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Otolaryngeal system (ENT)

Meniere’s disease

Inner ear disorder characterised by episodic vertigo with neurovegetative symptoms, sensorineural hearing loss, and tinnitus or aural fullness.

Inner ear disorder characterised by episodic vertigo with neurovegetative symptoms, sensorineural hearing loss, and tinnitus or aural fullness.

History:

Prosper Menière
Prosper Menière (1799 – 1862) was a French doctor who first identified that the inner ear could be the source of a condition combining vertigo, hearing loss and tinnitus, which is now known as Ménière’s disease.

Prosper Ménière brought the attention of vertigo and its possible relationship to the inner ear to light in his controversial paper presented in 1861 to the Imperial Academy of Medicine. His series of articles challenged the existing theories of vertigo as a cerebral disease. The eventual development and acceptance of criteria used to describe the disease with Ménière’s namesake has increased efforts in understanding the disease. The Committee on Hearing and Equilibrium of the American Academy of Otolaryngology – Head and Neck Surgery established diagnostic criteria and reporting guidelines for treatment. Their guidelines, published in 1995, define Ménière’s disease (MD) as “an idiopathic syndrome of endolymphatic hydrops“.


Epidemiology

The prevalence of Meniere’s disease varies between 3.5-513 per 100.000 and occurs more often in older, white and female patients.

Cumulative age distribution of onset of symptoms in patients with Meniere’s disease
Cumulative age distribution of onset of symptoms in patients with Meniere’s disease: The mean age at onset of symptoms was estimated to be 44.0 years on the basis of data from 1,035 patients in Finland. | Pyykkö, I., Nakashima, T., Yoshida, T., Zou, J. & Naganawa, S. Meniere’s disease: a reappraisal supported by a variable latency of symptoms and the MRI visualisation of endolymphatic hydrops. BMJ Open 3, e001555 (2013). MRI frequently reveals EH in the monosymptomatic ear.

Etiology

Aetiologies of endolymphatic hydrops
Aetiologies of endolymphatic hydrops. | Oberman, B. S., Patel, V. A., Cureoglu, S., & Isildak, H. (2017). The aetiopathologies of Ménière’s disease: a contemporary review TT – L’eziopatogenesi della Sindrome di Ménière: stato dell’arte. Acta Otorhinolaryngologica Italica : Organo Ufficiale Della Societa Italiana Di Otorinolaringologia e Chirurgia Cervico-Facciale, 37(4), 250–263. https://doi.org/10.14639/0392-100X-793

Endolymphatic hydrops (ELH)

Pathologically extended endolymphatic space (ELS)
Endolymphatic hydrops (EH)
Endolymphatic hydrops (EH) is characterized by an accumulation of fluid (endolymph), leading to an expansion of the endolymphatic space. The progression of Meniere’s disease is associated with the advancement of EH. The thin membrane bordering the endolymph and the perilymph is flaccid. When a rupture of the membranous labyrinth and subsequent collapse of the endolymphatic space is observed at some point in the inner ear, EH is usually observed in other parts inside the inner ear. | Nakashima, T., Pyykkö, I., Arroll, M.A., Casselbrant, M.L., Foster, C.A., Manzoor, N.F., Megerian, C.A., Naganawa, S., & Young, Y.-. (2016). Meniere’s disease. Nature Reviews Disease Primers, 2.

Variants:

  • Cochlear hydrops: Hearing loss is primary feature. Vertigo is absent.
  • Vestibular hydrops: Repeated episodes of vertigo without hearing loss

Presentation

Cardinal Symptoms:

  1. Episodic vertigo
  2. Fluctuating hearing loss
  3. Tinnitus
  4. Aural fullness
Clinical symptoms associated with Meniere’s disease
Clinical symptoms associated with Meniere’s disease: In most patients, the initial symptoms of Meniere’s disease (MD) present after a considerable accumulation of endolymph has occurred. Other patients have clinical symptoms earlier in the course of the disease, but the variability in symptomatology has not yet been explained. Not all patients have all the typical symptoms of MD (tinnitus, hearing loss, vertigo and aural fullness) and may only have one or some of these symptoms for years. Hyperacusis may also be the first symptom of MD. Symptoms are often transient for a long time — that is, fluctuations in the severity of endolymphatic hydrops are associated with a temporal alleviation of the clinical symptoms. Symptoms in the early stages of endolymphatic hydrops can disappear for some time, or even permanently, but symptoms in the advanced stages of MD are permanent. Importantly, permanent damage of the inner ear is associated with hearing loss as a result of the complete loss of the labyrinth function, but not with vertigo attacks | Nakashima, T., Pyykkö, I., Arroll, M.A., Casselbrant, M.L., Foster, C.A., Manzoor, N.F., Megerian, C.A., Naganawa, S., & Young, Y.-. (2016). Meniere’s disease. Nature Reviews Disease Primers, 2.

Special manifestations:

  • Tullio phenomenon: Sound-induced vertigo, dizziness, nausea or nystagmus due to distended saccule lying against stapes footplate
  • Recruitment phenomenon (hallmark of cochlear impairment): Perceptual phenomenon of sounds becoming rapidly louder with increasing sound level, leading to the somewhat paradoxical but common request of people with cochlear disorders “to speak louder” followed by the complaint to “stop shouting”
  • Tumarkin’s otolithic crisis (drop attack): Uncommon presentation with unknown mechanism in which patient suddenly drops without loss of consciousness
  • Lermoyez syndrome: Symptoms appear in reverse order with progressive hearing loss followed by vertigo

Diagnosis

Clinical examination:

  • Otoscopy
  • Nystagmus
  • Tuning fork tests

Audiometry:

  • Pure tone audiometry
  • Speech audiometry
  • Special audiometry tests:
    • Recruitment test positive
    • SISI (Short increment sensitivity index) test
    • Tone decay test

Electrocochleography + Glycerol test

Diagnostic test of choice. Glycerol, a dehydrating agent, reduces endolymph pressure and thus causes an improvement in hearing.
Electrocochleography: Meniere's disease
Electrocochleography demonstrates enhancing SP and SP/AP ratio in the left ear. | SP, summating potential; AP, action potential. | Vestibular Disorders. (2016). Retrieved 2 August 2022, from https://entokey.com/vestibular-disorders/

3D fluid-attenuated inversion recovery (3D-FLAIR) MRI

Can delineate the perilymphatic and endolymphatic spaces of the inner ear after intratympanic injection of Gadolinium DTPA (Gd-DTPA). In endolymphatic hydrops, the perilymphatic space surrounding the endolymph is either small or cannot be visualized.
MRI protocols in a patient with Meniere’s disease
MRI protocols in a patient with Meniere’s disease. High spatial resolution 3D-real inversion recovery (IR) image (a, 0.8 mm thick) and 3D-constructive interference in the steady-state (CISS) image (b, 0.4 mm thick), and their volume-rendered (VR) images (c, d). By comparing the perilymphatic VR image (c) and total lymphatic VR image (d), we can appreciate the degree of endolymphatic hydrops three dimensionally | Naganawa S, Nakashima T. Cutting edge of inner ear MRI. Acta Otolaryngol 2009;129:15-21

Differential diagnosis:

  • Basilar migraine: Associated with vertigo but without aural symptoms
  • Vestibular neuronitis: Associated with vertigo lasting for several days, no aural symptoms
  • Benign paroxysmal positional vertigo (BPPV): Associated with vertigo related to head movements, lasting seconds to minutes, no aural symptoms
  • Medications: Aminoglycosides, loop diuretics

Management

Different treatment options for Meniere disease exist but none are curative.

Treatment algorithm for Meniere's disease
Treatment algorithm for Meniere’s disease | Sajjadi, H. and Paparella, M. M. (2017) ‘Meniere’s disease’, The Lancet. Elsevier, 372(9636), pp. 406–414. doi: 10.1016/S0140-6736(08)61161-7.

 Meniere's disease
Nakashima, T., Pyykkö, I., Arroll, M.A., Casselbrant, M.L., Foster, C.A., Manzoor, N.F., Megerian, C.A., Naganawa, S., & Young, Y.-. (2016). Meniere’s disease. Nature Reviews Disease Primers, 2.

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