Occlusion of the Lateral Semicircular Canal, Endolymphatic Sac Surgery, and Cochlear Implantation: A Low Destructive Treatment for Unilateral Ménière's Disease and Deafness.

INTRODUCTION: Surgical treatment of Ménière's disease (MD) and deafness aims to treat vertigo and hearing disabilities. Current treatment options like labyrinthectomy and cochlear implantation (CI) have shown acceptable results but are destructive. Less destructive procedures, like the occlusion of the lateral semicircular canal and endolymphatic sac surgery, have been shown to be successful in vertigo control. The combination of both procedures with CI has not been investigated; therefore the objective of this study was to investigate the outcome of this combination in patients with single-sided MD and moderately severe to complete sensorineural hearing loss. METHODS: In this retrospective study, 10 patients with single-sided MD and moderately severe to complete sensorineural hearing loss were included. In all of them, a single-staged surgery, which consisted of CI, endolymphatic sac surgery, and occlusion of the lateral semicircular canal, was performed. The surgery was performed after a failed conservative therapy trial. The clinical outcome was evaluated by the Dizziness Handicap Inventory (DHI) and audiological tests. These were assessed preoperatively, 3 and 6 months after surgery. An MRI with a hydrops sequence was performed to support the clinical diagnosis. RESULTS: After the combined surgery, the mean DHI testing improved significantly from 71 to 30. Mean audiological monosyllabic speech testing outcome with the cochlea implant was 65% at 65 dB. The residual hearing of 2 patients could be preserved after the surgical procedure. CONCLUSION: The combination of occlusion of the lateral semicircular canal, endolymphatic sac surgery, and CI is an efficient low traumatic treatment for patients with a single-sided MD and moderately severe to complete sensorineural hearing loss.

Endolymphatic sac tumor misdiagnosed as metastatic renal cell carcinoma: Pitfalls in morphology and immunohistochemistry.

Endolymphatic sac tumor (ELST) is a rare disease that originates from the endolymphatic sac system of the inner ear. Being a low-grade malignant tumor, ELST has a mild morphology and is characterized by a slow but aggressive growth. Most clinicians and pathologists are unfamiliar with this disease. ELST can be misdiagnosed as metastatic renal cancer because of the similarity in morphology and expression of nephrogenic markers such as PAX8. The presented case of a 27-year-old man revealed that observing the characteristic location and confirming the absence of renal neoplasm to rule out the possibility of metastasis are critical for obtaining an accurate final diagnosis.

Functional outcomes and caloric response changes after endolymphatic sac decompression.

OBJECTIVE: To evaluate caloric response changes after endolymphatic sac decompression (ESD), together with hearing outcomes and the functional benefit of the operation. METHODS: A retrospective chart review of subjects who underwent endolymphatic sac decompression at a tertiary referral centre was performed. Data on audiological results, caloric testing, and functional level scale of the patients were analysed. RESULTS: Twenty-eight patients who met our criteria were eligible for enrolment in the study. The average follow-up after surgery was 25 months (range, 13-41). Postoperative pure-tone threshold averages and reduced vestibular response values (RVR) were not significantly altered by ESD; whereas, functional level scores improved significantly. CONCLUSION: Endolymphatic sac decompression is a surgical procedure that preserves hearing and vestibular function, and improves the daily functional level of patients with Ménière's disease. ESD can be preferred both in bilateral and unilateral disease because it does not alter vestibular function and preserves hearing.

Efficacy of endolymphatic duct blockage in treating Ménière's disease. / å† æ·‹å·´ç®¡å¤¹é—­æœ¯æ²»ç–—æ¢ å°¼åŸƒç— çš„æ•ˆæžœ.

OBJECTIVES: Ménière's disease (MD) is an idiopathic inner ear disorder characterized by recurrent episodes of episodic rotational vertigo, fluctuating hearing loss, tinnitus, and a feeling of ear stuffiness. Endolymphatic sac (ES)-related surgery is used primarily in patients with MD who have failed to respond to pharmacologic therapy. Endolymphatic duct blockage (EDB) is a new procedure for the treatment of MD, and related clinical studies are still scarce. This study aims to investigate the dynamic changes in endolymphatic hydrops (EH) and the long-term surgical outcomes in MD patients undergoing EDB, and to evaluate the impact of different types of ES on the surgical efficacy. METHODS: A retrospective analysis was conducted on 33 patients with refractory MD who underwent EDB. Based on the morphology of their endolymphatic sacs, patients were divided into a normal-type group (n=14) and an atrophic-type group (n=19). The frequency of vertigo symptoms, hearing, vestibular function, and the dynamic changes of gadolinium-enhanced MRI of the inner ear were compared were compared before and after surgery between the 2 groups. RESULTS: Compared with the atrophic-type group, the patients in the normal-type group had a higher rate of complete vertigo control, better cochlear and vestibular function, and a lower endolymph to vestibule volume ratio (all P<0.05). In addition, 7 patients in the normal-type group were found to have reversal of EH, while no reversal of EH was detected in the atrophic-type group after surgery. CONCLUSIONS: The response to EDB treatment varies between normal and atrophic MD patients, suggesting that the 2 pathological types of endolymphatic sacs may have different underlying mechanisms of disease.

A New Methodology for Evaluation of Large Vestibular Aqueduct in CT and MRI Images.

HYPOTHESIS: Development of a new method for large vestibular aqueduct (LVA)/large endolymphatic sac anomaly (LESA) assessment using magnetic resonance imaging (MRI) and computed tomography (CT)/cone beam CT (CBCT) images. The secondary objective was to compare both modalities. BACKGROUND: The gold standard for LVA diagnosis is the analysis of CT images using Valvassori and Clemis or Cincinnati criteria. The previous studies showed inconclusive results regarding the correlation between audiological and radiological data. METHODS: Retrospective analysis of radiological images from 173 patients (315 ears), who were diagnosed with LVA/LESA based on CT/CBCT and/or MRI images of the temporal bone. The images obtained using both techniques were used to measure the following dimensions of vestibular aqueduct (VA)/endolymphatic duct (ED)/intraosseous endolymphatic sac (ES): width of the opening, length, and width at external aperture. In MRI images, the maximal contact diameters of the extraosseous or intraosseous ES and dura mater were measured as well. RESULTS: LVA has been reported to be bilateral in 82% (142 patients) and unilateral in 18% (31 patients) of cases. Comparison of MRI and CT/CBCT measurements showed a moderate correlation (0.64) in external aperture, a moderate correlation (0.57) in the width of the VA opening, and a weak correlation (0.34) in length measurements (p < 0.05). CONCLUSION: We developed a new method to identify the heterogeneous pathology of LVA/LESA using reconstruction along the VA/ED/intraosseous ES axis, three measurements on two planes, and focus on the maximal contact diameter between the extraosseous or intraosseous ES and dura mater.

Endolymphatic Sac Tumour: A Case Report and Review of the Literature

Introduction Endolymphatic sac tumor (ELST) is a slow-growing, low-grade, locallyinfiltrative tumor arising from the endolymphatic sac/duct, which is located in the posterior part of the petrous temporal bone. It may be sporadic in origin, or may be associated with Von-Hippel Lindau (VHL) syndrome. Case description A 40-year-old female patient with an ELST without VHL syndrome who was treated successfully by microsurgical extirpation of the tumor. Discussion We discuss the radiological features and the histopathology of this rare tumor and review the relevant literature. Conclusion The case herein reported adds to the previously-reported cases of this rare tumor.

Descompressão do saco endolinfático: análise da vertigem e da audição / Decompression of the endolymphatic sac: vertigo and hearing analysis

Introdução: A cirurgia na doença de Ménière está indicada quando a vertigem é refratária ao tratamento clínico. Vários procedimentos cirúrgicos foram propostos para o tratamento da vertigem nessa labirintopatia. Nesse trabalho nosso interesse foi pela descompressão do saco endolinfático. Objetivo: Analisar os resultados pós-cirúrgicos de 18 pacientes com doença de Ménière, em relação ao controle da vertigem e a melhora ou estabilização da audição, confrontar os nossos achados com os da literatura internacional e discutir aspectos de interesse relacionados a essa cirurgia. Método: Neste estudo retrospectivo, dezoito pacientes com diagnóstico de doença de Ménière, sem melhora da vertigem com o tratamento clínico, foram submetidos à descompressão do saco endolinfático. Analisamos no pós-operatório o controle da vertigem, a ausência e a diminuição da frequência, duração ou intensidade das crises e a melhora ou estabilização da audição. Resultados: O controle da vertigem ocorreu em 66% dos pacientes-ausência de vertigem em 11 (61%) e diminuição da frequência,duração ou intensidade em 1(5%). Observou-se melhora da audição em 4 pacientes (22,2%) e estabilização em 8 (44,5%). Nenhum paciente teve piora da audição em decorrência da cirurgia. Conclusão: A cirurgia de descompressão do saco endolinfático é útil para o tratamento da vertigem na doença de Ménière e deve ser indicada como um dos primeiros procedimentos cirúrgicos, principalmente em pacientes com perda leve ou moderada da audição, pelos resultados que costuma alcançar e pelo baixo risco de complicação. Obtivemos 66% de controle da vertigem e 22,2% de melhora da audição nos pacientes operados.

Introduction: Surgery in the Meniere's disease is indicated when the vertigo is refractory to clinical treatment. Many surgical procedures have been proposed for the treatment of vertigo in this labyrinthopathy. In this work we aimed at the decompression of the endolymphatic sac. Objective: To analyze the postoperative outcomes of 18 patients with Meniere's disease as for the control of the vertigo and the improvement or stabilization of hearing, to compare our findings with the international literature and discuss aspects of interest regarding this surgery. Method: In this retrospective study, eighteen patients with Meniere's disease diagnosis, without improvement of the vertigo with clinical treatment, were submitted to decompression of the endolymphatic sac. In the postoperative we analyzed the vertigo control, the absence and diminishing of frequency, duration or intensity of the crises and the improvement or stabilization of hearing. Results: The vertigo control occurred in 66% of the patients, absence of vertigo in 11 (61%) and diminishing of the frequency, duration or intensity in 1 (5%). We observed an improvement of hearing in 4 patients (22.2%) and stabilization in 8 (44.5%). No patient had worsening of hearing as a result of the surgery. Conclusion: The endolymphatic sac decompression surgery is helpful for the treatment of the vertigo in the Meniere's disease and must be indicated as one of the first surgical procedures, specially in patients with light or moderate hearing loss, for the results it usually achieves and the low risk of complication. We obtained 66% of vertigo control and 22.2% of hearing improvement in the patients operated.

Enfrentamiento a un turmor de peñazco: estudio y posibilidad diagnóstica / Facing a petrous tumor: study and diagnostic possibility

Los tumores de punta de peñasco se pueden presentar con hipoacusia sensorioneural y un síndrome vertiginoso secundario a hidrops endolinfático. Dentro del diagnóstico diferencial se encuentran los tumores de saco endolinfático, los cuales han sido reportados cada vez con mayor frecuencia en la literatura. A continuación informamos el caso de un paciente de 50 años que se presenta a la consulta con hipoacusia sensorioneural fluctuante de oído derecho y síndrome vertiginoso. Las neuraimágenes muestran tumor de punta de peñasco que ha tenido un crecimiento progresivo en el tiempo erosionando secundariamente estructuras del oído interno. Se realizó biopsia que no fue concluyente, la cual no ha podido ser repetida por decisión personal de la paciente, por lo que se ha mantenido en manejo expectante. Dada la evolución y las imágenes se plantea diagnóstico de tumor de saco endolinfático, a la espera de una biopsia confirmatoria.

Tratamiento quirúrgico en la Enfermedad de Menière: la descompresión del saco endolinfático / Surgical treatment in Menière disease: endolymphatic sac decompression

La enfermedad de Menière se caracteriza por episodios de vértigo, asociados a hipoacusia, tinnitus y plétora aural. El tratamiento quirúrgico en esta enfermedad está indicado en aquellos pacientes en los que no hay una adecuada respuesta al tratamiento médico. En la actualidad los procedimientos quirúrgicos más comunes son: la descompresión del saco endolinfático, la neurectomía vestibular y la laberintectomía. En este artículo se describe la técnica quirúrgica para la cirugía de descomprensión del saco endolinfático.

Electrococleografía transoperatoria en cirugía de saco endolinfático / Intraoperative electrocochleography in endolumphatic sac surgery

Objetivos. Analizar los cambios en la relación potencial de suma-potencial de acción (PS/PA) en la Electrococleografía (EcoG) intraoperatoria a través de registros efectuados al descomprimir y abrir el saco endolinfático, tratar de identificar la posición del saco endolinfático en pacientes sometidos a cirugía de saco endolinfático con enfermedad de Meniére. Diseño. Prospectivo. longitudinal, comparativo, observacional. Material y métodos. En el periodo comprendido de Septiembre a Diciembre de 1998, en el servicio de Neurotología del Instituto Nacional de Neurología y Neurocirugía, se estudiaron 8 pacientes con diagnóstico de enfermedad de Meniére con falla a tratamiento médico, realizándoles audiometría, logoaudiometría, electronistagmografía y electrococleografía. Aceptaron se practicara cirugía de descompresión y apertura de saco endolinfático y 4 registros de electrococleografía transoperatoria. Resultados. La comparación de los valores obtenidos en el EcoG durante la descompresión y apertura del saco endolinfático mostró valores absolutos PS/PA, con una significancia del 99.9 por ciento, lo cual equivale a P. Los 8 (100 por ciento) pacientes mostraron disminución de la amplitud del PS en la EcoG intraoperatoria en algún momento del monitoreo. Todos los pacientes en su EcoG de control postquirúrgico mostraron una relación PS/PA a 30 por ciento, cuando en el registro prequirúrgico tenían registro a 35 por ciento. Conclusiones: Aunque nuestra muestra es pequeña, es significativa, ya que encontramos en el 100 por ciento de los pacientes disminución de la relación PS/PA en la EcoG intraoperatoria.

Descompresión del saco endolinfático en la enfermedad de Meniere experiencia en el Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez / Descompression of the endolinphatic sac in the treatment of Meniere disease. Experience at the National Neurology Institute

La enfermedad de Meniere fue descrita desde finales del siglo pasado, las opciones terapéuticas son farmacológicas y quirúrgicas, reservándose esta última para los pacientes que no responden al tratamiento farmacológico. En este trabajo se presenta la experiencia en el tratamiento de 47 pacientes, con diagnóstico de enfermedad de Meniere a quienes se realizó descompresión de saco endolinfático durante los años de 1975 a 1994, con un seguimiento promedio de 5.7 años.Se muestran los resultados en el control de vértigo en 85 por ciento de los casos. La audición se preservó y no se demostró efecto del procedimiento sobre el acúfeno.

Adenocarcinoma do saco endolinfático: relato de caso / Endolymphatic sac adenocarcinoma: case report

Apresenta-se um caso de adenocarcinoma do saco endolinfático e revê-se a literatura. O quadro clínico manifestou-se por tonteira e perda progressiva da audiçäo consequentes a tumor do saco endolinfático. A remoçäo cirúrgica foi completa e realizada por abordagem retro e translabiríntica. Os tumores do saco endolinfático säo raros, localmente invasivos e comprometem a mastóide e parte petrosa do osso temporal e o ângulo pontocerebelar. A remoçäo cirúrgica completa apresenta bom prognóstico

Anatomia macroscópica da parte extra-óssea do saco endolinfático em brancos e negros / Macroscopic anatomy of the extraosseous part of the endolymphatic sac in whites and blacks

Foram examinadas oitenta peças anatômicas de 40 cadáveres, com idades entre 13 e 72 anos, 21 homens e 19 mulheres, com o saco endolinfático (SE) inflado em metade das peças e injetado nas restantes. Os tipos de contorno do SE encontrados foram: piriforme (56,3 por cento), triangular (21,2 por cento), elíptico (15 por cento), circular (5 por cento) e oval (2,5 por cento). A simetria bilateral do contorno foi observada em 55 por cento dos casos. As distâncias das margens medial e lateral do SE à face externa do osso temporal foram maiores no homem do que na mulher. Foram medidas as dimensoes do SE e as distâncias da abertura externa do aqueducto do vestíbulo às seguintes estruturas: seio petroso superior, seio sigmóide, meato acústico interno e bulbo superior da veia jugular interna. A superposiçao do SE ao seio sigmóide tendeu a ocorrer com o aumento da idade. Foram observados feixes de reforço da dura-máter dispostos sobre a parede posterior do SE.

Tratamiento quirúrgico del vértigo: revisión bibliográfica / Surgical treatment of vertigo: a review

Se hace una revisión bibliográfica crítica de los procedimientos quirúrgicos usados para curar el síntoma vértigo, con objeto de aclarar sus indicaciones en función de resultados y de efectos nocivos. Las conclusiones a que se llegó fueron las siguientes: 1. En el vértigo producto de fístula perilinfática, se debe intervenir quirúrgicamente tan pronto sea diagnosticada y aún en casos de que solo exista una razonable sospecha. 2. En el vértigo postural paroxístico benigno incapacitante y resistente a tratamiento médico y rehabilitación física, se puede intervenir con obliteración del conducto semicircular posterior, informando al paciente, de preferencia por escrito, que existe riesgo, aunque poco probable, de que aparezca hipoacusia y acúfeno. 3. El vértigo ocasionado por la enfermedad de Meniére o por otros padecimientos, en caso de ser incapacitante y no susceptible a tratamiento médico, debe intervenirse siguiendo dos criterios: Cuando la audición ha disminuido hasta el punto de ya no ser útil, se puede emplear la gentamicina intratimpánica o la derivación del saco endolinfático; si estas fracasan, debe efectuarse laberintectomía. Cuando hay buena audición, o audición recuperable con auxiliar auditivo se puede hacer derivación del saco endolinfático; si ésta fracasa, neurectomía vestibular. 4. Hay razones para ser escéptico sobre la existencia y sobre la posibilidad diagnóstica del vértigo como producto de compresión vascular al tronco del nervio octavo, por lo que la descompresión microvascular del nervio debe considerarse como en fase experimental, y no efectuarse en ambiente clínico.