endolymphatic hydrops including Meniere's disease

see also:

• ED Mx of vertigo and BPV
• tinnitus
• deafness / hearing loss

• 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

• 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
  • +/- tinnitus
    • 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

• 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 to:
  • endocrine abnormalities
  • trauma
  • electrolyte imbalance
  • autoimmune dysfunction
  • medications such as non-steroidal anti-inflammatory drugs (NSAIDs)
  • 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

• 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
• transient ischaemic attack (TIA)
  • 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
  • see wikipedia
• 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
  • see wikipedia