Professional Documents
Culture Documents
ENT
APPROACH TO A
MEDICINE ORTHO
PATIENT WITH VERTIGO
NEURO
NEUROLOGIST
SURGEON
QUIZ
APPROACH TO A PATIENT
WITH VERTIGO
BENIGN PAROXYSMAL
POSITIONAL VERTIGO
MENIERE’S DISEASE
QUIZ
Hello readers,
Welcome to the second issue of Volume 2 of our e newsletter.
Advisor
Dr. A.K. Agarwal
Editorial Board
Pooja Kataria
Sumit Mrig
Ankush Sayal
OSSEOUS LABYRINTH —The osseous labyrinth consists of three parts: the vestibule, semicircular canals, and cochlea.
THE SEMICIRCULAR CANALS: 3 in number- superior, horizontal and posterior. They contain the semicircular ducts. They
open into the vestibule through 5 openings. The anterior ends have a dilated portion called the ampulla which opens independ-
ently into the vestibule. The posterior ends, which are not dilated, of the superior and posterior SCC fuse together and then open
into the vestibule whereas the posterior end of the lateral SCC opens independently. The lateral canal of one ear is very nearly in
the same plane as that of the other; while the superior canal of one ear is nearly parallel to the posterior canal of the other.
MEMBRANOUS LABYRINTH :- The membranous labyrinth consists of the cochlear duct, the semicircular canals, the
endolymphatic sac and duct.
The membranous labyrinth is lodged within the bony cavities just described, and has the same general form as these; it is, how-
ever, considerably smaller, and is partly separated from the bony walls by a quantity of fluid, the perilymph. In certain places it
is fixed to the walls of the cavity. The membranous labyrinth contains fluid, the endolymph, and on its walls the ramifications of
the acoustic nerve are distributed.
Within the osseous vestibule the membranous labyrinth does not quite preserve the form of the bony cavity, but consists of two
membranous sacs, the utricle, and the saccule.
The utricle receives the openings of the 5 Semicircular canals. It is concerned with linear acceleration. The saccule also is con-
cerned with linear acceleration. Both have sensory epithelium which is called Macula.
The semicircular ducts correspond to the semicircular canals. They are concerned with angular acceleration and deceleration.
The cochlear duct lies between the scala tympani and scala vestibule, also known as the scala media.
The functions of the vestibular system are to sense angular acceleration, linear acceleration and to coordinate head and eye move-
ments as well as maintain the antigravity and lower body muscles in relation to the head. The semicircular canals provide sensa-
tion for angular acceleration. The membranous labyrinth moves with head motion while the endolymph does not, causing a rela-
tive flow of endolymph and deflection of the cupula. Each hair cell has a resting potential and its associated neuron has a sponta-
neous discharge. Movement will cause an increase in the discharge rate on one side and a decrease from the paired canal on the
opposite side.
The otolith organs are sensitive to linear acceleration. Movement of the otoconia mass deflects the hair cells attached to it. The
maculae have hair cells oriented in many directions so linear acceleration in any direction may be sensed. The saccule is most sen-
sitive to gravity since it is in the vertical plane.
The reflexes involving the vestibular system include the vestibulo-ocular reflex and the vestibulospinal reflex. The vestibule-
ocular reflex helps maintain fixation of the eyes on an object with movement of the head. Both angular and linear acceleration sig-
nals are use in the vestibule-ocular reflex. Projections from the vestibular nuclei to the extraocular muscle nuclei allow for eye
movements that counteract head movements for gaze stabilization. The vestibulospinal reflex allows for input from the vestibular
organs to be use for posture and stability in a gravity environment. The projections from the vestibular nuclei travel to antigravity
muscles for coordinated movements to maintain posture.
Figure 1 and 2 depicts the various supranuclear oculomotor centres involved in maintainance of Vesticulo-ocular re-
flex. The abbreviations have been detailed below along with the various functions these regions perform.
Figure 1:
It has been observed that the ratio of central to peripheral vertigo is greater than 50% and in more than 50% cases of central cause of
vertigo, associated symptoms such as cerebellar signs are absent.
The following are clinical tests that need to be performed in addition to the routine ENT examination to differentiate between central
and peripheral vertigo.
COVER- UNCOVER TEST: This is to test malalignments of the visual axis. The prerequisite for all of these tests is foveal
fixation. The unilateral cover test reveals heterotropia (manifest strabismus), i.e. a malalignment of the visual axis when
both eyes look at a single target. First the patient has to fixate either a near target (at a distance of 30 to 40 cm e.g. exam-
iner’s nose) or one 5 to 6 mts away. Then the examiner covers one eye and looks for movements of the uncovered eye
(correction movements). If the uncovered eye moves from the inside outward, esotropia is present; if it moves from the out-
side inward, exotropia; if it moves from above downward, hypertropia; if it moves below upwards, hypotropia. The other eye
is then examined.
Check for spontaneous nystagmus: Ask the patient to fix eye over an object 25- 30cm away and check nystagmus in the primary
position. This allows us to rule out horizontal, vertical (upbeat/downbeat) and torsional nystagmus. Remember nystagmus gets
suppressed by fixation in peripheral vestibular dysfunction and remains stationary or increases in central vestibular dysfunc-
tion; a congenital nystagmus beats as a rule, horizontally at various frequencies and amplitudes and increases during fixation
After checking for possible eye movements in primary position and the misalignment of the axis of the eyes (cover test), the exam-
iner should then establish the range of eye movements monocularly and binocularly in the nine end positions; deficits found
here can indicate, e.g., ocular muscle or nerve palsy. Gaze holding deficits can also be determined by examining eccentric gaze
position.
When the eye is taken to the end positions, look for ability of gaze holding and any gaze evoked nystagmus. Horizontal gaze
evoked nystagmus can indicate a structural lesion in the area of brainstem or cerebellum (flocculus, NPH, Vestibular nucleus).
Vertical gaze evoked nystagmus is observed in lesions of the INC. Most important cause of gaze evoked nystagmus in all di-
rections is medications (e.g., phenytoin, tegretal) and alcohol intoxication. Remember that down beat nystagmus is most
prominent in the lateral gaze.
Look for any deficits in convergence reaction i.e., when an object is moved from a distance of 50cm towards the patients eye, this
causes vergence, accommodation and miosis. The neurons important for this lie in the mesencephalic reticular formation and
oculomotor nucleus. Lesions of rostral midbrain lesions and tumors of pineal region and thalamus lead to abnormal conver-
gence reaction. Convergence retraction nystagmus is seen with lesions of posterior commissure.
The above mentioned clinical examination for spontaneous nystagmus, range of eye movements, gaze evoked nystagmus can be
performed using frenzel’s glasses. These 20 diopters glasses prevent visual fixation. Remember spontaneous nystagmus indi-
cates a tone imbalance of the vestibule-ocular reflex; if it is caused by a peripheral lesion e.g. vestibular neuritis, the nystagmus
is typically damped by visual fixation.Head shaking nystagmus shows a latent asymmetry of the so called velocity storage,
which can be due to peripheral and central vestibular disorders.
In summary, 4 basic tests that have been found to be helpful in differentiating central from peripheral vertigo include:
Gaze evoked nystagmus
Skew deviation
Head impulse test
Saccadic smooth pursuit
Remember that any patient presenting with acute onset nystagmus with normal head impulse test is most likely due to central lesion.
Author details:
Dr. Tripti Brar, Senior Resident, Department of ENT & Head and Neck surgery, Maulana Azad Medical
College, Lok Nayak Hospital & Associated Hospitals.
Dr. Ankush Sayal, Senior Resident, Department of ENT & Head and Neck surgery, Maulana Azad Medi-
cal College, Lok Nayak Hospital & Associated Hospitals.
DIZZINESS
RECURRENT COUNTINOUS
SINGLE IMBALANCE
ATTACKS
EPISODE
NO NO CO- NO CO-
1.
COCHLEAR COCHLEAR CHLEAR CHLEAR
COCHLEAR
SYMP- SYMP- SYMP- SYMP-
SYMP-
TOMS TOMS TOMS
TOMS TOMS
2. 4. 5. 6. 7. 8.
NO ASSOC NO ASSOC ASSOC. NO ASSOC ASSOC NO ASSOC
SYMP- SYMP- SYMP- SYMP- SYMP- SYMP-
TOMS TOMS TOMS TOMS TOMS TOMS
3. 9.
ASSOC ASSOC
SYMP- SYMP-
TOMS TOMS
ENT
MEDICINE ORTHO
NEURO
NEUROLOGIST
SURGEON
Patients of vertigo are generally first seen by a general practioner and are referred from
one speciality to other without making any diagnosis.
Vertigo
A sense of feeling the environment moving when it does not. Persists in all positions.
Aggravated by head movement.
Dysequilibrium
A feeling of unsteadiness or insecurity without rotation. Standing and walking are
difficult.
Giddiness
Swimming, floating, giddy or swaying sensation in the head or in the room.
Unsteadiness
Sense of imbalance
Visual
Proprioceptive
Vestibular
CNS
Efferent
Dizziness Oculomotor
Sceletal muscles
Vegetative
Any mismatch between the afferent & the efferent causes vertigo.
Pathophysiology
Balance requires –
Normal functioning vestibular system
Input from visual system (vestibulo-ocular)
Input from proprioceptive system (vestibulo-spinal)
Disruption of balance between inputs results in :
vertigo (acute)
disequilibrium (chronic)
DISORDERS OF BALANCE
Differential diagnosis
Onset of symptoms
Chronologically
Periods of complete freedom
Speed of both onset and resolution
Ensure on same wavelength
Protocol for evaluation of vertigo patient includes clinical examination and battery
of tests :
Examination
♦ Ears
♦ Hearing
♦ Nystagmus
♦ Balance
♦ BP
♦ Positional testing
♦ Neurological examination
(iii) Haematology
a. FBC + ESR
b. VDRL/TPHA
(iv) Radiology
a. Mastoid X-rays
b. Internal auditory meatus X-rays
c.CTscan/MR scan
Apart from routine examination of the ear few bed side clinical tests helps in reaching to
a diagnosis of vertigo
HEAD SHAKE TEST
Head-shaking nystagmus (HSN) is a jerk nystagmus that may follow a prolonged sinusoidal head oscillation. It is not
new -- it was first described in 1907 by Robert Bárány (1907). Moritz called it "kopfschutteinnystagmus" (1951). Subse-
quent studies (Borries 1923; Klestadt 1936; Vogel 1929) described different techniques of eliciting the nystagmus and
advanced hypotheses regarding its pathophysiology (Fetter et al. 1990; Hain and Spindler 1993; Halmagyi and Curthoys
1988; Katsarkas et al. 2000; Minagar et al. 2001; Perez et al. 2004)
It is most commonly elicited in the following way:
The patient is positioned upright and instrumented so that fixation is removed but horizontal and vertical eye movements
can be observed. The test is best performed using video Frenzel goggles. The test cannot be done without a method
of eliminating fixation.
Eye movements are observed in darkness for 10 seconds to obtain a baseline. Next, the examiner grasps the patient’s
head and moves it briskly back and forth in the yaw plane (around the vertical axis) , aiming for a frequency of about 2
Hz and a displacement of the head of approximately 30 degrees to either side. Ideally, the head should be pitched about
20 degrees downward with respect to vertical so that the axis of rotation is close to being parallel to the axes of the lat-
eral canals, but practically the results of the test are not sensitive to this procedure. The head-shaking is continued for
20 cycles and then abruptly stopped.
In normal subjects or persons with symmetrical vestibular loss (such as bilateral vestibular loss), no nystagmus is ex-
pected. In persons with a dynamic imbalance between the ears (such as due to unilateral vestibular neuritis or an
acoustic neuroma), a nystagmus is often seen (usually beating towards the “better” ear (Hain et al. 1987; Katsarkas et al.
2000)) which decays over about 30 seconds. This is referred to as the first phase of nystagmus, because in some cases
it is followed by a second phase of nystagmus that is weaker, decays more slowly, and is directed towards the “bad” ear.
The main value of seeing a secondary phase is that one can clearly identify the primary phase (sometimes the primary
nystagmus is very short). Rarely, horizontal head-shaking produces a non-horizontal nystagmus, such as vertical nystag-
mus (Wu et al. 2005) or torsional nystagmus (Califano et al. 2001). This is called a “perverted” head-shaking nystagmus.
A vibratory stimulus has been applied not only to the mastoids but also to the vertex and neck 1,2. In 1999, Hamann and
Schuster 3 found that the sensitivity of the test was increased using a frequency of 60 Hz instead of 100 Hz. In 2004, Du-
mas et al. 7reported complete concordance between MVT, the head shaking test (HST) and the caloric test for detecting
the side with total vestibular loss. It was also suggested that MVT elicits vibratory nystagmus (VN) in healthy subjects 2 5.
It is not clear how mastoid vibration causes nystagmus in patients with peripheral vestibular deficit. Vibration has been
shown to excite semicircular canals and otolithic afferents in various animal species6-8 and in humans 1 3 9,10, indicating
that the effects on eye movement can be attributed to direct stimulation of intact vestibular receptors. Kalberg et al., in
particular, showed that vibration to the mastoid bone induces eye movements similar to those seen acutely, without vi-
bration, after unilateral vestibular loss and that the eye rotation axis of vibration-induced nystagmus is related to the ex-
tent of the unilateral vestibular deficit 9. The vibratory test, according to some authors, is not unlike the simultaneous ca-
loric test 4. Vibration also seems to increase afferent activity from stimulated muscles 11, suggesting that the effects may
be due to interaction between neck proprioceptors and the vestibular system 12,13
The aim of the present study was to determine the sensitivity and specificity of MVT in patients with damage caused by
vestibular neuritis, comparing results with those of the caloric test, the current gold standard for identifying unilateral defi-
cit in vestibular function. MVT was also compared with the head shaking test (HST) and with the head thrust test (HTT),
other conventional bedside tests used in the detection of vestibular asymmetry.
Apart from bed side clinical test which help in diagnosing cause of vertigo, balance regulation of the body can be evalu-
ated with the help of CDP.
It is generally classified into static & dynamic posturogaphy.
Computerized dynamic posturography (CDP) is an established test of postural stability. While ENG and rotation testing
assess visual-vestibular interactions, CDP provides information about motor control or balance function under varying
environmental conditions.
The ability to maintain balance depends not only on vision and the vestibular system, but also on information that the
brain receives from the muscles and joints which provide clues such as the direction of head turn and the texture and
slope of the walking surface.
CDP tests the relationships among all balance system components--eyes, somatosensory system, and vestibular
system. It measures the person's response to environments in which the amount of reliable information from the eyes
and somatosensory system is varied.
Posturography gives information about how well balance is maintained during challenging situations. It can help doctors
plan other vestibular testing, as well as assist in treatment design.
ROLE OF ENG
It helps in documenting the type of nystagmus with effect of maneuvers on the nystagmus.
The eyes are closely linked to the inner ear; these organs depend on each other for good balance and clear vision. Head
movement or other stimulation of the inner ear sends signals to the muscles of the eyes via the nervous system; this is
called thevestibulo-ocular reflex, or VOR.
The VOR normally generates eye movements that maintain clear vision with head move-
ment. Electronystagmography (ENG) is a battery (group) of eye-movement tests that look for signs of vestibular dys-
function or neurological problems by measuring nystagmus (a type of involuntary eye movements). ENG tests are the
most common ones administered to people with dizziness, vertigo, and/or balance disorders, although the test battery
and some testing methods vary widely.
During ENG, eye movements are recorded and analyzed via small electrodes placed on the skin around the eyes. The
electrodes attach to the skin with an adhesive, much like a small bandage. Alternatively, eye movements may be re-
corded by videonystagmography (VNG) using an infrared video camera mounted inside goggles that the patient wears
instead of sticky-patch electrodes.
One ENG test evaluates the movement of the eyes as they follow a moving target. Another observes eye movements as
the head is positioned in different directions.
During the caloric test warm or cold water or air is circulated in the ear canal. The temperature change stimulates the
inner ear in order to test the nystagmus response.
PRINCIPLES OF TREATMENT:
Medical treatment is advisable only in acute cases of vertigo with emphasis on rehabilitation exercises once the acute
crises is over.
Lot of vestibular suppressants are available in the market , but caution – not to add two or more vestibular suppres-
sants together as they not only make the patient more dizzy but also delay brain compensation.
Diazepam is the best vestibular suppressant & is used when other drugs fail to control vertigo.
Stemetil should not be given for more than a wk as it delays brain compensation.
Author details:
Dr. Sumit Mrig. Senior Consultant, Department of ENT & Cochlear Impalnt Surgery, Primus Super-
speciality Hospital, New Delhi.
BPPV can appear at any time from childhood to senility, but at least the idiopathic form is typically a disease of old age.
Male to female ratio is 1:1. Although the most common cause is head trauma but BPPV also occurs after excessive bed
rest in connection with other illnesses or after operation. About 10% of the spontaneous cases and 20% of the trauma
cases show bilateral BPPV. It is called benign because it resolves spontaneously within weeks to months, in some
cases, however it can last for years.
PATHOPHYSIOLOGY
According to the histologically based cupulolithiasis model of Schuknecht (1969), heavy, anorganic particles (Otoconia)
of specific weight, which become detached as a result of trauma or spontaneous degeneration from the utricular otoliths
of the cupula, settle in the underlying ampulla of the posterior canal. Whereas the cupula normally has the same specific
weight as the endolymph, it is heavier with these particles (heavy cupula), i.e., the canal is transformed from a sensor of
rotatory acceleration into a transducer of linear or angular acceleration. Despite its widespread acceptance, this theory
failed to explain many of the typical criteria of nystagmus in cases of positional vertigo. Various studies have shown that
when a heavy cupula is created by alcohol or deuterium ingestion, the resulting nystagmus, in a particular head position,
is sustained for a longer period of time, rather than transient as in the classical nystagmus.
Based on the observation by Pames And McClure, Epley, in 1992, proposed the canalolithiasis hypothesis which could
explain all symptoms of positioning nystagmus. According to this hypothesis, otoconia or canaliths float freely within the
endolymph of the semicircular canal instead of being firmly attached to the cupula and the heavy conglomerate, which
almost fills the canal, is assumed to be the cause of the positioning vertigo. The movement of the conglomerate causes
either an ampullofugal or ampullopetal deflection of the endolymph depending on the direction of the sedimentation.
CLINICAL PRESENTATION
The main symptoms of BPPV include severe attacks of rotatory vertigo with or without nausea, which are caused by
rapid changes in head position relative to gravity. Typical triggers include lying down or sitting up in bed, turning around
in bed and also bending over to tie shoe laces or extending the head in order to look up or do something above the
head. They frequently occur in the morning and are most pronounced during the first change in position after sleep; re-
peated changes in position cause a transient lessening of the attacks.
BPPV is elicited by extending the head or positioning the head or body toward the effected ear. Rotatory vertigo and nys-
tagmus occur after such positioning with a short latency of seconds in form of a crescendo/decrescendo course of maxi-
mally 30-60 seconds. The beating direction of nystagmus depends on the direction of gaze; it is rotatory when the gaze
is to the undermost ear and mostly vertical (to the forhead) during gaze to the uppermost ear. The nystagmus corre-
sponds to the ampullofugal excitation of the posterior canal of the undermost ear.
TREATMENT
When correctly performed, all three physical liberatory maneuvers (Simont or Epley liberatory maneuvers, Brandt-Daroff
exercises) are successful in almost all patients (Figure 1, 2 and 3 respectively).
Simont’s maneuver is quickly performed in three steps with the aid of a therapist as the patient lies on the examination
couch. It is important that the head of the sitting patient is turned by 45 degrees to the healthy ear, in order to put the re-
sponsible canal into a position parallel to the movement during the positioning. Relief is thus achieved in about 50% of
the cases with one single maneuver. Epley’s liberatory maneuver requires that the patient’s head and trunk be rotated
after being tilted backward into slightly head-holding position.
Fig 1. Schematic drawing of the Simont’s maneuver of a patient with right BPPV. (1) In the sitting position, the head
is turned horizontally 45 degree to the unaffected ear. (2) The patient is made to lie down on the side of affected ear
first with the nose pointing upwards. The patient maintains this position for 3 minutes. (3) The Patient is made to lie
down on the side of unaffected ear with nose pointing downwards and maintain this for 3 minutes, causing the clot to
exit the canal. The patient is then slowly moved into sitting position; this causes the clot to move into the utricular
cavity.
2. SURGERY
In patients refractory to liberatory meneuvers, singular neurectomy can be offered. Such selective neurectomy is diffi-
cult to perform and carries a risk of permanent hearing disorder. Neurectomy has been replaced by plugging of the
posterior canal. It is safer and more effective than nerve sectioning.
3. ANTIVERTIGINOUS THERAPY
Drug treatment of BPPV with antivertiginous substances is neither possible nor sufficiently effective long term against
the symptoms, because of the pathomechanism of the disorder. Patients who suffer from severe nausea after a sin-
gle repositioning maneuver can be prescribed dimenhydrinate (100mg) half an hour before performing the liberatory
maneuver.
BPPV of the horizontal canal is less frequent than posterior BPPV but is still diagnosed too seldom. Its cardinal features
are:
It can be induced by turning the head along the longitudinal axis of the supine body (either to the right or the left).
This results in an ampullopetal deflection of the cupula (with more severe vertigo and nystagmus) when the head
is turned to the side of the affected ear.
The nystagmus beats linear and horizontal to the undermost ear.
Repeated positioning maneuvers cause little fatigue of the positioning nystagmus.
The duration of the attacks and the nystagmus is longer because of the so called central storage mechanism of ve-
locity in the horizontal canal.
The striking feature of horizontal BPPV, i.e., it does not fatigue, agrees with the assumption as does the general experi-
ence that horizontal BPPV is difficult to treat by a single positioning maneuver.
1. Forced prolonged position on the healthy side: Vannuchi et al have adviced patients with h-BPPV to lie down on
the healthy side for 12 hours to allow otolithic debris to gravitate to the vestibule, by maintaining the affected side
uppermost.
2. 270 degree barbecue maneuver: this consists of turning the patients head and body from the supine position by
three 90 degree step rotations (total 270) towards the unaffected side to assume the lying position on the affected
side and then sit up (Fig 4).
3. 360 degree yaw rotation: Baloh proposed that treatment of h-BPPV can be achieved by this maneuver in which
the patients head and body is rotated by 360 degrees in rapid 90steps and towards the unaffected ear. The time
interval between each step is 30 seconds or until nystagmus subsides.
4. Liberatory maneuvers have been described in which the patient lies supine with head lifted by 30 degrees, turned
to the affected side and maintained in that position for five minutes. The head is then turned as quickly as possi-
ble 180 degrees to the other side and maintained there for five minutes. After the maneuver the patient is asked
to avoid head shaking and not to lie down for the next 48 hours.
This is rare and occurs as a consequence of treatment of posterior canal BPPV by single step maneuvers. Brandt Daroff
exercise have been found to be effective in a- BPPV.
Barany R, Cited by Dix R, Hallpike CS. Diagnose von krankheitserscheinungen im bereiche des otolithenapparatus.
Acta otolaryngol 1921; 2: 434-7.
Dix R, Hallpike CS. The pathology, symptamatology, and prognosis of certain common disorders of the vestibular sys-
tem. Proc R Soc Med 1952;54:341-54.
Schuknecht HF (1969) Cupulolithiasis. Arch Otolaryngol 90: 765-778.
Parnes ES, McClure JA (1991) Posterior semicircular canal occlusion in normal hearing ear. Otolaryngol Head Neck
Surg 104: 52-57
Epley JM (1992) The canalith repositioning procedure: For treatment of benign paroxysmal positioning vertigo. Oto-
laryngol Head Neck Surg 10: 299-304.
Brandt T, Daroff RB (1980) Physical therapy for benign paroxysmal positional vertigo. Arch Otolaryngol 106: 484-485.
Brandt T, Steddin S, Daroff R.B. (1994) Therapy for benign paroxysmal positioning vertigo, revisited. Neurology 44.
796-800.
Brandt T, Steddin S (1993) Current view of the mechanism of benign paroxysmal positioning vertigo: Cupulolithiasis or
canalolithiasis? J Vestib Res 3: 373-382.
Levat E, van Melle G, Monnier P, Maire R (2003) Efficacy of the Semont maneuver in benign paroxysmal positional
vertigo. Arch Otolaryngol Head Neck Surg 129: 629-633.
Semont A, Freyss G, Vitte E (1988) Curing the BPPV with a liberatory manoevre. Adv Otorhinolaryngol 42: 290-293.
Herdman SJ, Tusa RJ, Zee DS, Proctor LR, Mattox BE (1993) Single treatment approaches to benign paroxysmal ver-
tigo. Arch Otolaryngol Head Neck Surg 119: 450-454.
Gacek RR (1978) Further observations on posterior ampullary nerve transection for positional vertigo. Ann Otol Rhinol
Laryngol 87: 300-306.
Baloh RW, Jacobson K, Honrubia V (1993) Horizontal semicircular canal variant of benign positional vertigo. Neurology
43: 2542 – 2549.
Lempert Th, Tiel-Wilck K (1996) A positional maneuver for treatment of horizontal-canal benign positional vertigo, La-
ryngoscope 106: 476-478.
Bisdorff AR, Debatisse D (2001) Localizing signs in positional vertigo due to lateral canal cupulolithiasis. Neurology 57:
1085-1088.
Vanucchi P, Giannoni B, Pagnini P (1997) Treatment of horizontal semicircular canal benign paroxysmal positional ver-
tigo. J Vestib Res 7: 1-6.
Author details:
Dr. Ankush Sayal, Senior Resident, Department of ENT & Head and Neck surgery, Maulana Azad Medi-
cal College, Lok Nayak Hospital & Associated Hospitals.
Etiology
• Multiple causes of dysfunction of the vestibular system are known.
• No single entity is known to be responsible for Ménière's disease. It is currently thought to be due to overaccumulation of endo-
lymph in the cochlear duct.
Ménière's disease must be distinguished from other causes of endolymphatic hydrops such as post-traumatic, post-infectious, oto-
syphilis, and Cogan's syndrome (interstitial keratitis).20
An autoimmune etiology has been postulated after it was found that there was an association with presence of thyroid autoantibodies
in patients with Ménière's disease.21,22
GLOBAL VIEW
Reported prevalence of Ménière's disease varies widely, from 15 per 100,000 in the United States to 157 per 100,000 in the United
Kingdom.3 This difference in prevalence based on geographic area is likely due to reporting biases and not geographic patterns of
disease.
Bilateral disease is found in 10% of patients with Ménière's disease at initial diagnosis but, with disease progression, it may increase
to over 40%.9
A genetic predisposition appears to exist.10
A familial predisposition seems to exist; approximately one half of all patients have a family history of the disease.
Migraine-Associated Vertigo
Vertigo
ο Vertigo is a subjective sensation of motion while motionless.
At least 2 definitive episodes of vertigo of at least 20 minutes duration must have occurred to make the diagnosis.14
ο Duration is usually several hours long.
Horizontal or rotatory nystagmus is always present during attacks of vertigo.14
ο Symptoms are often accompanied with nausea, vomiting, and anxiety.
Acute attacks may be accompanied with sudden falls without loss of consciousness. These are termed crises of Tumarkin or drop
attacks.15 Most studies find the incidence of drop attacks to be less than 10%. In one case series, self-reporting of drop attacks was
72% among patients with diagnosis of Ménière's disease.16
In patients with the symptom of vertigo, Ménière's disease is the cause in 10%.17
Hearing loss
ο Sensorineural hearing loss must be documented audiometrically in the affected ear at least once during the course of the disease.
ο There may be fluctuation in the degree of hearing loss superimposed on a gradual decrement in function.
ο Hearing loss affects low frequencies initially but at a later stage flattening of the curve is seen .
PTA
ECOG NIHL
Diuretics
Medications with diuretic-like properties (eg, hydrochlorothiazide and triamterene , hydrochlorothiazide , acetazolamide
[Diamox], methazolamide ) decrease fluid pressure in the inner ear. These medications help prevent attacks but do not help
after the attack is triggered.
Diamox can be started as 250 mg once a day after baseline electrolyte are done and repeated every 2 wks to keep a watch on the
potassium level.
Diuretics help best in reducing aural fullness than relieving tinnitus or improving hearing loss
Intravenous fluid support can also help prevent dehydration and replace electrolytes.
Steroids
They have also been helpful in treating endolymphatic hydrops because of their anti-inflammatory properties.
Steroids can reverse vertigo, tinnitus, and hearing loss, probably by reducing endolymphatic pressure.
Steroids can be given orally, intramuscularly, or even transtympanically. Although the transtympanic route is controversial, it is
gaining wider acceptance throughout the otologic community.
Oral prednisolone started 1mg/kg after breakfast for a period of 2 wks→ 0.75 mg/kg for 1 wk →0.5mg/kg for 1 wk →0.25mg/kg
for 1 wk .
Before starting patients on steroids they must be evaluated for hypertension & diabetic status.
Aminoglycosides
They are a class of antibiotics that were serendipitously discovered to be preferentially toxic to the vestibular (balance) end or-
gan.
Destruction of the vestibular end organ renders the brain insensitive to fluctuations in inner ear pressure brought on by Ménière
disease. Given systemically, aminoglycosides affect both ears.
Although aminoglycosides can be used to treat extremely severe bilateral Ménière disease, such treatment leaves the patient with
little or no balance function. The resulting complete loss of inner ear function (ie, Dandy syndrome) can be debilitating.
The Meniett device delivers pulses of pressure to the inner ear via the tympanostomy tube. Although no one knows exactly why
this works, some patients have symptomatic relief when the device is used on a daily basis. Because it is new, long-term results have
not been fully evaluated.
Histamine agonists
Medications such as betahistine hydrochloride are widely used in Europe and South America. Although its mechanism of action is
somewhat controversial, many have reported success with its use in mitigating symptoms of Ménière disease. Unfortunately, since
betahistine is not US Food and Drug Administration approved, it is not discussed much in the United States.
A new molecule Betahistine mesilate 6/12mg has been used & the dosage is less as compared to betahistine hydrochloride with
more potency. Most of the studies are from Japan with no Indian studies are available .
Betahistine hydrochloride 16mg tid or 24 mg bid is the therapeutic dose for patients with vertigo and gradually reduced to bid or od
dose once the symptoms starts improving.
BASIC AIM IS NOT TO MAKE PATIENT DEPENDENT ON VESTIBULAR SUPPRESENT DRUGS BUT TO ENCOURAGE
THEM FOR REHABILITATION EXERCISES .
LATEST GUIDELINES
RECENT GUIDELINES IN MENIERE’S MEDICAL TREATMENT :
1. Betahistine Hydrochloride – 48mg three times a day. Even upto 480-540 mg can be given.
2. Treatment for Meniere’s disease is required upto a period of 3 – 4 months & majority of the cases of poor response to betahistine is because
of inadequate treatment .
Role of Prochlorperazine
Known by the name of stemetil , it is used in acute crisis to control vomiting but should not be used round the clock for more than 5
– 7 days as it delays brain compensation.
THE BEST VESTIBULAR SUPPRESENT IS diazepam, given when other drugs fail to control vertigo.
Surgical management
Indications
• Surgery is indicated for Ménière disease that is refractory to medical management. Typically, failure to respond to 3-6
months of medical therapy is an indication of surgery. However, patients with severe debility may undergo surgery sooner.
• Any underlying medical causes for Ménière disease should be treated before surgical therapy is undertaken. The diseased ear
must be clearly identified.
Historically, multitudes of clever surgical procedures have been invented, tested, and discarded. This article covers the 4 most
generally accepted management options:
♦ Endolymphatic sac decompression or shunt placement,
♦ Transtympanic medication perfusion,
♦ Vestibular nerve sectioning,
♦ Labyrinthectomy.
Labyrinthectomy
Labyrinthectomy has the advantage of a high cure rate (>95%) and is useful in patients in whom Ménière disease has destroyed
their hearing on the affected side.
Labyrinthectomy involves ablation of the diseased inner-ear organs but does not require entry into the cranial cavity. Therefore, it
is less complex than vestibular nerve sectioning.
Labyrinthectomy can be accomplished through 2 approaches: transcanal and basic mastoidectomy.
Transcanal approach
It is done through the external ear canal. First, a tympanomeatal flap is elevated. Next, a right angle pick is inserted through the
oval window and maneuvered to disrupt and scramble the nerve tissues of the labyrinth.
Sometimes, a drill is used to connect the round and oval windows to improve exposure to the neuroepithelium.
Mastoidectomy approach
Extension of the mastoidectomy by drilling through the semicircular canals allows for more complete ablation of the labyrinthine
neuroepithelium than does the transcanal approach.
Labyrinthectomy is a bit less invasive than vestibular nerve sectioning. Craniotomy is not required; therefore, the risk of CSF leakage
and meningitis is reduced. Patients typically require a few days of inpatient care. Adaptation to the surgical loss of 1 vestibular appa-
ratus usually takes weeks to months. Vestibular rehabilitation during this period is also helpful.
References
1. Meniere P. Observations de maladies de I'oreille interne caracterisee par des symptomesde congestion apoplectiforme. Gaz Med
de Paris. 1861;16:379.
2. Meniere P. Maladies de l'oreille interne offrant les symptomes de la congestion cerebraleapoplectiforme. Gaz Med de
Paris. 1861;16:88.
3. Minor LB, Schessel DA, Carey JP. Ménière's disease. Curr Opin Neurol. Feb 2004;17(1):9-16.
4. Meniere P. Nouveaux documents relatifs aux lesions de l'oreille interne caracterisee pardes symptomes de congestion cerebrale
apoplectiforme. Gaz Med de Paris. 1861;16:239.
5. Sajjadi H, Paparella MM. Meniere's disease. Lancet. Aug 2 2008;372(9636):406-14.
6. Paparella MM. Pathogenesis and pathophysiology of Meniére's disease. Acta Otolaryngol Suppl. 1991;485:26-35.
7. Paparella MM, Djalilian HR. Etiology, pathophysiology of symptoms, and pathogenesis of Meniere's disease. Otolaryngol Clin
North Am. Jun 2002;35(3):529-45, vi.
8. Merchant SN, Adams JC, Nadol JB Jr. Pathophysiology of Meniere's syndrome: are symptoms caused by endolymphatic hy-
drops?. Otol Neurotol. Jan 2005;26(1):74-81.
9. Kitahara M. Bilateral aspects of Meniére's disease. Meniére's disease with bilateral fluctuant hearing loss. Acta Otolaryngol
Suppl. 1991;485:74-7.
10. Klockars T, Kentala E. Inheritance of Meniere's disease in the Finnish population. Arch Otolaryngol Head Neck
Surg. Jan 2007;133(1):73-7.
11. Stephens D, Pyykko I, Varpa K, Levo H, Poe D, Kentala E. Self-reported effects of Ménière's disease on the individual's life: a
qualitative analysis. Otol Neurotol. Feb 2010;31(2):335-8.
12. Morrison AW, Johnson KJ. Genetics (molecular biology) and Meniere's disease. Otolaryngol Clin North Am. Jun 2002;35(3):497-
516.
13. Mancini F, Catalani M, Carru M, Monti B. History of Meniere's disease and its clinical presentation. Otolaryngol Clin North
Am. Jun 2002;35(3):565-80.
14. Monsell EM. New and revised reporting guidelines from the Committee on Hearing and Equilibrium. American Academy of Otolar-
yngology-Head and Neck Surgery Foundation, Inc. Otolaryngol Head Neck Surg. Sep 1995;113(3):176-8.
15. Baloh RW, Jacobson K, Winder T. Intratympanic gentamicin for Meniere's disease: effect on quality of life as assessed by Glas-
gow benefit inventory. J Laryngol Otol. Oct 2006;120(10):827-31.
16. Kentala E, Havia M, Pyykko I. Short-lasting drop attacks in Meniere's disease. Otolaryngol Head Neck Surg. May 2001;124
(5):526-30.
17. Bhattacharyya N, Baugh RF, Orvidas L, Barrs D, Bronston LJ, Cass S. Clinical practice guideline: benign paroxysmal positional
vertigo. Otolaryngol Head Neck Surg. Nov 2008;139(5 Suppl 4):S47-81. .
18. White J. Benign paroxysmal positional vertigo: how to diagnose and quickly treat it. Cleve Clin J Med. Sep 2004;71(9):722-8. .
19. Bronstein A. Visual symptoms and vertigo. Neurol Clin. Aug 2005;23(3):705-13, v-vi.
20. Grasland A, Pouchot J, Hachulla E, Bletry O, Papo T, Vinceneux P. Typical and atypical Cogan's syndrome: 32 cases and review
of the literature. Rheumatology (Oxford). Aug 2004;43(8):1007-15. .
21. Fattori B, Nacci A, Dardano A, Dallan I, Grosso M, Traino C. Possible association between thyroid autoimmunity and Menière's
disease. Clin Exp Immunol. Apr 2008;152(1):28-32.
Nacci A, Dallan I, Monzani F, Dardano A, Migliorini P, Riente L. Elevated antithyroid peroxidase and antinuclear autoantibody titers in
Ménière's disease patients: more than a chance association?. Audiol Neurootol. 2010;15(1):1-6.
Author details:
Dr. Sumit Mrig. Senior Consultant, Department of ENT & Cochlear Impalnt Surgery, Primus Super-
speciality Hospital, New Delhi.
Q 3. 26 yr/m c/o vertigo with gait & postural with normal neurological examination .
vertigo attacks get worse in crowds & departmental store & improve in the night . the
patient is suffering from ?
a. B/l vestibulopathy
b. Cerebellar lesion
c. Phobic postural vertigo
d. Demyelinating disease.
http://enttrends.webs.com/
The opinions published in this publication are those of the contributors and do not
necessarily reflect the opinion or recommendations of the publishers. The publisher
assumes no liability for any injury and or damage to persons and property as a matter of
products liability, negligence or otherwise, or from use of operation of any methods,
products instructions or ideas contained in the material herein. No warranties, either ex-
press or implied, are made with respect to its accuracy, completeness, or timeliness. Due
to rapid advance in medical science, an independent verification of the methodologies and
references quoted here is strongly recommended.
………………………………………………………………………………………………………………………Publisher