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MedicineToday 2014; 15(3): 18-26

PEER REVIEWED FEATURE 2 CPD POINTS Ménière’s A stepwise approach

MELVILLE DA CRUZ FRACS, MSc, MD Key points Ménière’s disease is an uncommon cause of recurrent that • Ménière’s disease is should be distinguished from other causes of disturbance such characterised by recurrent as vestibular . Stepwise introduction of dietary restriction of attacks of vertigo, fluctuat­ing loss, and a sodium, lifestyle changes and can reduce the frequency and sensation of aural fullness. severity of attacks in most patients. • Attacks are sudden in onset, typically accompanied by an énière’s disease is characterised by CLINICAL FEATURES AND NATURAL intense sensation of move­ recurrent attacks of vertigo accom- HISTORY ment, , , panied by fluctuating sensorineural The hallmark of an acute Ménière’s attack is diarrhoea and sweating, and , tinnitus and a of prolonged vertigo. The vertigo is characterised can last hours. M aural fullness. Prosper Ménière in 1861 cor- by a sudden unheralded intense sensation of • Treatment options for acute rectly attributed the attacks to a disorder of the movement, most commonly rotation or spinning, attacks include ondanse­tron, inner , suggesting that the mechanism of lasting at least 20 minutes and accompanied by and causation could be similar to migraine or inner nausea, vomiting, diarrhoea and sweating. The . ear , a which duration and character of the vertigo is impor- • In most patients, attack is still relevant for the disease today.1 tant in the diagnosis of Ménière’s disease frequency and severity can because episodes lasting a few seconds or min- be reduced by a stepwise MÉNIÈRE’S DISEASE TODAY utes are more likely to be due to benign parox- approach of dietary restriction The differential diagnosis of vertigo is broad, ysmal positional vertigo (BPPV) or, if longer of sodium, lifestyle changes often leading to all cases of vertigo being labelled than eight to 12 hours, to vestibular n­ euritis or and medi­ca­­tion; surgical as Ménière’s disease. To clarify the diagnosis, cerebellar . Feelings of light-headedness, treatment is rarely required. treatment and prognostication of patients with pressure or vague disturbances of balance are • Referral to a specialist is Ménière’s disease, the American Academy of more likely to be due to non-otological causes, best for initial diagnosis and Otolaryngology – Head and Neck Surgery such as postural hypotension, hyperglycaemia, evaluation. (AAO-HNS) has published guidelines for the hyperventilation, panic attacks or . • GPs have an important role classification of Ménière’s disease (Box 1).2 In the early stages of Ménière’s disease, in ongoing management of Although the category of ‘certain Ménière’s patients may report only the vertigo symptoms, patients with Ménière’s disease’ is used only as a definition, as as the fluctuations in hearing thresholds are disease. it requires postmortem histological examina- minor and may go unnoticed. However, as the tion of the temporal bone, the other categories disease progresses the fluctuating hearing loss are clinically useful. (generally affecting low tones) becomes more

Copyright _LayoutAssociate 1 17/01/12 Professor 1:43 da PM Cruz Page is an 4 Ear Nose and Throat Surgeon at Westmead Hospital, University of Sydney, and a Cochlear Surgeon at Sydney Centre, Sydney, NSW.

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Certain Ménière’s disease • Definite Ménière’s disease plus ­histopathological confirmation Definite Ménière’s disease • Two or more definite spontaneous episodes of rotational vertigo for 20 minutes or longer • Audiometrically documented hearing loss (unilateral or bilateral) on at least one occasion • Tinnitus or aural fullness in the affected ear • Other causes excluded, such as v­ estibular Probable Ménière’s disease • One definite episode of rotational vertigo © CHRISTY KRAMES, 2014 • Audiometrically documented hearing loss (unilateral or bilateral) on at least one balance control causes them to drop to the occasion ground without losing . • Tinnitus or aural fullness in the affected The natural history of Ménière’s disease is ear highly variable. Attacks of vertigo can occur • Other causes excluded daily in clusters over periods of several weeks Possible Ménière’s disease or as isolated episodes interspersed with variable • Episodic vertigo of the Ménière’s type periods of remission, sometimes lasting many without documented hearing loss, or months or several years. In the later stages of • Sensorineural hearing loss (unilateral or the disease, the episodes of vertigo tend to ‘burn bilateral), fluctuating or fixed, with out’, and severe hearing loss dominates the disequilibrium but without definite clinical picture. In long-term follow-up studies episodes of vertigo of patients with Ménière’s disease, 30% to 45% • Other causes excluded developed the disease in the contralateral ear * American Academy of Otolaryngology-Head and Neck within a period of 30 years. Foundation. Otolaryngol Head Neck Surg 1995; 113: 181-185. There are many variants of classical Ménière’s disease, in which vertigo precedes the fluctua- marked, and a permanent hearing loss may tions in hearing loss. The reverse occurs in persist between attacks (middle stages of the Lermoyez syndrome: hearing loss precedes disease). Later in the disease course, the hearing vertigo and improves following vertigo attacks. loss becomes permanent and nonfluctuating. Some patients experience a fluctuation in their At this stage, the attacks of vertigo usually hearing thresholds (usually low tone) accom- lessen, and hearing loss becomes the dominant panied by tinnitus and fullness but no vertigo. symptom (‘burnt out’ Ménière’s disease). The This has been labelled ‘cochlear hydrops’. sensation of tinnitus similarly may be less noticed in the early stages, only to become more EPIDEMIOLOGY prominent in the later course of the disease. Several epidemiological studies of Ménière’s Late in the disease, patients may experience disease have been performed over the past few drop attacks (causedCopyright by dysfunction _Layout of1 the17/01/12 ves- 1:43decades PM Page with 4 widely contrasting results. tibulospinal reflex), where a sudden loss of ­Estimated prevalence rates range from as low

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late in the disease, and the responses to various treatments, particularly surgery Allergic responses to the endolymphatic system. Obstructed drainage Vertigo Although no single theory of Ménière’s Excess endolymph production Endolymphatic Hearing loss causation is accepted by all workers in Autonomic imbalances hydrops Tinnitus this field, a more contemporary concept Viral Aural fullness of the disease is that it represents a failure Dietary deficiencies of the complex homeostasis Vascular irregularities caused by any of a range of pathologies. The symptoms during an attack can be Figure 1. Proposed causation of Ménière’s disease. more ­usefully viewed as a syndrome than as a single disease entity. The inner ear as 3.5 per 100,000 population to as high as In 1964, further histological studies changes leading to symptoms may be 513 per 100,000 population. The wide range showed evidence of ruptures of Reissner’s caused by a range of pathologies (see Fig- is likely to result from methodological dif- membrane, one of the membranous divi- ure 1). They include autoimmune ferences, changes over time in criteria for the sions between the potassium-rich endo- (Cogan’s syndrome and systemic lupus diagnosis of Ménière’s disease, difficulty in lymph and the perilymph of the . erythematosus), congenital and distinguishing Ménière’s d­ isease from related The ensuing ‘rupture theory’ proposed other viral infections (possibly herpes conditions such as migraine-associated that the acute mixing of potassium-rich simplex) of the inner ear (viral labyrinthi- ­vertigo, and differences in the populations endolymph with perilymph to tran- tis), , chronic ear disease, acoustic surveyed. However, it is clear that Ménière’s sient dysfunction within the coch- , trauma, and hormonal disease is more common in women, and lear and vestibular end organs, resulting changes with the menstrual cycle. In most the prevalence increases dramatically with in the attacks seen in Ménière’s disease. patients the cause is unknown. age, peaking in the 60 to 69 years age On the basis of this theory, many different group.3,4 It is very rare in people younger forms of surgery to alter the function of DIAGNOSIS than 20 years. the endolymphatic system were designed, In the vast majority of cases, Ménière’s with the aim of preventing pressure build disease can be diagnosed on the basis of PATHOPHYSIOLOGY up within the inner ear and minimising an accurate history and a few relevant tests History the membrane ruptures implicated in to exclude differential causes of recurrent Before Ménière’s description of the disease attacks of Ménière’s disease. vertigo. Clinical examination of patients in 1861 correctly attributed it to a disorder with Ménière’s disease often finds no of the inner ear, vertigo attacks were Current theories abnormalities or at most shows evidence thought to be due to a form of .1 As the understanding of the complex of a unilateral sensorineural hearing loss Ménière suggested the mechanism could physiology of the inner ear evolved and (or bilateral asymmetrical hearing loss in be similar to migraine or vasospasm the outcomes of various surgical and med- the case of bilateral disease). Clinical tests within the inner ear. Early treatments, ical treatments for Ménière’s disease were of balance such as Romberg’s test (stand- including cervical sympathectomy and reviewed, it seemed less likely that the ing feet together with eyes closed) and vasodilators such as nicotinic acid, were rupture theory could explain the attacks Unterberger’s stepping test (walking on based on this understanding. of vertigo and fluctuating hearing loss. the spot with eyes closed) may show only In 1938, the first reports of temporal Contemporary understanding of the a mild disturbance of balance. This is bone histology in patients with Ménière’s radial and longitudinal endolymph flow because most patients are examined only disease were published, showing an patterns in the healthy and diseased coch- between attacks, long after their symp- increased fluid volume in the endolymph lea led to a theory in 1991 that Ménière’s toms have resolved. If there are associated compartment of the cochlea, termed endo- disease is caused by episodic disturbances persisting neurological signs (cranial lymphatic hydrops. It was assumed that of endolymph formation and resorption.5 nerve palsies or ) then other increased endolymphatic volume led to the The theory encompasses current anatom- intracranial pathologies need to be attacks of Ménière’s disease. However, fur- ical, physiological and pathophysiological considered. ther histological analysis of human tempo- knowledge about the functions of the In the few cases where patients have ral bones showed many examples of endo- cochlear and balance system. It also been observed during an acute attack, clin- lymphatic hydropsCopyright in patients _Layout with 1 no17/01/12 explains 1:43 PM the Page stages 4 of Ménière’s disease, ical examination has varying results. In clinical symptoms of Ménière’s disease. including the occurrence of drop attacks general, patients appear quite unwell

20 MedicineToday x MARCH 2014, VOLUME 15, NUMBER 3 Downloaded for personal use only. No other uses permitted without permission. © MedicineToday 2014. First test Second test because of the unpleasant sensation of ver- Figure 2. Serial Air conduction tigo. They may be sweaty and pale, unable Bone conduction [ [ pure tone to stand up safely, nauseated and violently of vomiting. There may be horizontal nystag- 0 [ [ [ the right ear in a mus that changes direction as the attack [ [ [ patient with uni­ progresses. Following an attack, patients [ lateral Ménière’s 20 [ are left with a sense of ‘hangover’ for a [ disease showing day or two before recovering to normal fluctuating function. 40 [ sensorineural ­ [ [ low-tone hearing INVESTIGATIONS loss in that ear. Pure tone 60 The most useful investigation for diagnos- ing Ménière’s disease is a standard pure tone Hearing loss (dB) audiogram. In classical Ménière’s disease, 80 this may show varying degrees of sensori- neural hearing loss. Most commonly the 100 hearing loss affects low tones, although 250 500 1000 2000 4000 8000 other patterns are also seen (Figure 2). Frequency (Hz) Serial audiograms recorded at intervals during the progression of Ménière’s dis- Figure 3. A ease may show fluctuations in the degree contrast- of hearing loss. In the earliest stages of enhanced MRI Ménière’s disease, an audiogram (typically scan showing a recorded after the attack has abated) usu- large acoustic ally appears normal. As the disease pro- neuroma (arrow) gresses, the hearing loss becomes more in a patient who marked with some degree of hearing loss presented with persisting between attacks (middle stages low-tone hearing of the disease). Later in the disease course, loss and a mild the hearing loss becomes permanent and disturbance of nonfluctuating. balance.

Vestibular function tests and electrocochleography Vestibular function tests are highly spe- be useful in confirming the diagnosis of demonstrated dilation of the endolymph cialised tests of the balance system and are Ménière’s disease in atypical cases.6 compartments in some patients with particularly useful in evaluating patients advanced Ménière’s disease.7 Further whose cases are unusual, for example with Imaging studies refinements of these imaging techniques an atypical clinical history or bilateral Imaging studies such as MRI and CT scans may allow a more definite diagnosis in disease.6 Vestibular testing is mandatory are useful in excluding acoustic patients with Ménière-type symptoms, before considering interventions that (Figure 3) and other intracranial pathol- and strengthen the evidence base for the involve permanent ablation of vestibular ogies that disturb balance and hearing (e.g. many treatments available for patients with function (e.g. surgery, labyrinthectomy or acoustic tumours, and Ménière’s disease. division). Vestibular func- ). At current diagnostic tion tests are best ordered and interpreted resolutions, MRI imaging has no specific Other tests by a specialist (a neurologist or ear nose findings to indicate the presence of endo- General haematological and biochemistry and throat surgeon) with experience in lymphatic hydrops. However, there have tests show no specific abnormalities in managing otological conditions. been recent advances in MRI imaging Ménière’s disease but are useful in evaluat- An electrocochleogram,Copyright which _Layout records 1 17/01/12 using 1:43 intratympanic PM Page 4 injections of gado- ing patients for other causes of vertigo. A the hair cell responses to sound, may also linium contrast material, which have full blood count may show anaemia. Renal

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distinguished by its response to antimi- that the two be distinguished. If doubt 2. SOME DIFFERENTIAL DIAGNOSES FOR BALANCE DISTURBANCE graine medication (see below). exists, a trial of antimigraine management Simultaneous loss of vestibular and should be undertaken before considering Nonvestibular (most likely cochlear function is most commonly due irreversible interventions for Ménière’s nonvertigo) to Ménière’s disease but occasionally can disease. Vestibular migraine was discussed Anaemia have other causes. Herpes zoster causes in detail in a previous issue of Medicine Anxiety , vertigo and hearing loss, with vesicles Today.8 Cardiac arrhythmias in the ear canal (and if accompanied by Medication and other drug side effects facial palsy is termed Ramsay Hunt syn- MANAGEMENT Panic attacks drome). due to or fungi, Modern management of patients with Postural hypotension carcinoma, lymphoma or sarcoid can cause Ménière’s disease aims to reduce the vestibular and cochlear dysfunction asso- ­frequency and severity of symptoms and Vestibular (with and without hearing loss) ciated with other cranial nerve lesions. to improve the quality of life of patients Benign paroxysmal positional vertigo Vasculitides, including some ear- and and their families. A flexible management Cerebellar stroke eye-specific syndromes such as Cogan’s strategy needs to be formulated for each Ménière’s disease syndrome and Susac’s syndrome (retino- patient and for different stages of the dis- Migraine cochleocerebral vasculopathy), and syphilis ease. Treatments are best considered from Viral can mimic Ménière’s disease. a symptom control viewpoint, leading to lesions involving the vestibular nerve root a stepwise introduction of available ther- or nucleus, such as multiple sclerosis, rarely apies depending on their toxicities and function tests are important for patients cause a similar syndrome. ease of administration (Figure 4). who may require treatment with diuretics In general, episodic vertigo can be con- as part of their overall management. MIGRAINE AND MÉNIÈRE’S DISEASE trolled in most patients by current inter- Over the past 25 years, awareness has ventions (70% controlled within two years DIFFERENTIAL DIAGNOSIS increased that migraine can be associated of presentation), but it may take time to Patients with disturbances of balance are with disturbances of balance, including establish a satisfactory treatment regimen. extremely common in general practice, , imbalance and vertigo, with or In the advanced stages of Ménière’s disease, with many of the underlying causes being without headache, mimicking early especially when it is bilateral, hearing loss nonvestibular (see Box 2). The key to eval- Ménière’s disease. The overall prevalence has greater impact, often requiring pow- uating the differential diagnosis of dis- of migraine in the general population is erful hearing aids or at times a cochlear turbed balance lies in obtaining an accurate 13%, and a quarter of migraine patients implant. Tinnitus and the sensation of history of the balance problem and asso- experience dizziness or vertigo along with aural fullness are more difficult to manage. ciated symptoms (e.g. hearing loss, tinnitus other more typical migraine symptoms. Patients often habituate to these symp- and a feeling of aural fullness). Descriptions Because the prevalence of migraine in the toms, but they can persist and remain such as light-headedness, disorientation general population is far greater than that troublesome. and floating are likely to have nonvestibular of Ménière’s disease, a patient presenting causes, whereas a history of vertigo (a true with recurrent vertigo is much more likely Treatment of acute vertiginous sense of movement, spinning, rocking or to have migraine-associated vertigo than attacks tilting) is highly likely to be due to a dis- Ménière’s disease. The dominant symptom during acute turbance of the , including The distinction between vestibular attacks is vertigo. This is usually associated Ménière’s disease. migraine and Ménière’s disease may not with intense nausea, vomiting, sweating Further clues in the history, such as the be considered important in the early stages and sometimes diarrhoea. As the attacks duration of the vertigo, its frequency (rate of Ménière’s disease as initial management are usually unheralded, it is wise to advise of recurrence), positional elements, and strategies for the two disorders overlap (diet patients of strategies: the association of hearing loss, tinnitus and lifestyle modification), and prescribed • to ensure their safety (especially and aural fullness, allow Ménière’s disease generally have few side effects. while driving or working in situa- to be distinguished from other vestibular However, in the later stages of Ménière’s tions of danger) causes of vertigo such as BPPV, vestibular disease, when more invasive and irrevers- • to allow the attack to pass (which and cerebellar haemorrhage.6 ible treatments are being considered (e.g. may take several hours) and recovery Vestibular migraineCopyright can mimic _Layout early1 17/01/12 1:43 PM or Page surgical 4 labyrinthectomy or to ensue (often after a period of a day Ménière’s disease but can usually be endolymphatic sac surgery), it is crucial or two of feeling ‘washed out’).

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Complete destruction of the inner ear Figure 4. 3. SUPPORT SOCIETIES AND Ladder of INFORMATION SOURCES FOR Vestibular nerve section treatment MÉNIÈRE’S DISEASE options for Aminoglycoside injections preventing Support societies are an important attacks in source of information and reassurance Surgery of endolymphatic sac patients with to patients and their families. They can Ménière’s let patients and their families know that Micropressure disease. they are not alone or helpless in the Diuretics// Surgical and/or destructive challenge of managing their symptoms. They commonly provide authoritative guidelines on low-salt diets and Low-salt diet exercise programs, and updates on new management strategies as they Conservative become available. Much information of a general nature is available from their Once an attack is established, little can individual requirements can be helpful. A websites and regular newsletters. be done to alter its natural course. Vestib- regular and vigorous exercise program is Support societies and sources of ular suppressants with antinausea effects also useful in helping patients regain con- information on Ménière’s disease (ondansetron, prochlorperazine) are fidence in their . A regular include: ­useful. My preferred strategy is to use sub- brisk walk of 30 minutes, three to five days • Meniere’s Australia lingual ondansetron wafers (4 to 8 mg each week, is achievable by most patients. (www.menieres.org.au) three times daily), as these can be taken Formal vestibular exercises, conducted by • US National Institute on despite intense nausea and have a rapid a physiotherapist, may be useful for those and Other Communication Disorders onset of action. Diazepam 2 mg orally who need help designing a specific and (www.nidcd.nih.gov/health/balance) twice daily for 24 hours is a reasonable sustainable training program. • UK Ménière’s Society alternative. Rarely, hospital admission for Support societies such as Meniere’s (www.menieres.co.uk) a severe or prolonged attack is necessary Australia (www.menieres.org.au) provide for intravenous rehydration, especially for useful and accurate information for elderly patients or during hot weather. patients about vertigo, hearing loss, tinni- processed and fast foods. Although food tus and management of Ménière’s disease. may initially bland and unappetising, Preventive strategies They can also provide reassurance for most patients who persist habituate to the Education, lifestyle changes and patients and their families (see Box 3). new dietary conditions after a few weeks. support societies Input from a dietitian or an NAS recipe Patient education is an important part of Low-salt diet book can be useful. Reducing caffeine, the treatment of Ménière’s disease. Knowl- A simplistic understanding of diet and caffeinated soft drinks, chocolate and alco- edge about the disease, likely natural his- Ménière’s disease suggests that dietary salt hol intake seems to make a difference in tory and treatment options and dispelling intake is related to the degree of endolym- some patients. of myths surrounding treatment are phatic hydrops and influences the frequency important to improve the quality of life of and severity of vertiginous episodes. Diuretics patients and their families. Accurate infor- Although there is little formal evidence to Diuretics have been a mainstay for treatment mation can help alleviate the feelings of support this view, there is anecdotal evidence of Ménière’s disease since the early 1900s. frustration and helplessness experienced from clinical practice, with some patients Theoretically, the sodium loss produced by by many patients at first diagnosis. reporting acute attacks of Ménière’s disease diuresis reduces the extracellular fluid in Simple interventions such as regularity following a salt binge. the body and in turn the amount of endo- with diet, sleep and exercise go a long way Sensible recommendations for dietary lymphatic hydrops. Hydrochlorothiazide is to improving patients’ coping mechanisms. sodium restriction suggest a salt intake the most widely used diuretic, but frusemide, , both physiological and emotional, ranging from 1 to 2 g/day. In practice, this and combination diuretics plays a role in triggering attacks. Formal involves following a ‘no added salt’ (NAS) such as hydrochlorothiazide plus triamterene stress management Copyrightprograms _Layout delivered 1 17/01/12by diet, 1:43 avoiding PM Page obviously 4 salty foods, taking are also used. Careful monitoring of serum a psychologist and tailored to patients’ note of food labelling and avoiding electrolyte levels is required. Despite the

24 MedicineToday x MARCH 2014, VOLUME 15, NUMBER 3 Downloaded for personal use only. No other uses permitted without permission. © MedicineToday 2014. widespread use of diuretics, a recent Hearing aids and tinnitus inner ear from a portable pressure gener- Cochrane review found that there is no good management ator. Its use is based on the observation that evidence to support or reject their use in Later in the course of Ménière’s disease, pressure changes applied to the inner ear patients with Ménière’s disease.9 hearing loss starts to become intrusive. result in improved vertigo control in has limited impact patients with Ménière’s disease. A standard Betahistine in quiet listening environments but ventilation tube (or pre-existing perforation Vascular insufficiency to the inner ear has becomes significant with background of the ear drum) is required for a micro- long been proposed as a mechanism for . Directional hearing and music pressure device to be used. A treatment Ménière’s disease. Betahistine (a appreciation are also impaired. Bilateral cycle takes a few minutes and is repeated analogue) has been used in this context for hearing loss in bilateral Ménière’s disease three times a day. Several studies have sug- decades with the aim of improving inner is more intrusive. Hearing aids can be use- gested beneficial vertigo control with min- ear perfusion. Testing in animals suggests ful in this situation, but a particular chal- imal risk of complications. that this improves blood flow in the stria lenge is the fluctuating hearing loss seen vascularis of the cochlear duct. A recent in Ménière’s disease. This has been partially Aminoglycoside treatment clinical trial in humans showed betahistine addressed with self-programming and For patients in whom disabling vertigo to be useful in reducing the frequency and adjustable hearing aids, which allow continues to be the dominant symptom severity of vertiginous episodes and to some patients to adjust the power and processing despite reasonable trials of medical man- degree in helping tinnitus. There was no setting of the to suit their cur- agement, chemical labyrinthectomy should effect on hearing loss or aural fullness. rent hearing thresholds. If hearing loss is be considered. Gentamicin is the most Betahistine has minimal side effects severe then cochlear implants are highly commonly used ototoxic agent. The aim (gastric irritation) and the dose is easily effective in restoring hearing.10 of treatment is to greatly reduce hair cell varied (8 to 32 mg/day), allowing it to be Tinnitus can be a particularly distress- function in the vestibular system of the titrated against the frequency and severity ing symptom. Management strategies affected ear. Gentamicin is relatively ves- of vertiginous attacks. An initial regimen include education, sound therapy, short- tibulotoxic but leaves the cochlear hair cells of 16 mg twice daily is a useful starting term drug therapy with benzodiazepines intact, making it suitable for treatment of point, with the dose reduced by half each or sedating antidepressants, tinnitus intractable vertigo in with functional month. There is very little interaction with retraining therapy and cognitive behav- hearing. Gentamicin can be administered other medications, making it safe for use ioural therapy.11 under local anaesthesia by a transtympanic in older patients with Ménière’s disease. injection or via a grommet to the affected Semi-invasive and surgical ear, thereby sparing toxicity to the unaf- Corticosteroids treatments fected ear in unilateral disease or the inac- treatments for Ménière’s Grommets tive ear in bilateral disease. ­disease are a more recent intervention, based Several nonevidence-based surgical oper- A commonly used fixed-dose transtym- on the possible autoimmune basis of the ations and ‘sham’ procedures have been panic protocol involves a single injection disease and recent experience with use of used in the past with some benefit, most of gentamicin (40 mg in a 2 mL solution) intratympanic corticosteroids to treat sudden likely because of a strong placebo effect. to the , with a second injection sensorineural hearing loss. In addition to Most have now become historical, but four weeks later if the vertiginous episodes their possible immune-modulating effects, insertion of grommets (tympanostomy continue. Most outcome studies of gen- corticosteroids are likely to influence the tubes) is still used and may have a benefit tamicin report greatly improved vertigo sodium and fluid dynamics of the inner ear on vertigo control and aural fullness. As it control. The effect on the vestibular hair through their mineralocorticoid properties, is a simple procedure able to be performed cells is permanent and irreversible, with a making them a theoretically attractive treat- under local anaesthesia, is temporary and small risk of associated hearing loss. ment. However, few clinical trials have inves- has a low risk of complications (persistent tigated the use of corticosteroids for vertigo perforation, otorrhoea) grommet insertion Endolymphatic sac surgery, control to date. It may be reasonable to is worth considering. labyrinthectomy and vestibular nerve administer a short course of oral cortico­ section steroids (prednisone 1 mg/kg/day for 10 days) Micropressure therapy Surgery on the endolymphatic sac has been or to consider intratympanic injection Micropressure therapy is a minimally inva- a major treatment for intractable vertigo. ­( 4 mg, single dose) for sive therapy for Ménière’s disease that uses However, more recent analysis of the out- patients with Ménière’sCopyright disease _Layout who experi 1 17/01/12- a soft 1:43 probe PM insertedPage 4 into the external ear comes of sac surgery have shown it to be ence a sudden drop in hearing thresholds. canal. This delivers pressure pulses to the no better than placebo treatments (simple

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changes can go a long way to improving 4. PRACTICE POINTS ON MÉNIÈRE’S DISEASE patients’ quality of life. Referral to a specialist is preferable to • Ménière’s disease is characterised by recurrent attacks of vertigo accompanied by establish the correct diagnosis and to fluctuating sensorineural hearing loss, tinnitus and a sense of aural fullness. It is ­formulate a treatment plan. Some practice caused by a disturbance of inner ear physiology. points about Ménière’s disease are sum- • The prevalence of Ménière’s disease increases with age, peaking at ages 60 to marised in Box 4. 69 years; it is rare under the age of 20 years. • The hallmark of an acute Ménière’s attack is prolonged vertigo, which is characterised CONCLUSION by a sudden unheralded intense sensation of movement, most commonly rotation or An attack of Ménière’s disease can be a spinning, lasting at least 20 minutes. frightening experience for patients and • The differential diagnosis of recurrent vertigo includes migraine-associated their families. It occurs suddenly and with- vertigo, which is far more common than Ménière’s disease and should be strongly out warning. Later in the disease, hearing considered in the initial assessment and treatment of patients with vertigo. loss and tinnitus can become intrusive, • Management aims to reduce the frequency and severity of symptoms and to particularly in bilateral cases. In patients improve the quality of life of patients and their families. A combination of lifestyle with classical Ménière’s disease, a careful changes, medical and surgical interventions should be considered. history and a simple audiogram are usually • Episodic vertigo can be controlled in most patients by current interventions sufficient to make the diagnosis. A care- (70% controlled within two years of presentation), but it may take time to establish fully designed, stepwise treatment plan a satisfactory treatment regimen. involving dietary and lifestyle changes and medication can stabilise the symptoms and greatly improve patients’ quality of mastoidectomy), leading to a decline in its This is usually because of insufficient resid- life. MT popularity as a treatment of Ménière’s ual vestibular function in longstanding disease. end-stage disease. REFERENCES Complete unilateral surgical deaffer- A current experimental intervention entation of the vestibular system in the is the vestibular electronic implant. This A list of references is included in the website version affected ears via labyrinthectomy or comprises an implanted device with three (www.medicinetoday.com.au) and the iPad app ­vestibular nerve section have also lost electrodes, one placed in each of the end version of this article. popularity. Although highly effective in organs of the affected , controlling vertigo, these procedures carry attached to a microprocessor (analogous COMPETING INTERESTS: None. the risk of total hearing loss, facial nerve to a cochlear implant), which can sense palsy and neurosurgical complications changes in head posture and position. The Online CPD Journal Program related to entering the posterior fossa. aim of the device is to simulate vestibular Surgical treatment of Ménière’s disease function during head movement with the has been largely replaced by transtym- hope of improving balance control and panic application of gentamicin, because quality of life in patients with end-stage of the latter’s ease of administration and vestibular disease. relative safety. ROLE OF THE GP Experimental treatments GPs have an important role in the man- Management of active Ménière’s disease agement of patients with Ménière’s disease. focuses on reducing the frequency and It is important to recognise the chronic What are the characteristic features severity of vertiginous attacks. This can be nature of Ménière’s disease and to differ- of vertigo in Ménière’s disease? satisfactorily achieved in most patients with entiate it from more common causes of a combination of the interventions transient vertigo, such as BPPV, vestibular Review your knowledge of this topic and described above. However, in some patients neuritis and vestibular migraine. Provid- earn CPD points by taking part in MedicineToday’s Online CPD Journal Program. with severe disease and particularly those ing education about the natural history of with bilateral Ménière’s disease, disabling Ménière’s disease, ways of implementing Log in to disequilibrium (poorCopyright balance) _Layout persists 1 17/01/12 and 1:43 sustaining PM Page dietary 4 (low sodium) and www.medicinetoday.com.au/cpd between each cluster of vertiginous attacks. lifestyle (regular sleep and exercise)

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MELVILLE DA CRUZ FRACS, MSc, MD

REFERENCES

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