Lermoyez syndrome (LS) is generally regarded as a version of Meniere’s disease (MD), but this classification is not completely accepted and is under investigation. MD first reported in 1861 by Meniere, is usually referred to as idiopathic endolymphatic hydrops; the latter is due to increased pressure in the endolymph of the inner ear and can cause attacks of vertigo, tinnitus, and aural fullness, and result in hearing loss. Nausea and emesis also accompany the attacks. MD has several variants apart from the classic form; it can manifest without vertigo, or without hearing loss, or it can appear as Tumarkin otolithic catastrophe [1]. LS is characterized by essentially the same symptoms as MD (and therefore is likely to be a variant of it), but with one striking difference: a temporary improvement in hearing ensues at the time of the vertigo attacks [2].

LS is a very rare medical entity, and consequently, the number of in-depth descriptions of the disease is small [3]. In a study, which examined 12 patients with LS, some had hearing alterations without vertigo, while others had vertigo without alterations in hearing, indicating a similarity in the origin of LS and MD [3] [4]. Another report on a patient with bilateral LS also came to the conclusion that endolymphatic hydrops was the source of the problem, based on electrocochleography measurements [5], although dissenting opinions have been voiced [6]

The attack may last for hours, with both the improvement in hearing in low frequencies during the episode and the deterioration of hearing occurring before the next attack lasting from days to months [4]. The improvement in hearing can occur during or more commonly within a few hours following the attack [4]. Hearing loss may be fast at the initial stages and slow down later [3]. However, attacks of vertigo may increase in frequency as the disease progresses, as in the case of a patient with stabilized MD in one ear, and newly developed LS in the other [3].

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• Macula

  A sudden pressure increase in the saccule induces an abrupt change of the macula nerve action potential, lowering the threshold of nystagmus and dizziness and leading to an episode of vertigo. [jstage.jst.go.jp]

  Uzun-Coruhlu H, Curthoys IS, Jones AS (2007) Attachment of the utricular and saccular maculae to the temporal bone. Hear Res 233:77–85 PubMed CrossRef Google Scholar 17. [link.springer.com]

  All of the above dislodge the otoconia from the macula utriculae, & these gravitate to the most dependant part of the labyrinth, the ampulla of the posterior semi-circular canal. [drabrdesai.com]

• Impulsivity

  Some hyperactive-impulsive or inattentive symptoms that caused impairment were present before age 7 years. C. Some impairment from the symptoms is present in two or more settings (eg... ‎ [books.google.de]

  These include evaluation of utricular and saccular function by determination of ocular and cervical vestibular evoked myogenic potentials, and assessment of the vestibulo-ocular response by using the video head impulse test. [symptoma.com]

  Other testing including rotational chair, video head impulse test (vHIT), cervical and ocular VEMPs were considered to be within normal limits. [hearinghealthmatters.org]

  MacDougall HG, Weber KP, McGarvie LA, Halmagyi GM, Curthoys IS (2009) The video head impulse test: diagnostic accuracy in peripheral vestibulopathy. Neurology 73:1134–1141 PubMed CrossRef Google Scholar 12. [link.springer.com]

  These delicate hair cells transmit nerve impulses (via the eighth cranial nerve [vestibulocochlear nerve]) that result in the interpretation of sound and the maintenance of balance. [rarediseases.org]

• Vertigo

  In a study, which examined 12 patients with LS, some had hearing alterations without vertigo, while others had vertigo without alterations in hearing, indicating a similarity in the origin of LS and MD. [symptoma.com]

  […] and additional synonyms H81.39 Other peripheral vertigo Synonyms: Lermoyez' syndrome Otogenic vertigo Peripheral vertigo NOS Subcategories: H81.391 Other peripheral vertigo, right ear H81.392 Other peripheral vertigo, left ear H81.393 Other peripheral [averbis.com]

  […] by hearing improvement that accompanies an acute attack of vertigo. patients experience abrupt and brief attacks such that they find themselves thrown to the ground with no warning and with little or no vertigo after the initial fall. [xuqsrpbx.cf]

  This monograph on vertigo is based on the personal, hands-on, practical and clinical experience of managing vertigo in a large university-affiliated hospital. [books.google.de]

  Almost every one of the 12 patients showed, besides the Lermoyez attacks, also hearing fluctuations without vertigo, and vertigo without hearing fluctuations. [unboundmedicine.com]

• Dizziness

  […] symmetric during and following an episode of dizziness. [hearinghealthmatters.org]

  A sudden pressure increase in the saccule induces an abrupt change of the macula nerve action potential, lowering the threshold of nystagmus and dizziness and leading to an episode of vertigo. [jstage.jst.go.jp]

  Lermoyez nasal punch Lermoyez syndrome - increasing deafness interrupted by a sudden attack of dizziness after which the hearing improves. Synonym(s): labyrinthine angiospasm [medical-dictionary.thefreedictionary.com]

  Attacks of dizziness may come on suddenly or after a short period of tinnitus or muffled hearing. Some people have single attacks of dizziness once in a while. Others may have many attacks close together over several days. [icdlist.com]

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• Neglect

  The neglected but substantial problem of paediatric vertigo is addressed, as are the many pathologies in the elderly population that can lead to disequilibrium and repeated falls. [books.google.de]

Laboratory tests are routinely performed to exclude metabolic and hormonal diseases. Specific tests for LS (or for MD) have not yet been found. Magnetic resonance imaging (MRI) imaging is used for excluding anatomical abnormalities and growths, for example, acoustic neuromas. Three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) MRI is capable of quantitative evaluation of endolymphatic hydrops [7].

A number of acoustic exams are available for the evaluation of hearing loss and vertigo. Pure tone audiometry and acoustic impedance determine the hearing threshold and the acoustic reflex threshold; lower frequencies are usually affected more than higher ones, for example in one patient the threshold of hearing was found to increase in the range of 200-2000 hertz (Hz), whereas the values at high frequencies were relatively stable [3]. The glycerol test, which detects an improvement in air-conductive hearing threshold following oral glycerol administration, is characteristic of both MD and LS [1] [3]. Electrocochleography detects the expansion of the basilar membrane by measuring the ratio of the summing potential from the movement of the membrane and the nerve action potential following auditory stimulation. An increased value is often observed in MD and LS [1] [3]. Auditory brainstem response and the caloric test, which forms part of the assessment for vestibular function, are also used [3].

Newer methods are also available for testing dynamic otolith and horizontal semicircular canal function. These include evaluation of utricular and saccular function [8] by determination of ocular and cervical vestibular evoked myogenic potentials, and assessment of the vestibulo-ocular response by using the video head impulse test [9].

1. Ataman T, Enache A. Our experience with the medical treatment of endolymphatic hydrops. Int Tinnitus J. 2007;13(2):138-142.
2. Manzari L, Burgess AM, Curthoys IS. Vestibular function in Lermoyez syndrome at attack. Eur Arch Otorhinolaryngol. 2012 Feb;269(2):685-691.
3. Zhang Q, Xu M, Zhang X, Ren T, Anniko M, Duan M. A case of Meniere's disease in the left ear and Lermoyez syndrome in the right ear--a 32-month longitudinal observation and literature review. Acta Otolaryngol. 2010 Sep;130(9):1084-1088.
4. Schmidt PH, Schoonhoven R. Lermoyez's syndrome. A follow-up study in 12 patients. Acta Otolaryngol. 1989 May-Jun;107(5-6):467-473.
5. Schoonhoven R, Schmidt PH, Eggermont JJ. A longitudinal electrocochleographic study of a case of long-standing bilateral Lermoyez's syndrome. Eur Arch Otorhinolaryngol. 1990;247(6):333-339.
6. Maier W, Marangos N, Aschendorff A. Lermoyez syndrome--electrocochleographic studies. Laryngorhinootologie. 1996 Jun;75(6):372-376. [German]
7. Sepahdari AR, Ishiyama G, Vorasubin N, Peng KA, Linetsky M, Ishiyama A. Delayed intravenous contrast-enhanced 3D FLAIR MRI in Meniere's disease: correlation of quantitative measures of endolymphatic hydrops with hearing. Clin Imaging. 2015. Jan-Feb;39(1):26-31.
8. Curthoys IS. A critical review of the neurophysiological evidence underlying clinical vestibular testing using sound, vibration and galvanic stimuli. Clin Neurophysiol. 2010 Feb;121(2):132-144.
9. MacDougall HG, Weber KP, McGarvie LA, Halmagyi GM, Curthoys IS. The video head impulse test: diagnostic accuracy in peripheral vestibulopathy. Neurology. 2009 Oct 6;73(14):1134-1141.