M e n i e re s D i s e a s e i n t h e El d e r l y

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

KEYWORDS  Elderly  Menieres disease  Drop attacks  Otolith organs  Endolymphatic hydrops
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, Katsarkas1 found that 55.30% of them suffered from vertigo caused by an inner ear disease such as positional vertigo (47.20%), vestibular neuronitis (4.07%), and Menieres disease (4.07%). The typical criteria of Menieres 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 Menieres 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 Menieres disease ranges from 10 to 1000 per 100,000 patients of the ear, nose, and throat population.36 Menieres disease usually begins in adults ranging in age from 20 to 60 years.79 It is rarely described in children, who represent about 1% of Menieres patients.4,1014 However, that the real incidence of Menieres disease focuses on older patients was first reported by Ballester and colleagues.6 They found that among 432 patients suffering from Menieres 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, Uneri and Polat15 found a similar percentage of 12.5% of patients suffering from Menieres disease. These 2 studies tend to demonstrate that Menieres 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: dominique.vibert@insel.ch Otolaryngol Clin N Am 43 (2010) 10411046 doi:10.1016/j.otc.2010.05.009 0030-6665/10/$ see front matter 2010 Elsevier Inc. All rights reserved. oto.theclinics.com

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frequently than previously thought in patients older than 65. Although it seems that both 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 That women are more afflicted in this age range might be directly related to their longer life span compared with that of men. Ballester and colleagues6 distinguished 2 different groups of patients in their study. One group of patients from 65 to 75 years suffered from a reactivation of longstanding Menieres disease, which represented 40.9% of the cohort, and a second group of patients demonstrated the first manifestations of Menieres disease occurring between the ages of 65 and 82 years. The percentage of this de novo Menieres disease reaches 59.1% of all patients. In both groups, the clinical manifestations were similar to the classic vertigo spells lasting from minutes to hours, with nausea and sometimes with vomiting as well as the sensorineural hearing loss with fluctuation of hearing and tinnitus. However, the drop attacks were more frequent in the de novo group, occurring in 25.6% of patients compared with 11.1% in patients with a reactivation of their longstanding Menieres disease. This study underlined 2 interesting facts: the preponderance of women and the high frequency of drop attacks in patients older than 65. In the general population of Menieres disease with patients younger than 65 years, the incidence of drop attacks varies between 5% and 10%1618; however, Kentala and colleagues19 reported an extremely high incidence of drop attacks in 72% of their patients with Menieres disease aged from 17 to 79 years. In this study, the mean age at onset of the disease was 44 years, and they classified the drop attacks in 3 degrees (mild, moderate, severe) depending on the ensuing daily disturbances. Nine percent of the patients suffered severe disturbances. This percentage is therefore consistent with those in the literature with studies performed in the general population of patients with Menieres disease. Kentala and colleagues19 explained this high prevalence of drop attacks was because patients would probably not have spontaneously reported that the drop attacks caused mild or moderate disability if they had not been specifically asked. Thus, compared with the literature data, the group of patients with de novo Menieres disease in the elderly population showed a higher incidence of 25.6% of drop attacks. Feelings of erroneous movements such as the sensation of being pushed from behind or of a sudden movement of the environment are frequently described by patients with drop attacks. These symptoms are attributed to a dysfunction of the otolithic organs that measure the linear accelerations in the horizontal and vertical axes as well as the gravitational vector. Several pathophysiological mechanisms are thought to be implicated in the otolithic catastrophe of Tumarkin: sudden shift of the utricular macula, sudden changes in the endolymphatic fluid pressure, and sudden electrolyte changes secondary to the rupture of the membrane labyrinth. Thus, the inappropriate stimulation of the otolithic organs might generate a failure of the vestibulospinal reflex with the loss of postural tonus and, consequently, the falling.16,1922 To explain the higher incidence of drop attacks, particularly in patients with de novo Menieres disease, Ballester and colleagues6 assumed that it could be linked to a decreased compliance of the otolithic structures with a lower tolerance of the hydrops, owing to a limited capacity of the endolymphatic compartment distension. They also took into account the progressive decline of postural control and gait and visual difficulties of the elderly as factors able to influence the onset of falls. However, based on several recently published articles, new hypotheses might be proposed 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 Menieres disease or the histopathologic diagnosis of hydrops, Merchant and colleagues23 suggested that endolymphatic hydrops must be considered as an epiphenomenon of Menieres disease rather than being directly its cause. They considered that hydrops should be a marker for disordered homeostasis of the labyrinth. Indeed, Ichimiya and colleagues,24 Nadol and colleagues,25 and Shinomori and colleagues26 demonstrated cytochemical changes and ultrastructural lesions within type I and type II fibrocytes of the spiral ligament in experimental endolymphatic hydrops. They are involved in the recycling of K1 ions within the scala media.27,28 Furthermore, the role of calcium homeostasis implicated in endolymphatic hydrops has been suspected for several decades.2931 Several studies have shown that an induced endolymphatic hydrops in guinea pigs generated a number of biochemical changes,25 and particularly a marked decrease of immunoreactivity in calcium-binding proteins such as calmodulin, caldesmon, osteopontin, and S-100 among the type I fibrocytes.24 It was also suggested that the dysfunction of type I fibrocytes may be involved in regulating Ca11 levels in cochlear fluids.24
ON THE VESTIBULAR SIDE

Several experimental studies demonstrated the presence of calcium at all levels of the ultrastructure of the otolithic organs.3236 These findings pointed out the important role that calcium plays in the otolithic organs and their function. The recurrent benign paroxysmal positional vertigo, attributed to a dysfunction of the otolithic organs, was suspected to be related to a disturbance of calcium metabolism such as osteoporosis/osteopenia in women older than 50.37 This hypothesis was then corroborated by the results of an experimental study performed in female adult rats showing ultrastructural 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 Menieres disease as well as the high incidence in women and drop attacks in patients aged 65 or older might be related to the specific impact of the role of calcium metabolism in the elderly. Furthermore, drop attacks seem to occur more frequently in women than men (Dominique Vibert personal unpublished data, 2009). Thus, this difference might be because the disturbances of calcium metabolism that generate osteopenia or osteoporosis are more predominant in postmenopausal women compared with men. The treatment of Menieres disease in the elderly represents a challenge because of the polymedication that is very often administered for other concomitant systemic diseases. On one hand, in most cases, antivertiginous drugs such as betahistine and cinnarizin give good results with minor secondary effects. On the other hand, neuroleptics and antihistaminics are more difficult to administer because of their side effects, such as parkinsonism and depression, particularly in cases of longterm treatment. Chemical labyrinthectomy, by instillation of gentamycin into the middle ear, can also be proposed,3941 but unfavorable evolution with incapacitating ataxia may sometimes be observed.6 Minor surgical procedures such as insertion of transtympanic ventilation tubes and transcanalar sacculotomy are reported as to be effective and suppress the vertigo attacks in more than 70% of cases.6,4244 Nevertheless, sacculotomy represents a 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 selective vestibular neurectomy, represent optional procedures but must be carefully evaluated from case to case for patients with intractable recurrent attacks of vertigo resistant to other treatments. Ablative procedures remain the efficient treatment of drop attacks, taking into account the potential risks of severe injuries occurring in cases of sudden falls. When the general physical condition of the patient is good without comorbidity, such as sensory ataxia, cerebellar dysfunction, and poor vision, older patients are able to satisfactorily compensate the peripheral vestibular deafferentation.6,4649
SUMMARY

Menieres disease occurs more frequently than previously thought in patients older than 65 years. Two different groups of patients are distinguishable: patients with reactivation of a longstanding Menieres disease and patients with de novo disease. Compared with general Menieres patients, this specific population shows a high incidence of women as well as a high incidence of drop attacks, especially among patients with de novo Menieres disease. Because of the even lengthier life span of the occidental population, the potential risks of severe injuries caused by the drop attacks and their social consequences might represent a real problem for public health in terms of suitable care proposed to these patients.
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