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Meniere’s Disease in the Elderly Dominique Vibert, MD*, Marco Caversaccio, MD, Rudolf Ha ¨ usler, MD Dizziness and vertigo are common complaints in the elderly population. However, these symptoms may be a result of multiple causes, such as cardiovascular disease, secondary effects of medication, and pathologies of the central nervous system, as well as inner ear diseases. Among a population of 3427 patients 70 years of age or older, Katsarkas 1 found that 55.30% of them suffered from vertigo caused by an inner ear disease such as posi- tional vertigo (47.20%), vestibular neuronitis (4.07%), and Meniere’s disease (4.07%). The typical criteria of Meniere’s disease include the onset of recurrent attacks of vertigo lasting for a few hours with nausea and vomiting. The patients also complain of fluctuating hearing loss, an intermittent sensation of fullness, and a transient or permanent tinnitus within the impaired ear. Drop attacks, consisting of sudden falls without loss of consciousness, first described by Tumarkin, 2 can also occur in patients suffering from Meniere’s disease. They are attributed to a sudden dysfunction of the otolithic organs and are also named ‘‘otolithic catastrophe of Tumarkin.’’ Depending on the studies, the incidence of Meniere’s disease ranges from 10 to 1000 per 100,000 patients of the ear, nose, and throat population. 3–6 Meniere’s disease usually begins in adults ranging in age from 20 to 60 years. 7–9 It is rarely described in children, who represent about 1% of Meniere’s patients. 4,10–14 However, that the real incidence of Meniere’s disease focuses on older patients was first reported by Ballester and colleagues. 6 They found that among 432 patients suffering from Meniere’s disease, 15.3% were 65 years or older. In a recent retrospec- tive study about the origin of vertigo and dizziness in 677 patients older than 65, U ¨ neri and Polat 15 found a similar percentage of 12.5% of patients suffering from Meniere’s disease. These 2 studies tend to demonstrate that Meniere’s disease occurs more Department of Otorhinolaryngology, Head and Neck Surgery, Inselspital, University of Berne, 3010 Berne, Switzerland * Corresponding author. Neurotology, University Clinic of ENT, Head and Neck Surgery Inselspital, 3010 Berne, Switzerland. E-mail address: [email protected] KEYWORDS Elderly Meniere’s disease Drop attacks Otolith organs Endolymphatic hydrops Otolaryngol Clin N Am 43 (2010) 1041–1046 doi:10.1016/j.otc.2010.05.009 oto.theclinics.com 0030-6665/10/$ – see front matter ª 2010 Elsevier Inc. All rights reserved.

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Page 1: Meniere’s Disea

Meniere’s Diseasein the Elderly

Dominique Vibert, MD*, Marco Caversaccio, MD,Rudolf Hausler, MD

KEYWORDS

� Elderly � Meniere’s disease � Drop attacks � Otolith organs� Endolymphatic hydrops

Dizziness and vertigo are common complaints in the elderly population. However,these symptoms may be a result of multiple causes, such as cardiovascular disease,secondary effects of medication, and pathologies of the central nervous system, aswell as inner ear diseases.

Among a population of 3427 patients 70 years of age or older, Katsarkas1 found that55.30% of them suffered from vertigo caused by an inner ear disease such as posi-tional vertigo (47.20%), vestibular neuronitis (4.07%), and Meniere’s disease (4.07%).

The typical criteria of Meniere’s disease include the onset of recurrent attacks ofvertigo lasting for a few hours with nausea and vomiting. The patients also complainof fluctuating hearing loss, an intermittent sensation of fullness, and a transient orpermanent tinnitus within the impaired ear. Drop attacks, consisting of sudden fallswithout loss of consciousness, first described by Tumarkin,2 can also occur in patientssuffering from Meniere’s disease. They are attributed to a sudden dysfunction of theotolithic organs and are also named ‘‘otolithic catastrophe of Tumarkin.’’ Dependingon the studies, the incidence of Meniere’s disease ranges from 10 to 1000 per100,000 patients of the ear, nose, and throat population.3–6

Meniere’s disease usually begins in adults ranging in age from 20 to 60 years.7–9 It israrely described in children, who represent about 1% of Meniere’s patients.4,10–14

However, that the real incidence of Meniere’s disease focuses on older patientswas first reported by Ballester and colleagues.6 They found that among 432 patientssuffering from Meniere’s disease, 15.3% were 65 years or older. In a recent retrospec-tive study about the origin of vertigo and dizziness in 677 patients older than 65, Uneriand Polat15 found a similar percentage of 12.5% of patients suffering from Meniere’sdisease. These 2 studies tend to demonstrate that Meniere’s disease occurs more

Department of Otorhinolaryngology, Head and Neck Surgery, Inselspital, University of Berne,3010 Berne, Switzerland* Corresponding author. Neurotology, University Clinic of ENT, Head and Neck SurgeryInselspital, 3010 Berne, Switzerland.E-mail address: [email protected]

Otolaryngol Clin N Am 43 (2010) 1041–1046doi:10.1016/j.otc.2010.05.009 oto.theclinics.com0030-6665/10/$ – see front matter ª 2010 Elsevier Inc. All rights reserved.

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frequently than previously thought in patients older than 65. Although it seems thatboth sexes are almost equally affected in adult patients,8 Ballester and colleagues6

described a strong preponderance in women, with a sex ratio of 0.43 in their patients.This sex preponderance was also reported in the study of Uneri and Polat.15 Thatwomen are more afflicted in this age range might be directly related to their longerlife span compared with that of men. Ballester and colleagues6 distinguished 2different groups of patients in their study. One group of patients from 65 to 75 yearssuffered from a reactivation of longstanding Meniere’s disease, which represented40.9% of the cohort, and a second group of patients demonstrated the first manifes-tations of Meniere’s disease occurring between the ages of 65 and 82 years. Thepercentage of this ‘‘de novo’’ Meniere’s disease reaches 59.1% of all patients. Inboth groups, the clinical manifestations were similar to the classic vertigo spells lastingfrom minutes to hours, with nausea and sometimes with vomiting as well as the senso-rineural hearing loss with fluctuation of hearing and tinnitus. However, the drop attackswere more frequent in the ‘‘de novo’’ group, occurring in 25.6% of patients comparedwith 11.1% in patients with a reactivation of their longstanding Meniere’s disease. Thisstudy underlined 2 interesting facts: the preponderance of women and the highfrequency of drop attacks in patients older than 65.

In the general population of Meniere’s disease with patients younger than 65 years,the incidence of drop attacks varies between 5% and 10%16–18; however, Kentala andcolleagues19 reported an extremely high incidence of drop attacks in 72% of theirpatients with Meniere’s disease aged from 17 to 79 years. In this study, the meanage at onset of the disease was 44 years, and they classified the drop attacks in 3degrees (mild, moderate, severe) depending on the ensuing daily disturbances.Nine percent of the patients suffered severe disturbances. This percentage is there-fore consistent with those in the literature with studies performed in the general pop-ulation of patients with Meniere’s disease. Kentala and colleagues19 explained thishigh prevalence of drop attacks was because patients would probably not have spon-taneously reported that the drop attacks caused mild or moderate disability if they hadnot been specifically asked. Thus, compared with the literature data, the group ofpatients with ‘‘de novo’’ Meniere’s disease in the elderly population showed a higherincidence of 25.6% of drop attacks.

Feelings of erroneous movements such as the sensation of being pushed frombehind or of a sudden movement of the environment are frequently described bypatients with drop attacks. These symptoms are attributed to a dysfunction of theotolithic organs that measure the linear accelerations in the horizontal and verticalaxes as well as the gravitational vector. Several pathophysiological mechanisms arethought to be implicated in the otolithic catastrophe of Tumarkin: sudden shift ofthe utricular macula, sudden changes in the endolymphatic fluid pressure, and suddenelectrolyte changes secondary to the rupture of the membrane labyrinth. Thus, theinappropriate stimulation of the otolithic organs might generate a failure of the vesti-bulospinal reflex with the loss of postural tonus and, consequently, the falling.16,19–22

To explain the higher incidence of drop attacks, particularly in patients with ‘‘de novo’’Meniere’s disease, Ballester and colleagues6 assumed that it could be linked toa decreased compliance of the otolithic structures with a lower tolerance of thehydrops, owing to a limited capacity of the endolymphatic compartment distension.They also took into account the progressive decline of postural control and gait andvisual difficulties of the elderly as factors able to influence the onset of falls.

However, based on several recently published articles, new hypotheses might beproposed to explain these 2 characteristics within this specific population of patients,ie, the high incidence of drop attacks and the prevalence in women.

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ON THE COCHLEAR SIDE

On review of 107 archival temporal bone cases with the clinical diagnosis of Meniere’sdisease or the histopathologic diagnosis of hydrops, Merchant and colleagues23 sug-gested that endolymphatic hydrops must be considered as an epiphenomenon ofMeniere’s disease rather than being directly its cause. They considered that hydropsshould be a marker for disordered homeostasis of the labyrinth. Indeed, Ichimiya andcolleagues,24 Nadol and colleagues,25 and Shinomori and colleagues26 demonstratedcytochemical changes and ultrastructural lesions within type I and type II fibrocytes ofthe spiral ligament in experimental endolymphatic hydrops. They are involved in therecycling of K1 ions within the scala media.27,28 Furthermore, the role of calciumhomeostasis implicated in endolymphatic hydrops has been suspected for severaldecades.29–31

Several studies have shown that an induced endolymphatic hydrops in guinea pigsgenerated a number of biochemical changes,25 and particularly a marked decrease ofimmunoreactivity in calcium-binding proteins such as calmodulin, caldesmon, osteo-pontin, and S-100 among the type I fibrocytes.24 It was also suggested that thedysfunction of type I fibrocytes may be involved in regulating Ca11 levels in cochlearfluids.24

ON THE VESTIBULAR SIDE

Several experimental studies demonstrated the presence of calcium at all levels of theultrastructure of the otolithic organs.32–36 These findings pointed out the important rolethat calcium plays in the otolithic organs and their function. The recurrent benignparoxysmal positional vertigo, attributed to a dysfunction of the otolithic organs,was suspected to be related to a disturbance of calcium metabolism such as osteo-porosis/osteopenia in women older than 50.37 This hypothesis was then corroboratedby the results of an experimental study performed in female adult rats showing ultra-structural changes on the utricles of the osteoporotic rats, in terms of size and density,as well as aspect of otoconia.38

Thus, the high incidence of ‘‘de novo’’ Meniere’s disease as well as the high inci-dence in women and drop attacks in patients aged 65 or older might be related tothe specific impact of the role of calcium metabolism in the elderly. Furthermore,drop attacks seem to occur more frequently in women than men (Dominique Vibertpersonal unpublished data, 2009). Thus, this difference might be because the distur-bances of calcium metabolism that generate osteopenia or osteoporosis are morepredominant in postmenopausal women compared with men.

The treatment of Meniere’s disease in the elderly represents a challenge because ofthe polymedication that is very often administered for other concomitant systemicdiseases. On one hand, in most cases, antivertiginous drugs such as betahistineand cinnarizin give good results with minor secondary effects. On the other hand,neuroleptics and antihistaminics are more difficult to administer because of theirside effects, such as parkinsonism and depression, particularly in cases of long-term treatment.

Chemical labyrinthectomy, by instillation of gentamycin into the middle ear, can alsobe proposed,39–41 but unfavorable evolution with incapacitating ataxia may some-times be observed.6

Minor surgical procedures such as insertion of transtympanic ventilation tubes andtranscanalar sacculotomy are reported as to be effective and suppress the vertigoattacks in more than 70% of cases.6,42–44 Nevertheless, sacculotomy representsa risk, with profound postoperative hearing loss reported in 10% to 20% of cases.43,45

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Definitive vestibular surgical deafferentations, such as labyrinthectomy and selec-tive vestibular neurectomy, represent optional procedures but must be carefully eval-uated from case to case for patients with intractable recurrent attacks of vertigoresistant to other treatments. Ablative procedures remain the efficient treatment ofdrop attacks, taking into account the potential risks of severe injuries occurring incases of sudden falls. When the general physical condition of the patient is goodwithout comorbidity, such as sensory ataxia, cerebellar dysfunction, and poor vision,older patients are able to satisfactorily compensate the peripheral vestibulardeafferentation.6,46–49

SUMMARY

Meniere’s disease occurs more frequently than previously thought in patients olderthan 65 years. Two different groups of patients are distinguishable: patients with reac-tivation of a longstanding Meniere’s disease and patients with ‘‘de novo’’ disease.

Compared with general Meniere’s patients, this specific population shows a highincidence of women as well as a high incidence of drop attacks, especially amongpatients with ‘‘de novo’’ Meniere’s disease.

Because of the even lengthier life span of the occidental population, the potentialrisks of severe injuries caused by the drop attacks and their social consequencesmight represent a real problem for public health in terms of suitable care proposedto these patients.

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