endolymphatic_hydrops
Table of Contents
endolymphatic hydrops including Meniere's disease
introduction
- 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
- +/- 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
-
- 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
- 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
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
- 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
endolymphatic_hydrops.txt · Last modified: 2013/09/27 08:40 by 127.0.0.1