiotherapists can guide you during any phase or flare of your Meniere’s Disease so that you understand what you are experiencing and are able to make responsible decisions about your care.
Audiogram
Audiogram testing is necessary to determine the exact frequency of your hearing loss. With Meniere’s Disease, we expect to find one-sided sensorineural hearing loss of the lower frequencies.
Your physiotherapist can refer you to an audiologist for your hearing test.
ENG
Electronystagmography (ENG) is a specialised audiology test that identifies functional problems with the vestibular system by measuring eye movement with vestibular stimulation, by moving your eyes, head or a caloric test. With Meniere’s Disease, we expect to see differences between the functioning of your ears.
MRI
An MRI scan can be used to rule out any other pathology, like a space-occupying vestibular schwannoma. This image can only be ordered by your specialist and is not needed to confirm Meniere’s Disease.
Problems we encounter with vertigo from Meniere’s Disease
Misdiagnosis
Most general practitioners treat vertigo as a diagnosis, and not the symptom it is. For many healthcare practitioners, any positional vertigo is diagnosed as BPPV. This may lead to patients not undergoing the necessary testing to establish the correct diagnosis.
Misconceptions about treatment
Because Meniere’s disease causes short, recurrent vertigo attacks, there is no real chance for the brain to compensate and therefore traditional vestibular rehabilitation exercises may not be effective. Treatment is aimed at understanding your diagnosis, managing your symptoms and the quickest recovery between vertigo attacks.
Complications from surgical intervention
Hearing loss is a real risk from surgical intervention and cannot be reversed.
Physiotherapy Meniere’s treatment
Our priority is to determine the extent of your functional impairment from your Meniere’s Disease. We determine if you have compensated for the mismatch of sensory information you are receiving from your vestibular apparatus. The severity of damage to the vestibular organs or recovery from surgical intervention will determine how we approach your vestibular rehabilitation.
Phases of rehabilitation for Meniere’s Disease
Early Meniere’s Disease treatment: Empower
With a thorough history, we can guide you to have the right investigations to get the correct diagnosis. You will recover completely between Meniere’s Disease attacks, and we might find very little dysfunction on your evaluation. However, knowledge is power. If you understand what is happening, you know how to deal with it. You can expect a lot of information regarding your disease when first diagnosed and starting your treatment. You are essentially employing your rehabilitation team now; choose wisely.
Meniere’s Disease Flare treatment: Recalibrate
Knowing how to handle a flare in your symptoms is essential for you and your loved ones. You need to feel confident with your medication use, rest duration and scheduling treatment about 72 hours after your symptoms start. Simple vestibular exercises, like gaze tracking and VOR training to establish safe balance as quickly as possible is all that is needed now. Tracking your symptom severity between attacks is a good way to identify regression as early as possible.
Progressed Meniere’s Disease treatment: Compensate
Once your Meniere’s Disease has progressed and you are not returning to pre flare hearing and vestibular function, the real work of compensation is necessary. Now we will incorporate balance drills, gaze tracking and a combination of the two, maybe walking while you target track, to make sure you are safe during your every day activities.
You may need to see us on a more regular basis now while we navigate how your vestibular system can compensate, and essentially reprogram itself to make up as best it can for the deficit caused by your Meniere’s Disease.
Rehabilitation for surgical intervention for Meniere’s Disease treatment
Surgical intervention, or “surgical destruction”, aims to eliminate your vertigo symptoms. It does this by eliminating all information received from the system. You will need to relearn input from your gyroscope. Rehabilitation will be customised to your circumstances, but you can expect balance, both static and dynamic, training. Position changes, surface changes and different visual inputs during your rehabilitation sessions.
Healing time for Meniere’s Syndrome
Meniere’s disease is a chronic condition, and unfortunately, there is no cure. The aim of treatment for Meniere’s Disease is to ease symptoms by reducing the intensity and duration of vertigo attacks.
Understanding Meniere’s Disease empowers you to make responsible decisions regarding your treatment options and lifestyle.
Other forms of treatment for Meniere’s Disease
- Your audiologist will prescribe hearing aids. This is not only important for having conversations, but also for your spacial awareness, and can decrease the volume of your tinnitus.
- Your doctor (ENT/GP) will prescribe betahistidine, diuretics, antiemetics and motion sickness oral medication to aid pressure balance from fluid, nausea and dizziness.
- Injections, like corticosteroids or antibiotics, can be done with your ENT if needed during acute attacks.
- A psychologist may assist you with implementing cognitive behavioural therapy (CBT) into your lifestyle.
Is surgery an option for Meniere’s Disease?
Surgery is necessary when vertigo attacks become more frequent and debilitating, and your symptoms do not respond to medication and rehabilitation. The risk for complete hearing loss of the operated ear must be considered when deciding on a surgical intervention. Your ENT surgeon will discuss which surgical options you have, and may include:
- Transtympanic steroid injection
Steroids can be injected into your ear to decrease inflammation and pressure and aid in better fluid regulation. You will be required to lie flat with your head turned away from the affected ear for 45 min after your injection.
- Endolymphatic sac surgery
A shunt can be placed in the endolymphatic sac to drain excess fluid and better regulate fluid levels and pressure.
- Labyrinthectomy
If hearing is severely affected, a labyrinthectomy may be considered. The entire labyrinth, semicircular canals and vestibule are removed. This leads to complete hearing loss. With the complete removal of the balance organ, the aim is to eliminate the compensation for faulty signals from this side.
- Vestibular neurectomy
Dissection of the vestibular part of the vestibulocochlear nerve may eliminate vertigo attacks.
What else could cause my vertigo?
- BPPV
Free floating otoconia cause benign paroxysmal positional vertigo of sudden onset, extreme intensity and of short duration, with head movement.
- Vestibular migraine
Intense headache attacks that are associated with aural fullness, sensitivity to noise, pressure changes and tinnitus.
- Post Concussion Syndrome
Dizziness and headaches after whiplash or a concussion injury.
- Vestibular neuritis
An acute viral infection of the vestibular nerve that can also cause aural fullness, hearing loss and intense dizziness. The vertigo is generally present for as long as the infection is active. If left untreated, it may lead to an uncompensated vestibulopathy and episodic dizziness of less intensity.
- Vestibular Schwannoma
A benign tumor on the vestibular nerve may lead to aural fullness, hearing loss and dizziness.
Meniere’s Disease is also known as:
- Meniere’s Syndrome
- Auditory vertigo
- Meniere’s vertigo
- Aural vertigo
- Otogenic vertigo
