Meniere Disease Flashcards
Meniere Disease: An overview
Meniere disease is a multifactorial disorder probably caused by a combination of genetic and environmental factors. The disease is associated with an accumulation of endolymph in the cochlear duct and vestibular organs. Endolymphatic hydrops do not explain all of the clinical features of the disease. The clinical features of the disease include progressive hearing loss and a high frequency of vertigo crisis.
Clinical characterization of Meniere
The disease is characterized by episodic, spontaneous vertigo usually accompanied by fluctuating sensorineural hearing loss, tinnitus, and aural fullness. Cochlear symptoms may occur between episodes of vertigo. Vertigo crises are more frequent during early years of the disease. Hearing loss and vestibular hypofunction (reduced function) show great variability among patients, which can lead to difficulty establishing an accute phenotype. There is no blood test or other exact test for the disease. The disease is rather diagnosed based on a constellation of symtpoms, i.e. roaring tinnitus and changes in hearing. Meniere disease can be confused with other conditions which include migraine, atypical BPPV, etc.
Diagnostic criteria
The diagnostic criteria include two or more episodes of spontaneous vertigo lasting between 20 minutes and 12 hours. Vertigo is a sensation of movement in the absence of movement or an altered sensation of movement during normal movement of the head. The time is defined by how long the patient must remain immobile. Additionally, the criteria include low frequency sensorineural hearing loss documented with audiometry in one ear, defined at least one occasion before, during, or after one of the episodes of vertigo. Hearing loss/tinnitus/aural fullness symptoms usually fluctuate. Last, no other diagnosis can exist that better explains the vestibular symptoms. Some patients may describe triggers for their symptoms to include caffeine, sodium, sound (tullio phenomenon), or Hennebert symptom/sign (nystagmus) from pressure changes. These resulting episodes may occur later in the disease process, when hydrops have advanced bringing the membranous labyrinth closer to the stapes footplate.
Determining the affected ear
Thresholds will shift at least 35 dB HL at 2 consecutive frequencies below 2kHz or a 30 dB shift compared to contralateral ear. The hearing loss should be sensorineural. Some patients may have bilateral hearing loss with Meniere, but this is less common and autoimmune inner ear disease should be considered. If the hearing loss progresses slowly over years, the audiologist should consider the possibility of it being independent of vestibular symptoms, including migraines or DFNA6/14 (autosomal dominant variation for LF HL). The sensorineural hearing loss may precede the vertigo by months or years. Tinnitus and aural fullness are usually associated with the first episode of vertigo.
Differential diagnosis
DFNA9, DFNA6/14, autoimmune inner ear disease, stroke, cogan syndrome, ELST, CPA mass, susac syndrome, infectious causes (otosphyillis), third window syndromes, vestibular migraine, vestibular schwannoma, Vogt-Koyangi-Harada syndrome
Epidemiology
34-190 cases per 100,000 with an age of onset between the 3rd and 7th decade of life. There is a small predominance for women. Odds ratio increases 1.5 per 10 years, by 1.7 if you are caucasian, and by 1.7 if you are obese. Meniere can also be associated with arthritis (1.5 odds ratio), IBS (2.1 odds ratio), or migraine (2.0 odds ration).
Familial Meniere disease
Familial Meniere disease should be considered when at least one first or second relative has definitive or highly likely Meniere disease; it may occur in 8-9% of cases of Meniere disease in European populations, although it has been described in nearly all ethnic groups. It typically fits the AD inheritance pattern although mitochondrial recessive has been described (DTNA, FAM136A)
Other considerations
Meniere disease adversely impacts mental health, emotional state, and life satisfaction. Hydrops alone does not = Meniere. There is likely no single cause, but rather a common endpoint for a variety of anatomic or physiologic variables.
