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LESSON 142

Meniere's Disease and Endolymphatic Hydrops

Year 2
Spring semester
Disorders of the Auditory and Vestibular Systems
Inner ear and retrocochlear disorders
Duration: 11:32

We explain Meniere's disease and the endolymphatic hydrops that underlies it. With examples, we cover vertigo attacks lasting 20 minutes to 12 hours, fluctuating low-frequency sensorineural loss, tinnitus and aural fullness, and the Barany Society diagnostic criteria.

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Hello, and welcome to the AIHEAR Audiology Academy.
In this lesson, we learn about Meniere's disease and the endolymphatic hydrops that underlies it.
We will cover vertigo attacks, fluctuating hearing loss and the Barany Society diagnostic criteria.
Meniere's disease is a chronic disorder of the inner ear that comes in attacks.
Its main symptom is attacks of vertigo, a spinning sensation, that start spontaneously and last minutes to hours.
The attacks come with a fluctuating sensorineural hearing loss in the same ear that affects the low frequencies.
Tinnitus and a feeling of fullness in the ear complete the picture; these symptoms can increase before or during an attack.
The disease is named after Prosper Meniere, the French physician who linked these symptoms to the inner ear.
Endolymphatic hydrops is considered the key underlying finding.
The scala media, in the middle of the cochlear duct, is filled with endolymph, a fluid rich in potassium.
Endolymphatic hydrops is an increase in endolymph volume in this compartment, which then expands.
The expanding scala media pushes Reissner's membrane toward the scala vestibuli.
Hydrops usually affects the region near the cochlear apex first, the low-frequency region, which explains the low-frequency loss.
However, hydrops can be present without symptoms, so hydrops and Meniere's disease are not the same thing.
Why hydrops develops is not fully known; genetic, autoimmune and anatomical factors have been proposed.
Endolymph is found not only in the cochlea but also in the vestibular labyrinth.
After the cochlea, hydrops most often affects the saccule.
The utricle and the semicircular canals are affected less often and later.
How the spinning arises during an attack is not fully known; pressure and ion changes in the membranous labyrinth have been proposed.
That is why the severity of the attacks does not always parallel the degree of hydrops.
The disease most often begins between the ages of forty and sixty, but it can occur at any age.
At first, usually only one ear is affected.
Over the years, the second ear is also affected in some patients, and the disease becomes bilateral.
Attacks can come in clusters or be absent for months or years; the course varies greatly between people.
Hearing that recovers between attacks in the early stage may worsen permanently in later years.
In the late stage, the loss often becomes moderate to severe, with a flatter curve.
Before an attack, many patients feel fullness in the ear, louder tinnitus or reduced hearing.
The spinning sensation that follows lasts between twenty minutes and twelve hours, according to the Barany criteria.
Nausea, vomiting and being unable to stand are common during the attack.
On examination, a nystagmus with horizontal and torsional components is often seen; its direction can change during the attack.
After the attack, fatigue and a sense of imbalance can remain for hours or days.
Spinning that lasts a few seconds, or dizziness that continues without a break for days, is not a typical Meniere's attack.
The tinnitus of Meniere's disease is often described as a low-pitched hum or roaring sound.
Aural fullness is the feeling that the ear is blocked or under pressure, and it may become prominent before an attack.
Patients notice that their hearing is better on some days and worse on others.
When these ear symptoms rise in the same ear and at the same time as the vertigo, it is valuable for the assessment.
A diary that records the timing of symptoms helps both the physician and the audiologist.
In advanced disease, some people have sudden drop attacks, falling to the ground without losing consciousness.
These are called Tumarkin's otolithic crises and are explained by sudden stimulation of the otolith organs.
In the affected ear, sounds may be heard at a different pitch or distorted; this is called diplacusis.
Because of recruitment, an abnormally rapid growth of loudness, loud sounds can become uncomfortable.
As the disease progresses, speech discrimination scores may also fall.
In this example, the right ear shows a loss that is clear at the low frequencies and improves toward the highs.
Bone conduction thresholds overlap with air conduction, so the loss is sensorineural.
The left ear is within normal limits; this one-sided picture may be consistent with early Meniere's disease.
Barany wants bone conduction at two adjacent frequencies below two thousand hertz to be at least thirty decibels worse than the other ear.
Here, the bone conduction difference is thirty five decibels at two hundred fifty and five hundred hertz, so this criterion is met.
Some patients show a curve where low and high frequencies are both affected, peaking around two thousand hertz.
One of the most distinctive features of Meniere's disease is that hearing fluctuates over time.
In this sample table, the low-frequency thresholds of one ear during a symptomatic period are between forty and fifty decibels.
At the check two weeks later, the thresholds have clearly improved.
At the measurement six months later, the thresholds have worsened again.
A single audiogram cannot show this fluctuation, which is why serial audiograms are so valuable.
Doing serial measurements with the same equipment and the same method makes the comparison reliable.
A clinical tip: a complaint of aural fullness is often taken for a middle ear problem.
If the tympanogram is normal, do not stop there; always measure air and bone conduction thresholds at the low frequencies.
A low-frequency sensorineural loss with a normal tympanogram is an important finding that calls for referral to a physician.
Also tell the patient they can come to the clinic when symptoms increase; an audiogram taken then is very informative.
Electrocochleography, or ECochG, may show an increased ratio of summating potential to action potential in the affected ear.
On caloric testing, a weak response on the affected side is common.
In contrast, the video head impulse test often stays normal; we will discuss this dissociation in later lessons.
VEMP responses can also be affected, but the findings vary.
Magnetic resonance imaging is used to rule out retrocochlear causes; with special techniques, hydrops can also be shown.
None of these tests establishes the diagnosis on its own; diagnosis rests on clinical criteria.
In 2015, the Barany Society and related international societies published joint diagnostic criteria.
Definite Meniere's disease requires at least two spontaneous vertigo attacks, each lasting twenty minutes to twelve hours.
In addition, the affected ear must have an audiometrically documented low- to mid-frequency sensorineural loss.
Fluctuating ear symptoms in the same ear, such as changes in hearing, tinnitus or fullness, are also required.
In probable Meniere's disease, attacks last twenty minutes to twenty four hours, and no audiometric evidence is required.
In both cases, the picture must not be better explained by another vestibular diagnosis.
The condition that most resembles Meniere's disease is vestibular migraine, and the two can also occur in the same person.
Vestibular migraine can also bring attacks and ear symptoms, but a clear and permanent hearing loss is not expected.
One-sided hearing loss and tinnitus should also bring to mind retrocochlear causes such as vestibular schwannoma.
Autoimmune inner ear disease is considered especially in bilateral, rapidly progressive losses.
Sudden hearing loss and labyrinthitis are usually single, non-recurring events.
Making this distinction is the physician's task; audiological findings make an important contribution to the process.
The audiologist's first task is to document hearing accurately, especially during symptomatic periods.
Serial audiograms show the fluctuation and help in assessing the diagnostic criteria.
Vestibular tests reveal the affected side and the state of vestibular function.
For permanent hearing loss and tinnitus, rehabilitation options such as hearing aids and counseling can be planned.
Treatment is planned by the physician, and it ranges widely, from lifestyle changes to medication and interventional procedures.
That is why the audiologist works in constant contact with the ENT physician.
Meniere's disease is based on endolymphatic hydrops, an expansion of the scala media.
Vertigo attacks last twenty minutes to twelve hours.
The hearing loss begins at the low frequencies and fluctuates, which is why serial audiograms matter.
The Barany criteria look for attacks, audiometric loss and fluctuating ear symptoms together.
Test yourself: According to the Barany criteria, how long does a vertigo attack last in definite Meniere's disease?
The correct answer is B: twenty minutes to twelve hours.
Positional spinning that lasts seconds, or dizziness that continues for days without a break, suggests other conditions.
In the next lesson, we will examine vestibular schwannoma and cerebellopontine angle tumors, together with their audiological findings.
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