Menier's Disease

MENIER'S DISEASE
  • Meniere’s disease (Endolymphatic hydrops) is a disorder of the inner ear.
  • Characterized by tetrad of symptoms
  1. Episodic vertigo
  2. Fluctuating sensorineural hearing loss (more severe in lower frequencies).
  • Meniere's disease is associated with Cochlear deafness .
  • Patients often have transient hearing loss in the affected ear during the vertigo episodes. With repeated episodes, the vertigo often improves, but the hearing loss can become permanent.
  • Permanent Hearing loss is the most common morbidity associated with Menier's Disease.
  1. A sensation of aural fullness
  2. Tinnitus (low- pitched and roaring)
  • Hyperacusis may be seen.
  • Hennebert’s sign is positive.(Also positive in Perilymph Fistula)
  • Tulio’s phenomenon is present .
  • Normal intact tympanic membrance with negative fistula test.
  • Lermoyez syndrome is a variant of Meniere's disease, where initially there is deafness and tinnitus, vertigo appears later when deafness improves.
CAUSE OF MENIER'S DISEASE
  • Meniere's disease is idiopathic, but it is believed to be linked to endolymphatic hydrops, an excess of fluid in the inner ear due to obstruction of Endolymphatic duct or too much endolymph secreted by stria vascularis.
  • Edolymphatic fluid bursts from its normal channels in the ear and flows into other areas, causing damage. This is called "hydrops."
  • The membranous labyrinth, a system of membranes in the ear, contains a fluid called endolymph.
  • The membranes can become dilated( there may be marked bulging of the Reissner’s membrane ) like a balloon when pressure increases and drainage is blocked.
  • The underlying cause of Meniere's disease is most often unknown, sometimes it can be attributed to allergy, vascular insult, physical trauma, syphilis, acoustic neuroma, viral insult or other causes.
INVESTIGATIONS IN MENIER'S DISEASE 1) Tuning fork tests
  • Tuning fork tests show sensorineural hearing loss :-
  1. Rinne test → Positive
  2. Weber test → Lateralized to better ear
  3. ABC test → Reduced in affected ear
2) Pure tone audiometry
  • There is sensorineural hearing loss with affection of lower frequencies in early stages and the curve is of rising type. When higher frequencies are involved, curve becomes flat or a falling type.
3) Speech audiometry
  • Discrimnation score is usually 55-85%.
4) Special audiometry tests
  1. Indicate cochlear lesion :-
  2. Recruitment test is positive
  3. SISI (short increment sensitivity index) score is better than 70%
  4. Tone decay test shows decay of less than 20 dB.
5) Electrocochleography
  • Normally, ratio of summating potential (SP) to action potential (AP) is 30%. In Meniere's disease, SP/AP ratio is greater than 30% and is Gold standard diagnostic test of Meniere's disease.
6) Caloric test
  • It shows reduced response on the affected side. Often, it reveals a canal paresis on the affected side (most common) but sometimes there is directional preponderance to healthy side or a combination of both canal paresis on the affected side and directional preponderance to opposite side.
7) Glycerol test
  • Glycerol reduces endolymphatic pressure due to its dehydrating property and thus causes an improvement in hearing.
8) Reverse glycerol test
  • Performed by using acetazolamide. Shows deterioration in the pure tone threshold and speech discrimination score.
9) BERA-
  • reduced latency of wave V
TREATMENT OF MENIER'S DISEASE
Medical treatment:

  • Salt restriction,a low caffeine diet,avoidance of nicotine.
  • Diuretics,labyrinthine sedative
  • Vasodilators
  • Streptomycin therapy is given in bilateral cases to induce the labyrinthine damage
Surgical treatment
  • Indication :Severe in capacitating vertigo or tinnitus or to prevent further deterioration of hearing
  • Intermittent low pressure pulse therapy (Meniett device therapy)
  • Decompression of endolymphatic sac and insertion of a shunt between the membranous labyrinth and subarachnoid space
  • Vestibular neurectomy.
  • Stellate ganglion block or cervical sympathectomy
  • Intratympanic gentamycin
  • Total transmastoid labyrinthectomy-which relieves vertigo in over 90 percent of cases.
  • Sacculotomy(Fick's operation and Cody Tack's procedure)
Exam Question
  • Meniere’s disease is characterized by Vertigo,tinnitus,sensation of aural fullness and fluctuating hearing loss. 
  • Endolymphatic Hydrops is seen in Menier's Disease.
  • In meniere’s disease there will be marked bulging of the reissner’s membrane.
  • Salt restricted diet is advised in Menier's Disease.
  • Permanent Hearing loss is the most common morbidity associated with Meniere's disease.
  • Menier's Disease and Perilymph fistula present with positive Hennebert sign.
  • Electrocochleography is the gold standard investigation for diagnosis of Menier's Disease.
  • Recruitment test is positive in Menier's Disease.
  • Meniere's disease is associated with Cochlear deafness .
  • Hyperacusis may be seen in Menier's Disease.
  • Glycerol test is used for diagnosis of Menier's Disease.
  • Fick's operation and Cody Tack's procedure is for Menier's Disease.
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