Gadolinium-enhanced MRI reveals dynamic development of endolymphatic hydrops in Ménières disease / Ressonância magnética com gadolínio revela o desenvolvimento dinâmico da hidropsia endolinfática na doença de Ménière

Abstract Introduction: Meniere's disease is associated with impaired hearing, tinnitus, vertigo, and aural fullness. Many anatomical studies have suggested idiopathic endolymphatic hydrops as the pathological basis of Meniere's disease, which now can be visualized by using gadolinium -enhanced magnetic resonance imaging of the inner ear. Objective: To investigate the development of endolymphatic hydrops in Meniere's disease by monitoring the vestibules and cochleae of affected patients. Methods: Inner ears of 178 patients with definite unilateral Meniere's disease diagnosis were visualized by 3-dimensional fluid-attenuated inversion recovery and three-dimensional real inversion recovery magnetic resonance imaging following bilateral gadolinium intratympanic injection. The scans were used to evaluate the presence and degree of endolymphatic hydrops in the vestibules and cochlear structures, including the cochlear apical turn, the cochlear middle turn, and the cochlear basal turn. The correlation of endolymphatic hydrops occurrence between the various parts of the inner ear was determined. Results: Symptomatic endolymphatic hydrops was detected on the affected side in all patients, whereas asymptomatic endolymphatic hydrops was detected on the unaffected contra-lateral side in 32 patients (18.0%). On the affected side, the cochlear apical turn and the cochlear middle turn demonstrated significantly higher rates of endolymphatic hydrops than the cochlear basal turn and the vestibule. The severity of endolymphatic hydrops gradually decreased from the cochlear apical turn to the cochlear basal turn. On the contra lateral side, the incidence and degree of the detected asymptomatic endolymphatic hydrops were significantly greater in the cochleae than in the vestibules (p < 0.05), with no significant difference detected between the cochlear turns. Conclusion: Progression of endolymphatic hydrops appears to be directional, initiated in the cochlea. The order of endolymphatic hydrops severity gradually decreases from the cochlear apical turn to the cochlear basal turn and then to the vestibule. Endolymphatic hydrops in the vestibule is associated with symptomatic Meniere's disease.

Resumo Introdução: A doença de Ménière está associada a deficiência auditiva, zumbido, vertigem e plenitude auricular. Muitos estudos anatômicos sugerem hidropsia endolinfática idiopática como a base patológica da doença, que agora pode ser visualizada através de estudo por imagem da orelha interna por ressonância magnética com gadolínio. Objetivo: Investigar o desenvolvimento da hidropsia endolinfática na doença de Ménière com monitoramento dos vestíbulos e das cócleas dos pacientes afetados. Métodos: Orelhas internas de 178 pacientes com diagnóstico definitivo de doença de Ménière unilateral foram visualizados através de imagem de recuperação de inversão atenuada por fluidos em ressonância magnética tridimensional, 3-D FLAIR, e por inversão real após injeção intratimpânica bilateral de gadolínio. Os exames foram usados para avaliar a presença e o grau de hidropsia endolinfática nos vestíbulos e nas estruturas cocleares, inclusive o giro coclear apical, o giro coclear médio e o giro coclear basal. A correlação da ocorrência de hidropsia endolinfática entre as várias partes da orelha interna foi determinada. Resultados: Hidropsia endolinfática sintomática foi detectada no lado afetado em todos os pacientes, enquanto hidropsia endolinfática assintomática foi detectada no lado contralateral não afetado em 32 pacientes (18,0%). No lado afetado, o giro apical da cóclea e o giro coclear médio demonstraram taxas significativamente mais altas de hidropsia endolinfática do que o giro basal e o vestíbulo. A gravidade da hidropsia endolinfática diminuiu gradualmente do giro apical da cóclea para o giro basal. No lado contralateral, a incidência e o grau da hidropsia endolinfática assintomática detectada foram significantemente maiores nas cócleas do que nos vestíbulos (p < 0,05), sem diferença significante entre os giros cocleares. Conclusões: A progressão da hidropsia endolinfática parece ser direcional, iniciando-se na cóclea. A sua ordem da gravidade diminui gradualmente do giro apical da cóclea para o giro basal e, em seguida, para o vestíbulo. A hidropsia endolinfática no vestíbulo está associada à doença de Ménière sintomática.

Menière's disease and disorders of the carbohydrate metabolism involving the inner ear

Introduction: Menière's disease was described in 1861, but there are still uncertainties regarding its pathophysiology and treatment. Endolymphatic hydrops is recognized as a fundamental pathological characteristic of the disease, as a result of an inadequate absorption of the endolymph. A milder type of endolymphatic hydrops results from an altered chemical composition of the endolymph, due to disorders of the carbohydrate metabolism. Objective: To describe the association of both types of hydrops in patients with Menière disease. Methods: This was a retrospective study of 98 patients with Menière's disease, 62 of whom also presented disorders of the carbohydrate metabolism, and 5 patients with delayed endolymphatic hydrops, 2 of whom also presented disorders of the carbohydrate metabolism. Results: The follow-up of these patients showed that the correction of the metabolic disorders may help in the clinical treatment of Menière's disease and of delayed endolymphatic hydrops, but this does not happen in the more severe types of the diseases. Conclusion: Patients with Menière's disease may present simultaneous disorders of the carbohydratemetabolism, affecting the inner ear. The correction of these disorders helps the clinical treatment but does not preclude the progression of the more severe cases of Menière disease (AU)

Intratympanic Gentamicin for Intractable Ménière's Disease - A Review and Analysis of Audiovestibular Impact

Abstract Introduction Intratympanic gentamicin regulates the symptoms in most patients with incapacitating Ménière's disease. The treatment protocols have changed over the years from medical labyrinthectomy to preservation of vestibular function. Objectives This study aims to review the audiovestibular response related to the effect of the drug in controlling vertigo. Data Synthesis Articles were identified by means of a search in the PubMed database using the key words Meniere and intratympanic or transtympanic gentamicin. Total 144 articles were reviewed after excluding those that were technical reports, those based on experimental animal studies, those that focused on outcomes other than vertigo (tinnitus or aural fullness), those with delivery methods other than tympanic membrane injection, and those with bilateral cases. If there was more than one article by the same author(s) or institution, only the most recent one matching the aforementioned criteria and those that were not overlapping were included. Conclusion Titration methods or multiple injections on a daily basis can be preferred if the patients have profound or non-serviceable hearing, since these methods have significant incidence of hearing loss. Treatment protocols with a frequency of injection not shorter than once a week, or those with injections on a monthly basis as "needed" provide the same level of vertigo control with better preservation of hearing. Caloric testing is not an ideal tool to analyze the correlation between vertigo control and the effect of gentamicin as compared with gain asymmetry of the vestibulo-ocular reflex. Vestibular-evoked myogenic potentials and the head thrust test are more reliable than other vestibular tests for the follow-up of patients undergoing gentamicin treatment.

Crisis otolítica de Tumarkin: revisión de la literatura / Tumarkin's otolithic crisis: a review

Las crisis de Tumarkin consisten en caídas bruscas al suelo sin pródromos previos ni pérdida de conciencia y de segundos de duración que pueden ocurrir con frecuencia relativa en pacientes con enfermedad de Ménière. Si bien pueden presentarse de manera aislada durante la evolución de la enfermedad, existen casos con crisis recurrentes que comprometen significativamente la calidad de vida de los pacientes. Se postula que las crisis se producen por una alteración de la función del órgano otolítico, específicamente del sáculo. El tratamiento puede ser desde el manejo expectante hasta el uso de laberintectomía química o quirúrgica.

Tumarkin´s otolithic crisis is a sudden fall that comes with no loss of consciousness, and without warning or prodrome. It has a short duration and can occur with relative frequency in patients with Meniere disease. When it is present, it significantly compromises life quality of patients. There is no certainty about its mechanism, but it is assumed that the crises are caused by an otolithic organ disfunction, specifically a collapse of the saccule. Treatment can range from observation to chemical or surgical labyrinthectomy.

Hearing function after betahistine therapy in patients with Ménière's disease / Função auditiva após terapia com beta-histina em pacientes com doença de Ménière

ABSTRACT INTRODUCTION: Preventing or reversing hearing loss is challenging in Ménière's disease. Betahistine, as a histamine agonist, has been tried in controlling vertigo in patients with Ménière's disease, but its effectiveness on hearing problems is not known. OBJECTIVE: To examine the effect of betahistine on hearing function in not-previously-treated patients with Ménière's disease and to define possible contributors in this regard. METHODS: A total of 200 not-previously-treated patients with definite unilateral Ménière's disease received betahistine by mouth (initial dose, 16 mg three times a day; maintenance dose, 24-48 mg daily in divided doses). Changes in indicators of hearing status before and six months after treatment were documented. Hearing loss was considered as the mean hearing level >25 dB HL at five frequencies. RESULTS: The mean duration of disease was 3.37 years. Six months after treatment the mean hearing level decreased by 6.35 dB compared to that at the baseline (p < 0.001). Both patients' age and the duration of disease correlated negatively with the improvement in hearing function. Post treatment hearing loss was independently associated with age, the initial hearing level and the chronicity of disease. The corresponding optimal cut-off points for predicating a persistent hearing loss 6 months after treatment were 47 years, 38 dB HL, and 1.4 years, respectively. CONCLUSION: Oral betahistine was significantly effective in preventing/reversing hearing deterioration in patients with Ménière's disease. Age, the hearing level on admission, and the disease duration were independent predictors of hearing status after treatment.

Resumo Introdução: Prevenir ou reverter a perda auditiva é um desafio na doença de Ménière. A betahistina, um agonista de histamina, tem sido testada no controle de vertigem em pacientes com doença de Ménière, mas sua eficácia em problemas de audição ainda não é conhecida. Objetivo: Analisar o efeito da betahistina na função auditiva em pacientes com doença de Ménière não tratados previamente, e definir possíveis contribuintes a esse respeito. Método: Um total de 200 pacientes sem tratamento prévio, e com diagnóstico definido de doença de Ménière unilateral, recebeu beta-histina por via oral (dose inicial de 16 mg três vezes ao dia; dose de manutenção de 24-48 mg por dia, em doses divididas). Alterações dolimiar auditivo antes e após seis meses de tratamento foram documentadas. Considerou-se como perda auditiva uma média do nível de audição > 25 dB NA em cinco frequências. Resultados: A média de duração da doença foi de 3,37 anos. Seis meses após o tratamento, a média do limiar auditivo diminuiu em 6,35 dB, em comparação com o valor da linha de base (p < 0,001). Tanto a idade dos pacientes quanto a duração da doença apresentaram correlação negativa com a melhora da função auditiva. A perda auditiva após o tratamento foi independentemente associada à idade, ao nível inicial de audição e à cronicidade da doença. Os pontos de corte ótimos correspondentes para prever uma perda auditiva persistente seis meses após o tratamento foram 47 anos, 38 dB HL e 1,4 ano, respectivamente. Conclusão: A betahistina oral foi significantemente eficaz na prevenção/reversão da deterioração auditiva em pacientes com doença de Ménière. Idade, nível de audição na admissão e duração da doença foram fatores preditivos independentes da condição auditiva após o tratamento.

Corticoides en la Enfermedad de Meniere / Steroids in disease Meniere

La Academia Americana de Otorinolaringología-HNS en195 define a la Enfermedad de Meniere como un síndrome idiopático de hidrops endolinfático que clínicamente se manifesta por crisis vertiginosas esporádicasespontáneas y recurentes asociadas a hipoacusia, plenitud ótica y acufenos.El vértigo se define como un trastorno del equilbriocaracterizado por una sensación de movimiento rotatorio del cuerpo o los objetos que lo rodean.El acúfeno es la percepción de sonido en ausencia deuna señal simultánea acústica o eléctrica. Sería el resultado de una actividad aberante producida en una ovarias localizaciones de la vía auditva desde la cócleahasta la corteza cerebral, que es procesada de modoanómalo y que es interpretado eróneamente por loscentros superiores como un ruido. Se hacen patentescuando alcanzan una intensidad que supera el enmascaramiento del sonido ambiental que nos rodea.Objetivo Determinar los resultados en la audición,acufeno y vértigo luego tratamiento intratimpánico concorticoides en la Enfermedad de Meniere Definitva.Determinar el impacto en la calidad de vida luego deltratamiento intratimpánico.Material y método Se realizó un estudio prospectivo,utilzando el método estadístico, que incluyo a 13 pacientes de ambos sexos mayores de 14 años que consultaron por Enfermedad de Meniere al Servicio de Otorinolaringología del Hospital Nacional de Clínicas Córdoba Argentina durante el periodo comprendido entrelos años 209-2012. Conclusión Concluimos que el vértigo en los pacientesque presentan Enfermedad de Meniere mejora en todoslos aspectos, siendo este el síntoma que motiva la consulta, proponemos este tratamiento en los pacientes quepresentan vértigo incapacitante.

The American Academy of Otolaryngology-HNS in 195defined Meniere's disease as an idiopathicendolymphatic hydrops syndrome manifests clinicalysporadic, spontaneous and recurent vertiginous crisesasociated with hearing los, tinitus and aural fulnes.Vertigo is defined as a balance disorder characterized bya sensation of rotational movement of the body orsurounding objects.Tinitus is the perception of sound in the absence of anacoustic or electric signal simultaneously. Would resultfrom aberant activity produced in one or more locationsof the auditory pathway from the cochlea to the cerebralcortex, which is procesed and anomalously beingmisinterpreted by the higher centers as noise. Becomeaparent when they reach an intensity that exceds theambient sound masking around us.Objetive Determine the results in hearing, tinitus anddizines after intratympanic corticosteroid therapy inMeniere 's Disease Final.Determine the impact on quality of life after intratympanictreatment.Materials and methods A prospective study wasperformed using the statistical method ,which included13 patients of both sexes over 14 years consulting forMeniere's disease the Department of Otolaryngology,National University Hospital -Córdoba Argentina for theperiod betwen 2009 -2012. Conclusion We conclude that he vertigo in patients withMeniere's disease improved in al aspects, which is thesymptom that motivates the query, we propose thistreatment in patients with disabling vertigo.

Manobra de Epley na vertigem posicional paroxística benigna associada à doença de Ménière / Epleys maneuver in benign paroxysmal positional vertigo associated with Menieres disease

Os efeitos da manobra de Epley na vertigem posicional paroxística benigna (VPPB) associada à doença de Ménière são controvertidos. OBJETIVO: Avaliar a vertigem e o nistagmo de posicionamento após uma ou mais manobras de Epley na vertigem posicional paroxística benigna (VPPB) associada à doença de Ménière e na recorrência da VPPB. MATERISL E MÉTODO: Estudo retrospectivo de 62 pacientes com VPPB associada à doença de Ménière submetidos à manobra de Epley e acompanhados durante 12 meses após a extinção do nistagmo de posicionamento. RESULTADOS: Para abolir o nistagmo de posicionamento, foi necessária uma manobra de Epley em 80,7 por cento dos pacientes, duas em 16,1 por cento e três em 3,2 por cento. A vertigem foi eliminada em 71,0 por cento dos pacientes, melhorou em 27,4 por cento e permaneceu inalterada em 1,6 por cento. Quatro semanas após a extinção do nistagmo de posicionamento, todos os pacientes ficaram assintomáticos. Recorrência da VPPB foi observada em 19,4 por cento dos casos, com eliminação da vertigem e nistagmo de posicionamento à manobra específica para o canal afetado. CONCLUSÕES: Na VPPB associada à doença de Ménière, vertigem e nistagmo de posicionamento foram eliminados com uma, duas ou três manobras de Epley. A recorrência da VPPB foi resolvida com uma manobra para o canal envolvido.

The effects of Epleys maneuver in benign paroxysmal positional vertigo (BPPV) associated with Menières disease are controversial. AIMS: To evaluate the progression of positional vertigo and nystagmus after one or more of Epleys maneuvers in BPPV associated with Menières disease, and the recurrence of BPPV. METHOD: a retrospective study of 62 patients with BPPV associated with MenièreÆs disease, that underwent Epleys maneuver, and that were monitored during 12 months after elimination of positional nystagmus. RESULTS: One Epleys maneuver was required to eliminate positional nystagmus in 80.7 percent of the patients, two in 16.1 percent, and three in 3.2 percent; after elimination of nystagmus, positional vertigo was suppressed in 71.0 percent of the patients, improved in 27.4 percent and remained unaltered in 1.6 percent. Four weeks after elimination of positional nystagmus, all patients were asymptomatic. Recurrence of BPPV was seen in 19.4 percent of the cases, with elimination of the positional vertigo and nystagmus by means of the specific maneuver for the involved canal. CONCLUSION: In BPPV associated with Menières disease, vertigo and positioning nystagmus were eliminated with one, two or three Epley maneuvers. BPPV recurrence was resolved by using a specific maneuver for the affected canal.

Qualidade de vida em pacientes com vertigem posicional paroxística benigna e/ou doença de Ménière / Quality of life in patients with benign paroxysmal positional vertigo and/or Ménière's disease

Pacientes com vertigem posicional paroxística benigna e/ou doença de Ménière relatam prejuízos na qualidade de vida. Objetivo: Comparar o impacto da tontura na qualidade de vida destes pacientes e avaliar a influência do gênero, faixa etária e canal semicircular afetado. Forma de estudo: clínico com coorte transversal. Material e Método: Estudo prospectivo realizado na Universidade Federal de São Paulo, em 2003/04. O Dizziness Handicap Inventory foi aplicado em 70 pacientes com vertigem posicional, 70 com doença de Ménière e 15 com ambas. Utilizou-se o teste de igualdade de duas proporções e a análise de variância para a avaliação estatística. Resultados: Os escores obtidos com a aplicação do questionário foram superiores, na crise e fora dela, no grupo com doença de Ménière, em relação ao com vertigem posicional, mas apenas na crise em relação ao grupo com associação (p<0,05). Não houve correlação com a faixa etária, gênero e nos casos de vertigem posicional, com o acometimento do canal semicircular. Conclusões: Os pacientes com doença de Ménière apresentaram pior qualidade de vida, na crise e fora dela, em relação aos com vertigem posicional paroxística benigna e aos com associação de ambas labirintopatias, quando na crise da vertigem posicional. O prejuízo da qualidade de vida foi independente do gênero, da faixa etária e nos casos com vertigem posicional, do canal semicircular acometido.

Patients with benign paroxysmal positional vertigo and/or Ménière's disease relate damages in quality of life. Aim: To compare the impact of dizziness on quality of life, in patients with benign paroxysmal positional vertigo and/or Ménière's disease, in crisis and out of crisis, and to evaluate the influence of gender, age and impaired semicircular canal. Study design: clinical with transversal cohort. Material and Method: The prospective study was realized in 2003/04 at Federal University of São Paulo. The Dizziness Handicap Inventory was applied in seventy patients with positional vertigo, seventy with Ménière's disease and fifteen with both. Two-proportion equality test and the Analysis of variance were employed in this study. Results: When comparing the groups, Dizziness Handicap Inventory results evidenced higher averages in crisis and out of crisis for Ménière's disease group than for positional vertigo group. The same occurred only during the crisis period in the group when comparing with both disorders (p<0,05). No significant statistical differences were observed, when comparing the results considering age, gender and, in the group with positional vertigo, affection of posterior semicircular canal as variables. Conclusions: Ménière's disease patients presented worse quality of life when compared to BPPV patients, in and out of crisis, and during the crisis when regarding the patients with association of both disorders. The damage on quality of life was independent of gender, age and in the BPPV cases it was independent of posterior canal affection.

Utriculostomia: apresentaçäo de uma nova técnica para tratamento cirúrgico da vertigem / Utriculusstomy: presentation of a new technique for the treatment of vertigo

Objetivo: apresentar uma nova técnica alternativa para o tratamento cirúrgico de vertigem causada pela Doença de Menière, a utriculostomia, desenvolvida no HCPA...

Vertigem no idoso / Vertigo in the elderly

Este trabalho tem por objetivo mostrar que uma análise cuidadosa das vertigens, tonturas, zumbidos e hipoacusias pode levar ao diagnóstico de moléstias importantes, como tumores cerebrais, numa fase em que o tratamento cirúrgico tenha baixa mortalidade e mínima morbidade. Foram submetidos ao exame otoneurológico 290 pacientes portadores de distúrbios labirínticos e auditivos, sem diagnóstico conhecido, sendo encontradas 70 (20,1 por cento) com patologias graves, como tumores cerebrais, esclerose múltipla etc., e 220 (75,9 por cento) com moléstias nåo localizadas no sistema nervoso central

Hipoacusia de conducción en hidropesia endolinfática / Conduction deafness in endolymphatic hidropesy

The audiograms of 33 patients suffering from Méniére's disease were studied. A conductive hearing loss was found in about fifty per cent of then. The following criteria was used to consider that a patient had a conductive hearing loss: First, an air-bone gap of at least 20 dB in one frequency, excluding 250 Hz. If in 250 Hz there is an air-bone gap of 20 dB, it is necessary an addtional air-bone gap in other frequency of at least 10 dB. Second, when an air-bone gape of 15 dB exists in one frequency, it must be another air-bone gap of at least 10 dB in other frequency. These conditions must be found in two or more audiograms in the same patient. Two groups were made with the 33 patients. One was conformed by 24 patients that had one or more of the following conditions: 1. A positive glucrol test. 2. Fluctuating hearing loss. It means that the patinte had an improved audiogram in respect to a previous one. 3. An ascending profile in the low frequencies. 3. An ascending profile in the low frequencies. The second group of nine patients had not any of the previous chacracteristics. In the first group the authors found that 62.5% had a conductive hearing loss while in the second group 22.2% had the same condition. This difference is statistically significant (p = 0.05). Nine impedanciometric test belonging to eight of the first group and three of equal number of patients of the second group were analyzed. Only few cualitative changes in the tympanometric curve were found. Five of the eight patients of the first group and none of the three patients of the second group showed an increase of the static compliance