MENIÈRE’ S DISEASEForm B

Information leaflet

What is Menière’s Disease?Menière’s disease (MD) is a condition of the inner ear. It consists of attacks of vertigo, fluctuating hearing loss, tinnitus and a sensation of fullness or pressure in the ear. MD is named after a French physician by the name of Prosper Menière who described this condition it in 1861. Famous people in history like for instance Vincent Van Gogh and Julius Caesar are believed to have suffered from Menière’s disease. Menière’s syndrome is another term often used in the literature and refers to Menière’s disease like symptoms but with a known cause. A few known conditions can present with these symptoms. When the term Menière’s disease is used it implies that the cause is not (yet) known. In fact in the majority of cases the cause is unknown. Menière’s disease is not a life threatening disease.

Relevant anatomyThe inner ear converts sound and movement into neural signals. These signals are transmitted to the brain via the cochleovestibular nerve. The inner ear consists of the cochlea for hearing and the vestibular apparatus for balance control and image stabilization during head movement. It is hosted in the temporal bone on the side of the skull. The outside part of the inner ear is called the otic capsule and consists of very hard dense bone. Inside this bony labyrinth there is a membranous system, consisting of ducts. This membranous system is surrounded on the outside by fluid called perilymph. The fluid on the inside of the membranous system is called endolymph. Peri- and endolymph are different, an important fact in order for all the structures of the inner ear to work properly. If it mixes, function is lost and the inner ear cannot work. The stria vascularis and dark cells produce the endolymph. It circulates and is eventually drained through the endolymphatic duct and absorbed by the endolymphatic sac, all different parts of the membranous endolymphatic system. This is an ongoing and dynamic process.

Endolymphatic hydropsEndolymphatic hydrops is defined as the increase in pressure due to endolymph fluid builds up in the inner ear. It is believed by many that endolymphatic hydrops is what happens in the inner to produce the symptoms of Menière’s disease. Others argue that it is not the cause but rather the result of some unknown cause of the symptoms of Menière’s ’s disease. The pressure and accumulation of the endolymph can occur due to the increase in production, impairment in circulation and eventual reduction in absorption. During the attack the endolymphatic hydrops is responsible for the symptoms and when it settles the symptoms disappear and the attack is over. As mention before some disputes this, believing that the endolymphatic hydrops is only a marker of Menière’s disease and not responsible for the symptoms. It is also known that endolymphatic hydrops is found on autopsy in some patients who never had Menière’s disease. Some experts

Dr. LM Hofmeyr MEDICLINIC MUELMED 13 Fairway StreetOTOLOGIST AND NEUROTOLOGIST Room 505 BellvilleMBChB(UP) MMED ENT (UP) 577 Pretorius Street 7530HPCSA no. 0408042 Arcadia 0815 Cape TownPr. No. 0041637 Pretoria (Opp MEDICLINIC LOUIS LEIPOLDT)Prof. LM HOFMEYR NEUROTOLOGIST INC T 012 341 8924 T 021 946 3620Reg. no. inc 2013 / 017579 / 21 F 086 618 1804 F 086 618 1804Reg. no. VAT 4410261004 C 082 339 4926 C 082 339 4926lmhofmeyr@surgeon.co.zawww.lmhofmeyr.co.za

are of the opinion that the sensory organs and nerves of the inner are responsible and that endolymphatic hydrops is not the reason for the symptoms at all!

Be it as it may, endolymphatic hydrops is still currently the most acceptable theory of what happens in the inner ear during a Menière attack.

The fact of the matter is that we still do not know what exactly causes Menière’s disease and which parts of the inner ear is really involved.

The endolymphatic sac as the causeIt is known that the endolymphatic sac has immunological properties. This means it can respond to certain triggers with a defense mechanism called the immune response. It entails the recruitment of immunocompetent cells and the secretion of chemicals to protect the body. The problem however is that this reaction and chemicals can also damage the inner ear leading to hearing loss and dizziness, in fact all the symptoms of Menière’s disease. Autoimmune ear disease (AIED) is the recognized term for this process. Whether it is the body’s immune system that goes haywire or a virus that triggers the response the end result is the same namely damage to the inner ear. Autoimmune reactions are unlikely the cause of Menière’s disease in one ear but may be a cause if present in both ears.

Polyganglionitis episodica (PGE)Polyganglionitis episodica (PGE) is the episodic or recurrent inflammation in more than one ganglion (collection of nerve cell bodies) of specifically the cranial nerves. It is believed by some experts that the herpes simplex type 1 virus (HSV-1) is the root cause of Menière’s disease and that it explains all the symptoms of Menière’s disease. The herpes simplex type 1 virus resides in the nerve cell bodies, especially of the cranial nerves and has been associated with diseases such as cold sores (V cranial

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nerve) Bell’s palsy (VII cranial nerve) and vestibular neuritis (part of the VIII cranial nerve). The majority of people are contaminated with the herpes simplex type 1 virus in any case but because there are so many different strains, no one presents in the same way. If affected, the herpes simplex type 1 virus stays in the nerve cell body for life. It is usually dormant, quiet and is only awakened by stress in the host. By far the majority of infected persons will not have problems but in some patients stress and illness lowers the bodies immune response which then causes the virus to replicate, step out of line and attack the parts that is supplied by the affected nerve where it resides. In some patients it may only be the cochlea with hearing loss and tinnitus and in some patients only the vestibular organ with dizziness and loss of balance. Replication of the herpes virus is often seen in HIV and AIDS patients, known to have suppressed immunity. Also read the very interesting and comprehensive review by Dr Herman Hamersma (link)

Who gets Menière’s diseaseMenière’s disease is not that common. The prevalence is about 200 per 100 000. This represents about 0,2% of the population. There may be a genetic link with a familial predisposition. It is often misdiagnosed and this was clearly demonstrated by the Framingham study in the US that found that 2% (10 times more) patients believed that they suffered from Menière’s 's disease. This is clearly much higher than the expected number. Menière’s ’s disease is more common in female patients with a female to male ratio of around 3:2. In my practice it is most commonly seen in patients between 30 and 70 years, increasing every decade. It is rare in children and I have not seen it in a child. It is reported to be more common in white patients and in some studies in overweight patients. In my practice I see it more and more in black patients and other ethnic groups and I get the idea that social standing plays a more important role. I also cannot support the fact that it is more common in obese patients.

The risk of getting it in both ears (bilateral Menière’s ‘s disease) is controversial and varies greatly in the literature between 17% and 50%. In my own series and that of Dr H Hamersma we found the incidence to be around 15% to 20%. This is a realistic assumption as Dr Hamersma has managed patients with Menière’s disease since 1958. It would seem that the longer a patient has got it in one ear the smaller the risk is to develop it in the second ear. I support the findings of Silverstein that of those patients that will develop it in the second ear, 75% will do so within 5 years of the first ear.

What causes Menière’s ’s disease?The answer to this is unknown but it is believed that there may be an underlying genetic susceptibility or inner ear abnormality in a patient combined with some environmental factor. Possibilities include:1. Virus infections (especially herpes simplex type 1 or HSV-1)2. Injuries3. Blood circulation problems4. Allergies and food intolerance5. Immune reactions Menière’s syndrome and other causes that may mimic Menière’s disease

1. Vestibular migraine2. Recurrent labyrinthitis 3. Perilymph fistula (PLF), enlarge vestibular aquaduct (EVA) and superior canal dehiscence

(SCD)

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4. Acoustic neuroma (vestibular schwannoma)5. Multiple sclerosis6. Syphilis7. Congenital ear malformations8. Impaired blood supply in the distribution of the anterior inferior cerebellar artery, transient

ischaemic attacks (TIA) and stroke.9. Autoimmune inner ear disease10. Cogan syndrome11. Endolymphatic sac tumour12. Vestibular paroxysmia (neurovascular compression syndrome)13. Autosomal dominant sensorineural hearing loss 14. Susac syndrome

Links with other known diseasesThere is a link between Menière’s 's disease and conditions such as: migraine, motion sickness, hypothyroidism, autoimmune disease, allergy and diabetes. Migraine affects about 14% of the population and is almost 2 orders of magnitude more common than Menière’s 's disease. Migraine variants with prominent ear symptoms are more common than Menière’s 's disease. Patients with Menière’s 's have migraine 50% of the time. In research studies it has been demonstrated that some of the above conditions are more common in patients with Menière’s 's disease than in the rest of the population.

How is Menière’s ’s disease diagnosed?The following criteria are used to diagnose Menière’s 's disease (MD).

1. Two or more episodes of vertigo or dizziness, each lasting 20 minutes to 24 hours2. 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

1. Sensitivity to visual stimuli and bright light2. Sound distorsion and sensitivity in the affected ear3. Headaches4. Anxiety and fear of falling5. Diarhoea, sweating, nausea, vomiting and increased heart rate during the attacks.6. Loss of balance and disequilibrium7. Exhaustion after an attack and sleepiness

The problem with the diagnosis of Menière’s ’s disease is that it is very rare and that it often occurs after hours and with specialists not always available. The emergency doctor is most often confronted during an attack. Because life threatening conditions such as heart attacks and stroke can present with similiar symptoms, priority is given to exclude and manage these conditions, often forgetting about Menières disease.

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Drop attacksDrop attacks (Otolithic crisis of Tumarkin) is a form of Menière’s ’s disease where patients experience sudden unpredictable falls whilst maintaining full consciousness. They are attributed to sudden mechanical deformation of the otolith organs (utricle and saccule), causing a sudden activation of vestibular reflexes. Some patients report that the whole world suddenly turns upside down.

Triggers of attacksSome patients identify triggers that may set of an attack. These include stress, overwork, fatigue, emotional distress, additional illnesses, altitude changes, seasonal changes, certain foods( see Migraine diet), and too much salt in the diet. Caffeine can aggravate the tinnitus and alcohol always aggravates the symptoms. If not, it is unlikely Menière’s disease.

The natural course of Menière’s ’s diseaseThis is very unpredictable. Menière’s disease can occur as single attacks separated by months or even years. It may also occur in clusters of many attacks per week. Sometimes a patient’s attacks can stop, never to return. Patient need not present with the full picture from the onset. Some patients with hearing loss in one ear my only develop vertigo and the other symptoms of Menière’s disease years later. This is sometimes referred to as delayed endolymphatic hydrops. Other patients may present with only vertigo and later develop hearing fluctuation and hearing loss. After recurrent attacks patients may develop permanent hearing loss and vertigo attacks may become less and even disappear often leaving a patient with permanent imbalance, disequilibrium and tinnitus. This happens due to the recurrent and permanent damage that occurs in the inner ear over time. Non- specific symptoms like neck stiffness, motion sickness, queasiness, unsteadiness, vision disturbance and concentration problems may later become evident. Early on in the disease process a low-frequency sensorineural hearing loss that fluctuates is commonly found. This usually changes into either a flat loss or a peaked pattern over time. Menière’s disease rarely leads to total deafness. Hearing loss can usually be aided with a hearing aid or cochlear implantation (CI).

Clinical examination and findingsThe clinical examination may not reveal much. This is especially true if a patient is not in an attack. The ideal scenario is to see a patient in an attack although this is not always possible. In an attack a patient will have difficulty with their balance function with a tendency to veer to a specific side whilst walking and even falling. Patients are usually very sick, may be pale, nauseous and vomit. They may be sweaty and even have diarrhoea. In an emergency setting a heart attack and stroke are usually excluded. Patients will usually demonstrate nystagmus (abnormal involuntary repetitive jerking eye movements). They may demonstrate sensorineural hearing loss with tuning fork testing. The ideal is to perform a proper hearing test, if possible. Other neurological findings are absent. In later stages of Menière’s ’s disease especially after permanent damage have occurred to the inner ear, abnormalities of the vestibular system may be picked up in addition to the hearing loss even if the patients is not in an attack.

Special tests for Menière’s ’s disease The following tests may be of use in the diagnosis and management of Menière’s disease1) Diagnostic hearing tests including speech discrimination scores2) Immittance and reflex testing of the ear.

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3) Auditory brainstem response audiometry (ABR). 4) Balance and vestibular testing (VNG, DVA, VHIT and VORTEQ). 5) Vestibular evoked myogenic potentials (VEMP).6) Computed tomography (CT scan) of the temporal bone.7) Magnetic resonance imaging(MRI) with gadolinium to exclude central causes and tumours8) Blood tests.

The psychological impact of Menière’s ’s diseaseIn my practice I have seen many patients with Menière’s ’s disease over the years and can testify to the high number of psychological problems present in these patients. Depression, anxiety, fear, anger and avoidance behaviour are common. People change their life style, develop avoidance behaviour, stop going out shopping and to restaurants and is afraid of driving. They feel that they cannot plan anything for the future. I have seen adult men crying out of desperation. Relationships suffer and spouses are often non sympathetic. When one partner has Menière’s ’s disease the other one also suffers from it in an indirect way.

How can Menière’s ’s disease be managed. The best result is for the patient to go into spontaneous remission.Never say “there is no treatment” and “you must learn to live with this disease”.The dilemma is that until we know exactly what causes Meniere’s disease all our treatments are aimed only at symptoms control and in a certain way at prevention of progressive damage to the inner ear structures. Any treatment modality that claims a success rate of 60% is exactly the same as placebo. As long as the treatment is not harmfull and not expensive, I suppose helping 60% of patients is better than nothing. However, the point that I would like to make is to take care not to accept any claimed success with a specific treatment. Not a single treatment has shown to be superior to others in all scenarios and in all patients. There are many trials where some of the different treatment modalities have been compared with others. The outcome of a treatment depends on different aspects and is not the same for each patient.

The management of Menière’s ’s disease can be devided in:1. Life style modification2. Medication3. Devices4. Surgery5. Vestibular rehabilitation 6. Psychological support7. Hearing loss and tinnitus

Life style modificationThis usually does not work but is advised for the minimal benefit to a few. It includes:

1. Salt restriction 2. Caffeine restriction 3. Stress relieve4. Alcohol restriction

MedicationTreatment with medication can be divided in:

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1. Medication to stop the attacks and reduce the symptoms.2. Medication to prevent the attacks.3. Medication to destroy the inner ear function.

DevicesDevices like the Menniet device are no better than placebo. As a myringotomy and grommet placement is required for using the device it is advised that before buying a device a myringotomy and a grommet placement is performed first to see if the symptoms do not resolve. Improvement after myringotomy and grommet placement has been documented in the literature.

Surgery for Menière’s disease

Historically a number of surgical procedures were described to treat Menière’s ’s disease of which many are not preformed anymore. Patients should not receive any surgery unless their vestibular system has been properly assessed and the function documented. The most common procedures and operations performed nowadays include:

1. Myringotomy with placement of a grommet (ventilation tube)2. Myringotomy with intratympanic administration of medication3. Endolymphatic sac surgery4. Labyrinthectomy

a. Transcanal (TC) approachb. Transmastoid (TM) approach

5. Vestibular neurectomya. Middle cranial fossa (MCF) approachb. Retrosigmoid (RS) approachc. Retrolabyrinthine (RL) approach

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Vestibular rehabilitation

Vestibular rehabilitation will not cure Menière’s ’s disease. It consists of specific exercises aimed at helping the brain to compensate for the lost or reduced function of the inner ear due to Menière’s ’s disease. Although some of these programs can be found on the Internet it is strongly advised to consult a vestibular rehabilitation specialist (physiotherapist or audiologist trained in vestibular rehabilitation) for maximal benefit. Part of vestibular rehabilitation includes information on life style modification and ways to reduce the risk of loss of balance and injuries at home. Hearing loss and tinnitus

At any stage of Menière’s ’s disease, patients may benefit from hearing amplification. Hearing aids, bone conduction devices and cochlear implantation (CI) are available options. It is suggested to obtain hearing aids from a trained audiologist and a trial period should always be offered. A patient should have a choice of more than one hearing aid.

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Tinnitus can be managed in many ways. Tinnitus counseling and retraining therapy are offered by some trained audiologists and work well. Often hearing aids may improve the tinnitus. There is always something to be done and patients should never be told to live with the tinnitus. Patients should be warned against treatments, devices and medication on the market that claim to cure tinnitus and should consult with an ENT surgeon or audiologist before using these.

Psycholocical support

Menière’s ’ disease is not a psychological disorder but it is common for patients with Menière’s ’s disease to develop psychological problems. A psychologist may have to be consulted and if symptoms are severe a psychiatrist may need to prescribe medication. A partner of a patient with Menière’s ’s disease may also benefit from psychological support.

Dr Hofmeyr’s experienceDr Hofmeyr and his team have managed more than 350 patients with acoustic neuromas and have succesfully operated on more than 250 patients. He shares his experience mainly with Dr Tommy Bingle (neurosurgeon). Dr Hofmeyr has surgical experience in the middle cranial fossa (MCF), translabyrinthine (TL) and retrosigmoid (RS) approaches

Further reading1) http://www.medifocus.com/2009/landingp2.php?gid=TL008&?a=a 2) https://en.wikipedia.org/wiki/Ménière's_diseases3) http://dizziness-and-balance.com/disorders/menieres/menieres.html 4) http://www.entnet.org/content/menieres-disease 5) http://www.earsinus.com 6) http://www.mdjunction.com/forums/menieres-disease-discussions/general-support/3068476-

acyclovir-treatments-dr-gacek7) http://www.entinstitute.co.za/C_library.asp

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