menieres disease

Meniere’s disease

Meniere’s disease is a chronic disease of the inner ear which causes episodes of vertigo and hearing loss. This disease affects many people and is very important because it causes to very severe vertigo. The severity of vertigo is so high that some patients in the first attack of Meniere’s disease feel that they are dying. The positive point is that this severe vertigo will not be repeated so strongly and will subside overtime in a period of 7-10 years.

The disease is largely known and there are many articles about it. However, in some cases the person’s disease is misdiagnosed as the Meniere’s disease. The reason is that although the prevalence of this disease is high, its diagnosis is difficult and a specialized team and appropriate diagnostic tools are required. One of the issues that make it difficult to diagnose this disease is the comorbidity of it with other diseases like vestibular neuritis (VN), benign paroxysmal positional vertigo (BPPV) and migraine. On the other hand, this disease has a progressive course in which in the early stages, all symptoms of the disease are not evident. Moreover, in some patients, all signs are not seen together. Therefore, high knowledge and clinical experience with this disease and other illnesses associated with hearing impairment and dizziness play an important role in diagnosing it.

Introduction

This disease was first explained by a French physician, Prosper Meniere (1799-1862), in 1861. Before this scientist, it was thought that Meniere’s symptoms were caused by a brain lesion. Prosper Meniere linked these symptoms to a disorder in the inner ear. He and other scientists called it “inner ear glaucoma”[1, 2].

In most cases, these patients with symptoms of sudden onset of dizziness present to the emergency rooms. At this stage, due to accompaniment of hearing impairment with these symptoms, they may be mistakenly diagnosed as “labyrinthitis” or “sudden sensorineural hearing loss”. In these cases, if Vertigo is repeated, Meniere’s disease will be considered in the diagnosis.

Meniere’s disease is divided into two categories. The first one is typical Meniere’s disease in which all cochlear (hearing loss, tinnitus and ear fullness) and vestibular (vertigo and ear fullness without hearing loss or tinnitus) signs are seen. In atypical Meniere’s disease, cochlear signs or vestibular signs are seen[3].

Due to the difficulties in the diagnosis of Meniere’s disease, it is difficult to determine its prevalence and the results of studies are also different. Prevalence of Meniere’s disease has been reported from 3.5 in 100000 to 513 in 100000[4]. Its prevalence in women is 1-3 times higher than in men. This disease is more common in adults and in fourth and fifth decades of their life. Of course, this disease has also been reported in children. In most patients, a family history of the disease is reported. It seems that the disease affects white people of North European descent more than the African and Black races[5, 6].

Pathophysiology

The main histopathologic correlate of this disease is endolymphatic hydrops which results as the excessive production of endolymphatic fluid or its lower than needed re-absorption. Endolymphatic Hydropse can happen secondary to high blood pressure, metabolic disorders or taking especial medications. Meniere’s disease is a type of episodic and recurrent endolymphatic hydropse that is idiopathic.

Diagnosis

The main sign of this disease is its fluctuations and changes in its severity. Common symptoms of typical Meniere’s disease include episodic vertigo which lasts from minutes to hours (with positional vertigo in and between attacks). Moreover, almost all patients, report the sense of pressure in the ear or ear fullness and tinnitus.

Most of patients experience unilateral symptoms and they will experience bilateral symptoms several years after the disease onset.

Although numerous tests and evaluations are used in the diagnosis of this disease, its diagnosis is still clinical. That is, a precise history taking and complete physical evaluation is required. The main necessary evaluations for Meniere’s disease include a thorough audiometric assessment, CHAMP, videonystagmography and electrocochleography. In all of the unilateral cases, Brain MRI with and without contrast is required to rule out other retrocochlear lesions which can lead to hearing loss, tinnitus and vertigo. Temporal bone CT-scanning is of less value in the diagnosis of the Meniere’s disease.

Most of these patients show up-sloping low frequency sensorineural hearing loss which is fluctuating and becomes flat over time. Serial audiograms are very helpful in diagnosis of this disease because in addition to determining the type and form of hearing loss, its fluctuations are confirmed over time.

Patients who manifest the signs of Meniere’s disease must complete hematological tests, including fat and blood glucose tests in order to rule out more common causes of their symptoms.

References:

  1. Knapp, H., A clinical analysis of the infl ammatory aff ectation of the inner ear. Arch Ophthalmol Otolaryngol, 1871. 4: p. 79.
  2. Portmann, G., Vertigo: surgical treatment by opening of the saccus endolymphaticus. Arch Otolaryngol, 1927. 6.
  3. Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere’s disease. American Academy of Otolaryngology-Head and Neck Foundation, Inc. Otolaryngol Head Neck Surg, 1995. 113(3): p. 181-5.
  4. Alexander, T.H. and J.P. Harris, Current epidemiology of Meniere’s syndrome. Otolaryngol Clin North Am, 2010. 43(5): p. 965-70.
  5. Caparosa, R., Medical treatment of Meniere’s disease. Laryngoscope Investig Otolaryngol, 1963. 73: p. 6.
  6. Nsamba, C., A comparative study of the etiology of vertigo in the African. J Laryngol Otol, 1972. 86: p. 8.