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Vestibular Disorders A-Z GLOSSARY OF TERMS Acknowledgements Whirled Foundation thanks the Meniere's Society (UK) for allowing us to reprint this series, which originally appeared in their journal "Spin". Some changes and additions have been made to suit Australian conditions by David Brigden, who has also provided references. 1 Contents A .............................................................................................................................................................. 3 B .............................................................................................................................................................. 4 C .............................................................................................................................................................. 6 D .............................................................................................................................................................. 8 E ............................................................................................................................................................ 10 F............................................................................................................................................................. 12 G ............................................................................................................................................................ 14 H ............................................................................................................................................................ 15 I ............................................................................................................................................................. 17 L ............................................................................................................................................................. 18 M ........................................................................................................................................................... 19 N ............................................................................................................................................................ 22 O ............................................................................................................................................................ 23 P ............................................................................................................................................................ 25 R ............................................................................................................................................................ 26 S............................................................................................................................................................. 26 T ............................................................................................................................................................ 28 U ............................................................................................................................................................ 29 V ............................................................................................................................................................ 30 Z............................................................................................................................................................. 31 2 A Acoustic Neuroma A rare non-cancerous tumour affecting the vestibulocochlear (acoustic) nerve which carries auditory and balance information from the inner ear to the brain. Symptoms of progressive hearing loss, dizziness or imbalance (and occasionally severe vertigo) and tinnitus 1 2 are similar to those of Meniere's. When Meniere's is first being diagnosed in a patient, a CT scan or an MRI is often ordered to exclude the possibility of an acoustic neuroma. Acrophobia Acrophobia is an extreme or irrational fear of heights. Vertigo (loss of balance/dizziness) is often incorrectly used to describe the fear of heights. Acupuncture Acupuncture literally means ‘to prick with a needle’. It can be used to treat a variety of conditions from anxiety, arthritis and asthma, through to back pain, circulatory problems, depression and digestive problems. Agoraphobia Agoraphobia is often loosely defined as a fear of open spaces. More precisely, agoraphobia is an anxiety disorder which consists of the fear of experiencing a difficult or embarrassing situation from which the sufferer cannot escape, such as situations where they feel trapped, insecure, out of control, or too far from their personal comfort zone. Panic attacks are a common feature of agoraphobia. Alcohol Alcohol can be a problem for some people with Meniere's disease as it can cause the tiniest blood vessels at the very end of the system to contract and so restrict the blood supply to the inner ear. Small amounts of alcohol – one standard drink, such as a glass of beer (285ml), a glass of red wine (100ml) or a pub measure of spirits (30ml) - can actually improve the peripheral circulation, but any more has the opposite effect. Audiogram An audiogram is a test to see if your hearing is impaired and which ear is affected. Using a set of headphones, you will be asked to listen to sounds produced at different frequencies and levels of loudness. The audiologist will ask you to indicate each time you hear a sound, and will record the softest sound you can hear at each frequency. This is known as your hearing ‘threshold’. One ear is tested and then the other. 1 2 Royal Australian College of General Practitioners Complete Home Medical Guide, Dorling Kindersley Aust P/L, 2006. Professor Paul Fagan, Clinical Assessment of Vertigo, Meniere's disease Brochure-2, Meniere's Support Group of NSW Inc., 2005. 3 Audiologist Audiologists are university graduates with postgraduate qualifications in audiology or equivalent training. The Audiological Society of Australia, the professional society for audiologists, has a clinical certification program and members participate in ongoing professional development to retain their Certificate of Clinical Practice. Audiologists have knowledge, training and experience in: assessing hearing performing complex diagnostic tests to determine the cause of the hearing problem helping clients choose suitable hearing aids and/or assistive listening devices taking ear impressions for custom-made ear pieces prescribing and fitting hearing aids checking that devices are comfortable and adjusting the sound so it is as clear as possible teaching clients how to use their hearing aids designing tailored hearing improvement programs3 Your neurologist or ENT specialist may refer you to an audiologist for diagnostic testing. Audiologists are also the appropriate health professionals for people with Meniere's disease to consult for hearing aid selection and fitting. B Balance System The sense of balance is controlled by a ‘balance system’ which brings together information from three different sources: 1. Vision – you can literally see where you are and where you are going. 2. All the sensors in the body – the joints, muscles, soles of the feet and so on. With these sensors you can feel where you are and how you are moving. 3. A set of fluid-filled tubes in the inner ear known as the vestibular organ. This gives out signals whenever the head is moved or tilted. Information about your position and movements from these three sources is fed to the brain. The brain acts like a computer, combining the information from all the senses to give a stable picture of the world and to control head, body and eye movements. If any part of this balance system is giving out unusual or faulty information, then dizziness and imbalance may result. 3 Australian Hearing website: www.hearing.com.au/clinicians 4 Benign Paroxysmal Positional Vertigo (BPPV) The definition of BPPV can be broken down as follows: Benign - because it is not due to serious disease, though it can be quite disabling. Paroxysmal - because it occurs in short bursts of up to a minute only. Positional - because it is provoked specifically by movement to or from certain positions. Vertigo - the type of dizziness defined as an illusion of movement, either of the sufferer or of their surroundings (not just a fear of heights!). BPPV is caused when loose chalk crystals get into the wrong part of the inner ear. These microscopic crystals (otoconia or otoliths, literally ear stones) should be embedded in a lump of jelly. Their function is to weigh down the jelly and make that part of the ear sensitive to gravity. That is how we tell which way is up. The crystals are constantly being re-absorbed and reformed and in the course of time fragments come loose. Lying flat can then occasionally cause some of the loose debris to fall into one of the three semicircular canals - the parts of the ear responsible for sensing rotation. Movement in the plane of the affected canal will then cause the crystals to move along the canal, stimulating it and giving rise to the sensation of rotation. Anything which loosens the chalk crystals can give rise to a bout of BPPV. It can occur as a result of any ear disease, including viral infections (vestibular neuritis) and Meniere's disease. "Generally, BPPV resolves by itself within six months or so. Treatment options in the meantime could include medications to help control nausea and special manoeuvres designed to dislodge otoconia. These manoeuvres boast an 80% success rate"4. Most commonly the Epley manoeuvre (or sometimes the Semont manoeuvre) would be used to treat BPPV. The diagnosis, and the treatment, can be carried out by your ENT specialist. Physiotherapists with an interest in vertigo and vestibular rehabilitation would also be familiar with the treatment manoeuvres. There are also home exercises, but it is important to have your BPPV diagnosed initially and the correct exercise prescribed. Benzodiazepines Benzodiazepines such as diazepam (Valium) and lorazepam (Ativan) have some use in relieving the anxiety associated with the initial Meniere’s attacks and preventing stress from affecting the patient. Many of these medications also act as vestibular suppressants. All these drugs are addictive and must be prescribed cautiously.5 4 http://www.betterhealth.vic.gov.au/bhcv2/bhcarticles.nsf/pages/Vertigo_benign_paroxysmal_positional_vertigo Professor WPR Gibson, The Treatment of Meniere's Disease by the General Practitioner, Meniere's Disease Brochure-3, Meniere's Support Group of NSW Inc., 2005 5 5 Betahistine Betahistine is also known by the brand name Serc. It is a histamine analogue and is one of the most commonly prescribed drugs for Meniere's disease, vertigo or dizziness in the UK. Betahistine is used to treat vertigo, tinnitus and hearing loss associated with Meniere's disease. Betahistine works by acting on histamine receptors that are found in the walls of blood vessels in the inner ear. By activating these receptors, a process is started which is thought to reduce the pressure of the fluid that fills the labyrinth in the inner ear. This helps to relieve the symptoms associated with Meniere's disease. Serc is readily available in Australia. It is a prescription only medication. C Caffeine Coffee, tea, and other drinks containing caffeine can sometimes be a problem for sufferers of Meniere's disease. Caffeine can cause the tiniest blood vessels at the very end of the system to contract and so restrict the blood supply to the inner ear. Too much caffeine can make Meniere's symptoms worse. Calcium Channel Blockers Calcium is a major ion within the inner ear. It competes with potassium for receptor sites and also has a catalytic effect. Calcium disturbance may be involved in Meniere’s disease.5 Calcium channel blockers, sometimes called calcium antagonists, are drugs that prevent, at different parts of the body, the activity of calcium. The calcium channels in our body are different depending on where they are. The calcium channels in our body are different depending on where they are, (for example) whether they are on the blood vessels of our head, our inner ear or on our heart. Therefore, the drug that is given has to be specific to those channels. Stugeron® (cinnarizine) is a calcium antagonist, although not a powerful one. It acts mainly on small blood vessels and prevents constriction on these vessels. In theory it therefore improves the micro circulation of the ear. Caloric Test The caloric test investigates the function of the horizontal semicircular canal in the inner ear. It involves stimulating the semicircular canal, by introducing warm or cold water or air into the outer ear canal, and recording the eye movements (nystagmus) produced. It is a test that looks at the balance function and it may help to differentiate which ear is affected. In Meniere's disease it can be vital to have this information if surgery is being considered. 6 Cinnarizine (Stugeron®) Cinnarizine is a medication that has been used in Europe since 1966 for motion sickness, to block vertigo, and for Meniere's disease.6 Cinnarizine is an antihistamine, a calcium channel blocker, probably a vestibular suppressant, and has dopamine antagonist characteristics.7 "Cinnarizine has been shown to be effective in controlled trials. It seems to help between 30-50% of patients. The main side effect of cinnarizine is weight gain, and other effects are drowsiness, gastric upsets and possible depression. The suggested dose is 25mg three times daily until vertigo is controlled."5 Stugeron® (cinnarizine) is imported into Australia in 25mg tablets. As the Consumer Medicine Information (CMI) leaflet is not available in Australia, patients or doctors should download or print the South African electronic package insert A. 5.7 available from: http://home.intekom.com/pharm/janssen/stugeron.html Cochlea The cochlea is part of the inner ear. The cochlea is the sense organ that translates sound into nerve impulses to be sent to the brain. The cochlea contains three fluid chambers: two contain the same type of fluid but the middle one, the endolymph chamber, contains a different type of fluid – this is the one that gets swollen in Meniere's disease. Tiny bones in the middle ear transmit sound from the eardrum across the middle ear and vibrate against the cochlea. Vibrations cause tiny hair cells in the fluid inside the cochlea to vibrate. This generates nerve impulses that then travel to the brain. The cochlea is shaped like a spiral and gets its name from the Greek word 'Kokhlias' meaning snail. Cognitive Behaviour Therapy Cognitive Behaviour Therapy (CBT) is a short-term treatment which can help you to change how you think (cognitive) and what you do (behaviour). Changes in these areas can help you to feel significantly better in coping with your day-to-day life. CBT is used to solve a variety of problems, from anxiety, sleeping difficulties and depression to drug and alcohol addiction. CBT is also used to help people cope with Meniere's in general, and tinnitus in particular. Counselling Counselling sessions are designed to help self-exploration and understanding, to identify thoughts, emotions and behaviours that lead to a greater sense of personal resources and self-determined change. 6 7 Haybach PJ. Meniere's disease – what you need to know. Portland (OR): Vestibular Disorders Association; 1998. p.179. Hain TC, Yacovino D. Pharmacologic treatment of persons with dizziness. Neurol Clin 2005 23 831-853. p. 836. 7 Counselling helps you to find ways to adapt, make the most of your situation and gain confidence in yourself to try new ways of thinking and feeling that can improve the quality of your life. Counsellors are trained to listen. You can express your thoughts and feelings freely knowing that the counsellor is there for you and will understand your situation. The counsellor will not impose his or her views on you; it is your individual perception of the situation that is important. Often family and friends are themselves too involved in your life to allow you to express yourself freely. The relationship with the counsellor is confidential. CT Scan A CT (or CAT) scan stands for computerised tomography. The scan takes pictures of the body from different angles using x-rays and uses a computer to put them together. The CT scanner is a large machine. You will be required to lie down inside the scanning machine. The machine will move you backwards or forwards a small distance after each x-ray. You will be asked to lie very still, but breathe normally. The radiographer operating the machine will leave the room during the scan, but will be able to talk to you via an intercom. Several scans will be carried out. It is a painless procedure and may last from a few minutes to 30 minutes. D Decibel (dB) The decibel is a logarithmic unit of measurement that expresses the magnitude of a physical quantity. It is used to measure the magnitude of a sound, or sound pressure level (dB SPL). It is also used in hearing tests, where hearing is measured in decibels of hearing level (dB HL) at different frequencies, and recorded on an audiogram. Dexamethasone Professor Marcus Atlas describes the use of this steroid in the treatment of Meniere's: "An emerging treatment for Meniere’s disease is the use of dexamethasone medication applied to the middle ear, either through a small ventilation tube (grommet) or with a syringe through the ear drum. Patients are asked to lie with their head turned to allow [the dexamethasone] to reach the round window membrane, a partition between middle and inner ear, and therefore the medication diffuses into the inner ear. Dexamethasone is a steroid and may be particularly valuable in patients in whom an underlying immune cause of Meniere’s disease is suspected. 8 Dexamethasone treatment to the middle ear avoids the side effects of oral or injected steroid treatment. However, long term controlled clinical trials have not been completed for this treatment. Early studies are encouraging."8 Diagnosis In 1995 the American Academy of Otolaryngology, Head and Neck Surgery published guidelines9 to assist the diagnosis of Meniere's. Under these guidelines, a diagnosis of definite Meniere's disease requires all of the following: * Two or more definitive spontaneous episodes of vertigo lasting 20 minutes or longer. * Audiometrically documented sensorineural hearing loss on at least one occasion. * Tinnitus or aural fullness in the affected ear. * Exclusion of other causes, such as vestibular migraine or acoustic neuroma. In Australia, diagnosis to this standard would usually be done by an ENT specialist or a neurologist. The specialist would arrange hearing tests, and possibly also balance tests such as the caloric test, and may order an MRI or CT scan to exclude other causes. Diet Coffee, tea and alcohol can be a problem as they cause the tiniest blood vessels at the very end of the system to contract and so restrict the blood supply to the inner ear. Following a low-salt diet can help to reduce the frequency and severity of attacks of vertigo. Detailed information on reducing salt intake can be found on Dr. Trevor Beard's website: www.saltmatters.org Disequilibrium Disturbance of balance, instability. Diuretics Diuretic medicine increases the production and flow of urine from the body, used to remove excess fluid from the body. In Meniere’s disease diuretics are used to help remove the excess salt in the fluid from the body. Atlas MD. The surgical management of Meniere's disease, Meniere’s disease brochure-4, Meniere’s Support Group of NSW Inc., 2005. Monsell EM, Balkany TM, Gates GA, et al. Committee on hearing and equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere's disease. Otolaryngol, Head & Neck Surg 1995; 113: 181 -185 8 9 9 Dizziness & Vertigo Although these two words overlap in meaning in everyday speech, it is best to reserve the term 'vertigo' for illusions of movement, such as the sensation that the external world is spinning. Vertigo is one of the symptoms considered in the diagnosis of Meniere's. The term 'dizziness' is a broader term and includes physical unsteadiness, imbalance, and lightheadedness. Drop Attacks Drop attacks, also known as Tumarkin's Otolithic Crisis, are when a person will fall to the ground with no warning. The attacks are severe, but quite brief, less than a minute in duration. The person will remain awake and will not lose consciousness. A fall due to a loss of consciousness would be due to some other cause, and should be medically investigated. Drop attacks are sometimes experienced in the later stages of Meniere's disease. They do not affect everyone, and if they do occur, they usually settle after one or two years.10 E Ear Wax T. C. Hain states: "Ear wax is a normal product of the ear which protects the skin of the ear from water and infection. Ear wax is formed from wax glands in the external ear canal as well as other components such as dead skin, sweat, and oil. "There are two types described, wet and dry, which are inherited. Dry wax is common in Asia, while wet wax is common in Western Europe. Wet wax can be either soft or hard, the hard wax being more likely to be impacted. Too little ear wax increases the risk of infection. Too much wax also increases the incidence of infection and hearing loss. So, you want just enough."11 Ear wax is said to be impacted if it plugs up the ear, causing hearing to be reduced, and a full feeling in the ear. Ear wax is also one of the most common causes of hearing aid malfunction. If you are subject to impacted ear wax, the best way of having it removed is by an ENT specialist (ear doctor). The ear doctor would use an examining microscope and will remove wax with a cerumen spoon, or suction, or both. Do not try to use a cotton tipped applicator (cotton bud) to remove impacted wax, as this will push the wax deeper into the ear canal. 10 11 Gibson WPR. The natural course of Meniere’s disease, Meniere’s disease brochure-1, Meniere’s Support Group of NSW Inc., 2005. www.dizziness-and-balance.com/disorders/hearing/wax2.html 10 Electrocochleography This test evaluates the signal that comes from the ear when a loud sound is put into the ear. Electrodes are used to measure the part of the brain wave that comes from the inner ear and if it is abnormal, it provides evidence for Meniere’s disease or hydrops. Electronystagmography (ENG Test) This is a test where eye movements are measured and observed under various stimulus conditions. The purpose of the ENG is to determine if there is something wrong with the vestibular (balance) portion of the inner ear. If dizziness is not caused by the inner ear, it might be caused by disorders of the brain, by other medical disorders such as low blood pressure, or by psychological problems such as anxiety.12 Endolymph (and Endolymphatic Hydrops) Within the inner ear there are two types of fluid separated by membranes. Endolymph is rich in potassium and is similar in chemical makeup to intracellular fluid. Perilymph is rich in sodium and is similar to spinal fluid. In Meniere’s disease there is too much endolymph fluid in the inner ear. This condition of excess fluid is referred to as endolymphatic hydrops.13 14 Endolymphatic Sac The endolymphatic sac is part of the membranous inner ear. The role of the endolymphatic sac in Meniere's disease is described by Professor Gibson: "The endolymphatic sac secretes substances which increase endolymph production and osmotically attract fluid into its cavity. One theory is that a narrow endolymphatic duct becomes blocked by debris in Meniere’s disease. Episodes of over secretion of substances by the endolymphatic sac cause a sudden restoration of longitudinal flow and result in attacks of vertigo.” "Gradually as the disease progresses the endolymphatic sac becomes so damaged that it can no longer clear the debris, and then the attacks of vertigo cease but hearing is permanently affected."15 Endolymphatic Sac Surgery Surgical treatment of the endolymphatic sac can take a number of forms, and may include drainage, shunts, decompression, or removal of the sac. In 2006, Professor Gibson reported an 84% success rate in eliminating vertigo attacks completely or reducing them to manageable levels, in a series of 200 endolymphatic sac removal operations. He described the operation as follows: www.dizziness-and-balance.com/testing/ENG/engrot.html www.dizziness-and-balance.com/anatomy/endolymphatic-sac.htm 14 www.menieres.org.au/causes.php 12 13 15 Gibson WPR. The natural course of Meniere’s disease, Meniere’s disease brochure-1, Meniere’s Support Group of NSW Inc. 2005. 11 "The surgery is not demanding for the patient. A small incision is made in the scalp in the crease behind the ear. There is no need to remove any noticeable amount of hair. The mastoid bone is drilled away to reveal the dura or lining of the brain cavity. The endolymphatic sac is located and peeled away from the dura. The exact position of the neck of the endolymphatic sac can be determined by using an endoscope. "The surgery takes about 1½ hours to perform. Afterwards the patient may feel unsteady but rarely has any significant vertigo. Most patients can leave hospital within 24 hours. "It seems that removal of endolymphatic sac surgery hastens the ear towards the ‘burnt out’ stage when the attacks of vertigo become insignificant and the hearing is permanently impaired. "The surgery is indicated when other less invasive treatments have proved to be of no avail."16 Epley Manoeuvre This is the most common procedure to relieve the symptoms of BPPV (Benign Paroxysmal Positional Vertigo). The Epley Manoeuvre begins by making the patient dizzy with the appropriate Hallpike test. The patient is then rolled over (in stages, pausing for about half a minute in each position) onto the opposite side, before being sat up again. This manoeuvre floats the chalk crystals that cause the problem, around the affected canal and out the far end, back to where they belong. F Family and Friends An important part of the management of vestibular disorders is the ability of relatives and friends to understand what their loved one or friend is experiencing, as Meniere's does not have the usual visible symptoms and medicinal cures of more common complaints. Fear There is often fear and loss of confidence associated with Meniere's disease which prevents many people from leading a normal life. This feeling is real and understandable because no one can predict when an attack is going to occur. Social contact often becomes limited because of the worry of experiencing an attack in public places. All this fear boils down to the one fear of not being able to cope. When you know how to handle things, they look different. The anxiety doesn’t go away but you know you have some control and this is the key to dealing with fear. Eventually you will learn how to handle both Meniere's disease and the fear. Gibson WPR. Endolymphatic Sac Surgery, The Balancer Newsletter No. 44, Meniere’s Support Group of NSW Inc. June 2006. Reprints available from Meniere's Australia – quote item code: IS046-09/06 16 12 Fight or flight When you feel particularly stressed, anxious, angry or fearful, you may be more likely to experience dizziness. This is because some of your body’s automatic reflexes are linked to your emotions and thoughts through a process called the “fight or flight” response. Your brain interprets any strong emotions or frightening thoughts as a signal that you are in danger, and automatically prepares your body to either fight or run away. This means that your heart rate increases and your breathing gets faster as blood is pumped to your muscles. A side effect of this is that you may feel sick or dizzy, since breathing too fast causes you to take in too much oxygen, which can make you dizzy. Practising relaxation or meditation regularly can help in reducing fear and anxiety. Discussion of these issues with a psychologist can also be helpful. Flying In our experience, most people with Meniere's disease report no difficulty at all with flying, indeed some report feeling better for the experience. If your main problem when flying is ear pain, it is not likely to be a result of Meniere's disease. Frequent attacks of vertigo, or the uncertainty of when an attack might occur, may make you reluctant to travel, but many members have reported being able to travel successfully. Meniere's Australia has a number of useful information sheets on travelling with Meniere's. Forgetfulness The balance system is a complex integrated system that normally functions subconsciously. When your balance is working well, you don’t need to think about where your arms and legs need to be, it happens automatically. When your automatic balance mechanisms are not working well, you have to think about your balance and it becomes a conscious process. You need to concentrate on how you are going to get across the room to the door, or where you are going to put your feet. While your brain is consciously occupied with balance, your memory and other thought processes may be less efficient than normal. Fullness Many people who have Meniere's disease describe a feeling of ‘fullness’, or a feeling of pressure in their ear. Meniere's disease involves an excess of the sodium-rich fluid (called endolymph) in the inner ear. That is why reducing salt in your diet may be helpful, because it may reduce the accumulation of that fluid. The build up of endolymph is thought to account for the feeling of fullness in people’s ears before an attack. 13 G Genetics According to Professor T C Hain, "about one in three patients with Meniere's disease have a firstdegree relative with Meniere's disease"17 although other estimates of familial occurrence tend to be somewhat lower, ranging between 5% and 20%18. Although genetic investigations are being carried out, so far no conclusive genetic causes have been identified19. Gentamicin Gentamicin is an antibiotic belonging to a family of similar drugs called aminoglycosides. When it is applied behind the ear drum by intratympanic injection, it damages or destroys the balance organs of that ear, reducing or eliminating vertigo attacks and drop attacks20. Grommet A grommet is another name for a tympanostomy tube. Grommets are inserted into the eardrum to allow air into the middle ear space. Professor Tumarkin in Liverpool, England reported success using grommets in the 1950’s when some sufferers reported a lessened sensation of aural fullness and fewer attacks. There is a hypothesis that small changes of ear pressure can be transmitted into the inner ear. Small pressure changes may be beneficial but larger pressure changes may irritate the inner ear causing an increase in endolymph volume. The extra volume of endolymph may then result in attacks of vertigo. The grommet connects the middle ear cavity to the ear canal. This prevents the middle ear from developing any positive or negative pressure differences. A grommet is a tiny plastic tube. The specialist makes a tiny incision in the eardrum and then inserts the grommet to prevent this incision from healing. Hain TC. Etiology (cause) of Meniere's Syndrome [Online] 2010 Jan 17 [Accessed 2011 May 2 URL: www.dizziness-andbalance.com/disorders/menieres/men_eti.html 18 Medifocus Guidebook on Meniere's Disease. Updated Jan 10, 2011 Available from: www.medifocus.com 19 Jen JC. Genetics of vestibulopathies. Adv Otorhinolaryngol. 2011;70:130-4. Epub 2011 Feb 24. Abstract available at: www.ncbi.nlm.nih.gov/pubmed/21358195 20 Murofushi T, Halmagyi GM, Yavor RA. Intratympanic gentamicin in Ménière's disease: results of therapy. Am J Otol. 1997 Jan;18(1):527. Abstract available at: www.ncbi.nlm.nih.gov/pubmed/8989952 17 14 H Hair cells – in relation to balance Hair cells are the sensory receptors in both the auditory system and the vestibular system. They are topped with hair-like structures called stereocilia, which transform the mechanical energy of movement of the endolymphatic fluid into nerve impulses. Hair cells in the inner ear send balance signals back to your brain via the vestibular nerve. Your whole inner ear is filled with endolymphatic fluid. The fluid moves around your inner ear when you move. The hair cells in your balance organ are activated to send signals to your brain when they are moved by this fluid.21 Hair cells – in relation to hearing Like the balance organ, the hearing organ is also covered in tiny sensory hair cells, and is filled with the same endolymphatic fluid. In Meniere's disease, the sensory hair cells that are moved by low frequencies are damaged first, causing tinnitus and hearing loss for low pitched sounds. As the disease progresses, the hair cells on the rest of your hearing organ can also become damaged, eventually affecting your ability to hear all sound frequencies.21 Hearing loss In Stage 1 of Meniere's there may be hearing distortion and some temporary hearing loss at the time of a vertigo attack but often the effects on hearing may not be noticed, as the vertigo, nausea and vomiting demand one's full attention. If Meniere's progresses to Stage 2, the hearing loss does not return to normal in between attacks.10 The permanent hearing loss from Meniere's starts as a low-tone loss, but slowly spreads to other frequencies if the attacks continue. Hearing aids Hearing aids are very successful for people with Meniere’s disease providing the individual characteristics of the hearing loss are properly addressed. The wearing of a hearing aid also reduces tinnitus perception in the affected ear. Unfortunately, there is still a current belief that a hearing aid is not very helpful for people with Meniere’s disease. There are many people with Meniere's who would greatly benefit from a properly adjusted hearing aid.22 If Meniere's progresses to involve the other ear (bilateral Meniere's) a hearing aid should be fitted to each ear, for best results. 21 22 Professor Lucy Yardley & Dr Sarah Kirby, The Balance System and Meniere's Disease. Spin 63, Meniere's Society (UK) 2008. Celene McNeill (audiologist), Hearing loss in Meniere’s disease – the overlooked symptom. Equilibrium, Summer 2009. 15 Hormones Hormones are chemicals released by cells that affect cells in other parts of the body. There are a group of Meniere's sufferers who report an in increase in symptoms around menstruation. The exact mechanism is uncertain. One potential explanation is that the change in the concentration of female hormones oestrogen and progesterone immediately before a period, together with an increase in a further hormone called aldosterone, leads to fluid retention, and this increases the volume and pressure of the endolymph within the inner ear hence worsening the vertigo. Other explanations have suggested that the hormonal change at this time alters the blood’s viscosity hence there is a reduction in the inner ear’s microcirculation with a loss of function.23 Hydrops Endolymphatic hydrops is a disorder of the vestibular system of the inner ear. It stems from abnormal fluctuations in the fluid called endolymph, which fills the hearing and balance structures of the inner ear. In Meniere's disease there is too much endolymph fluid in the inner ear. This condition of excess fluid is referred to as endolymphatic hydrops. It is often just called "hydrops". Hyperacusis When a person is unable to tolerate everyday sounds that don’t seem to bother other people the medical term given to this is hyperacusis. About 8% of the population report hyperacusis. There are many causes of hyperacusis. Migraine needs to be considered as a cause. In addition to hyperacusis, persons with migraine often have sensitivity to bright light, motion intolerance and sensitivity to strong smells. Some Meniere's patients report abnormal sensitivity to everyday sounds and may have hyperacusis. A popular treatment approach is TRT (Tinnitus Retraining Therapy) which is a mixture of psychotherapy and masking (when used for tinnitus) or sound generators (when used for hyperacusis). TRT requires considerable time commitment. Audiologists may conduct TRT sessions. Hyper pressure treatment Professor Gibson explains hyper pressure treatment (positive pressure pulses) for Meniere's: "An extension of the grommet concept is to deliver small beneficial pulses of hyper pressure into the middle ear. The pulses gently act on the windows of the inner ear and milk some of the excess endolymph away. "The concept was very carefully researched in Sweden and this has led to a commercially available machine, the Meniett® device. 23 Mr Ian Johnson, Questions and Answers. Spin 33, Meniere's Society (UK). 16 "This device delivers a series of calibrated hyper pressure pulses during a cycle lasting about three minutes. Meniere's sufferers use the machine three times daily while the disease is active and once a day while the disease is in remission. "A grommet must be present to use the device. The machine costs around $5000 and is not available through the Medicare system." 24 As noted by Associate Professor Stephen O'Leary, "the major advantage of the Meniett…is that there is no risk to hearing through the treatment (apart from the very small risk associated with the placement of the grommet. In this respect the Meniett has an advantage over other non-medical treatments, most of which carry a small risk of damaging the hearing. The major disadvantage of the Meniett is the need to have a grommet in the eardrum, which must be replaced at regular intervals. With a grommet, water must be kept out of the ear, and there is a chance of developing an ear infection and discharge."25 I Idiopathic Idiopathic means arising spontaneously, or from an obscure or unknown cause. Meniere’s disease is commonly regarded as an idiopathic disorder, as the cause is yet to be determined. Meniere’s syndrome is the term more correctly used for those minority of cases where the cause is known. Conditions believed to cause Meniere’s syndrome include trauma (such as head injury or ear surgery), viral infection (such as mumps or measles), late-stage syphilis, and perhaps some autoimmune disorders that affect the inner ear.26 Incidence The incidence of a disease is an epidemiological measure. Incidence measures the rate of occurrence of new cases of a disease or condition. Incidence is calculated as the number of new cases of a disease or condition in a specified time period (usually a year) divided by the size of the population under consideration who are initially disease free. Incidence should not be confused with prevalence which is a measure of how common a disease or condition is in a population at a particular point in time. Prevalence is a record, or estimate, of how many people have the condition (not just new cases). Professor WPR Gibson, Minor Surgical Treatments, Meniere's Disease Brochure-9, Meniere's Support Group of NSW Inc., 2005 Assoc. Professor Stephen O'Leary, Pressure Pulse Therapy – What is the Meniett? in the booklet Understanding Meniere's Disease, edited by Lynn Polson, 2009 (obtainable from Meniere's Australia). 26 Schwaber MK, Vestibular Disorders, Chapter 27 in Clinical Otology, 3rd edition. Editors: Hughes GB, Pensak ML. Thieme Medical Publishers, Inc., New York, 2005 24 25 17 Inner Ear Meniere’s disease is a condition of the inner ear. The inner ear has two main parts: the cochlea, which is responsible for hearing, and the vestibular apparatus, which is responsible for balance and motion. Intratympanic Within the middle ear. An intratympanic injection is an injection through the eardrum, into the middle ear. L Labyrinth The labyrinth is a system of fluid passages in the inner ear that includes both the organ of hearing (the cochlea) and the organs of balance (the semicircular canals and the utricle & the saccule). Labyrinthitis Labyrinthitis is inflammation of the labyrinth. This may be due to a viral or bacterial infection of the inner ear. Labyrinthitis can cause vertigo (often with nausea and vomiting), dizziness & loss of balance, and temporary hearing loss. In most cases, labyrinthitis will resolve by itself within three weeks or so. However complications of labyrinthitis can include permanent balance problems and hearing loss.27 Treatment for labyrinthitis can include anti-nausea medications, medications to dampen the sensations of dizziness, antibiotics (in the case of bacterial infection), antiviral drugs & corticosteroids (in the case of viral infection), and balance exercises, if needed.27 Labyrinthectomy A labyrinthectomy is a surgical procedure that destroys the whole inner ear – that is, the operation destroys both the hearing and the vestibular functions. Labyrinthectomy may be considered for Meniere's patients in whom there is no hearing in the ear which is causing vertigo. Labyrinthectomy offers excellent control of vertigo28, but destruction of the labyrinth will initially impair overall balance to some extent. Meniere's in both ears usually precludes destructive procedures24 because good balance function in the non-operated ear is needed to compensate for the loss of balance function due to the destruction of the balance organs in the operated ear. 27 28 www.betterhealth.vic.gov.au www.dizziness-and-balance.com/treatment/surg.html 18 Lifestyle Certain changes in lifestyle can be beneficial to people with Meniere's disease and can help them in controlling their symptoms. Dietary changes, such as reduction of salt, sugar and caffeine can be helpful, as can control of stress. Meniere's Australia has a number of information sheets on diet29 and lifestyle improvements30 that may help people with Meniere's disease manage their symptoms. Lip-reading Lip-reading is ‘seeing the sound of speech’. The movements of the lips and the tongue, together with facial expression and body language, are all clues for understanding speech. Lip-reading is a useful skill for those who are hearing impaired. Local branches of the non-profit organisation Better Hearing Australia31 teach lip-reading as part of their Hearing Loss Management classes. CD-ROMs, DVDs, and other home lip-reading learning aids are also available.32 33 M Mal de Debarquement Syndrome (MdDS) Mal de Debarquement Syndrome is a condition which causes sufferers to have a constant feeling of imbalance and unsteadiness after exposure to motion such as an ocean cruise. After getting off the boat (debarquement) they develop a rocking sensation, as if they are still on the boat. Although most people have this rocking sensation for only a short period, in some cases it may persist for months or years. After MdDS has started, most medications that work for other forms of dizziness or motion sickness are ineffective.34 Professor T C Hain has an interesting discussion of possible mechanisms on his website34, and favours a theory that the brain constructs a predictive model of the boat's motion in order to maintain balance on board ship. This internal model would normally extinguish within a short time after getting off the boat but for some people it persists, causing long-term unsteadiness and rocking sensations. 29 30 www.menieres.org.au/cat-diet.php www.menieres.org.au/cat-lifestyle.php www.betterhearingaustralia.org.au www.lipread.com.au/ 33 www.betterhearingwa.org.au/products/index.htm 34 www.dizziness-and-balance.com/disorders/central/mdd.html 31 32 19 Medications There is no specific medication for Ménière’s disease; however, medication may be given to treat the symptoms. betahistine (Serc), prochlorperazine (Stemetil) and cinnarizine (Stugeron) are most commonly prescribed to people with Ménière’s disease/vertigo. Some people may also be prescribed a diuretic. Meniere’s disease Meniere’s disease is a condition of the inner ear. The cause of Meniere’s disease is unclear and there is currently no known cure. Meniere's symptoms may include: * Attacks of vertigo (the room appears to spin) – the attacks last more than 10 minutes and may last up to several hours. * Nausea and vomiting (and sometimes diarrhoea) often accompany the vertigo attacks, especially in the early stages of the disorder. * A feeling of fullness in the ear at the time of the attack. * Tinnitus (ringing in the ear) at the time of the attacks. Some degree of tinnitus may become permanent. * Hearing loss (and distortion of sound) in the affected ear at the time of the attack. Some degree of hearing loss may become permanent if Meniere's progresses to a later stage. The criteria for the diagnosis of definite Meniere's were set out in the glossary for the letter D (Equilibrium, Spring 2010). The criteria require exclusion of other possible causes of the symptoms, such as vestibular migraine or acoustic neuroma. Meniett The Meniett® device is a portable, battery operated, low pressure pulse generator. More details about low pressure pulse therapy can be found under the item for hyper pressure treatment, in the glossary for the letter H. Meniere's patients interested in the Meniett should discuss it with their ENT specialist, who will need to insert a grommet in the eardrum. The current price in Australia for a new Meniett device is $5566.00 (including GST). It may be possible to trial the device. This can be discussed with your specialist or with a company representative.35 Middle Ear The middle ear is that part of the ear that includes the eardrum and the three tiny bones of the middle ear, ending at the round window that leads to the inner ear. Medtronic Australasia P/L., 97 Waterloo Road, North Ryde, NSW, 2113. Ph: (02) 9857-9000 Fax: (02) 9878-5100 Toll Free: 1800 668 670 Web: www.medtronic.com.au/ 35 20 Migraine associated vertigo (MAV) Migraine is quite common – approximately 13% of adults in the US suffer from migraine. Migraine can cause vertigo – clinical studies indicate that at least 25% of people with migraine report episodic vertigo. Migraine associated vertigo is one of the most common causes of vertigo36. Other common causes of vertigo are BPPV, vestibular neuritis & labyrinthitis. Although a headache is usual, you can have migraine without a headache. Migraines are often accompanied by visual symptoms and are relieved by sleep. There is often sensitivity to various types of sensory inputs: light, sound, smells (such as perfume), and motion (people with migraine are prone to motion-sickness). Symptoms may be triggered by weather changes. Auditory symptoms (if they occur) are usually bilateral if caused by migraine.36 If migraine is suspected a referral to a neurologist should be considered. A trial of anti-migraine medication can be used to confirm a diagnosis of migraine associated vertigo.2 Diet and lifestyle are important. Migraine is sensitive to a greater variety of triggers than Meniere's. Medications for migraine can be for acute attacks or for prevention. Medications for migraine are different to those for Meniere's (except for acute vertigo attacks), so expert advice is important. Australian websites on migraine can be found via a search on www.healthinsite.gov.au/ and US oriented information can be found on T C Hain's webpages. 36 37 Motion sickness "Motion sickness is the nausea, disorientation and fatigue that can be induced by head motion. The first sign is usually pallor (a pale appearance). Yawning, restlessness and a cold sweat forming on the upper lip or forehead often follow. As symptoms build, an upset stomach, fatigue or drowsiness may occur. The final stages are characterized by nausea and vomiting."38 MRI – Magnetic Resonance Imaging One of the tests that may be performed during diagnosis is an MRI scan. The scan uses a strong magnetic field and radio waves to produce detailed pictures of the inside of your body. When investigating patients suffering from dizziness, an MRI may be used to look at the inner ear. It is mainly used to exclude an acoustic neuroma, which is a rare condition that can produce symptoms somewhat similar to Meniere’s. Acoustic neuromas were covered in the glossary for the letter A (Equilibrium, Summer 2009). Recently, Professor Gibson and others have used an MRI, together with an intra-tympanic injection of gadolinium, to enable endolymphatic hydrops to be visualised and measured.39 36 www.dizziness-and-balance.com/disorders/central/migraine/mav.html 37 www.dizziness-and-balance.com/disorders/central/migraine/index.html www.dizziness-and-balance.com/disorders/central/motion.htm 38 39 Nakashima T et al. Grading of endolymphatic hydrops using magnetic resonance imaging. Acta Otolaryngol Suppl. 2009 Feb; (560):58. Abstract available at: www.ncbi.nlm.nih.gov/pubmed/19221900 21 N Nausea Nausea is a feeling of sickness, with loathing of food and inclination to vomit. Nausea and vomiting can be due to a variety of causes40. In Meniere’s, nausea and vomiting are reflex responses to the attacks of vertigo. Neurectomy A neurectomy is the surgical removal of a nerve or section of a nerve. A vestibular neurectomy is one surgical procedure which may be performed for Meniere’s disease. The vestibular nerve carries information from the balance organ to the brain. In this procedure the nerve is cut stopping the abnormal information reaching the brain. Neurologist A neurologist is a specialist physician who treats diseases of the brain, spinal cord, nerve and muscle. Neurologists undergo extensive training in general medicine and clinical neurology, and are members of the Royal Australasian College of Physicians. Your GP may refer you to a neurologist when you first get vertigo attacks, as vertigo can be caused by a variety of medical conditions apart from Meniere’s, including migraine (which is common), and acoustic neuroma (which is rare, but which does need to be excluded as a cause). Neuroma A tumour on a nerve or in nerve tissue. Nystagmus Nystagmus is usually present during a vertigo attack. “Nystagmus is a term denoting a rapid movement of the eye from side to side where movement to one side is slower than to the other. When one inner ear loses its function either completely or incompletely, the contralateral [opposite] ear becomes predominant. If the left ear is damaged then the right ear becomes dominant and forces the eye to move slowly to the left side. When a certain point is reached the eyes flick back to the non-diseased side, either because of elastic recoil or because of the effect of the nearby part of the brain, the cerebellum. This form of nystagmus is due to a paralysis of the affected side. The fast phase of the nystagmus is towards the normal side. In an acute episode of Meniere’s disease, sometimes the nystagmus can be of a reversed variety”2. Nystagmus is deliberately induced in the caloric test in order to investigate the function of the horizontal semicircular canal in the inner ear. Metz A, Hebbard G. Nausea and vomiting in adults – a diagnostic approach. Aust Fam Physician 2007;36(9):688–92. www.racgp.org.au/afp/200709/200709metz.pdf 40 22 O Oscillopsia Oscillopsia is the bouncing and blurring of vision. If it is caused by a loss of vestibular function, then the oscillopsia will occur when the person is moving. When oscillopsia occurs with head movement, it is almost certainly due to the failure of the vestibulo-ocular reflex.41 The vestibulo-ocular reflex keeps our eyes directed at the object we are looking at despite movements of our head, thanks to feedback from our vestibular balance organs. If we lose most or all of our vestibular function, this reflex will not work well (and may not work at all) and our eyes will not automatically make the necessary movements to counteract our head movements. The subjective effect is bouncing or blurred vision when moving. Oscillopsia is one of a number of effects of loss of vestibular function. Others are unsteady gait & posture, difficulty in walking on uneven surfaces, and difficulty in maintaining balance in the dark. Otitis Inflammation of the ear. Otitis externa Inflammation of the external ear canal (the tube between the outer ear and eardrum). Otitis externa can be caused by an infection or allergic reaction. It is sometimes referred to as ‘swimmer’s ear’ because it can be caused by getting water into the ear canal. Otitis media Otitis media is inflammation of the middle ear. Acute otitis media is infection of the middle ear that comes on suddenly, often with pain and a raised temperature. It is more common in children under six years of age. Otoacoustic Emissions Otoacoustic emissions (OAEs) are low-intensity sounds produced by the inner ear in response to clicks or tone bursts. OAEs can be measured with a sensitive microphone placed in the ear canal. OAEs are used in clinical audiology and ENT practice and in basic research. Otolaryngologist & Otologist An otolaryngologist (or ENT specialist) is a doctor who diagnoses and treats diseases of the head and neck, especially ears, nose, and throat. An otologist specializes only in diseases, disorders and operations relating to the ear. Da Cruz M. Swimmer’s ear and differential diagnoses. MedicineToday Dec 2007 Vol 8 No. 12. Full text at: www.medicinetoday.com.au/cpd/files/articles/200712/Module%203.pdf 41 23 Otoconia Otoconia are microscopic calcium carbonate crystals embedded in a protein matrix within the otolith organs42. They are also called otoliths (literally “ear stones”). If otoconia come loose they can end up in one of the semicircular canals and cause the brief episodes of vertigo known as BPPV. Otolithic Crisis of Tumarkin This is a sudden fall that occurs without warning. It is likely due to inappropriate activity of the otolith organs, usually associated with Meniere’s disease43. The attacks are severe, but quite brief, less than a minute in duration. It is also called a drop attack. The person will remain awake and will not lose consciousness. A fall due to a loss of consciousness would be due to some other cause, and should be medically investigated. Otolith organs In addition to the semicircular canals, the vestibular labyrinth of the inner ear has two otolith organs, the utricle and the saccule. The utricle is contained within a swelling adjacent to the semicircular canals, and the saccule is close to the cochlea. The otolith organs sense gravity and linear acceleration. A set of hair cells are coupled to a mass of stones. When the stones move, with respect to the hairs, they exert a shearing force on the hairs. This force is detected by the hair cells and sent to the brain via branches of the vestibular nerve. The otolith organs sense motion according to their orientation. The utricle is largely horizontal in the head, and largely registers accelerations acting in the horizontal plane of the head. The saccule is largely vertical and registers accelerations in the vertical plane (for example, in an elevator). Motion sensations from the utricle and the saccule are used for many reflexes and damage to these organs can impair ocular and body stabilization.42 Otosclerosis Otosclerosis is an abnormal growth of bone of the middle ear. This bone prevents structures within the ear from working properly and causes hearing loss. The hearing loss may become severe. If the hearing loss is a conductive type of loss, then hearing aids or surgery can be very helpful.44 Hain TC. Otoliths. April 2010 (Graphics courtesy of Timothy C. Hain, M.D., Chicago Dizziness and Hearing) URL: www.dizziness-andbalance.com/disorders/bppv/otoliths.html 43 Hain TC. Meniere’s Disease. July 2012 (Graphic courtesy of Timothy C. Hain, M.D., Chicago Dizziness and Hearing) URL: www.dizziness-andbalance.com/disorders/menieres/menieres.html 44 Hain TC. Otosclerosis. Feb 2011 URL: http://www.dizziness-andbalance.com/disorders/hearing/otoscler.html 42 24 Ototoxicity Ototoxicity refers to drug or chemical-related damage to the inner ear. Such damage can lead to temporary or permanent hearing loss, and/or loss of balance. Ototoxic substances include several therapeutic medicines.45 P Physiotherapist Physiotherapy is a healthcare profession that assesses, diagnoses, treats, and works to prevent disease and disability through physical means. Physiotherapists are experts in movement and function who work in partnership with their patients, assisting them to overcome movement disorders.46 A carefully planned individualised exercise program can help with many balance problems. A program can be devised by a physiotherapist specialising in vestibular rehabilitation.47 Presbycusis Presbycusis is hearing loss associated with the degenerative processes of aging. The term comes from the Greek “presbys” meaning “elder” and “akousis” meaning “hearing.” A similar use of “presbys” is found in ophthalmology with “presbyopia” referring to “vision of the elderly.” Proprioception Proprioception consists of sensations from joints, tendons and muscles that provide awareness of position and movement. Proprioception and vision supplement the signals from the vestibular balance organs to form our overall balance system. Psychologist Psychologists are university qualified experts in human behaviour, and apply their expertise using reliable and scientifically supported methods. Research shows that psychological treatments are as successful as medication in treating conditions such as depression and anxiety. Vestibular disorders can be quite stressful and may precipitate bouts of anxiety or depression. Also there is the difficulty of coping with an unpredictable chronic condition. Talking with a psychologist may be of help. Virtual Medical Centre. Ototoxicity. July 2008 URL: www.virtualmedicalcentre.com/diseases/ototoxicity/885 https://www.physiotherapy.asn.au/ 47 Sharpe MH. Understanding Vestibular Rehabilitation, in Understanding Meniere's Disease (Updated) by Lynn Polson OAM (editor) 2009 (obtainable from Meniere's Australia). 45 46 25 R Recruitment Recruitment causes your perception of sound to be exaggerated. Even though there is only a small increase in the noise levels, sound may seem much louder and it can distort and cause discomfort. Someone with recruitment can have problems only with specific sounds and frequencies or may have problems with all sound in general. Remission Remission is a lessening in the intensity of a medical disorder, or the temporary disappearance of symptoms. Remission occurs in Meniere's. In Stage 1, the ear often returns to normal during these periods of remission. Remissions of Meniere's can last from weeks to many years. Frequent remissions in a disease make it more difficult for medical researchers to verify the effectiveness of new treatments. Round Window The round window is one of the two openings into the inner ear. It is closed off from the middle ear by the round window membrane, which vibrates with opposite phase to vibrations entering the inner ear through the oval window. It allows fluid in the cochlea to move, which in turn ensures that hair cells of the basilar membrane will be stimulated and that audition will occur. S Saccule The saccule is one of the two otolith balance organs in the inner ear that sense gravity & linear acceleration. Saccus decompression This is a surgical procedure used for people with Meniere’s disease. The aim of the procedure is to stop the vertigo attacks, while preserving the hearing in that ear. There are several variations; all aim to reduce the pressure of the fluid in the endolymphatic sac. Schwannoma A schwannoma is a tumour of the tissue that covers nerves. These tumours develop (and take their name) from a type of cell called a Schwann cell. Schwannomas are often benign (not cancerous). 26 A vestibular schwannoma is a benign, slow growing tumour that develops on the balance and hearing nerves of the inner ear. A vestibular schwannoma is also called an acoustic neuroma. It can cause symptoms similar to Meniere's. When Meniere's is first being diagnosed in a patient, a CT scan or an MRI is often ordered to exclude the possibility of an acoustic neuroma Semicircular canals There are three semicircular canals within the inner ear; known as the ‘horizontal’, ‘posterior’, and ‘anterior’ canals. The three canals are all at right angles to each other so that together they can detect movement across all the different angles you can move through. The semicircular canals are covered in tiny sensory hair cells which send balance signals back to your brain (via your vestibular nerve). Sensorineural hearing loss Sensorineural hearing loss accounts for about 90% of all hearing loss. It is sometimes called (not quite accurately) "nerve deafness". Sensorineural hearing loss is due to damage to either the cochlea (often the hair cells) or to the auditory nerve. Common causes include ageing (presbycusis), Meniere's disease, ototoxic medications, immune disorders, and noise exposure.48 Serc See Betahistine Stemetil A commonly prescribed medication for an acute vertigo attack with accompanying nausea and vomiting is Stemetil (prochlorperazine) either as tablets, or an injection, or a suppository. Stemetil controls vomiting and it is a vestibular suppressant so it reduces the vertigo. It has a general sedative effect, which is usually an advantage in an acute vertigo attack.5 Stress Stress is not believed to be the cause of Meniere’s disease, but it can exacerbate symptoms. Studies have shown that your body is less able to cope with dizziness when you are stressed. When you are under stress your body gets ready to fight or run away, known as the ‘fight or flight’ stress reflex. So you may become more dizzy and sick than you would if you were stress free. Stugeron See Cinnarizine. 48 www.dizziness-and-balance.com/disorders/hearing/sensorineural.htm 27 T Temporomandibular joint The temporomandibular joint (TMJ) is the area directly in front of the ear on either side of the head where the upper jaw and lower jaw meet. TMJ disorders are problems of the jaw joint and can be caused by a number of behaviours or conditions. Common symptoms of TMJ disorders include headache, ear pain, jaw sounds, dizziness, ear fullness and tinnitus Tinnitus The word 'tinnitus' comes from the Latin word for 'ringing' and refers to the perception of sound in the absence of any corresponding external sound. Tinnitus noises are described variously as ringing, whistling, buzzing and humming.The sound may be heard in one ear, in both ears, or in the middle of the head, or it may be difficult to pinpoint its exact location. The sound may be low, medium, or high-pitched. There may be a single sound or two or more components. The sound may be continuous or it may come and go.49 Tinnitus is very common in the general population – 90% have tinnitus (some may need to be in a sound-proof room to be able to hear it), about 30% become aware of tinnitus, about 15% report constant tinnitus, and around 2% report that their tinnitus is distressing.50 Tullio's phenomenon Tullio's is a symptom, not a disease – it is when loud noises make one literally dizzy, as in spinning vertigo. It is important to determine which ear is the problem. An otologist (an ENT who specialises in ear problems) will likely do a hearing test, take a careful history, and get a temporal bone CT scan to look for superior canal dehiscence (where part of the bony roof of the top semicircular canal is missing), or other ear problems that may be causing Tullio's. Structural changes to the inner ear from Meniere's, such as pathologic dilation of the otolithic sac, may possibly be a cause. However, Tullio's is uncommon in Meniere's disease.51 Tumarkin's Otolithic Crisis See Drop Attacks British Tinnitus Association. What is tinnitus? Dec 2011. URL: www.tinnitus.org.uk/ Westcott M. Tinnitus Management. Equilibrium, Summer issue, December 2011 51 Hain TC. Tullio's Phenomenon. April, 2011. URL: www.dizziness-and-balance.com/disorders/symptoms/tullio.html 49 50 28 Tympanometry Tympanometry is a method of measuring the stiffness of the ear drum. A probe is inserted in the ear canal. You feel a pressure change and hear a tone. It is part of most basic hearing assessments. Much useful information about the middle ear can be gained from this short and easy test.52 Tympanic membrane The tympanic membrane, also known as the ear drum, is located between the ear canal and the middle ear. Normally it completely separates these two spaces, preventing air or fluid from moving across.53 Tympanic membrane The thin membrane separating the middle ear and external ear is known as the tympanic membrane (eardrum). The name derives from the Latin term ‘tympanum auris’ meaning drum. U Unilateral Unilateral means one-sided. When a person with Meniere’s disease is referred to as having unilateral Meniere’s, this means only one ear is affected. Urea Urea is a natural substance related to urine. Professor Gibson in Australia calls it the Cinderella drug and in his many years of practice he has never known a patient have an attack of Meniere’s disease within four or five hours of taking an oral dose of Urea. It is an osmotic diuretic so it helps to lose fluid quickly. It is not readily available in the United Kingdom at present. If it is available and you’ve got a very important function, like a ball to go to, then at least you know that that evening you will get home without having had an attack. Utricle The utricle is a one of the two otolithic organs of the inner ear. 52 Hain TC. Tympanometry. Aug, 2010. URL: www.dizziness-and-balance.com/testing/hearing/tymp.htm 53 Hain TC. Tympanic membrane. Feb, 2010. URL: www.dizziness-and-balance.com/anatomy/tm.html 29 V Vasodilators Vasodilators widen blood vessels and allow increased blood flow. Vasodilators have been used to treat Meniere’s disease for over fifty years on the expectation that they will lead to resorption of endolymph.5 An example of a vasodilator used to treat Meniere's is Serc (betahistine). A recent animal study has demonstrated that Serc does increase blood flow in a particular part of the inner ear, and that the magnitude of the increase depends on dosage.54 Vertigo In a medical context, vertigo refers to an illusion of movement, such as a sensation that the person (or the room) is spinning. Attacks of vertigo are common in the early stages of Meniere's. Vestibular Migraine Some symptoms such as vertigo, which may have been ascribed to Meniere's, can be due to vestibular migraine. Treatments are different if the cause is migraine so if migraine is suspected it is important to get good advice, usually from a neurologist. Vestibular Nerve Section A vestibular nerve section, or VNS, is a surgical method of eliminating vertigo attacks and drop attacks (provided Meniere's is only in one ear). The procedure involves dividing the balance nerve supplying the inner ear affected by Meniere’s, but preserving the hearing nerve. Patients need to have normal balance in the other ear.8 The VNS is a major operation and time is needed for recovery. There is also some risk of possible complications55. As with other destructive procedures (labyrinthectomy, endolymphatic sac surgery, and intratympanic gentamicin), some deterioration in overall balance is to be expected after the procedure but vestibular rehabilitation therapy can help overcome this. Vestibular Neuritis In vestibular neuritis, dizziness and vertigo are attributed to a viral infection of the vestibular nerve. Vestibular neuronitis (implying damage to sensory neurons) is another term that is used for the same pattern of symptoms. Ihler F et al. Betahistine exerts a dose-dependent effect on cochlear stria vascularis blood flow in guinea pigs in vivo. PloS one 7(6), 2012: e39086. 55 Hain TC. Vestibular Nerve Section. Sept 2011. URL: www.dizziness-and-balance.com/treatment/vn_section.html 54 30 Acutely, vestibular neuritis is usually treated symptomatically, meaning that medications are given for nausea (anti-emetics) and to reduce dizziness (vestibular suppressants). When a herpes virus infection is strongly suspected, an anti-viral medication called "Acyclovir" or a relative may be used. It usually takes three weeks to recover from vestibular neuritis. If it is taking longer, vestibular rehabilitation therapy may help speed recovery.56 Vestibular rehabilitation Vestibular rehabilitation is a programme of head, eye and neck movements, often led by a physiotherapist, to assess balance function and re-educate the balance system so the person becomes more stable. The more customised this balance training is to the individual, the better the results. Z Zofran wafers Professor Gibson says: “The wonder drug to stop an attack of Meniere’s disease is ondansetron (Zofran 8mg wafers). Zofran® wafers - undoubtedly the best current treatment to lessen an acute attack. Like the old time fizzers, it is placed under your tongue and the tablet is rapidly absorbed into the blood stream. “Zofran wafers dissolved under the tongue, are more effective than an injection of Stemetil and if you take it early in the attack you can usually greatly subside the attack and make it less intense. The dose can be repeated within an hour if you think you need more than 8mg. Side effects are extremely rare.”57 56 57 Hain TC. Vestibular Neuritis and Labyrinthitis. March 2014. URL: www.dizziness-and-balance.com/disorders/unilat/vneurit.html Professor Bill Gibson, Address opening the Meniere’s Australia Symposium, Sydney 2009, as reported in Equilibrium, Autumn 2010. 31