Labyrinthine Disorders Research Articles

Case Report: - Sudden sensorineural hearing loss and vertigo due to inner ear hemorrhage

Sudden deafness is a common cause of emergency consultation in otology. Usually, despite investigations, no etiology is known. Intracochlear hemorrhage is a rare cause of sudden sensorineural hearing loss (SSNHL) and may be associated with various labyrinthine disorders. In such cases, magnetic resonance imaging (MRI) is the clef of the diagnosis. We report the case of a 70-year-old patient who was referred to our department for sudden hearing loss, tinnitus, and vertigo. Otoscopic and neurological examinations were normal, and pure-tone audiometry revealed left profound sensorineural hearing loss. Videonystagmography (VNG) revealed left vestibular deficit. MRI demonstrated a high signal intensity inside the cochlea on unenhanced T1-weighted images, and no other abnormalities were found; in particular, no enhancement after intravenous administration of gadolinium. No etiology was identified. Vertigo disappeared rapidly with corticosteroid treatment and hyperbaric oxygen therapy, but hearing did not improve. Intra-labyrinthine hemorrhage causing SSNHL is rare, and the hearing prognosis is poor.

Case Report - Sudden sensorineural hearing loss and vertigo due to inner ear hemorrhage

Sudden deafness is a common cause of emergency consultation in otology. Usually, despite investigations, no etiology is known. Intracochlear hemorrhage is a rare cause of sudden sensorineural hearing loss (SSNHL) and may be associated with various labyrinthine disorders. In such cases, magnetic resonance imaging (MRI) is the clef of the diagnosis. We report the case of a 70-year-old patient who was referred to our department for sudden hearing loss, tinnitus, and vertigo. Otoscopic and neurological examinations were normal, and pure-tone audiometry revealed left profound sensorineural hearing loss. Videonystagmography (VNG) revealed left vestibular deficit. MRI demonstrated a high signal intensity inside the cochlea on unenhanced T1-weighted images, and no other abnormalities were found; in particular, no enhancement after intravenous administration of gadolinium. No etiology was identified. Vertigo disappeared rapidly with corticosteroid treatment and hyperbaric oxygen therapy, but hearing did not improve. Intra-labyrinthine hemorrhage causing SSNHL is rare, and the hearing prognosis is poor.

Seltene Erkrankungen des vestibulären Labyrinths: von Zebras, Chamäleons und Wölfen im Schafspelz

The differential diagnosis of vertigo syndromes is a challenging issue, as many – and in particular – rare disorders of the vestibular labyrinth can hide behind the very common symptoms of “vertigo” and “dizziness”. The following article presents an overview of those rare disorders of the balance organ that are of special interest for the otorhinolaryngologist dealing with vertigo disorders. For a better orientation, these disorders are categorized as acute (AVS), episodic (EVS) and chronic vestibular syndromes (CVS) according to their clinical presentation. The main focus lies on EVS sorted by their duration and the presence/absence of triggering factors (seconds, no triggers: vestibular paroxysmia, Tumarkin attacks; seconds, sound and pressure induced: “third window” syndromes; seconds to minutes, positional: rare variants and differential diagnoses of benign paroxysmal positional vertigo; hours to days, spontaneous: intralabyrinthine schwannomas, endolymphatic sac tumors, autoimmune disorders of the inner ear). Furthermore, rare causes of AVS (inferior vestibular neuritis, otolith organ specific dysfunction, vascular labyrinthine disorders, acute bilateral vestibulopathy) and CVS (chronic bilateral vestibulopathy) are covered. In each case, special emphasis is laid on the decisive diagnostic test for the identification of the rare disease and “red flags” for potentially dangerous disorders (e. g. labyrinthine infarction/hemorrhage). Thus, this chapter may serve as a clinical companion for the otorhinolaryngologist aiding in the efficient diagnosis and treatment of rare disorders of the vestibular labyrinth.

A travel between the Corpus Hippocraticum and the Gazette médicale de Paris: vestibology from its origins to the nineteenth century

Background: In ancient times, dizziness was divided into two different forms: ílingos (true and proper vertigo) and skotódinos (vertigo associated with blurred vision). For centuries, it was considered as a cerebral affection: the otologic aetiology remained unthinkable. At the end of the sixteenth century, we find the first written documentation of the otovestibular triad pathognomonic for endolymphatic hydrops: the jurist Bonaventura Angeli from Ferrara, thanks to his symptoms’ description, can rightfully be considered the first patient with Ménière’s syndrome whom we have historical information. From that, the anatomical and physiological studies of balancing system continued: after Valsalva, a first evolution of medical thinking came with the recognition of the presence of liquid, and not air, inside the labyrinth. With Flourens, the principle that the semicircular canals were a vestibular organ, began to emerge.Purpose: The rationale to perform this research lied in the need to investigate a fascinating chapter of the history of medicine, regarding a difficult subject of otorhinolaryngology: the vestibular system.Methods: We collected a variety of documents, from Ancient Greek time to nineteenth century, related to the studies of vestibular pathologies, highlighting the earliest evidences of the association between hearing disorders and balance complaints. The finally selected papers were methodologically evaluated.Results: Because of the frequent association between vestibular and hearing disorders, the hypothesis that ear could be involved both in the sense of hearing and balance gradually advanced across the centuries, until Prosper Ménière’s observations: from 1861 onwards, vertigo was recognized not only as a brain alteration, but also as an otologic disorder.Conclusions: Although there has been an obvious medical developments regarding the inner ear function, labyrinthine disorders remain a challenging field yet to be explored.

Increased Velocity Storage in Subjects with Meniere's Disease.

Velocity storage mechanism is a multisensory rotation estimator; it compensates for errors in the information provided by the peripheral vestibular organs by means of an adjustment in the duration of the vestibular signal. The aim of this study was to determine the activity of the velocity storage mechanism in the presence of a labyrinthine disorder, using galvanic vestibular stimulation to cause direct activation of the vestibular afferent neurons. Forty-one subjects with definite Meniere's disease (MD) and 36 healthy volunteers were evaluated using a 20-s galvanic vestibular stimulation. We found a post-stimulus nystagmus overshoot exclusively in subjects with MD (47% in subjects with unilateral disease and 82% in subjects with bilateral disease), but no overshoot in healthy subjects. Because post-stimulus nystagmus overshoot is caused by the velocity storage mechanism, this finding suggests an increase in the velocity storage in subjects with a labyrinthine disease.

Chapter 18 - Benign paroxysmal positional vertigo and its variants

Benign paroxysmal positional vertigo is a common labyrinthine disorder caused by a mechanic stimulation of the vestibular receptors within the semicircular canals. It is characterized by positional vertigo and positional nystagmus, both provoked by changes in the position of the head with respect to gravity. The social impact of the disease and its direct and indirect costs to healthcare systems are significant owing to impairment of daily activities and increased risk of falls. The first description of a patient with benign paroxysmal positional vertigo is from Robert Bárány in 1921, but the features of the syndrome and the diagnostic maneuver were well described by Dix and Hallpike in 1952. Since then, the gradually increasing interest of otolaryngologists and neurologists has led to a progressive advance in the knowledge of this labyrinthine disorder with regard to its epidemiologic, pathophysiologic, clinical, and therapeutic aspects. Despite the often effective diagnosis and treatment of most cases of benign paroxysmal positional vertigo, the physiopathologic explanations of the disease are mainly speculative. In this chapter, we describe the epidemiologic, pathophysiologic, clinical, and therapeutic aspects of benign paroxysmal positional vertigo.

Glucose metabolism disorders and vestibular manifestations: evaluation through computerized dynamic posturography

IntroductionGlobal sugar consumption has increased in the past 50 years; its abusive intake is responsible for peripheral insulin resistance, which causes the metabolic syndrome – obesity, diabetes mellitus, hypertension, and coronary heart disease. ObjectiveTo evaluate the effect of a fractionated diet without glucose as treatment for labyrinthine disorders associated with glucose–insulin index. MethodsThe study design was a prospective randomized controlled trial. Fifty-one patients were divided into two groups: the diet group (DG), which comprised subjects treated with a fractionated diet with glucose restriction, and the control group (CG), in which individuals were not counseled regarding diet. Patients underwent computerized dynamic posturography (CDP) and visual analog scale (VAS) on the first and 30th days of the study. ResultsThere was improvement in the assessed posturographic conditions and VAS self-assessment in the DG group after 30 days when compared to the control group. ConclusionThe fractionated diet with glucose restriction was effective for the treatment of vestibular dysfunction associated with glucose metabolism disorders.

P10. Magnetic vestibular stimulation (MVS) influences fMRI resting-state fluctuations. The modulation of the default-mode network as an exemplary case

Introduction Dizziness in the presence of strong magnetic fields has been noticed ever since the first magnetic resonance experiments at high field strengths (>1 Tesla) have been conducted. It was suggested that this was due to the changing magnetic flux in the inner ear induced by the gross subject-motion within the magnetic field and that the motionless presence in the static magnetic field could not affect subjects significantly ( Schenck, 1992 ). Recently however, Roberts et al. (2011) showed that healthy subjects at rest who are kept in total darkness and exposed to the static magnetic field of an MR tomograph developed a persistent nystagmus, while patients with bilateral peripheral vestibular failure did not. Furthermore, the nystagmus’ slow phase velocity was modulated systematically depending on the subject’s head orientation relative to the field. The authors argued that the ionic fluids in the inner ear, which are constantly flowing as cells maintain resting activity, will be diverted by a (magnetic) Lorentz-force and this creates a pressure onto the cupula “the rotatory motion sensor” in the inner ear, thus leading to a nystagmus akin to a constant (accelerating) rotatory stimulation. This model was further supported by a simulation study ( Antunes et al., 2012 ) and a recent study of patients with unilateral labyrinthine disorders ( Ward et al., 2014 ). It was speculated that this magnetic vestibular stimulation (MVS) effect might influence fMRI results, because a nystagmus is indicative for a vestibular imbalance. Methods The aim of the current study was to investigate if this MVS effect does indeed modulate the fluctuations of the BOLD signal in areas related to the vestibular system. Here we treat the case of MVS influencing resting-state networks, presenting results from resting-state experiments conducted at two different field strengths (1.5 Tesla & 3 Tesla) and focus in particular on the influence of MVS on the default mode network. Results Those subparts of the default mode network that were significantly modulated between field strengths were also commonly associated with vestibular functions. Furthermore, the signal-scaling behavior of these subparts was different from the other parts of the default mode network that were not significantly modulated. In fact, the signal-scaling behavior was in agreement with a modulation due to MVS as expected from a Lorentz-model perspective (i.e. linear increase) while the other parts that were not significantly modulated by the MVS effect showed a scaling behavior that was expected simply from considering fMRI signal-scaling behavior due to increased field strength (i.e. approximately square root increase, e.g. see ( Duyn, 2012 )). Conclusion The MVS effect significantly affected BOLD signal modulation. Those parts which were more closely connected to the vestibular peripheral sense-organs did modulate in accordance with the Lorentz-model prediction and those subparts which modulated less, but still modulated significantly above the expected increase due to field-strength related signal-increase, were further removed in terms of connectivity from the vestibular peripheral sense-organs and therefore were modulated less strongly by MVS.

Magnetic Vestibular Stimulation in Subjects with Unilateral Labyrinthine Disorders

We recently discovered that static magnetic fields from high-strength MRI machines induce nystagmus in all normal humans, and that a magneto-hydrodynamic Lorentz force, derived from ionic currents in the endolymph and pushing on the cupula, best explains this effect. Individuals with no labyrinthine function have no nystagmus. The influence of magnetic vestibular stimulation (MVS) in individuals with unilateral deficits in labyrinthine function is unknown and may provide insight into the mechanism of MVS. These individuals should experience MVS, but with a different pattern of nystagmus consistent with their unilateral deficit in labyrinthine function. We recorded eye movements in the static magnetic field of a 7 T MRI machine in nine individuals with unilateral labyrinthine hypofunction, as determined by head impulse testing and vestibular-evoked myogenic potentials (VEMP). Eye movements were recorded using infrared video-oculography. Static head positions were varied in pitch with the body supine, and slow-phase eye velocity (SPV) was assessed. All subjects exhibited predominantly horizontal nystagmus after entering the magnet head-first, lying supine. The SPV direction reversed when entering feet-first. Pitching chin-to-chest caused subjects to reach a null point for horizontal SPV. Right unilateral vestibular hypofunction (UVH) subjects developed slow-phase-up nystagmus and left UVH subjects, slow-phase-down nystagmus. Vertical and torsional components were consistent with superior semicircular canal excitation or inhibition, respectively, of the intact ear. These findings provide compelling support for the hypothesis that MVS is a result of a Lorentz force and suggest that the function of individual structures within the labyrinth can be assessed with MVS. As a novel method of comfortable and sustained labyrinthine stimulation, MVS can provide new insights into vestibular physiology and pathophysiology.

Audiovestibular impairment presenting as a portent of vertebrobasilar infarction

Acute audiovestibular symptoms (hearing loss and vertigo) can be caused by labyrinthine disorders such as viral labyrinthitis, cerebrovascular diseases, perilymphatic fistula and acoustic neuroma. If neurological symptoms are not present, it is more likely that viral labyrinthitis is the cause, especially in the absence of abnormal findings on imaging (Lee et al, 2002).

A possible mechanism to explain how ischaemia and the maintained function of gastric type proton pumps could lead to hydrops of the inner ear in Ménière's disease

A possible explanation of the genesis of Ménière's disease (MD) and other labyrinthine disorders on the basis of a direct involvement of the inner ear gastric type proton pump under ischaemia was recently outlined: maintained activity of the proton pump under acidic conditions could favour the onset of an endolymphatic hydrops through enhanced ion concentration in the endolymph and subsequent osmotic movement. The details of such a mechanism could be explained by considering the capability of sensory cells to extrude H+ into the endolymph in exchange for K+ through organellar isoforms that can transiently appear on plasma membranes and permit an electroneutral ion exchange. This capability appears to be rapidly activated under acidic conditions, which can be a consequence of a local ischaemia. This could mean that in an acidic milieu with an enhanced intracellular amount of H+, provided that the proton pumps maintain their activity as in other organs, a greater amount of K+ can finally be involved in the physiological cycling process and enter into the endolymph; this hypothesis seems reliable and could explain how an osmotic mechanism causing the onset of the hydrops could paradoxically be related to defensive responses to a vasospasm.

Vestibular rehabilitation using the Nintendo® Wii Balance Board – a user-friendly alternative for central nervous compensation

Conclusion: The Nintendo® Wii Balance Board is a cost-effective and user-friendly alternative to other popular frequently used systems that aid vestibular compensation, particularly in elderly patients. In addition, further treatment in the home environment is possible. Objective: This cohort study was designed to investigate the impact of the Nintendo® Wii Balance Board as a visual compensation device after acute vestibular neuritis. Methods: Subjects were randomly assigned to one of two treatment groups. Group A (n = 37) performed customized exercises with the Nintendo® Wii Balance Board. Group B (n = 34) performed only two elected exercises as a control group for comparison of the results. Both groups underwent additive therapy with steroids (intravenous) in decreasing doses (250 mg decreasing to 25 mg over 10 days). The Sensory Organization Test (SOT), Dizziness Handicap Inventory (DHI), Vertigo Symptom Scale (VSS), and Tinneti questionnaire were evaluated immediately before treatment (baseline), at the end of treatment, i.e. at day 5, and after 10 weeks. Results: The early use of a visual feedback system in the context of the balance training supports the central nervous vestibular compensation after peripheral labyrinthine disorders. Patients in group B (without training) required a longer in-patient stay (average 2.4 days, SD 0.4) compared with patients following early Wii rehabilitation. The absence of nystagmus under Frenzel's goggles in group A was observed 2.1 days (SD 0.5) earlier than in group B. Group A showed significantly better results in the SOT, DHI, VSS, and Tinneti questionnaire at all time points measured (p < 0.05).

The fragility of the hemodynamic balance in the elderly: possible influences on the inner ear

The possible occurrence of labyrinthine disorders linked to hemodynamic imbalances has been widely outlined [1-4] and well demonstrated by clinical observations concerning both young and healthy people [4] and subjects suffering from cardiovascular affections as hypertension the prevalence of tinnitus was studied in patients under antihypertensive [5] or heart failure of variable severity [6]. In all cases, a possible influence of a sharp decrease in the values of blood pressure followed by an abnormal vasomotor reaction was postulated to have some role in the genesis of the disorder, mostly when considering that blood supply of the inner ear is of terminal type. In the elderly, the problem is even more complicated by the frequent presence of isolated systolic hypertension, which is characterized by the lowered compliance of the vascular tree, presenting with an increase of the arterial stiffness and reported to be due both to age-related loss of distensibilty in the major central arteries and to endothelial dysfunction [7]: this increases, in general terms, the risk of transient hypoperfusion to an organ with a terminal circulation as the labyrinth and represents a crucial factor to deal with when treating hypertension in elderly. Moreover, the possibility of the so-called “hypertension-hypotension syndrome” [8], i.e. the coexistence of supine hypertension and orthostatic hypotension as a result of dysautonomia [7] represents a further complication in this sense. Hence the necessity of choosing, when possible, an antihypertensive therapy able to protect the endothelial wall and to avoid brusque falls of peripheral oxygenation which could damage the inner ear. Author details Department of Specialist Surgical and Anesthesiological Sciences, University of Bologna, Italy. Posgraduate School of Audiology and Phoniatrics, University of Bologna, Italy.

Antihypertensive Drug and Inner Ear Perfusion: An Otologist's Point of View.

A number of labyrinthine disorders with sensorineural hearing loss, vertigo, and tinnitus are known to occur to young people without vascular risk factors, thus being classified as “idiopathic” in the absence of satisfactory explanations; in the last decade, this phenomenon has found a reliable explanation by the adverse effect of a sharp decrease of blood pressure values followed by an abnormal vasomotor regulation. This model may not only be applied to healthy subjects, but even had some confirmation in conditions possibly affecting hemodynamic changes, such as heart failure or treated hypertension. In particular, the results of a recent study on the impact of different antihypertensive therapies, which was analyzed by monitoring the onset or enhancement of tinnitus as a symptom of inner ear sufferance, unequivocally demonstrated an increased prevalence of tinnitus in subjects submitted to more “aggressive” treatments. This seems in agreement with recent observations about the model of fluid homeostasis of the inner ear, and suggests, when possible, to resort to treatments with modulatory effects in order to maintain a steady perfusion to the labyrinth thus protecting its function.

Lateral Canal Paroxysmal Positional Vertigo Revisited

The first reports of an involvement of the lateral canal (LC) in paroxysmal positional vertigo (PPV), were published in 1985, by Luciano Cipparrone et al., from Italy and Joseph McClure from Canada. The increasing interest of otolaryngologists and neurologists has led to a progressive advance in the knowledge of this labyrinthine disorder regarding its epidemiological, physiopathological, clinical, and therapeutic aspects. According to the most recent data, LC-benign PPV accounts for 17% of all PPV patients, regardless of gender and between the two labyrinths. The LC-PPV syndrome is characterized by intense positional vertigo and direction-changing geotropic horizontal nystagmus, both caused by rotation of the head in the supine position. Less frequently, it presents with apogeotropic nystagmus. In some patients nystagmus is also detectable in the sitting position, mimicking a spontaneous nystagmus. In most cases nystagmus is caused by displaced otoconia floating in the semicircular canal. The pathological side, which must be identified for successful treatment, is usually indicated by nystagmus intensity: the more intense positional nystagmus beats toward the affected ear. In a few cases, where there is no difference in nystgmus intensity, other indicators are necessary to determine the pathological side. Vestibular neuritis and posterior fossa lesions should be considered in the differential diagnosis. Treatment of LC-PPV relies on some physical maneuvers, the objective of which is to allow the otoconial debris to exit from the LC by centrifugal inertia and/or by gravitation.

Haemodynamic profile of young subjects with transient tinnitus

AbstractObjective: Our objective was to contribute to drawing a haemodynamic profile of healthy subjects prone to labyrinthine disorders of functional origin. The aim was to determine if some haemodynamic aspects could characterize young people with a history of transient tinnitus, considered as an early symptom of cochlear damage possibly derived from hypoperfusion. In one year we studied 60 consecutive subjects (28 ± 5.2, range 18–40 years): 24 who experienced transient tinnitus, and 36 without tinnitus. Exclusion criteria were a history of audiological and otological impairment, ear surgery, and known cardiac abnormalities. A clinical and echographic cardiac evaluation was performed, with assessment of blood pressure, heart rate, and left ventricular structure and function. Results: All results were within the normal range in both groups. The tinnitus group had a slightly lower body mass index (BMI) (p = 0.05) and body surface area (BSA) (p <0.05), while age, blood pressure and heart rate were similar ...

Nausea and vomiting

Nausea and vomiting are symptoms resulting from a triggered emetic reflex. Many endogenous and exogenous triggering factors can activate the emetic reflex, making understanding difficult and therapy challenging. The key to managing most cases of nausea and vomiting lies in a good history and a detailed physical examination. Most episodes of acute vomiting (lasting < 48 hours) have an evident triggering factor (eg, infection, viral illness, or food poisoning) and can be managed by removing the triggering agent and via supportive therapy. Chronic and unexplained nausea and vomiting can be a challenge. The cause is often obscure and requires special investigation. Functional gastroduodenal disorders such as cyclic vomiting syndrome, functional vomiting, and chronic idiopathic nausea should be considered if investigations are unrevealing. Knowledge regarding various emetic pathways and the specific neurotransmitters involved helps to target therapy. Histamine-1 receptor antagonists and muscarinic antagonists are suitable candidates for motion sickness and labyrinthine disorders. Phenothiazines, 5-hydroxytryptamine-3 receptor antagonists, corticosteroids, and benzodiazepines have a role in postchemotherapy and postoperative nausea and vomiting. Cannabinoid and neurokinin-1 receptor antagonists are best reserved for refractory cases of nausea and vomiting. Motilin agonists and metoclopramide are useful for treating impaired gastric motility disorders.

Gastric type proton pump of the inner ear: Its possible involvement in labyrinthine disorders

It was recently suggested that the favourable effect of proton pump inhibitors on gastric reflux could threaten the homeostasis of the inner ear in several different ways. Moreover, when it is considered that a gastric type proton pump has been found in the inner ear, it could be hypothesized that this treatment has a direct effect. As ischaemia threatens gastric mucosa even by acid secretion, thus implying resistance of the proton pump to damaging factors, a similar kind of mechanism could be imagined in the inner ear, where it could even cause the osmotic changes possibly involved in Meniere's disease hydrops. This hypothesis, if proved, could open new perspectives on some not fully understood phenomena and lead to finding more satisfactory therapeutic options.

Estimulação calórica gelada com ar nas vestibulopatias periféricas crônicas com nistagmo espontâneo

OBJETIVO: analisar o efeito da estimulação gelada com ar a 10ºC sobre o nistagmo pós-calórico em pacientes com vestibulopatias periféricas crônicas que apresentam nistagmo espontâneo com olhos fechados. MÉTODOS: 61 pacientes foram submetidos às estimulações com ar a 42, 18 e 10ºC. RESULTADOS: em 42 casos (69,8%) foram encontrados valores anormais de preponderância direcional e/ou de predomínio labiríntico a 42 e 18ºC. A prova a 10ºC apresentou valores de assimetria dentro dos padrões de normalidade em 52,5% dos casos e valores anormais de assimetria em 16,4% (p=0,012), confirmou hiporreflexia unilateral em 11,5% e identificou anormalidades não evidenciadas a 42 e 18ºC em 8,2%. CONCLUSÃO: a estimulação gelada com ar a 10ºC possibilitou retirar a influência do nistagmo espontâneo de olhos fechados sobre o nistagmo pós-calórico em pacientes com vestibulopatias periféricas crônicas.

Nystagmus as a Sign of Labyrinthine Disorders: Three-Dimensional Analysis of Nystagmus

In order to diagnose the pathological condition of vertiginous patients, a detailed observation of nystagmus in addition to examination of body equilibrium and other neurotological tests are essential. How to precisely record the eye movements is one of the goals of the researchers and clinicians who are interested in the analysis of eye movements for a long time. For considering that, one has to think about the optimal method for recording eye movements. In this review, the author introduced a new method, that is, an analysis of vestibular induced eye movements in three-dimensions and discussed the advantages and limitations of this method.