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Introduction to Meniere's Disease

It is also called hydrops, endolymphatic hydrops. It is disease of inner ear, the main pathology is distension of endolymphatic system due to increase in volume of endolymph.
Atypical Meniere’s disease;
Is a term which has been suggested to describe patients who complain of some but not all of the
classical symptoms of the triad.
Variants of Meniere’s disease;
  1. Lermoyez syndrome (reversed symptoms)
    Sudden sensoruieural hearing loss which improve during or immediately after the attack of vertigo (Tinnitus, hearing loss vertigo)
  2. Tumarkin’s otolithic catastrophe
    Abrupt falling attacks of brief duration without loss of consciousness
  3. Cochlear Hydrops – only cochlear symptoms, no vertigo
  1. Female: Male —1:3
  2. Age of onset: 35-60 yrs (peak : 5"’ -6th decade)
  3. Familial tendency: 14-20 %
  4. Autosomal dominant
  5. Usually U/L
  1. Increased production of endolymph
  2. Decreased absorption of endolymph  
  3. Combination of both              
  1. Dilatation of the endolymphatic system. Cochlear hydrops is seen in all cases and saccular hydrops is seen in most. Utricular hydrops is rare.  
  2. Bulging of the Reissner’s membrane into scala vestibuli which is greatest in the apical turn
  3. Outpouchings of the wall of the saccule and sometimes the utricle
  1. Genetic factors:
    Familial tendency (14-20%), autosomal dominant
  2. Anatomical:
    Small vestibular aqueduct
  3. Traumatic:
    Biochemical dysfunction in the cells of the membranous labyrinth
  4. Viral infection: H. simplex -I notorious cause damage to the endolymphalic duct and sac
  5. Allergy:
    Food and inhalant allergens
  6. Autoimmunity:
    Circulating immune complexes may cause direct damage to the endolymphatic sac
  7. Psychosomatic and personality features
    1. Etiology of sec. endolymphatic hydrops:
    2. Abnormal metabolic/ endocrine state

Clinical features:
  1. Vertigo:
    1. Episodic, sudden onset
    2. Lasts 24 min to 24 hours
    3. Rotatory in nature
    4. Associated with nausea, vomiting, pallor, sweating and diarrohea
    5. No loss of consciousness    
  2. Hennebert’s sign:                                        
    Vertigo and nystagmus experienced by the patient during pressure induced excursion of the stapedial foot plate. Is a false positive fistula sign.
  3. Hearing loss:
    1. Fluctuant and progressive         
    2. Initially : low frequency losses          
    3. Later : high and low frequency losses                    
    4. Dysacusis: Sounds perceived has an abnormal tinny nature
    5. Diplacusis: Same sound perceived as a different pitch in the two ears
    6.  Loudness intolerance - candidates for hearing aid
Tinnitus: Roaring type (low pitched, during attack, change in character, intensity)
Aural fullness, may herald an attack
Extra Edge:

Fluctuating hearing loss is seen in otitis media with effusion (serous otitis media), Meniere’s disease, perilymph fistula and malingering.


MCQ. Tulio’s Phenomenon:
Subjective imbalance and nystagmus observed in response to loud, low frequency noise exposure


Pure tone audiometry:-
42% : Flat audiogram   32% : Peaked pattern
19% : Downward sloping  7% : Rising pattern

Recruitment: Present

Fig: (A) Audiogram in early Meniere’s disease. Note: Hearing loss is sensorineural and more in lower frequencies – the rising curve. As the disease progresses, middle and higher frequencies get involved and audiogram becomes flat or falling type (B & C).
  1. BERA
    Shows reduced latency of wave V
    * Tone decay - <20dB (N <15dB)  * Speech discrimination score to be between 55- 85 %
  2. Electrocohleography:
    Most sensitive and diagnostic. Records the action potential and the summating potential of the cochlea through a recording electrode placed over the round the window area.
    1. Normal width of summating potential/action potential== 1.2-1.8 m sec
      Widening greater than 2 m sec is usually significant
    2. Summating potential / action potential = 1:3 = 033                            
      Normal < 0.4                 
      In menieres>0.45

Fig: Electrocochleography. A. Normal ear; B. Ear with Meniere’s disease. Voltage of summating potential (SP) is compared with that of action potential (AP). Normally SP is 30% of AP. This ratio is enhanced in Meniere’s disease.
  1. Caloric test:
    Canal paresis in 75% (reduced response on affected side)
    1. Glycerol is given                             
    2. It produces a decrease in the intra labyrinthine pressure and also improves the cochlear blood flow
  2. Reverse glycerol test:
    1. Performed using acetazolamide                                                      
    2. Shows deterioration in the pure tone thresholds and speech discrimination scores
Table: Results of various tests to differentiate a cochlear form a retrocochlear lesion
  Normal Cochlear lesion Retrocochlear lesion
Pure tone audiogram Normal Sensorineural hearing loss Sensorineural hearing loss
Speech discrimination score 90-1 00% Below 90% Very poor
Rollover phenomenon Absent Absent Present
Recruitment Absent Present Absent
SISI score 0-15% Over 70% 0-20%
Threshold tone decay test 0-15 dB Less than 25 dB Above 25 dB
Stapedial reflex Present Present Absent
Stapedial reflex decay Normal Normal Abnormal
B. E.R.A Normal interval between wave I & V Normal interval
between wave I & V
Wave V delayed or absent
  1. Treatment (Medical Management)
    1. Acute phase:
      1. Reassurance
      2. Bed rest
      3. Vestibular suppressants:
        1. Phenothiazines  : Prochlorperazine (stemetil)
        2. Antithistamines  : Cinnarzine, Cyclizine, Promethazine
        3. Benzodiazepines:DiazepamLorazepam                                                            
      4. Vasodilators  
    2. Prophylaxis between acute episodes:
      1. Salt restricted diet
      2. Avoid smoking/tea/coffee/alcohol
      3. Diuretic therapy : To reduced endolymph volume and pressure
      4. Vasodilators: Given in the belief that meniere’s disease is the result of strial ischemia or endolymphatic sac hyperperfusion.
        1. Betahistine dihydrochloride                    
        2. Papavarine                                         
        3. Isoxsuprine                                                                    
        4. Amyl nitrate                      
        5. Nicotinic acid                                                
        6. 5% Co2 + 95% O2: Carbogen  
        7. Low molecular weight Dextran  
        8. Histamine
    3. Chronic Phase:
      1. Vestibular sedatives ; for 3 months
      2. Vasodilators
      3. Diuretics: Added to the above drugs if recurrent attacks/ if not controlled by vasodilators/vestibular sedatives.
      4. If allergic etiology: Elimination of allergen
      5. Steroids and immunotherapy : For autoimmune cases
  1. Other surgeries:
    1. Intermittent low pressure pulse therapy (Meniett device therapy). It is observed that intermittent positive pressure delivered to inner ear fluids Brings relief from the symp­toms of Meniere’s disease.
    2. Not only there is improvement in vertigo, tinnitus and ear fullness, but hearing may also improve. Intermittent positive pressure waves can be delivered through an instrument called Meniett device.

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