Meniere's disease is a disorder characterized by spontaneous attacks of vertigo accompanied by fluctuating sensorineural hearing loss, tinnitus, and aural fullness. It is caused by endolymphatic hydrops, which results from overproduction or malabsorption of endolymph fluid in the inner ear. Attacks typically involve three phases - an initial irritative phase with nystagmus beating towards the affected ear, followed by a paretic phase with nystagmus beating away from the affected ear, and then a recovery phase with nystagmus back towards the affected ear. Diagnosis involves documented hearing loss and attacks of vertigo lasting at least 20 minutes according to established criteria. Treatment aims to
1. Meniere’s disease(sbo-3)
Meniere’s disease is a disorder characterized by spontaneous attacks of vertigo ,with associated
fluctuating sensorineural hearing loss ,tinnitus &aural fullness .It is a common cause of the syndrome
of spontaneous vertigo.
Aetiology Meniere’s disease is said to be idiopathic,although a variety of predisposing factor have
been identified.Overproduction or malabsortion of endolymph resulting endolymphatic
hypertension ,in tern endolymphatic hydrops.
Many different inner ear disease disease mumps,csom,trauma cogan syndrome,autoimmunity to the
inner ear antigens.
Pathophysiology:Periodic ruptures of the membranous labyrinth which result in leakage of the
potassium-rich endolymph into perilymph are thought to be responsible for producing meniere’s
attacks.
Initial excitation of the hair cells as a result of the increased potassium concentration,
Subsequent paretic phase is thought blockade of neurotransmitter release.
Healing of the rupture allows restitution of the chemical composition of the endolymph &
perilymph resulting in termination of the attack& improvement in vestibular & auditory function.
The gradual deteriotion in inner ear function result from the effects of repeated ruptures.
Clinical manifestations
Recurrent attacks of spontaneous vertigo with nausea vomiting,attack are herald by lateralized low
frequency hearing loss,tinnitus &aural fullness.
Following onset ,the vertigo typically increases in intensity over a perioid of minutes then usually
lasts for several hours.The hearing impairment &tinnitus may persist for days.Patient may have
postural imbalance for several days.
Typical attack has three phase;Irritative phase; nystagmus horizontal,beat towards the affected ear.
Paretic phase; nystagmus beats away from the affected ear,last for several hours.Recovery phase
;nystagmus towards again affected side.
In the early stage of the disease ,most patients are well between the attacks.As the disease
progress ,patients may have persistent hearing loss,tinnitus&postural imbalance.
Later stage of disease,some patients develop drop attacks(tumarkin or otolithic crises).The patient
drops to ground without warning(patient experience some external force pushed to the ground.)
2. Diagnosis of meneire’s disease:
American Academy of Otolaryngology –head & neck surgery has published diagnostic guidelines for
Meneire’s disease.
Definite Meneire’s disease;two or more spontaneous attack of vertigo(each lasting 20minute or
Longer)Hearing loss documented by PTA at least one occasion,tinnitus or aural fullness on the
affected side.
Probable Meneire’s disease;two or more spontaneous attack of vertigo with unilateral loss,tinnitus,
Or aural fullness all at same time.
A possible meneire’s disease;two or more spontaneous attack of vertigo,no auditory symptom
during attack.
In early stages of Meniere’s disease,vestibular &auditory function tests are often completely normal
between attacks.A fluctuating low-frequency sensorineural hearing loss may be seen & is suggestive
of the diagnosis of the early stage of the disease. As the disease progresses,all frequencies are
affected resulting in a flat sensorineural hearing loss that is at least moderate in severity.
Electrocochleograpgy; most sensitive & specific for meniere’s disease when tone-burst &click stimuli
are used. Recorded transtympanically at the promontory. Giving 4gm of nacl for 3days prior to the
electrocochleogram may increase the sensitivity.
Management options: aim to decrease the production or accumulation of endolymph.Strict sodium
restriction (so that uninary sodium is less than 50mmol/day) &diuretic can be effective in reducing
the frequency of vertigo.
Surgery; Endolymphatic sac surgery,selective vestibular neurectomy.labyrinthectomy with cochlear
implantation.
Recently intra tympanic inj. Of gentamycin may be effective to control severe intractable vertigo in
most patients.
Outcomes &complications; the natural history of meniere’s disease is variable.Attacks may occur
days ,months ,even year apart, with little or no warning.
In most patients only one ear is affected in early stage of disease, unfortunately the second ear
involved in about half of the patients .
Treatment of meniere’s disease(p 3798)
The treatment of meniere’s disease should be preceded by diagnosis on the basis of strict criteria&
after exclusion of retrocochlear pathology.
3. 1)General measures:Level of anxiety correlates with the severity of symptoms in the patients with
Meniere’s disease,it is postulated that stress may contribute via autonomic mechanisms in the
genesis of endolymphatic hydrops.
Increase prevalence for both inhalant & food allergies in patients with meniere’s diseae& it has been
suggested that allergy immunotherapy & food elimination may be benefit in these cases.
2) Diet: to restrict salt intake to 1mg or 1.5 -2mg /day in order to address endolymphatic hydrops.
3)Drug therapy:Drug therapy for Meniere’s disease may include,
a)Acute vestibular symptoms suppression by anticholinergic ,antihistaminic,calcium channel
antagonist.
b)Drug that aim to influence endolymphatic hydrops which include diuretics & betahistine.
c)Suppression of immunological reactions with steroids or methotrexate.
d)Deastruction of the vestibular end organ by intratympanic injection of aminoglycosides.
Diuretics may be effective in the long term control of vertigo but not of cochlear symptoms.
Dyazide has been reported as effective in controlling vertigo.
Betahistine is a histaminic analogue with weak agonistic of both H1 & H2 ,moderate antagonistic
action of H3 histaminic receptors.
a) A reduction of the symmetric functioning of the vestibular end organ.
b) Improved microvascular circulation in the stria vascularis of the cochlear.
c) Inhibition of the activity in the vestibular nuclei.
d) Less alertness effects on alertness through cerebral H1 receptors.
Suppression of immunogical reaction:oral recommended treatments include high doses of
prednisolone for one month with slow tapering over 4 to 6 months.
Intratympanic injection of aminoglycoside.