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Background: Meniere disease, characterized by intermittent episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural pressure, is a common cause of vertigo in humans. The pathogenesis of Meniere disease remains unknown. The current study aimed to describe a novel pathological change discovered in the inner ears of patients with Meniere disease who underwent labyrinthectomy. Methods: This retrospective case-control study was conducted with 21 patients with MD who underwent labyrinthectomy. A total of 15 patients diagnosed with acoustic neuroma or glomus jugular tumor were review over the same period of time as control. The clinical information of the patients and the pathological features of the membrane are described. Results: The new pathological tissue was a morbid membrane structure sealing the round window, characterized by the formation of lymphatic capillaries. Histochemical and immunofluorescent staining was positive for D2-40, LYVE-1, podoplanin, and PROX1, which are the classical markers of the lymphatic vessels. Transmission electron microscopy revealed that the lymph capillaries lacked a typical basement membrane and that their ends were blind, composed of a single layer of endothelial cells with valval connection structures between adjacent capillary epithelial cells. Conclusion: This is the first report of lymphatic vessels in the human inner ear, and this pathological structure is a completely new discovery. The lymphatic vessels may develop due to inflammation or decompensation of pressure in the inner ear, suggesting that the inner ear can reactively form lymphatic vessels in some inflammation and fluid flow-dependent pathological conditions. The current findings help in improving our understanding of the pathogenesis of Meniere disease.
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Nystagmus induced by applying an intense vibratory stimulus to the skull (SVIN) indicates vestibular functional asymmetry. In unilateral vestibular loss, a 100 Hz bone-conducted vibration given to either mastoid immediately causes a primarily horizontal nystagmus. The test is performed in darkness to avoid visual fixation (VF) but there are no data about how much VF affects the often-intense SVIN. The aim is to analyze the amount of reduction in SVIN when VF is allowed during testing. Thus, all patients seen in a tertiary hospital for vertigo or dizziness with positive SVIN were included. SVIN was recorded for 10 s for each condition: without VF (aSVINwo) and with VF (aSVINw). We obtained an aSVINwo and an aSVINw as average slow-phase velocities (SPV) without and with VF. VF index (FISVIN) was calculated as the ratio of SPV. Among the 124 patients included, spontaneous nystagmus (SN) was found in 25% and the median slow phase velocity (mSPV) (without VF) of SN was 2.6 ± 2.4°/s. Mean FISVIN was 0.27 ± 0.29. FISVIN was 0 in 42 patients, and FISVIN between 0 and 1 was found in 82 (mean FISVIN 0.39 ± 0.02). Fixation suppression was found in all patients with SVIN in cases of peripheral vestibulopathy. FISVIN clearly delineates two populations of patients: with or without a complete visual reduction in nystagmus.
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Benign paroxysmal positional vertigo is the most common cause of peripheral vertigo. It is characterized by short and recurrent episodes of vertigo, trigged by specific head movements that displace otoconia within the semicircular canals. The movement of dislodge otoconia from the utricle cause abnormal positional endolymphatic currents. Primary treatment involves reposition maneuvers aimed at moving the displaced otoconia out the affected canal, therefore correct identification of the affected canal is essential for the diagnosis. The posterior semicircular canal (PSC) is the most frequently affected due to its spatial orientation and the force of gravity. Recent technological advances have allowed for better assessment of positional nystagmus during diagnostic and therapeutic maneuvers, revealing various possible scenarios of PSC involvement. Regarding the PSC, otoconia may be found in different parts of the canal, and not just in the expected location, floating in the long arm of the canal. The understanding of these variants is crucial, as the prognosis and the disease progression differ in such cases. This review aims to describe the six possible variants of PSC involvement described so far.
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Introduction: Cochlear implantation is currently regarded as a safe and minimally invasive procedure. However, cochlear implantation can have an impact on vestibular function, despite the lack of correlation between patient symptomatology and damage in vestibular tests. Thus, the present study aims to analyze the presence of hydrops and histological reactions at the level of the vestibule after cochlear implantation with dexamethasone pump delivery in Macaca fascicularis (Mf). Materials and methods: A detailed histological study was conducted on a total of 11 Mf. All 11 Mf were divided into three groups: 5 Mf were implanted with an electrode array HL-14 connected to a pump delivering FITC-dextran for 24 h (Group A); 4 Mf were implanted with a CI electrode array attached to a pump for FITC-dextran delivery for 7 days (Group B); and 2 Mf were considered the control group, without any kind of cochlear device implantation (Group C). After drug deliver, the selected macaques were euthanized to collect tissue samples for histological analysis. An experienced observer, focusing on the utricle and saccule areas, conducted a blinded inner ear histology analysis. Results: Surgical procedures were successfully performed in all cases. No signs of cochlear reaction to the device were observed, including neither collapse nor fibrosis. Endolymphatic sinus dilatation was observed in Mf4A and Mf3B, while cochlear hydrops was observed in Mf3A. The mean areas of the utricle and saccule exhibited some statistically significant differences, specifically, in the saccule between groups C and both groups A (p = 0.028) and B (p = 0.029); however, no significant differences were observed between groups A and B or among comparisons of the utricle. Discussion: A significant concern relates to the safety of cochlear implantation with regard to vestibular preservation and hearing. New advancements in electrode arrays, such as CI devices coupled with delivery pumps, pose a challenge in maintaining minimally traumatic surgical concept-based procedures without affecting the inner ear homeostasis. The implantation of this device may cause vestibular hydrops in the saccule, indicating that the longer the time of substance release, the greater the grade of hydrops evidenced at the saccular level. Apart from this finding, the risk of histological damage to the vestibule is low.
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BACKGROUND: Inner ear hemorrhage (IEH) is an increasingly recognized cochlear lesion that can cause sensorineural hearing loss (SNHL). Magnetic resonance imaging (MRI) is known to be the best imaging modality for clarifying the causes of SNHL and providing images that point to those causes. AIMS: Evaluate the lesional patterns in patients with presumed Inner ear hemorrhage (IEH) from radiological and functional aspects. MATERIAL AND METHODS: We retrospectively reviewed 10 patients performed in our institution from 2014 to 2020, with suspected labyrinthine hemorrhage based on radiological and functional examination. RESULTS: We included 8 patients with IEH and sensorineural hearing loss (SNHL). The median age was 55 years (range: 3 months - 78 years). The results from the MRI and functional tests were compared for each end-organ. Only three cases (37.5%) showed a correlation between signal abnormalities and dysfunction in the labyrinthine apparatus. CONCLUSIONS: In patients with SNHL inner ear hemorrhage needs to be ruled out in the differential diagnosis, so specific MRI sequences should be requested. It represents a way to a better understanding of the disorder and the variety of findings claim for a complete auditory and vestibular testing.
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Enfermedades del Oído , Oído Interno , Pérdida Auditiva Sensorineural , Pérdida Auditiva Súbita , Humanos , Persona de Mediana Edad , Estudios Retrospectivos , Pérdida Auditiva Súbita/diagnóstico , Oído Interno/diagnóstico por imagen , Enfermedades del Oído/complicaciones , Pérdida Auditiva Sensorineural/diagnóstico por imagen , Pérdida Auditiva Sensorineural/etiología , Imagen por Resonancia Magnética/métodos , Hemorragia/diagnóstico por imagen , Hemorragia/complicacionesRESUMEN
BACKGROUND: The 3D-REAL-IR MRI sequence allows for an in vivo visualization of endolymphatic hydrops. Qualitative assessment methods of the severity of vestibular and cochlear hydrops are the most commonly used. METHODS: A quantitative volumetric measurement of vestibular EH in patients with definite unilateral Ménière's disease using the 3D-REAL-IR sequence and the calculation of the endolymphatic ratio (ELR) was intended. RESULTS: Volumetric calculations of the vestibules, vestibular endolymph and vestibular ELR are performed in 96 patients with unilateral Ménière's disease and correlated with classic qualitative grading scales. CONCLUSIONS: Quantitative volumetric measurement of vestibular hydrops using the 3D-REAL-IR sequence is feasible and reproducible in daily clinical practice. Vestibular ELR values exceeding 60% defined radiologically significant vestibular hydrops, while values below 30% defined radiologically non-significant vestibular hydrops.
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Vestibular medicine "embraces a wide approach to the potential causes of vestibular symptoms, acknowledging that vertigo, dizziness, and unsteadiness are non-specific symptoms that may arise from a broad spectrum of disorders, spanning from the inner ear to the brainstem, cerebellum and supratentorial cerebral networks, to many disorders beyond these structures" [...].
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BACKGROUND: Rafael Lorente de Nó was a neuroscientist that worked alongside two of the giants of Medicine, the Nobel Prize winners Cajal and Bárány. OBJECTIVE: To describe the contributions of Lorente de Nó to vestibular neuroscience. METHODS: Detailed review of the publications of Lorente de Nó and analysis of the archives from Junta para Ampliación de Estudios e Investigaciones Científicas at Residencia de Estudiantes (Madrid, Spain), Casa de Salud Valdecilla at Hospital Universitario Marqués de Valdecilla (Santander, Spain), Becker Medical Library at Washington University (St. Louis, MO, USA), Rockefeller Archive Center (Sleepy Hollow, New York, NY, USA), Archivo Fernando de Castro (Madrid, Spain), Biblioteca Nacional de España (Madrid, Spain) and Legado Cajal at Instituto Cajal (Madrid, Spain). Most of this material is unpublished and includes over a hundred letters to or from Lorente. RESULTS: Lorente de Nó made a substantial contribution to our understanding of the vestibular system. Amongst these, he meticulously detailed the course of the vestibular nerve and its central projections. He described the vestibulo-ocular reflex as the consequence of an integration of the various nuclei and connections across the vestibular system, rather than a simple three-neuron arc. He also highlighted the role of the reticular formation in the generation of the fast phase of the nystagmus. CONCLUSIONS: Lorente de Nó was a pioneer of modern neuro-otology, having made outstanding contributions to vestibular neuroscience, forging novel discoveries that still burn true today.
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Benign paroxysmal positional vertigo (BPPV) and bilateral vestibulopathy (BVL) are two completely different forms of vestibular disorder that occasionally occur in the same patient. We conducted a retrospective review searching for that coincidence in our database of the patients seen over a 15-year period and found this disorder in 23 patients, that is 0.4%. More frequently they occurred sequentially (10/23) and BPPV was diagnosed first. Simultaneous presentation occurred in 9/23 patients. It was subsequently studied, but in a prospective manner, in patients with BPPV on all of whom a video head impulse test was performed to search for bilateral vestibular loss; we found it was slightly more frequent (6/405). Both disorders were treated accordingly, and it was found that the results follow the general trend in patients with only one of those disorders.
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Cochlear implantation surgery (CI) is considered a safe procedure and is the standard treatment for the auditory rehabilitation in patients with severe-to-profound sensorineural hearing loss. Although the development of minimally traumatic surgical concepts (MTSC) have enabled the preservation of residual hearing after the implantation, there is scarce literature regarding the vestibular affection following MTCS. The aim of the study is to analyze histopathologic changes in the vestibule after CI in an animal model (Macaca fascicularis). Cochlear implantation was performed successfully in 14 ears following MTCS. They were classified in two groups upon type of electrode array used. Group A (n = 6) with a FLEX 28 electrode array and Group B (n = 8) with HL14 array. A 6-month follow-up was carried out with periodic objective auditory testing. After their sacrifice, histological processing and subsequent analysis was carried out. Intracochlear findings, vestibular presence of fibrosis, obliteration or collapse is analyzed. Saccule and utricle dimensions and neuroepithelium width is measured. Cochlear implantation was performed successfully in all 14 ears through a round window approach. Mean angle of insertion was >270° for group A and 180-270° for group B. In group A auditory deterioration was observed in Mf 1A, Mf2A and Mf5A with histopathological signs of scala tympani ossification, saccule collapse (Mf1A and Mf2A) and cochlear aqueduct obliteration (Mf5A). Besides, signs of endolymphatic sinus dilatation was seen for Mf2B and Mf5A. Regarding group B, no auditory deterioration was observed. Histopathological signs of endolymphatic sinus dilatation were seen in Mf 2B and Mf 8B. In conclusion, the risk of histological damage of the vestibular organs following minimally traumatic surgical concepts and the soft surgery principles is very low. CI surgery is a safe procedure and it can be done preserving the vestibular structures.
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Implantación Coclear , Implantes Cocleares , Animales , Implantación Coclear/efectos adversos , Implantación Coclear/métodos , Macaca fascicularis , Implantes Cocleares/efectos adversos , Ventana Redonda/cirugía , Pruebas AuditivasRESUMEN
Abstract Introduction In patients with benign paroxysmal positional vertigo, BPPV; a torsional-vertical down beating positioning nystagmus can be elicited in the supine straight head-hanging position test or in the Dix-Hallpike test to either side. This type of nystagmus can be explained by either an anterior canal BPPV or by an apogeotropic variant of the contralateral posterior canal BPPV Until now all the therapeutic maneuvers that have been proposed address only one possibility, and without first performing a clear differential diagnosis between them. Objective To propose a new maneuver for torsional-vertical down beating positioning nystagmus with a clear lateralization that takes into account both possible diagnoses (anterior canal-BPPV and posterior canal-BPPV). Methods A prospective cohort study was conducted on 157 consecutive patients with BPPV. The new maneuver was performed only in those with torsional-vertical down beating positioning nystagmus with clear lateralization. Results Twenty patients (12.7%) were diagnosed with a torsional-vertical down beating positioning nystagmus. The maneuver was performed in 10 (6.35%) patients, in whom the affected side was clearly determined. Seven (4.45%) patients were diagnosed with an anterior canal-BPPV and successfully treated. Two (1.25%) patients were diagnosed with a posterior canal-BPPV and successfully treated with an Epley maneuver after its conversion into a geotropic posterior BPPV. Conclusion This new maneuver was found to be effective in resolving all the cases of torsional-vertical down beating positioning nystagmus-BPPV caused by an anterior canal-BPPV, and in shifting in a controlled way the posterior canal-BPPV cases of the contralateral side into a geotropic-posterior-BPPV successfully treated during the followup visit. Moreover, this new maneuver helped in the differential diagnosis between anterior canal-BPPV and a contralateral posterior canal-BPPV.
Resumo Introdução Em pacientes com vertigem posicional paroxística benigna, VPPB, um nistagmo vertical para baixo com componente de torção pode ser provocado no teste head hanging supino executado na posição reta ou no teste de Dix-Hallpike para qualquer um dos lados. Esse tipo de nistagmo pode ser explicado por uma VPPB do canal anterior ou por uma variante apogeotrópica da VPPB do canal posterior contralateral. Até agora, todas as manobras terapêuticas propostas abordam apenas uma possibilidade, sem antes fazer um diagnóstico diferencial claro entre elas. Objetivo Propor uma nova manobra para nistagmo vertical para baixo com componente de torção com uma lateralização clara que leve em consideração os dois diagnósticos possíveis, VPPB do canal anterior e VPPB do canal posterior. Método Um estudo de coorte prospectivo foi conduzido em 157 pacientes consecutivos com VPPB. A nova manobra foi feita apenas nos pacientes com nistagmo vertical para baixo com componente de torção, com lateralização nítida. Resultados Vinte pacientes (12,7%) foram diagnosticados com nistagmo vertical para baixo com componente de torção. A manobra foi feita em 10 (6,35%) pacientes, nos quais o lado afetado foi claramente determinado. Sete (4,45%) pacientes foram diagnosticados com VPPB do canal anterior e tratados com sucesso. Dois (1,25%) pacientes foram diagnosticados com VPPB do canal posterior e tratados com sucesso com a manobra de Epley após sua conversão para VPPB geotrópica de canal posterior. Conclusão Essa nova manobra mostrou-se eficaz na resolução de todos os casos de VPPB com nistagmo vertical para baixo com componente de torção causada por VPPB do canal anterior. E na mudança de forma controlada dos casos de VPPB do canal posterior do lado contralateral para uma VPPB geotrópica de canal posterior tratada com sucesso durante a consulta de seguimento. Além disso, essa nova manobra auxiliou no diagnóstico diferencial entre a VPPB do canal anterior e a VPPB do canal posterior contralateral.
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Benign paroxysmal positional vertigo (BPPV) is one of the most common disorders that causes dizziness. The incidence of horizontal semicircular canal (HSC) BPPV ranges from 5% to 40.5% of the total number of BPPV cases diagnosed. Several studies have focused on establishing methods to treat BPPV caused by the apogeotropic variant of the HSC, namely, the Appiani maneuver (App). In 2016, a new maneuver was proposed: the Zuma e Maia maneuver (ZeM), based on inertia and gravity. The aim of this study is to analyze the efficacy of App versus ZeM in the resolution of episodes of BPPV produced by an affectation of the horizontal semicircular canal with apogeotropic nystagmus (Apo-HSC). A retrospective, quasi-experimental study was conducted. Patients attended in office (November 2014-February 2019) at a third-level hospital and underwent a vestibular otoneurology assessment. Those who were diagnosed with Apo-HSC, treated with App or ZeM, were included. To consider the efficacy of the maneuvers, the presence of symptoms and/or nystagmus at the first follow up was studied. Patients classified as "A" were those with no symptoms, no nystagmus; "A/N+": no symptoms, nystagmus present during supine roll test; "S": symptoms present. Previous history of BPPV and/or otic pathology and calcium levels were also compiled. From the 54 patients included, 74% were women. The average age was 69. Mean follow-up: 52.51 days. In those patients without previous history of BPPV (n = 35), the probability of being group "A" was 63% and 56% (p = 0.687) when treated with App and ZeM, respectively, while being "A/N+" was 79% and 87% for App and ZeM (p = 0.508). Of the 19 patients who had previous history of BPPV, 13% and 64% were group "A" when treated with App and ZeM (p = 0.043), and 25% and 82% were "A/N+" after App and ZeM, respectively (p = 0.021). In conclusion, for HSC cupulolithiasis, ZeM is more effective than App in those cases in which there is a history of previous episodes of BPPV ("A": 64% (p = 0.043); "A/N+": 82% (p = 0.021)).
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PURPOSE: Current studies show that frequency tuning modification is a good marker for the detection of endolymphatic hydrops (EH) employing magnetic resonance imaging (MRI) in patients with Ménière's disease (MD). The purpose of the present study is to analyze the auditory and vestibular function with audiometric and vestibular-evoked myogenic potentials (VEMP) responses, respectively, in both the affected and unaffected ears of patients with unilateral MD using MRI as diagnostic support for the degree of EH. METHODS: We retrospectively reviewed the medical records of 76 consecutive patients with unilateral definite MD (age 55 (28-75); 39 women, 37 men). MRI was used through intravenous gadolinium administration, audiometry, and VEMPs. Functional tests were performed up to a week after the MRI. All were followed up one year after imaging utilizing clinical, auditory, and vestibular testing to rule out bilateral involvement. RESULTS: In the unaffected ear, the mean pure-tone average is normal even in cases with hydrops and, for a similar severity of hydrops is significantly lower than in the affected ear. Significant differences for the amplitude of the response at 0.5 kHz, at 1 kHz between the affected and unaffected ears were found to be lower in the affected ears. The relative amplitude ratio (1 Kz-0.5 kHz) was significantly lower in the affected ear and in the case of the oVEMP response depends on the degree of EH. The response in the unaffected ear was not modified by the presence or the degree of hydrops. CONCLUSION: In the unaffected ear, hydrops is not associated with hearing deterioration. For a similar degree of hydrops, hearing loss is significantly greater in the affected ear. The endolymphatic hydrops in the vestibule induces a frequency bias in the VEMP response only in the affected ear and not in the unaffected ear. Because of these findings we consider that hydrops does not represent an active disorder in the unaffected ear.
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Hidropesía Endolinfática , Enfermedad de Meniere , Potenciales Vestibulares Miogénicos Evocados , Masculino , Humanos , Femenino , Persona de Mediana Edad , Enfermedad de Meniere/complicaciones , Enfermedad de Meniere/diagnóstico , Estudios Retrospectivos , Hidropesía Endolinfática/diagnóstico , Hidropesía Endolinfática/diagnóstico por imagen , Potenciales Vestibulares Miogénicos Evocados/fisiología , Imagen por Resonancia Magnética/métodos , EdemaRESUMEN
BACKGROUND: Although diagnostic criteria have been established for superior canal dehiscence syndrome, cases in which the diagnosis is not easy are frequent. On those occasions, some tests such as vibration-induced nystagmus or vestibular-evoked myogenic potentials can offer invaluable help due to their high sensitivity and specificity. METHODS: We studied 30 patients showing superior canal dehiscence or "near-dehiscence" in a CT scan. Skull vibration-induced nystagmus and high frequency ocular vestibular-evoked myogenic potentials are performed in each patient. The aim of the study is to determine how useful both tests are for detection of superior canal dehiscence or near-dehiscence. RESULTS: Of the 60 temporal bones studied, no dehiscence was the result in 22, near-dehiscence in 17 and a definite finding in 21. In 10/30 patients, there was no SVIN (Skull vibration induced nystagmus) during otoneurological testing, while in 6/30, induced nystagmus was mainly horizontal, and in 14/30 there was vertical up-beating. All patients had a positive oVEMP (Ocular vestibular evoked myiogenic potentials) at 0.5 kHz in both ears and the HFoVEMP (High frequency ocular vestibular evoked myiogenic potentials) response was positive in 25/60 (41.6%) of the ears studied and in 19/30 of the patients evaluated (in 6 it was positive in both ears). Up-beat SVIN will point to a SCD (Superior Canal Dehiscence) mainly when HFoVEMP are present, and when this is negative there is a high probability that it is not a SCD. CONCLUSIONS: When SVIN and HFoVEMP results are added (or combined), they not only improve the possibilities of detecting SCD, but also the affected side.
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Background: The number of intratympanic gentamicin (ITG) injections needed to achieve vertigo control in patients with intractable Ménière's disease (MD) may vary from a single dose to several instillations. Changes in different vestibular test results have been used to define an endpoint of treatment, including the decrease of the vestibulo-ocular reflex (VOR) gain elicited by the head-impulse test. Objective: To assess the accuracy of the VOR gain reduction after horizontal canal stimulation, as measured with the video head-impulse test (vHIT) 1 month after the first intratympanic injection, in predicting the need for one or more instillations to control vertigo spells in the long term. Methods: The VOR gain reduction was calculated in 47 patients submitted to (ITG) therapy 1 month after the first instillation. Results: Single intratympanic treatment with gentamicin has a 59.6% efficacy in vertigo control in the long term. Hearing change in the immediate period after treatment (1 month) is not significant to pre-treatment result and is similar for patients who needed multiple doses due to recurrence. Chronic disequilibrium and the need for vestibular rehabilitation were less frequent in patients with a good control of vertigo with just one single injection of gentamicin. A fair accuracy was obtained for the VOR gain reduction of the horizontal canal (area under the curve = 0.729 in the Receiver Operating Characteristic analysis) in predicting the need for one or more ITG. Conclusions: Single intratympanic treatment with gentamicin is an effective treatment for patients with MD. That modality of treatment has very limited damaging effect in hearing. The degree of vestibular deficit induced by the treatment is significant as measured by the reduction in the gain of the VOR but not useful for prognostic purposes.
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OBJECTIVE: Flecainide is an oral class IC antiarrhythmic drug whose most common extracardiac adverse reactions are "dizziness" and "visual disturbances." We describe a case of flecainide associated- bilateral vestibulopathy and a literature review of this drug's effect on the vestibular system. PATIENT: Sixty-nine-year-old man with a 3-month history of unsteadiness and dizziness after an increase in the dose of flecainide. INTERVENTIONS: Otologic examination, video head-impulse test, vestibular evoked myogenic potentials, pure tone audiometry and high-resolution magnetic resonance imaging. RESULTS: Otologic examination, including the head-impulse test, and vestibular testing revealed bilateral vestibulopathy. CONCLUSIONS: Dizziness is a common extracardiac adverse reaction of Flecainide. Based on the clinical case that we present and the literature review carried out, we hypothesized that a possible mechanism by which flecainide might cause dizziness and visual disturbances is bilateral vestibulopathy.