Enfermedad de Meniere. Otorrinolaringologia.

1. Eponym
Physical examination undertaken between attacks was
unremarkable, with normal blood pressure and cardiac
rhythm. Results of neurological examination, including
cerebellar examination, were normal. Ear, nose, and
throat examination including vestibular examination was
unremarkable on every occasion.
During a recent admission involving acute vertigo,
horizontal nystagmus was noted with the direction of the
nystagmus being towards the affected ear. An audiogram
showed a unilateral sensorineural hearing loss.
Ménière’s disease
Clinical features
The natural history of Ménière’s disease is typically
variable in intensity and frequency; the initial attacks
being predominantly vestibular in nature, with later
attacks more marked by tinnitus and hearing loss.
Hearing loss might at first be transient in nature, but
later becomes progressive. The disease can evolve over a
period of months to years and is usually unilateral, but
may develop in the opposite ear.
“Ménière’s disease is capricious both in its behaviour
and its response to treatment. Criteria for diagnosis may
vary greatly. Its puzzling periodicity with characteristic
remissions and exacerbations make the evaluation of
treatment methods difficult and frustrating. A uniform
set of criteria for diagnosis and judging and reporting
the results of treatment is needed to facilitate and
validate clinical studies of this disease.”1
These are the introductory remarks to the 1972
American Academy of Ophthalmology and Otolaryn-
gology Committee on Hearing and Equilibrium
guidelines published more than 30 years ago.1
This
system was revised in 1985,2
and again in 1995,3
and was
designed specifically for the standardisation of the
diagnosis and reporting of results, and in the production
of guidelines by the American Academy of
Otolaryngology–Head and Neck Surgery Committee on
Hearing and Equilibrium (AAO–HNS CHE) guidelines
for the diagnosis and evaluation of therapy.
The AAO–HNS CHE guidelines for definite Ménière’s
disease specify at least two episodes of vertigo of at least
20 min duration, audiometrically confirmed sensori-
neural hearing loss on at least one occasion, tinnitus or
aural fullness during episodes, and exclusion of other
possible causes of vertigo.3
Provision in these guidelines
is also made for certain, probable, and possible Ménière’s
disease.3
Ménière’s disease is associated with endolymphatic
hydrops (raised endolymph pressure in the membranous
labyrinth, figure 1), but a causal relation remains
unproven.4
Other conditions associated with endolym-
phatic hydrops, vertiginous conditions associated with
inner ear disease, and central causes of vertigo need to be
excluded when making a diagnosis.
Medical treatment
Dietary and lifestyle modification, including the
elimination or reduction of caffeine, alcohol, tobacco, and
stress, have been recommended. It has been suggested
that a low-salt diet might reduce endolymphatic pressure
in endolymphatic hydrops,5
but this hypothesis does not
appear to have been tested.4
Evidence for the beneficial effect of psychological sup-
port comes from observations that study groups
receiving placebos and patients placed on surgical
waiting lists can show resolution of symptoms.6
Furthermore, sham procedures have been shown to be as
successful as surgical intervention.7
Hence, it is difficult
to separate the apparent benefit of intervention from the
natural resolution of symptoms.
Lancet 2005; 366: 2137–39
Published online
August 25, 2005
DOI:10.1016/S0140-6736(05)
67144-9
Western Memorial Regional
Hospital, Corner Brook, A2H
6C7 Newfoundland, Canada
(M A Thorp FCS[SA]ORL); and
Department of
Otolaryngology, Southmead
Hospital, Bristol, UK
(A L James FRCS[ORL])
Correspondence to:
Dr Marc Thorp
m_cthorp@nf.sympatico.ca
www.thelancet.com Vol 366 December 17/24/31, 2005 2137
Prosper Ménière
MarcAThorp,AdrianLJames
Case presentation
A 46-year-old woman presented with a 3-month history of
vertiginous attacks associated with nausea and vomiting.
Each episode lasted anything from 1 h to 3 h. She described
the feeling of “being on a roundabout”, where her
surroundings were spinning. Episodes generally began with
her affected ear feeling “blocked off” and with increased
sensitivity to noise. A roaring tinnitus was associated with
some attacks and she had noticed deafness following most
episodes. She had experienced as many as three episodes a
week and had been sent home from work on occasion. She
had been admitted to an emergency room as a result of
associated vomiting. Each attack caused her and her family a
great deal of distress.
Figure 1: Vertical section through the middle turn of a human cochlea
showing endolymphatic hydrops by means of a bulging Reissner’s
membrane (arrow)

2. Eponym
Pharmacological treatments are used for prophylaxis
against acute episodes, as well as for the symptomatic
relief of vertigo. Vestibular sedatives such as prochlor-
perazine and cyclizine are widely used in the acute control
of vertigo. Sublingual preparations are especially useful
for vertigo associated with vomiting.
Results of an observational study showed that
intravenous histamine injection ameliorated the acute
manifestations of Ménière’s.4
This led to the development
of the histamine analogue, betahistine, and its use in
prophylaxis against Ménière’s disease. Betahistine is
thought to alleviate symptoms either by reduction of
endolymphatic pressure through improved microvascular
circulation in the stria vascularis of the cochlea, or by
inhibition of activity in the vestibular nuclei.4
Although
widely used to prevent acute attacks of Ménière’s, good
evidence to confirm the efficacy of betahistine is scarce.8
Small randomised placebo controlled studies suggest the
possibility of reduction in vertigo without substantial side-
effects, but no relevant change in tinnitus, aural fullness,
or hearing loss has been demonstrated.4
Diuretics and
trimetazidine are also used in the prophylaxis of acute
attacks of Ménière’s disease, despite the absence of any
convincing evidence of efficacy.4
The natural history and remitting nature of Ménière’s
disease makes assessment of the response to treatment
difficult, and efficacy can only be established in large,
long-term trials. In the absence of such evidence a
pragmatic approach to pharmacological management
accounts for the continued use of drugs of unproven
efficacy, albeit with minimal side-effects.
Surgical treatment
Patients who have not responded to treatment with drugs
or whose symptoms are incapacitating are offered surgery.
Surgery can be divided into two categories: destructive and
conservative. Destructive procedures ablate both
vestibular function and hearing reserve. These procedures
include labyrinthectomy, retro-labyrinthine vestibulo-
cochlear nerve section, and translabyrinthine vestibular
neurectomy.6
Conservative procedures are aimed at preservation of
hearing and are consequently offered to patients with
useful cochlear reserve. These include intratympanic
gentamicin instillation, endolymphatic sac surgery, and
vestibular nerve section. Both the intratympanic and
intralabyrinthine delivery of medications, including
steroids and the aminoglycoside antibiotics in particular
gentamicin, are now considered first choice treatment
after failed medical treatment.9
Intratympanic aminoglycosides
The predominantly vestibulotoxic effects of aminogly-
coside antibiotics, such as streptomycin and gentamicin,
renders them useful in the management of intractable
vertigo. When, however, such drugs are administered
systemically, they can affect both labyrinths, and although
the patient might be free of episodic vertigo, they might be
disabled by oscillopsia and ataxia.6
The selective vestibulotoxic profile of gentamicin
popularised intratympanic use for the treatment of severe
vertigo. 29 studies with gentamicin have been published
in the past decade, and have provided a considerable body
of evidence to justify its use in unilateral Ménière’s
disease. Vertigo control rates, using AAO–HNS CHE
treatment criteria, have varied from 83% to 100% in
published studies.9
The end organ vestibular effects of gentamicin
instillation are largely irreversible, and with definite risk of
permanent sensorineural hearing loss, the natural history
of Ménière’s disease should be considered before offering
treatment to patients.
Endolymphatic sac surgery
Endolymphatic sac surgery, repopularised in the 1960s,
was originally described in the 1920s. It is the only surgical
procedure that aims to reverse the suspected pathophys-
iological process of endolymphatic hydrops. Such surgery
can be classified into three main types of procedure:
decompression, drainage, and ablation. Although control
of vertigo is reported in 74–90% regardless of the pro-
cedure used,9
no report has shown convincing improve-
ment in hearing.6
Good results reported by some surgeons
may be associated with the timing of surgery and patient
selection.6
Critically, one randomised controlled study has
shown endolymphatic sac surgery to be no better than a
placebo procedure.7
Prosper Ménière
Prosper Ménière was born in Angers, France, in 1799.10
He was educated at the Lycee, and later at the university,
in Angers, where he excelled in the classics. He completed
his studies at the Hôtel-Dieu in Paris—one of the most
prestigious hospitals in Europe at the time. He received
the gold medal in 1826 and his doctorate in 1828, and his
ultimate ambition was to become a professor at the Hôtel-
Dieu.10
Ménière attended Duchess Caroline Louise, the widow
of the second son of Charles X, who was pregnant while
imprisoned at Blaye. Although this ingratiated him with
the current political establishment, it may have con-
tributed to his marginalisation from mainstream
academic medicine.10
In 1835, Ménière organised regional health care against
a cholera epidemic, for which he gained the Chevalier of
the Legion d’Honneur (figure 2). During this period, on
two occasions, candidates were appointed professorships
at the Hôtel-Dieu, even though it was felt to be that they
were less qualified than him.11
The year of 1838 marked a turning point. Jean Marc
Gaspard Itard, one of the fathers of otology and education
of the deaf, died while serving as director of the Institute of
Deaf-Mutes in Paris.11
Although Ménière had no formal
otological training, he was appointed to the post. In the
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3. Eponym
same year he married the daughter of Becquerel, a
member of the Institute of Deaf-Mutes in Paris. Ménière
dedicated the rest of his life to the care of deaf-mutes and
in doing so revolutionised the approach to vertigo.
Although he was a devoted physician and worked 7 days a
week, he also found time for his interests in opera, botany,
and political commentary. His friends included Balzac,
Liszt, Hugo, and the literary critic, Janin.11
Ménière’s greatest contribution came in 1861, when he
presented a paper to the Imperial Academy of Medicine in
Paris. He questioned the current theory that vertigo was a
form of cerebral apoplexy or epilepsy. He argued that the
inner ear was responsible for balance, a theory that was
violently opposed. His theories were based on astute
observation of patients with vertigo associated with
hearing loss and tinnitus, and the work of Pierre Flourens
who demonstrated that systematic surgical ablation of the
semicircular canals in a pigeon resulted in loss of balance,
which was oriented to the direction of that canal. Ménière
concluded that: (1) the auditory apparatus could be
affected, resulting in episodes of tinnitus and loss of
hearing; (2) abnormalities of the inner ear might be
responsible for sudden attacks of vertigo, accompanied by
nausea, vomiting and syncope; (3) although attacks are
intermittent, they are associated with progressive hearing
loss; and (4) the lesion in the condition later named after
him is likely to be in the semicircular canals.
He correctly recognised that vertigo of central origin was
not associated with hearing loss, and that patients with
aural vertigo did not lose consciousness during attacks.12
Following his death as a result of pneumonia in 1862,
aged 62, the triad of vertigo, deafness, and tinnitus became
increasingly recognised. In the 1870s, the term “Maladie
de Ménière” was coined by Charcot (1874).12
McBride and
James described “Ménière’s disease” as paroxysmal or
constant vertigo with coincident deafness.13
Beasley and
Jones reported that Politzer felt that the term Ménière’s
disease be reserved for sudden deafness and vertigo
arising from a disorder in the labyrinth.12
The pathological findings of endolymphatic hydrops
believed to be responsible for Ménière’s disease were only
discovered in 1938, simultaneously by Hallpike and
Cairns in London and Yamakawa in Osaka.12
Ménière was a man of humility, who was largely over-
looked by his peers. He once said:
“I am certain that the best works have been burned, that
the sweetest verses have never been printed . . . whereas
the shameless, the impertinent, the pre-eminent show off
with insolence in the sun of publicity without any right to
do so.”14
Ménière would have been the last to predict that his
work would lead to lasting fame, by being linked to the
condition which he described.11
Conflict of interest statement
We declare that we have no conflict of interest.
References
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2 Committee on Hearing and Equilibrium. Meniere’s disease: criteria
for diagnosis and evaluation of therapy for reporting. AAO-HNS Bull
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3 Committee on Hearing and Equilibrium. Meniere’s disease: criteria
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4 James AL, Thorp MA. Meniere’s disease. Clin Evid 2003; 9: 565–73.
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effect of endolymphatic sac surgery in treatment of Ménière’s
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syndrome. Cochrane Database Syst Rev 2001; 1: CD001873.
9 Silverstein H, Lewis WB, Jackson LE, Rosenberg SI. Changing trends
in the surgical treatment of Meniere’s disease: results of a 10-year
survey. Ear Nose Throat J 2003; 82: 185–94.
10 Morrison AW. Prosper Ménière (1799–1862). A synopsis of his life
and times. Ear Nose Throat J 1997; 76: 626–31.
11 Baloh RW. Prosper Ménière and his disease. Arch Neurol 2001; 58:
1151–56.
12 Beasley NJ, Jones NS. Ménière’s disease: evolution of a definition.
J Laryngol Otol 1996; 110: 1107–13.
13 McBride P, James A. Epilepsy, vertigo and ear disease.
Edinburgh Med J 1880; 25: 702–08.
14 Pappas DG, McGuinn M. Unpublished letters of Prosper Ménière: a
personal silhouette. Am J Otol 1993; 14: 318–25.
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Figure 2: Prosper Ménière (1799–1862)
The only portrait of Prosper Ménière (circa 1833) by Guill Bodiner, hung in the
Paris Institute for over a century, until its disappearance some time ago. It has
not been recovered.