Meniere’s Disease: Still a Mystery

There have been symptom reports consistent with Meniere’s disease for many years yet it still remains poorly understood. There is some suspicion that historical figures such as Julius Caesar, Martin Luther, and even Vincent Van Gogh may have had the condition.⁴

The condition was first theorized to be an inner ear condition by French physician Dr. Prosper Meniere in 1861.³ Dr. Meniere’s theory has held true in identifying the inner ear as the source of the condition, but there is still no consensus on the pathophysiology behind the disease.

Treatments for Meniere’s disease are also variable due to the multitude of theories for its existence.

Theories Behind Meniere’s

Meniere’s disease causes episodes of vertigo with associated hearing loss, fullness/pressure, and tinnitus in the affected ear. With repeated episodes the hearing loss often becomes permanent and may even reach the point of total deafness in the impacted ear.

Meniere’s disease is often associated with atypical inner ear fluid levels, also known as endolymphatic hydrops.¹⁵ The initial theory of Meniere’s disease proposed that the endolymph levels fluctuated, which resulted in atypical stimulation of both the hearing and balance halves of the inner ear, resulting in hearing loss and vertigo. There are flaws in this theory though, as it has been shown that there are individuals with endolymphatic hydrops that do not have symptoms of Meniere’s disease.^6,8,10

This would suggest that endolymphatic hydrops may be associated with Meniere’s disease, but is unlikely to be the sole cause.

Other Possible Theories

There are numerous other proposed theories for Meniere’s disease including but not limited to: abnormalities of the endolymphatic sac/endolymph flow, endolymphatic sinus dysfunction, bone degeneration around the inner ear structures, autoimmune inner ear dysfunction, utriculoendolymphatic valve dysfunction, allergy, trauma, malformation of the cochlear or vestibular aqueducts, middle ear muscle spasms, intracranial hypotension, abnormal endolymph ionic concentrations, abnormal endolymph secretion, viral insult, genetic predisposition, displaced otoconia, migraine, and poor inner ear blood flow.^{6,7,10,11,14,16}

A more detailed review of some of the different theories for Meniere’s disease can be found here.

Despite medical and research advancement the exact pathophysiology of Meniere’s disease is still elusive.

Time Course of Meniere’s Symptoms

Meniere’s disease can be difficult to treat due to the general disagreement as to the cause for the condition. Thankfully, for most individuals the vertiginous symptoms are often temporary and tend to decrease in frequency with time.¹²

In general, the episodes of vertigo decrease in frequency and intensity within 5-10 years of onset.⁹ Another study showed that 57% of individuals had complete control of the vertigo by year 2 and 71% had complete control of the vertigo by year 8.¹³

Unfortunately, the hearing loss, tinnitus, and sensation of pressure or fullness in the ear are often permanent.

Many cases of Meniere’s disease impact just one ear, but as time passes from initial onset there is a higher risk for developing the condition in the other ear. Around 30% of cases go on to impact the other ear within the first 10 years of onset. If that time frame is backed out to 20 years or greater since the first ear was impacted, then there is roughly a 50% chance of the condition impacting the other ear.

In those cases that do impact both ears, often the vertiginous symptoms are less severe when the individual becomes symptomatic for the second ear.⁹

Demographics of Meniere’s Disease

Meniere’s disease has been shown to impact around .2% of the population and most individuals become symptomatic between the ages of 40-60. ^{1, 2} There are not currently any strong racial or gender correlations.⁵

There may be some genetic predispositions to developing Meniere’s disease but given the plethora of suggested causes, it is difficult to identify any one genetic factor.

Meniere’s Treatment Remains Varied – with Mixed Results

Given the poorly understood pathophysiology of Meniere’s disease, treatments are variable and have mixed levels of effectiveness. There is no immediate cure for Meniere’s disease and typically a treatment ladder is utilized.

Treatments at the bottom rung of the ladder are rather benign but with limited risk. If there is limited response to treatments lower on the ladder, then you may ascend to the “next rung” of the ladder so to speak.

The higher you ascend the ladder the greater the risks associated with the treatment.

Treatments can vary from dietary modification such as reducing the salt in your diet, betahistine, steroid injections, endolymphatic sac decompression, gentamicin injection and even nerve section in some cases. Procedures such as nerve section and gentamicin injection can leave the individual with significant inner ear vestibular and hearing deficits that are permanent.

These types of ablative treatments are really a tradeoff of more persistent dizziness and imbalance instead of the episodes of vertigo.

Summary

We have been aware of the symptoms of Meniere’s disease for over 2000 years and have known the inner ear was the culprit for over 150 years, yet the condition is still not fully understood. Due to the multitude of theories as to the generation of Meniere’s disease, treatments are variable and often focused on symptom control rather than a cure. Thankfully, the vertiginous symptoms do eventually resolve for most individuals but the hearing loss is often permanent.

Hopefully, additional research can allow for us to better understand Meniere’s disease, which in turn will allow for more effective treatment.

 

References

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2. Alexander, T.H., & Harris, J.P. (2010) Current Epidemiology of Meniere’s Syndrome. Otolaryngol. Clinics of North Am. 43(5): 965-970.
3. Baloh, R. (2001) Prosper Meniere and his Disease. Neurol.. 58(7): 1151-1156. doi:10.1001/archneur.58.7.1151
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12. Perez-Garrigues, H., Lopez-Escamez, J., Perez, P., Sanz, R., Orts, M., Marco, J.,…Ramirez, R. (2008) Time Course of Episodes of Definitive Vertigo in Meniere’s Disease. Arch Otolaryngol Head Neck Surg. 134(11): 1149-54. doi: 10.1001/archotol.134.11.1149.
13. Silverstein, H., Smouha, E. & Jones, R. Natural History vs. Surgery for Meniere’s disease. Head & Neck Surg. (1989) 100(1): 6-16.
14. Weiss, N.M., Breitsprecher, T.M., Dlugaiczyk, J., Kammerer, F., Lukas, C., Dazert, S.,…Bachinger, D. (2023) Meniere’s Syndrome Associated with Fibrous Dysplasia of the Temporal Bone. Otology & Neurotology. 44(6): e445–e448. doi: 1097/MAO.0000000000003891
15. Yamakawa, K. (1938) Temporal Bone Histopathology of Meniere’s Patient. Paper presented at the Annual Meeting Oto-Rhino-Laryngol Society; Japan.
16. Yamane, H., Sunami, K., Iguchi, H., Sakamoto, H., Imoto, T., & Rask-Andersen, H. (2012) Assessment of Meniere’s Disease from a Radiological Aspect – Saccular Otoconia as a Cause of Meniere’s disease?. Acta. Otolaryngol.. 132(10): 1054-1060.