Meniere’s Disease: Symptoms, Diagnosis, and Treatment
Ménière’s disease (pronounced “Men-YAIRS” or “MEN-EARS”) was first described by Prosper Ménière in the early 1800s.
Also known as endolymphatic hydrops, Meniere’s is a disorder of balance, hearing, or both—caused by an increase in endolymph, an inner-ear fluid.
Symptoms of Meniere’s:
- Fluctuating hearing loss, usually more so in the low frequencies
- Attacks of vertigo, often preceded by an “aura”
- Feeling of fullness in the affected ear in the absence of an acute middle ear disorder
- Low pitched “roaring” tinnitus in the affected ear
While there are many reasons for some of these symptoms, to truly diagnose your problem as Meniere’s, all four the above abnormalities must be present. If you have just hearing loss, tinnitus, or vertigo, check out the links above for more information on non-Meniere’s-related disorders of the inner ear issues.
What is the cause of Meniere’s Disease?
Unfortunately, we really don’t know exactly what causes Meniere’s, but we do know a lot about how it tends to behave and progress. Symptoms tend to emerge in the early forties. While not technically hereditary, it tends “run in families” particularly among the female members. Many patients have a sister, mother or aunt with similar symptoms. Despite not knowing exactly what causes it, physicians and audiologists who treat Meniere’s do know a lot about how to manage and treat the symptoms. More on that in a bit.
What’s going on in the ear with Meniere’s?
The inner ear consists of two connected organs: One for hearing called the cochlea and one for balance called the vestibular labyrinth.
Also known as endolymphatic hydrops, Meniere’s is a disorder of balance, hearing, or both caused by an increase in endolymph, an inner-ear fluid.
The cochlea allows us to perceive sound by converting fluid vibrations in the inner ear into nerve impulses. At the center of this process are the inner and outer hair cells within the Organ of Corti. The Outer Hair Cells (OHC) act as pre-amplifiers, effectively amplifying vibrations within the cochlear to help the Inner Hair Cells (IHC) to activate their bio-mechanical “light switch” sending the “I heard something” message to the brain via the auditory nerve. The OHCs also work in tandem with the brain to fine-tune pitch perception.
The brain estimates pitch by keeping track of which area of hair cells have the greatest response, and estimate loudness by counting how many areas are stimulated. When hearing loss occurs, we either have lost some hair cells altogether, or they have become distorted or malfunction for some reason. More on this and Meniere’s in a bit.
The Vestibular Labyrinth includes three semicircular canals as well as the utricle and saccule. These act like carpenter’s levels and accelerometers. The orientation of the semi-circular canals allow us to measure head movement in three planes: Twisting side to side, looking up or down, and tilting ear-to-shoulder. This tells our brain if our head is steady or if it is moving in one of these planes.
When your head moves, the brain triggers an eye movement with equal movement and opposite direction, called the vestibulo-ocular reflex (VOR)—so the world doesn’t appear to whoosh in front of us.
Finally, the brain gets information from a body-awareness system called proprioception. This measures relative weight distribution on feet, legs and buttocks to determine if we are standing, walking or sitting.
When it all works as planned…
A quick check of the other canals shows little to no movement, so the brain is pretty sure the head (and maybe body) moved in this plane.
The proprioceptive system reports about 90% of your weight on your tuchus (rear end) and 10% on your feet, so we know you’re sitting which means only your head moved UP.
Knowing this, your brain tells the VOR to move your eyes DOWN the same amount it thinks your head moved up. This allows you to change your eye gaze from the tablet to the family member without getting dizzy.
Imagine you’re sitting at the kitchen table, checking out the latest updates from HearingTracker on your tablet. You look up to talk to a family member is the room. Your brain senses movement in only the anterior (head nodding) semi-circular canal. This suggests to your brain that you either moved your head up, or your body rotated back.
What goes wrong with Meniere’s
Meniere’s disease is a disruption of the fluid pressure inside your two inner ear organs. It’s not entirely clear whether this is caused by an overproduction of fluid or if the natural absorption and recycling of fluid is somehow slowed down. The end result is too much fluid pressure, which reduces the brain’s ability to accurately figure out what’s going on.
The “classic” presentation of Meniere’s includes disruptions of both balance and hearing, however many patients lean toward one or the other as their primary set of symptoms.
- Vestibular Meniere’s - Usually begins with onset of sudden, often severe episodes of vertigo described as a spinning sensation. Unlike other vestibular disorders such as Benign Paroxysmal Positional Vertigo (BPPV), or motion sickness, these attacks come out of the blue and are generally not brought on by movement. Due to the high risk of falling, these have been called “drop attacks.” Many patients report a strange feeling or “aura” prior to the attack which can help them get to a safe place prior to onset. Because of the intensity of these episodes, nausea and vomiting is not uncommon.
- Cochlear Meniere’s - Generally begins with a sensation of fullness and a reduction of hearing in one ear. Rather than a sudden “attack”, changes in hearing tend to ebb and flow a bit more slowly. It is not uncommon for patients with this presentation of Meniere’s to also have a significant reduction in word recognition. This is a bit counter-intuitive since the hearing loss associated with Meniere’s is usually greater at low pitches which are less important for speech understanding than the higher pitches. Assuming no other hearing issues, low pitched hearing loss should allow that person to understand speech even though the perceived volume is lower. In Meniere’s however, this additional distortion of speech makes hearing aid fitting a bit more challenging, often requiring some research and consultation with others if the fitting audiologist hasn’t worked with a lot of patients with Meniere’s.
In addition to hearing loss, most Meniere’s patients experience low pitched “roaring” tinnitus which can fluctuate with the hearing loss. Because tinnitus is also affected by how we perceive it, negative thoughts about it can make it seem less variable and more bothersome than the hearing loss itself. Support for this is very important, and while tinnitus, like Meniere’s, is not curable at this time, learning how to manage it, including understanding that there are many others who have successfully habituated to it provides a toolkit of skills to manage it when the inevitable fluctuations come along. The American Tinnitus Association is a great resource for finding others to help with this part of the process.
Stages and progression of Meniere’s
One of the most frustrating things about Meniere’s is that it doesn’t behave the same all the time. In the early stages, symptoms tend to be very active and fluctuate rapidly and widely. This is the most difficult and often most frightening stage of the disorder. Having a good support system, including a network of others with Meniere’s is critical. The Vestibular Disorders Association provides a great collection of scientific articles, links to support groups, and providers familiar with Meniere’s and other balance disorders, and most importantly, a strong message that you are not alone.
For most patients, the active stage settles down after a few months or years and while there may be a progression of hearing loss, the frequency and intensity of the vertigo episodes usually decrease. Many Meniere’s patients eventually “burn out” and reach a maximum level of dysfunction for hearing or balance. On the hearing side, this usually means that word recognition becomes poor enough that hearing aids are not really effective and evaluation for a cochlear implant is indicated. When balance function burns out, most find that a specialized form of physical therapy called vestibular rehabilitation, allows them to adjust to the “drunken sailor” feeling that comes with only one side of the vestibular system shows up for work.
The beginning of the diagnosis can start with your primary care physician taking a careful case history to document the pattern and timing of your symptoms, but because Meniere’s can involve both hearing and balance impairment, it’s really a good idea to be seen by a specialty physician called an otologist. This is a subspecialty ear, nose, and throat (ENT) physician who sub-specializes in the ear and the inner ear. They may be Board Certified by the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS).
In addition to a general head and neck exam, your evaluation will most likely include a hearing test to determine if your hearing thresholds matches the pattern that we typically see with Meniere’s. Following that, the physician may order some blood work, magnetic resonance imaging (MRI) and a balance assessment.
The blood work is to assess your overall health and to check for other possible cause oft your symptoms such as high or low blood pressure, diabetes, or abnormal sodium levels.
While it is an unlikely cause for Meniere’s symptoms, the MRI will rule out the possibility that a non-cancerous tumor called a vestibular schwannoma is making you dizzy.
The balance assessment will include several tests to determine whether your disorder of balance is happening in the inner ear labyrinth (peripheral) or from a disturbance of communication between the ear and the brain (central).
Since Meniere’s symptoms fluctuate, it is possible that these tests all show normal results. Because of this, a Meniere’s assessment from an Otologist will often also include a specialized test called electrocochleography, or EcoG, which measures the electro-chemical behavior of the cochlea. Patients with active Meniere’s show a specific result on ECoG, which can aid in the diagnosis.
One of the challenges in diagnosing Meniere’s is the fluctuating nature of the disorder. While it would be ideal to perform all of the above test during an acute attack, most patients will not come into an office while the world is spinning and their ears are roaring. If you can provide a very accurate history including the approximate dates, duration and severity of the symptoms, your physician and audiologist can arrive at an accurate diagnosis even if your appointment is on a “good” day.
Treatment options vary by the patient and their individual needs and the severity of their symptoms. The majority of Meniere’s patients I have seen in my 30 years of clinical practice report that the hearing loss is inconvenient, but the vertigo is debilitating. Due to this, mitigating balance issue tends to dominate the treatment plan.
Handling acute episodes
For acute episodes, anti-vertigo medications like Meclizine or Dramamine may be prescribed along with anti-anxiety medicines like Valium or Xanax. Be very careful when taking these and discuss both the side effects (drowsiness, confusion) and the intended use with your physician. Generally these medicines are only intended as a response to acute attacks, not to be taken daily to “prevent” episodes. Fear of an attack is an absolutely valid concern, but using certain medications daily can actually make it harder for your body to habituate to the disorder and can mask symptoms that may assist your treatment team in making your life easier.
Addressing the underlying condition
Since the assumed underlying issue with Meniere’s is fluid buildup in the inner ear, the first attempt is to reduce that. Initial treatment includes a low-salt diet, which while no fun for the taste buds, often takes the edge off and can significantly reduce the severity of symptoms. Some patients are also very sensitive to caffeine, so this may be another dietary restriction suggested. Improving overall health by increasing hydration and engaging in regular physical activity between episodes may be suggested as a general lifestyle modification. This has the secondary benefit of keeping you flexible and strong in order to allow your brain to shift focus from the ear to the proprioceptive system to maintain balance and prevent falls. This can be as simple as walking, or as specific as Tai Chi or yoga. Be sure to practice any of these with a buddy who knows you are subject to sudden vertigo both for your own safety and to avoid frightening them.
If these do not reduce symptoms enough, you may be prescribed a mild diuretic to attempt to reduce the amount of fluid in your body generally—this may help to reduce the pressure in the ear.
Treatment for hearing loss symptoms usually involves hearing aids. Because Meniere’s may only affect one ear, a monaural (one-ear) fitting may suffice if the hearing in the unaffected ear is very good, however due to the connected / stereo nature of most hearing aids, you may perform better in adverse listening settings (distance, background noise, reverberation) with two hearing aids. Asking the prescribing audiologist to perform aided speech testing with competing multi-talker noise with no hearing aids, one on the affected ear only and on both ears, will allow you to compare how well you actually hear in each difficult setting rather than relying only on opinion or marketing information to determine which system is best for your hearing needs.
Because Meniere’s tends to progress and fluctuate, it important that the hearing aid on your affected ear have the ability to be set for both “good” and “bad” hearing days using user-selectable programs. You should also select a hearing aid that can be adjusted for significant changes in volume such as a Receiver In Canal (RIC) device where lower to higher power speakers can be exchanged in the office without having to send the device to the manufacturer for adjustment.
Because falls are one of the most significant health risks in Meniere’s, it is always a good idea to ask for a referral for Vestibular Rehabilitation even if you aren’t currently having drop attacks. This specialized form of physical therapy seeks to create a customized inventory of your strengths and weaknesses in balance and movement. Particular emphasis is placed on beefing up your proprioceptive system and how you use your vestibulo-ocular system to maintain stability regardless of what information is coming from your inner ears. In dormant phases of Meniere’s, this gives you super-duper balance. More importantly, in active phases, when your inner ears are completely unreliable, the lessons learned in vestibular rehabilitation give you the ability to ignore the “crazy talk” of the affected labyrinth and focus on the reliable and now very strong alternate systems for keep you upright, safe, and secure.
Treating more severe cases
In some cases, severe symptoms persist despite all of the above management strategies. For these cases, further medical assessment by an inner ear sub-specialist called a neurotologist is indicated. Some common treatments for “intractable” (hard to control) Meniere’s includes injections of Gentamicin which is known to diminish balance response to the treated ear, or endolymphatic sac decompression, which surgically decreases the volume and therefore pressure of the fluid in the vestibule by placing a shunt in the endolymphatic sac. In very severe cases, the vestibular nerve is severed to prevent disruptive signals from the affected ear from reaching the brain. In the most extreme cases, total removal of the vestibular labyrinth called a labyrinthectomy may be performed.
Research into Meniere’s treatment
Research into the cause and treatment of Meniere’s is ongoing and while a cure has yet to be found, places like the National Institute on Deafness and Other Communication Disorders (NIDCD) and the Mayo Clinic continue to fund and conduct research on the underlying cause, how other disorders and external factors affect symptoms and treatments to make living with Meniere’s easier and more effective. The National Institutes of Health (NIH) also funds research into Meniere’s.
Living with Meniere’s
Meniere’s is a significant issue, but it does not need to be a show stopper. The more you know about how your version of Meniere’s behaves, the better equipped you are to work with and around it. First, realize you are not alone. It is estimated that worldwide, 12 out of every 1,000 people have Meniere’s.
You have no control over where and when Meniere’s will take your hearing and balance, but you have 100% control over how you allow that reality to impact you.
Reach out to organizations like The Vestibular Disorders Association, The American Tinnitus Association, and the Hearing Loss Association of America to connect with others who are going through similar experiences with Meniere’s.
Taking control of your health and lifestyle habits not only helps your body adapt to Meniere’s, but it reinforces that you are an active collaborator with a not-so-nice visitor in your life rather than a passive responder to a chaotic, unpredictable, and unwanted intruder. Fear and frustration are valid and expected reactions to Meniere’s but they are not the only ones.