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25. van der Stappen A, Wuyts FL, van de Heyning PH. Computerized electronystagmography:
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26. Halmagyi GM, Cremer OF, Anderson J, Murofushi T, Curthoys IS.Isolated directional preponderance of caloric nystagmus: clinical signicance. Am J Otol. 2000;21:559.
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28. Hess K, Baloh RW, Honrubia V, Yee RD.Rotational testing in patients with bilateral peripheral
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29. MacDougall HG, Weber KP, McGarvie LA, Halmagyi GM, Curthoys IS.The video head impulse
test: diagnostic accuracy in peripheral vestibulopathy. Neurology. 2009;73(14):1134–41.
30. Stevens MN, Garrison DB, Kaylie DM.What is the potential clinical utility of vHIT when
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31. Alhabib SF, Saliba I. Video head impulse test: a review of the literature. Eur Arch
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2016 Jun 21.
32. Janky KL, Patterson J, Shepard N, Thomas M, Barin K, Creutz T, Schmid K, Honaker
JA.Video head impulse test (vHIT): the role of corrective saccades in identifying patients with
vestibular loss. Otol Neurotol. 2018;39(4):467–73.
33. Anson ER, Bigelow RT, Carey JP, Xue QL, Studenski S, Schubert MC, Agrawal Y.VOR gain
is related to compensatory saccades in healthy older adults. Front Aging Neurosci. 2016;8:150.
34. Hullar TE, Della Santina CC. Responses of irregularly discharging chinchilla semicircular canal vestibular nerve afferents during high-frequency head rotations. J Neurophysiol.
2005;93(5):2777–86.
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impulse test detects gentamicin vestibulotoxicity. Neurology. 2009;72(16):1417–24.
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40. Piker EG, Jacobson GP, McCaslin DL, Hood LJ.Normal characteristics of the ocular vestibular evoked myogenic potential. J Am Acad Audiol. 2011;22(4):222–30.
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https://doi.org/10.1007/s00405- 016- 4157- 4. Epub
A. Chern and L. Lustig
Chapter 4
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Menière’s Disease
HitomiSakano andBenjaminT.Crane
Learning Objectives and Key Points
• History of Menière’s disease and the current diagnostic criteria.
• Incidence of Menière’s disease and histological ndings.
• Vestibular tests and how they can be used to help with the diagnosis.
• Treatment options including conservative and ablative treatment.
Case Study
A 64-year-old female presents to the clinic with three episodes of spinning vertigo
over the past month. Each attack lasted 3h and associated with vomiting. Just prior
to the onset of vertigo, she noticed that her right ear had a buzzing sound and the
hearing seemed mufed. The ear continues to feel full. She then reveals that she has
had episodic vertigo off and on for the past 10 years but that they would occur less
than once a year. The only medication she takes is hydrochlorothiazide for
hypertension.
Introduction
Before 1860, the ear as the origin of balance and vertigo was unknown and dizziness as well as balance problems were thought to be an exclusively central disorder.
The cause was often given as “cerebral congestion” or lumped in with epilepsy. As
early as the 1820s, postrotation nystagmus was observed in mental patients after
rotation in cages as a means to subdue them. Jan E.Purkinje hypothesized that this
effect was central in origin. Vertigo symptoms during this period were often treated
with leaching, purging, and cupping. Around this time there were the rst hints that
H. Sakano · B. T. Crane (*)
Department of Otolaryngology, University of Rochester, Rochester, NY, USA
e-mail: Benjamin_Crane@urmc.rochester.edu
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2023
B. T. Crane et al. (eds.), Disorders of the Vestibular System,
https://doi.org/10.1007/978-3-031-40524-2_4
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the equilibrium may have a peripheral component. Pierre Flourens noticed that
pigeons would y in circles in the same orientation as an ablated semicircular
canal [1].
The existence of peripheral vestibular disorders was proposed by Prosper
Menière in 1861 [2]. Menière was the director of a large deaf mute institution in
Paris who saw patients develop both vertigo and deafness immediately after trauma
to the ear, allowing him to conclude that both symptoms have a common inner ear
origin [3]. In support of this conclusion, he presented the results of an autopsy of a
young girl who developed sudden hearing loss and acute vertigo. On autopsy,
Menière found her brain was normal but the semicircular canal was lled with
blood. Because of this nding it was commonly believed well into the twentieth
century that Menière’s disease was caused by hemorrhage. Prior to 1940, “Menière’s
disease” was used as a generic term for any peripheral vertigo, especially if it
involved hearing loss. The rst insight into the true pathophysiology of Menière’s
disease came a decade after his initial report, with Knapp’s hypothesis that inner ear
hydrops was similar to ocular glaucoma [4].
Early treatment of Menière’s disease focused on destruction of the end organ,
although criteria for diagnosis were not well dened at that time and many of the
patients described may not have had what would now be described as Menière’s
disease. In 1904, the techniques of both eighth cranial nerve section [5] and labyrinthectomy [6, 7] were described. The concept of drainage of the endolymph was rst
reported by Portmann in 1926 [8]. Dandy also proposed selective vestibular nerve
sectioning via a suboccipital approach during the 1930s, and treated over 600
patients [9]. In this early period, these procedures carried a high risk of deafness,
facial nerve paralysis and a signicant risk of mortality. It was not until 1938 after
examination of specimens taken from two nerve section patients who died in the
perioperative period that Hallpike and Cairns were able to report the dilation of the
endolymphatic system in patients with Menière’s disease. This was hypothesized as
a disruption of the resorptive mechanism [10]. This nding was also independently
made by Yamakawa [11].
Treatment of Menière’s disease has continued to evolve through the present day,
with several variations on the Portmann shunt being proposed, such as transmastoid decompression [12], subarachnoid drainage [13], and cochleosacculotomy
[14]. All of these procedures have had incomplete success at treating vertigo symp-
toms and carry a risk of hearing loss. A randomized controlled trial has suggested
that these procedures have an efcacy similar to the placebo effect of surgery [15],
although a more recent reanalysis of this data has suggested that endolymphatic
sac procedures may have a small effect on vertigo and tinnitus [16]. However,
shunts do not relieve the histologic nding of hydrops [17] and hydrops also persists on imaging [18]. With continuing controversy, these procedures are still performed and evolve [19] despite a lack of high quality evidence to support the
therapy [20].
In recent years, transtympanic therapy has become popular for Menière’s disease. Installation of aminoglycosides into the middle ear was described by
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Schuknecht in 1957 with streptomycin injection through a microcatheter placed
through the tympanic membrane [21]. Although control of vertigo was achieved in
these patients, severe hearing loss also occurred in most patients. The potential for
hearing preservation has led most to focus on gentamicin and dexamethasone as
transtympanic therapy options. Dexamethasone is the more conservative option
which allows good vertigo control for a limited time with minimal risk of hearing
loss [22, 23]. Although gentamicin is an ablative therapy, it is preferentially more
toxic to the vestibular hair cells and thus offers the potential for hearing preservation. Thus, it is possible to gain complete control of vertigo with only a small chance
of profound hearing loss [24].
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Clinical Course
The clinical presentation of Menière’s disease includes spontaneous, episodic
attacks of vertigo; sensorineural hearing loss which usually uctuates; tinnitus; and
often a sensation of unilateral aural fullness. Despite this well-known symptom
complex, Menière’s disease remains a controversial and often difcult disease to
diagnose, determine the pathogenesis, and dene optimal treatment. This is in part
due to the dramatic variability among patients and even within patients over time
and the absence of good diagnostic tests to conrm the disease. The AAO-HNS has
recently published a clinical practice guideline detailing the strength of evidence for
diagnostic tests and treatment modalities of Menière’s [25].
Background
Prosper Menière rst described the combination of hearing loss and vertigo in
1861 and proposed a common origin of both symptoms in the inner ear. Although
the idea that the ear could be the sensory organ responsible for both hearing and
balance was revolutionary at the time, he attributed the disorder to hemorrhage
of the semicircular canal [3]. It is Menière along with Flourens who recognized
that vertiginous symptoms could originate in the inner ear. However, hemorrhage into the inner ear which Menière himself believed to be the pathophysiology has proven to be erroneous. Knapp advanced the hypothesis that hydrops
was similar to ocular glaucoma [4], although this was not histologically demonstrated until 1938 [10, 11]. Prior to this time, “Menière’s disease” was used as a
generic term for any peripheral vertigo, and unfortunately some continue to
erroneously use it generically today. Understanding of Menière’s disease has
advanced considerably since these initial descriptions, yet the cause of the
underlying hydrops remains elusive and controversial despite a century of active
research.
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H. Sakano and B. T. Crane
Incidence
Signicant variation exists in the reported incidence of Menière’s disease. Reports
vary from 17 per 100,000in the Japanese population [26] to as much as 513 per
100,000in the population of southern Finland [27] with several studies reporting
intermediate values. Some of the variation may be explained by the diagnostic criteria used, and the access to health care in a population. The most recent data suggests an incidence in the United States of about 1in 500 [28] and in the United
kingdom of 13.1 per 100,000 person years [29]. The disease seems to be more
prevalent among Caucasians [30], with an approximately equal gender distribution
with some series claiming a slightly higher prevalence in women (see Fig.4.1) [28,
29]. The peak age of onset is in the fourth and fth decades, although presentation
can occur at almost any age. Most would agree that it is much less common than
some other sources of vertigo including benign paroxysmal positional vertigo
(BPPV) and vestibular migraine.
The frequency of bilateral disease is unclear and the published incidence is
extremely variable with a range of 2–78% [31]. The rate depends on the length of
follow up and the diagnostic criteria. The studies at the extreme ends of this range
were prior to 1980 when standardized diagnostic criteria were not employed. The
true incidence is probably in the range of 19–24% [30, 31]. The onset of bilateral
disease may occur many years or decades after the unilateral symptoms [32]. Some
have implicated autoimmune disease [33] and migraine [34] as related factors in
cases of bilateral Menière’s disease.
Familial occurrence of Menière’s disease has been reported in 10–20% of cases
[35, 36]. Autosomal dominant mode of inheritance has been suggested [37] although
500
400
300
200
100
# per 100,000 persons
0
Both
Male
Female
Fig. 4.1 Prevalence of Menière’s by age and gender. (Adapted from Alexander and Harris
2010 [28])
All <18 18-34 35-44 45-54 55-64 65+
190
136
240
10
Age (years)
9
9
61
41
80
176
114
233
299
206
386
411
318
497
440
355
504
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67
autosomal recessive and mitochondrial inheritance has also been described [38].
Migraine is strongly associated with Menière’s disease in familial cases [39]. Gluten
sensitivity has recently been implicated as a possible etiology [40]. The incidence is
elevated in individuals with specic major histocompatability complexes (MHC).
Human leucocyte antigens (HLA) B8/DR3 and Cw7 have been associated with
Menière’s disease [41, 42]. The etiology of the disease in these individuals may be
autoimmune [43], but no gene has yet been identied despite analysis of several
potential candidates [44].
Pathogenesis
Menière’s disease is characterized by recurring attacks of vertigo, sensorineural
hearing loss, tinnitus, and in some individuals, a uctuating fullness in the ear.
Acute attacks are superimposed on a gradual deterioration in sensorineural hearing
in the involved ear, typically in the low frequencies initially. Over time a reduction
in responsiveness of the involved peripheral vestibular system occurs.
The pathologic basis felt to underscore these ndings is a distortion of the membranous labyrinth. The hallmark of this is endolymphatic hydrops [10]. This reects
the changes in the anatomy of the membranous labyrinth as a consequence of the
over-accumulation of endolymph. This occurs at the expense of the perilymphatic
space (Fig.4.2).
Traditional thought has held that the endolymph, which is produced by the stria
vascularis in the cochlea and by the dark cells in the vestibular labyrinth, circulates
in both a radial and longitudinal fashion [45]. In the case of hydrops, the underlying
pathophysiology is controversial but, inadequate absorption of endolymph by the
endolymphatic sac is the prevalent theory [46]. The endolymphatic duct (and valve
Fig. 4.2 Photomicrograph
of a cross section of human
cochlea demonstrating
endolymphatic hydrops in
a patient with Menière’s
disease. Note the
distension of Reissner’s
membrane into the scala
tympani in the apical turn
of the cochlea (arrowhead).
(Courtesy of Drs. J.Rutka
and M.Hawke, University
of Toronto)
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H. Sakano and B. T. Crane
of Bast) may act as a valve to regulate endolymph homeostasis [47]. Temporal bone
histologic equivalents of hydrops have been produced in animals as a consequence
of disruption of the endolymphatic sac, thereby supporting the above theory of
pathogenesis [48].
Pathologic studies of the human endolymphatic sac in the hydrops patient remain
controversial. Some have reported perisaccular brosis [49] and decreased endo-
lymphatic duct size [50]. However, the underlying cause of these histologic ndings
is not certain.
Imaging studies of individuals with Menière’s disease also identify abnormalities of the endolymphatic drainage system. Such studies suggest hypoplasia of the
endolymphatic sac and duct reected in the decreased visualization of the vestibular aqueduct and reduction in periaqueductal pneumatization on computed tomographic (CT) imaging [51]. Individuals with Menière’s disease have signicantly
smaller and shorter endolymph drainage systems as measured by the distance
between the posterior semicircular canal and the posterior fossa on MRI [52]. These
anatomic variations develop by age 3, and may predispose these individuals to later
development of Menière’s disease. Enhancement of the endolymphatic sac [53] and
perilymphatic space [54] has been demonstrated on MRI after gadolinium enhancement. There have been ongoing studies in which hydrops has been imaged in
humans [55]. A more recent variation using 4-h delayed intravenous contrastenhanced three- dimensional uid attenuated inversion recovery (3D FLAIR) MRI
[56] shows endolymphatic hydrops on imaging in 90% of symptomatic ears
(Fig.4.3).
The presence of hydrops by imaging is not one of the current diagnostic criteria
for Menière’s disease. Positive ndings for endolymphatic hydrops can be seen in
22% in clinically silent ears [56]. Imaging may be useful in ruling in Menière’s
disease but not in ruling it out. The utility of 4h MRI protocol is still questionable
as it is not a feasible protocol outside of academic settings. However, there is a
well- established role of standard MRI to exclude other possible causes of dizziness
and unilateral hearing loss such as a vestibular schwannoma or endolymphatic
sac tumor.
Endolymphatic hydrops has been uniformly observed in the temporal bones
from individuals with Menière’s disease. However, not all patients found to have
hydrops, had a history of Menière’s disease [57, 58]. Hydrops has also been found
postmortem following labyrinthitis, autoimmune inner ear disease, otitis media,
head trauma, mumps, and meningitis. Asymptomatic hydrops has also been
described [59].
Ruptures in the membranous labyrinth of the Menière’s patient are thought to be
signicant to the pathophysiology of Menière’s disease. Membranous ruptures have
been found in nearly all parts of the inner ear. Healed scars, presumably after rupture, have also been identied [60, 61]. Their presence has supported one of the
more prominent theories of the pathogenesis of Menière’s disease. Schuknecht postulated that ruptures in the membranous labyrinth allow leakage of the potassiumrich endolymph into the perilymph, bathing the eighth cranial nerve and lateral
sides of the hair cells [62]. High concentrations of extracellular potassium
a
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b
c
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Fig. 4.3 Delayed 3D inversion recovery sequence 4h after intravenous contrast administration.
Panel a (top) normal inner ear. Interscalar septum (thin arrow), scala tympani (large arrow head),
osseous spiral lamina/cochlear duct (thick arrow), scala vestibuli (small arrowhead), saccule
(dashed arrow), and utricle (dotted arrow). Panel b: Cochlear hydrops grade I with irregular dilation and partial obstruction of the scala vestibuli (arrows). Vestibular hydrops grade I with dilation
of the endolymphatic space (dotted arrow) encompassing more than half of the vestibulum. A circular perilymphatic space (dashed arrow) remains visible. Panel c: Cochlear hydrops grade II, with
total obliteration of the scala vestibuli (arrows). In vestibular hydrops grade II, dilation of the
endolymphatic space leads to effacement of the perilymphatic space (dotted arrow). (Used with
permission, Barath etal. 2014 [56])
depolarize the nerve cells and cause their acute inactivation. This results in a
decrease in auditory and vestibular neuronal outow consistent with the hearing
loss and features of acute vestibular paralysis seen in a typical Menière’s attack.
Healing of the membranes is presumed to allow restitution of the normal chemical
milieu, with termination of the attack and improvement in vestibular and auditory
function. The chronic deterioration in inner ear function is presumably the effect of
repeated exposure to the effects of the potassium. This theory remains somewhat
controversial as others have suggested these ruptures rarely occur and do not adequately explain the observed symptoms [63].
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Etiology of Menière’s Disease
The triad of hearing loss, tinnitus, and vertigo constitutes Menière’s syndrome. If
the cause is unknown, it is dened as Menière’s disease [64]. However, if a disease
entity that is known to cause endolymphatic hydrops is associated with the syndrome, the diagnosis is one of secondary endolymphatic hydrops (i.e., otosclerotic
foci causing mechanical endolymphatic blockage [65]). Obstruction of the endolymphatic duct is the basis for development of hydrops in experimental animals.
This is accomplished by any lesion that can produce failure of duct function
including mechanical blockage, chemical brosis, viral inoculation, immunologically induced inammation, and ischemia [66]. However, these animal models
cannot be interpreted to explain the actual cause of the human disease. These models also do not completely reproduce the clinical and pathologic entity experienced
by humans.
Antibodies directed against normal inner ear elements have been suggested.
Patients with Menière’s disease have an increased incidence of specic types of
human leukocyte antigens [35, 67]. Most autoimmune processes, however, including those that affect the ear such as Cogan’s disease, have histopathology which
demonstrates inltration of white blood cells and cellular destruction. If an autoimmune mechanism is responsible, it would have to be a more indolent course or
intermittent and absent at the time of tissue sampling. Some patients with Menière’s
disease respond to allergic desensitization which further suggests a potential
immune etiology in some patients [68].
Viral infection has also been a suggested mechanism of Menière’s disease
[60]. The observed occurrence of symptomatic hydrops many years after unexplained deafness, so-called delayed endolymphatic hydrops, suggests that subclinical viral infection could cause hydrops many decades later [69]. No virus
has been conclusively identied, but a comparison of Menière’s disease patients
with controls has demonstrated higher antibody response to herpes simplex
virus [70].
Ischemia of the endolymphatic sac or inner ear has also been proposed as a
underlying mechanism of Menière’s disease [71]. Such a common vascular mechanism may link migraine and Menière’s disease [39, 72, 73].
Numerous factors are implicated as causative of Menière’s disease. Although
this in part reects our continued lack of understanding, it is also suggestive that
Menière’s disease may be multifactorial or may represent the common end point to
a variety of injuries or anatomic variables. We know of a number of processes that
seem associated with the development of hydrops (e.g., trauma, acute otitis media,
autoimmune inner ear disease, labyrinthitis, congenital inner ear deformity, idiopathic processes). These are not always associated with the development of symptoms [57]. It is possible that Menière’s disease is precipitated by a variety of events
such as autoimmune, viral, traumatic, vascular/ischemic, and even congenital anatomic and molecular variations which may act as triggers for the later development
of symptomatic hydrops.
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Diagnosis
There is no single test that makes the diagnosis of Menière’s disease. Rather, it is
most importantly a complete history, including a detailed description of the pattern
of disease presentation, supported by quantitative testing. The most recent denition
of the disease has been established by the Committee on Hearing and Equilibrium
of the American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS)
and is summarized in Box 4.1. This was a joint, multinational effort by the
Classication Committee of the Bárány Society, The Japan Society for Equilibrium
Research, the European Academy of Otology and Neurotology (EAONO), the
Equilibrium Committee of the American Academy of Otolaryngology-Head and
Neck Surgery (AAO-HNS) and the Korean Balance Society [74]. With these criteria
the diagnosis of “denite Menière’s” denes the maximum duration of vertigo episodes as 12h (down from 24in the 1995 AAO-HNS criteria). They also only consider hearing loss asymmetry in the mid and low frequencies.
Box 4.1 Updated AAO-HNS Criteria for Menière’s Diagnosis [74]
Denite MD
• Two or more spontaneous episodes of vertigo each lasting 20 min
to 12h.
• Audiometrically documented low- to medium-frequency sensorineural
hearing loss in one ear, dening the affected ear on at least one occasion
before, during or after one of the episodes of vertigo.
• Fluctuating aural symptoms (hearing, tinnitus or fullness) in the
affected ear.
• Not better accounted for by another vestibular diagnosis.
Probable MD
• Two or more episodes of vertigo or dizziness, each lasting 20 min
to 24h.
• Fluctuating aural symptoms (hearing, tinnitus or fullness) in the
affected ear.
• Not better accounted for by another vestibular diagnosis.
Clinical Presentation
The typical history consists of recurring attacks of vertigo (96.2%) with tinnitus
(91.1%) and ipsilateral hearing loss (87.7%) [75]. Attacks are often preceded by an
aura consisting of a sense of fullness in the ear, increasing tinnitus, and a decrease
in hearing. They may, however, be sudden in onset, with little or no warning. Acute
attacks typically last from minutes to hours, most commonly 2–3h [76]. Attacks
longer than a day are unusual and if present should cast doubt on the diagnosis.
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