Lermoyez syndrome is an infrequent disease of the auditory and vestibular system. It is likely to be caused by endolymphatic hydrops, and is defined by attacks of vertigo, together with tinnitus, aural fullness, and fluctuating hearing loss, followed by temporary hearing improvement. Surgery is considered for cases with frequent, severe episodes.
Presentation
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].
Entire Body System
- Falling
Drop attacks from Meniere's are more "active" -- the person involved actually ends up participating in the fall (because they feel as if they are flipped). Most other drop attacks are slower and are more of a "collapse" than an actively driven fall. [dizziness-and-balance.com]
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]
Some people with Meniere's disease have "drop attacks" during which the dizziness is so bad they lose their balance and fall. Scientists don't yet know the cause. [icdlist.com]
As the disease progresses, middle and higher frequencies get involved and audiogram becomes flat or falling type (B & C). BERA Shows reduced latency of wave V * Tone decay - Electrocohleography: Most sensitive and diagnostic. [gradestack.com]
- Weakness
Vestibular testing revealed a caloric weakness (reduced response of 35%) affecting the right side. Other testing including rotational chair, video head impulse test (vHIT), cervical and ocular VEMPs were considered to be within normal limits. [hearinghealthmatters.org]
Light-headedness & weakness. Uneasiness, insecurity & confusion. [drabrdesai.com]
A specific program is designed to improve the weaknesses of your balance system, which may include problems with vision, proprioception, and vestibular input. [eioftx.com]
Ablative procedures are generally effective in alleviating the disabling attack of vertigo, and patients typically recover quite well due to the principles of central compensation following a stable unilateral vestibular system weakness. [asha.org]
- Inflammation
As causes for this syndrome, which can affect the hearing, the aequilibrium or both at the same time, a number of pathologies have been found to be responsible: Autoimmune inflammation in the inner ear, in conjuction with those in kidneys, intestines, [meniereclinic.com]
Usually, the first symptom is rhinitis (nasal inflammation), with sneezing, a runny nose, congestion, and often a reduced sense of smell. This is usually followed by the development of asthma, and then the growth of polyps inside the nose. [health24.com]
It has been noted that viral infections sometimes caused inflammation of the inner ear that can develop symptoms of vertigo, a common and key characteristic of Meniere’s disease. [diseasedefinition.info]
Ocul Immunol Inflamm. 2009;17(4):292–294. [PubMed] [Google Scholar] 184. Anand R. Photodynamic therapy for diffuse choroidal hemangioma associated with Sturge Weber syndrome. Am J Ophthalmol. 2003;136(4):758–760. [PubMed] [Google Scholar] 185. [ncbi.nlm.nih.gov]
Respiratoric
- Pneumonia
[…] having ISBN 1 86487 271 3; “relevant service” means: (a) eligible war service (other than operational service); or (b) defence service (other than hazardous service); “terminal event” means the proximate or ultimate cause of death and includes: (a) pneumonia [legislation.gov.au]
The disease cannot be transferred or transmitted from one person to another by touching or sharing the object or by living in surrounding with the infected person. “ How long is a person with pneumonia contagious? “ [diseasedefinition.info]
Ears
- Hearing Impairment
At the pre-stage of vertigo, he noticed the severity of tinnitus and hearing impairment was used to increase. Then, the attacks of dizziness appeared. Meanwhile, both tinnitus and hearing impairment disappeared after the sudden onset of vertigo. [jstage.jst.go.jp]
The hearing loss may progress to a profoundly impaired degree as the disease process continues. However, the degree of hearing loss seldom exceeds a 70 dB average [ 21 ]. [intechopen.com]
Neurologic
- Vertigo
[…] 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]
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]
[…] 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]
Almost every one of the 12 patients showed, besides the Lermoyez attacks, also hearing fluctuations without vertigo, and vertigo without hearing fluctuations. [unboundmedicine.com]
Tinnitis and progressive hearing loss followed by an attack of vertigo, after which hearing improves. [medical-dictionary.thefreedictionary.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]
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]
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]
- Confusion
Uneasiness, insecurity & confusion. [drabrdesai.com]
[…] erroneous attempts at descriptive terminology such as encephalotrigeminal angiomatosis, or designations such as venous angioma, caput medusae, or cavernoma, along with a plethora of eponymous appellations including Fegeler, Cobb, and others, served to confuse [ncbi.nlm.nih.gov]
- Ataxia
[…] movement BPPV,vascular loop Foods-caffeine,cheese,wine Stress /lack of sleep Fluoroscent lights Migraine Loud sound, Pressure changes:valsalva,sneezing,coughing SSC dehiscence Perilymhatic fistula Enlarged vestibular aqueduct Alcohol,exercise Episodic ataxias [xuqsrpbx.cf]
Workup
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].
Treatment
THE TREATMENT OF VERTIGO The treatment is dependent upon the cause of the vertigo. MEDICAL TREATMENT IS INITIALLY RECOMMENDED. The treatment of various central nervous & psychogenic disorders is beyond the scope of this discussion. [drabrdesai.com]
Finally, the patient will be seen by an otolaryngologist to determine a diagnosis and discuss treatment options. Treatment Options Much research has been done to determine the efficacy of treatment options for patients with Ménière's disease. [asha.org]
This patient is currently under the care of one of the neurotologists at our clinic and is considering utilizing betahistine for treatment. For those unfamiliar with Menieres disease and the so called “treatment ladder”, click here. [hearinghealthmatters.org]
Treatment of of Lermoyez Treatment is generally the same as Meniere's disease. If one accepted some of the other theories mentioned above, one might consider vascular treatments, or migraine treatments. [dizziness-and-balance.com]
Prognosis
Prognosis Hydrops is a chronic disease, like diabetes or hypertension. It needs to be treated medically (low sodium diet and diuretic) like other disease processes for many years. [eioftx.com]
Prognosis • Prognosis is variable, since the disease pattern of exacerbation and remission makes evaluation of treatment and prognosis difficult to predict. – In general, Ménière symptoms tend to stabilize spontaneously with time. [slideshare.net]
MR staging of acute dural sinus thrombosis: correlation with venous pressure measurements and implications for treatment and prognosis. AJNR. 1995;16(5):1021–1029. [PMC free article] [PubMed] [Google Scholar] 111. [ncbi.nlm.nih.gov]
Etiology
The etiology is also discussed in these cases. We believe that vasospasms is the main etiological factor in producing the sequence of events in Lermoyez's syndrome. [jstage.jst.go.jp]
Middle Aged Syndrome Tinnitus / etiology From MEDLINE®/PubMed®, a database of the U.S. [biomedsearch.com]
They compare 25 cases of aspirin nasal polyposis with 26 other cases of various etiologies. Other substances than aspirin seem to be charged. The complications are regular with severe asthma and infection. [read.qxmd.com]
If allergic etiology: Elimination of allergen Steroids and immunotherapy : For autoimmune cases Other surgeries: Intermittent low pressure pulse therapy (Meniett device therapy). [gradestack.com]
Observations on the etiology and treatment of Meniere's syndrome. JAMA 1941; 116: 1753–60. Google Scholar | Crossref 11. Koskas, HJ, Linthicum, FH, House, WF. Membranous ruptures in Meniere's disease: Existence, location, and incidence. [journals.sagepub.com]
Epidemiology
It covers all aspects of the subject, offering the latest information on epidemiology, aetiopathogenesis, diagnostics, the battery of vestibular and allied tests, treatment, rehabilitative management and ethical considerations. [books.google.de]
In the present study we conduct e d epidemiological and neurotological studies of 46 cases with Lermoyez's symptoms. Of these 46 cases,30 showed the typical course of Lermoyez's syndrome. [jstage.jst.go.jp]
Epidemiology of Meniere’s disease? The disease/disorder was firstly identified and characterized in year 1800 by a French doctor named Prosper Meniere. The disorder affects approximately 0.3-0.9 persons per 1000. [diseasedefinition.info]
Pathophysiology
Pathophysiology The pathophysiology of Ménière's disease is not clearly understood. [asha.org]
A reexamination of objective findings leads to a new, straightforward pathophysiologic mechanism. [ncbi.nlm.nih.gov]
Negative myoclonus: an overview of its clinical features, pathophysiological mechanisms and management. Neurophysiol Clin Neurophysiol 2006:36:337-343 Sheldon JH. On the natural history of falls in old age. [dizziness-and-balance.com]
Prevention
Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.de]
It is due to the presence of a clot which prevents the compressive action of superior constrictor muscle on the blood vessels. [neet.eckovation.com]
Gentamicin prevents the growth of microorganisms and decreases the balancing function of affected ear, so that the other ear can maintain the function. [diseasedefinition.info]
It’s not clear you can do anything to prevent Ménière’s disease. However, there are many things you can do the help manage your symptoms. In addition to eating a low-salt diet, you may want to cut down on alcohol and caffeine. [webmd.com]
References
- Ataman T, Enache A. Our experience with the medical treatment of endolymphatic hydrops. Int Tinnitus J. 2007;13(2):138-142.
- Manzari L, Burgess AM, Curthoys IS. Vestibular function in Lermoyez syndrome at attack. Eur Arch Otorhinolaryngol. 2012 Feb;269(2):685-691.
- 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.
- Schmidt PH, Schoonhoven R. Lermoyez's syndrome. A follow-up study in 12 patients. Acta Otolaryngol. 1989 May-Jun;107(5-6):467-473.
- 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.
- Maier W, Marangos N, Aschendorff A. Lermoyez syndrome--electrocochleographic studies. Laryngorhinootologie. 1996 Jun;75(6):372-376. [German]
- 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.
- 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.
- 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.