Ménière’s Disease
MÉNIÈRE’S DISEASE (MD) is an idiopathic labyrinthine condition now universally accepted to have an autoimmune foundation.
Typical symptoms are:
- violent rotational vertigo (varying in duration from 20 minutes to more than 24 hours)
- Sensorineural deafness
- tinnitus
- ear fullness
- autonomic symptoms (nausea, vomiting, photophobia)
The histopathological basis is the so-called ENDOLYMPHATIC COCHLEAR HYDROPE, an increase in endolymph in the labyrinth due to the prevalent dysfunction of the endolymphatic sac. It has a trend classically defined as fluctuating. There is no clear prevalence of incidence between males / females.
The age most affected of the onset is the fourth decade, however occurrences at a younger age are not infrequent. Onset after sixty years is rarer. Up to 50% of cases can undergo a bilateralization of the pathology.
Initially it may present an audiometric drop in the low frequencies, often accompanied by recruitment and annoying perception of particularly intense sounds.
Upon evaluation of the nystagmus in videoculonystagmoscopy, it is possible to detect a nystagmus defined as irritative, beating towards the affected side, and which over time can reverse its direction. In the evaluation phase during the vertigo crisis, a horizontal or horizontal-torsional nystagmus, counter lateral to the affected side is frequently encountered.
A high percentage of patients with Ménière’s disease may present the evolution into TUMARKIN SYNDROME, more frequent in the advanced stages of the disease, but not to be excluded at any of the pathological stages. In some cases, it can represent the early manifestation of the pathology.
TUMARKIN’S SYNDROME is a clinical condition that can be defined as serious, if not dramatic, which can also be defined as VESTIBULAR DROP ATTACK, or a sudden loss of the antigravitational postural tone responsible for the consequent fall or drop. Obviously, such patients are at very high risk of fracture or other traumatic consequences, even severe ones. Patients with Tumarkin typically have no loss of consciousness or cognition.
In summary, the condition, which causes patients to fall suddenly, is often associated with retropulsion (feeling that something is pushing back), or lateropulsion, without loss of consciousness, it is of short duration, and a consequence of saccular and utricular endolymphatic hydrops.
A variant of MÉNIÈRE’S DISEASE to take into consideration is OHRESSER’S SYNDROME which occurs in the premenstrual period. Vertigo events with or without auricular fullness is often accompanied by a decidedly disabling migraine.
A further Meniere-like variant to consider is the LERMOYEZ SYNDROME.
This is characterized by the temporal inversion of the typical symptoms of endolymphatic hydrops, and the onset of symptoms are followed by the disappearance of auditory symptoms. It is assumed that at the base of LERMOYEZ is an initial vasospasm of the internal auditory artery.
There are various classifications of MÉNIÈRE’S DISEASE.
An important point for the clinical trend is the 2003 PAGNINI CLASSIFICATION.
It includes:
1- CANAL MD
Of which there are two subtypes:
The first, characterized by:
- Rising hearing loss on low frequency tones
- Auditory fluctuation with return to normality
- Isolated vertiginous episodes
- No or modest labyrinthine deficit
- Lermoyez variant
The second:
- Peaked hearing loss (low and acute frequencies)
- Auditory fluctuation with no return to normal
- Ensuing vertigo episodes
- Partial labyrinthine deficit
2- MACULA MD
- Pantonal hearing loss
- Little or no hearing fluctuation
- Episodic labyrintholithiasis
- Tumarkin phase (or syndrome)
- Total or subtotal labyrinthine deficit
3- TORPID MD
- Stable pantonal hearing loss (60-85 dB)
- Recurrent postural instability without episodes
- Total labyrinthine deficit
Concerning MD, the Barany Society has considered various diagnostic criteria, which have been further defined over the years. These diagnostic criteria (updated to 2015) include:
1- DEFINITE MD
- Two or more episodes of vertigo lasting between 20 minutes and 12 hours
- Possible Tumarkin Stages
- Unilateral sensorineural hearing loss in low and medium tones.
- Fluctuating hearing loss, tinnitus and / or fullness
- Other vestibular pathologies excluded
2- PROBABLE MD
- Two or more episodes of vertigo lasting between 20 minutes and 12 hours
- Hearing loss, tinnitus and / or fluctuating fullness (where audiometry is not essential)
- Other vestibular pathologies excluded.
3- POSSIBLE MD
- Meniere-type vertigo episodes without documented hearing loss (vestibular Menière) or fluctuating or stable sensorineural hearing loss associated with imbalance but without vertigo (cochlear Menière).
- Other causes excluded.
4- CERTAIN MD
DEFINITE MD plus histopathology.
The evolution from MONOLATERAL to BILATERAL is subject to the duration and precocity of onset of MÉNIÈRE. On average, the time span for bilateralization is 7/8 years.In fact, the time range is extremely variable, from two to twenty-seven years, should the event occur, with the possibility that the second ear affected may develop only auditory symptoms, without vertigo symptoms.
DELAYED ENDOLYMPHATIC HYDROPS
A separate discussion is that related to DELAYED ENDOLYMPHATIC HYDROPS (DEH). It is characterized by profound hearing loss, generally from trauma or infection. Over time, the patient develops a fluctuating hearing loss with repeated vertiginous episodes. Although with a different genesis, DEH treatment is the same as that for MD.
