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endolymphatic hydrops including Meniere's disease


  • refers to conditions associated with excess fluid build up in the endolymphatic spaces of the inner ear
  • this typically causes vertigo, tinnitus, fullness in the ear(s) and possibly sensorineural deafness
  • some patients also hear echos

idiopathic endolymphatic hydrops (Ménière's disease (MD))

  • characterized by the following symptoms:
    • ED Mx of vertigo and BPV often accompanied by nausea and vomiting
      • characteristically rotatory spinning or a rocking sensation and may be associated with nausea and vomiting, and persists from 20 minutes to 24 hours duration
      • at least 2 definitive episodes of vertigo of at least 20 minutes duration must have occurred to make the diagnosis
    • sensorineural hearing loss, usually fluctuating
      • low frequency or combined low and high frequency sensory loss with normal hearing in the mid frequencies. Over time the hearing loss “flattens out
    • +/- sensation of pressure or fullness in the affected ear
      • usually low-tone roaring
  • most commonly occurs in middle-aged patients, usually between 20 and 50 years
  • affects ~1 in 1000 people
  • 8-15% appear to have an AD genetics
  • half also have migraine while 85% of those with bilateral disease have migraine!1)
    • 22-56% of patients with classical MD have Hx of migraine compared to 15-17% of women and 6% of men
    • in a small study, only 4% of those with MD without migraine have concurrent bilateral episodes, whereas, bilateral symptoms occur in about half of those with MD and migraine and 39% had a FH of episodic vertigo compared with only 2% of those with MD without migraine. The migraine group had FH migraine in 72% vs 12% for the non-migraine group. The migraine group had onset of MD peaking earlier at age 30-40yrs and none after age 60yrs 2)
    • migraine may cause vasospastic micro-vascular ischaemic damage to the inner ear, hearing loss, and susceptibility to developing endolymphatic hydrops
  • may involve one or both ears and usually exibits fluctuating hearing loss and episodic vertigo, although one symptom may precede the other by months or years.
  • rare for Ménière's disease to present with a severe sensorineural hearing loss
  • no specific diagnostic test for Meniere's disease and a definitive diagnosis can only be made postmortem

delayed endolymphatic hydrops (DEH)

  • first described, under the name of “unilateral deafness with subsequent vertigo”, by Wolfson and Lieberman and Nadol et al
  • characterized by a profound sensorineural hearing loss in one ear, found to have been present in most cases from early childhood, due to an unknown cause, trauma or viral infections.
  • after a prolonged period (usually many years) patients with DEH experience the onset of episodic vertigo from the deaf ear (Ipsilateral Delayed Endolymphatic Hydrops) or develop a fluctuating hearing loss and/or episodic vertigo in the opposite ear, previously with normal hearing (Controlateral Delayed Endolymphatic Hydrops).
  • vestibular symptoms are identical to those of Ménière's disease

secondary endolymphatic hydrops

  • secondary to:
    • endocrine abnormalities
    • trauma
    • electrolyte imbalance
    • autoimmune dysfunction
    • parasitic infections
    • hyperlipidemia
    • hypothesized to be a factor, not an etiology, influencing the clinical course of subjective idiopathic tinnitus (SIT)
      • ie. tinnitus rather than vertigo is the main feature of the classic tetrad of symptoms

other DDx of Ménière's type symptoms

  • migraine associated vertigo
    • 3-5% of population and thus much more common than Ménière's disease
    • vestibular migraine:
      • episodic vertigo in patients with a history of migraines
    • basilar type migraine (BTM)
      • vertigo plus more than one neurologic symptom of putative brainstem or bilateral cortical origin occur as part of a headache aura
    • rarely experience simultaneous vestibular and cochlear symptoms
    • do not cause persistent tinnitus or objective hearing loss
  • acoustic neuroma (vestibular schwannoma):
    • typically present with progressive asymmetric hearing loss but can sometimes have fluctuating hearing loss
    • rarely have true vertigo but may complain of imbalance
    • some will have tinnitus and imbalance with normal hearing
  • Cogan's syndrome
    • chronic inflammatory condition that occurs most commonly in young adults and can include vestibuloauditory symptoms that are similar to Meniere disease.
    • patients also have other features including eye disease and systemic vasculitis
  • patulous Eustachian tube
    • patients may have fullness in the ear, tinnitus and autophony (self hearing from inside, strongly amplified) but no vertigo or deafness
    • on otoscopy, the tympanic membrane vibrates with every breath taken by the patient
  • superior canal dehiscence syndrome (SCDS)
    • rare condition, 1st described in 1998
    • progressive condition
    • vertigo/imbalance
    • pulsatile tinnitus
    • autophony
    • Tullio phenomenon - sound-induced vertigo, disequilibrium or dizziness, nystagmus and oscillopsia
    • hyperacusis
    • low frequency conductive deafness
The relevance of migraine in patients with Meniere's disease. Brodsky at al. Acta Oto-Laryngologica (0001-6489) 20071201. Vol.127,Iss.12;p.1241-1245
endolymphatic_hydrops.txt · Last modified: 2013/09/27 18:40 (external edit)