Endolymphatic sac tumor with intralabyrinthine hemorrhage: a case report / 临床耳鼻咽喉头颈外科杂志

A 27-year-old female patient suffering endolymphatic sac tumor with intralabyrinthine hemorrhage was reported. The patient had hearing loss in the left ear with continuous tinnitus, and MRI showed the soft tissue shadow of endolymphatic sac. Considering that the tumor involved semicircular canal and vestibule,endolymphatic cyst tumor resection was performed by labyrinth route. After surgery, there was no cerebrospinal fluid leakage and facial nerve function was normal. More importantly, enhanced MRI of temporal bone showed no tumor recurrence 1 year after surgery.

Manifestações de imagem da doença de von Hippel-Lindau: um guia ilustrado com foco no sistema nervoso central / Imaging manifestations of von Hippel-Lindau disease: an illustrated guide focusing on the central nervous system

Abstract A doença de von Hippel-Lindau (VHL) é uma síndrome hereditária autossômica dominante rara que afeta a linha germinativa do gene VHL, um gene supressor tumoral. A doença de VHL é caracterizada pelo desenvolvimento multissistêmico de uma variedade de tumores benignos e malignos, especialmente no sistema nervoso central (SNC). Dentre eles, destacam-se hemangioblastomas retinianos e do SNC, e o tumor do saco endolinfático. Os diferentes locais dos tumores justificam a diversidade de sinais e sintomas relacionados à doença, que usualmente se manifestam com a idade média de 33 anos. Apesar dos avanços da medicina, a expectativa de vida média desses pacientes é de 49 anos. Exames de imagem têm papel fundamental no diagnóstico e são essenciais no seguimento dos pacientes com doença de VHL. Este ensaio iconográfico descreve as manifestações características dos tumores do SNC relacionados à doença de VHL que todos os residentes de radiologia devem saber.

Abstract Von Hippel-Lindau (VHL) disease is a rare, autosomal dominant inherited syndrome that affects the germline of the VHL gene, a tumor suppressor gene. VHL disease is characterized by the multisystemic development of a variety of benign and malignant tumors, especially in the central nervous system (CNS). Such tumors include retinal and CNS hemangioblastomas, as well as endolymphatic sac tumors. The various tumor sites are responsible for the diversity of signs and symptoms related to the disease. The mean age at symptom onset is 33 years. Despite medical advances, the average life expectancy of patients with VHL disease is 49 years. Imaging plays a pivotal role in the clinical diagnosis and is essential to the follow-up of patients with VHL disease. This pictorial essay describes characteristic CNS manifestations of VHL disease-related tumors that all radiology residents should be aware of.

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.

“Endolymphatic sac tumour”: A case report with review of literature

Endolymphatic sac tumour (ELST) is a non-metastasizing low grade adenocarcinoma of endolymphatic sac origin. It is also known as Heffner tumour, low grade adenocarcinoma of endolymphatic sac origin and aggressive papillary middle ear tumour. These tumours are closely associated with Von Hippel Lindau (VHL) disease. Here we report a case of Endolymphatic sac tumour in a 63 yr old lady who presented with left sided facial palsy. Since the tumour was highly vascular and required preoperative embolization, initial clinicoradiological diagnosis was Jugulotymphanic paraganglioma. Histopathology showed features of Endolymphatic sac tumour, which was confirmed by immunohistochemistry. Since this tumour is locally aggressive low grade adenocarcinoma, the diagnosis is difficult in advanced cases where there is erosion of petrous temporal bone or the lesion shows extension into cerebellopontine angle as in our case. Since the association of this tumour with VHL disease is well established, it is important to screen all the patients of VHL disease for this lesion and also all the patients of ELST should be screened for other lesions of VHL disease to aid in early diagnosis and treatment. The case is presented here for its rarity and difficulty in initial diagnosis.

Study of Sigmoid Sinus Variations in the Temporal Bone by Micro Dissection and its Classification - A Cadaveric Study

Abstract Introduction Sigmoid sinus (SS) variations have been classified variously in the literature. These classifications suffer from some form of shortcoming from a clinical point of view for their application. Objective We propose a clinically relevant classification of the SS in relation to the posterior semicircular canal (PSCC) and to the exposure of the presigmoid dural plate. The positioning of the SS was analyzed with reference to the volume of themastoid and to the level of mastoid pneumatization. Methods A total of 94 formalin-preserved human cadaveric temporal bones were microdissected to carry out a complete mastoidectomy. The SS, the presigmoid dural plate, and the PSCCwere exposed, and the position of the former was analyzed in relation to the latter two in order to classify the position of the SS into three grades. Results GradeI hadthebest exposureof the presigmoid dura andof the PSCC,while grade III had the poorest exposure of the presigmoid dura and of the PSCC. Grade I SS was associated with good pneumatization and highermastoid volumescompared with grades II and III. Conclusions The SS exhibits considerable anatomic variability. A favorable positioning of the SS is associated with a large mastoid volume and pneumatization. A careful preoperative study of the imaging may help in understanding the positioning of the SS and the safety of various transmastoid approaches.

Imaging characteristics of patients with large vestibular aqueduct syndrome and its relationship with the acoustically evoked short latency negative response / 中华耳鼻咽喉头颈外科杂志

Objective@#To explore the imaging characteristics of large vestibular aqueduct syndrome (LVAS) patients and their relationship with the acoustically evoked short latency negative response (ANSR), so as to provide reference for the diagnosis of LVAS.@*Methods@#Clinical data of 174 patients(334 ears) with LVAS diagnosed and treated by the Department of Otorhinolaryngology Head and Neck Surgery of the First Affiliated Hospital of Guangxi Medical University, from October 2009 to December 2017 were retrospectively analyzed, including 117 males and 57 females, aged from 5 months to 47 years old, with the median age of 4 years and 4 months. ABR and imaging data of patients were collected. Midpoint diameter and the outlet diameter of the vestibular aqueduct were measured on CT images, the midpoint diameter of the intraosseous parts and the extraosseous parts of enlarged endolymphatic sac(EES) were measured on MRI images. The correlation between the above measurements was analyzed by Pearson test using SPSS 17.0. According to whether ASNR was detected in ABR, the above data were divided into two groups, and the differences of the above imaging measurements were compared by the Independent-Sample Test.@*Results@#The average midpoint diameter of the vestibular aqueduct was (1.87±0.58) mm (±s, the following was the same), and the outlet diameter was (3.07±0.99) mm on CT; the average midpoint diameter of the intraosseous parts in enlarged endolymphatic sac(EES) was (2.39±1.37) mm, and the extraosseous parts was (2.50±2.18) mm on MRI. There was a correlation between the four measurements (P<0.05), among which the midpoint diameter of vestibular aqueduct was strongly positively correlated with the outlet diameter (r=0.760), and the remaining pairs were weakly correlated. ASNR was detected in 241 ears (72.16%,241/334) and undetected in 93 ears (27.84%, 93/334) of the 334 ears with LVAS. Midpoint diameter and the outlet diameter of the vestibular aqueduct in no ASNR group were smaller than the ASNR group, and the difference was statistically significant (t value was 2.814 and 2.754, P<0.05). There was no significant difference in the midpoint diameter of the intraosseous parts and the extraosseous parts of enlarged endolymphatic sac between the two groups, and the difference was no statistically significant(t value was 0.101 and 0.683, P>0.05).@*Conclusions@#There is a strong positive correlation between the midpoint diameter of vestibular aqueduct and the outlet diameter in LVAS patients. There is a certain correlation between the size of vestibular aqueduct and the size of endolymphatic sac. The smaller the diameter of vestibular aqueduct, the lower the occurrence rate of ASNR.

Analysis of the correlation between radiological and audiological features of patients with enlarged vestibular aqueduct / 中华耳鼻咽喉头颈外科杂志

Objective@#The radiological and audiological results of patients with hearing loss associated with enlarged vestibular aqueduct (EVA) were analyzed statistically to explore the association between them.@*Methods@#In this retrospective study, we screened 64 patients (128 ears) with EVA diagnosed in the Department of Otorhinolaryngology, Shengjing Hospital of China Medical University from January 2012 to June 2016, who met the inclusion criteria and the exclusion criteria at the same time, including 37 males (74 ears) and 27 females (54 ears), aged from 6 months to 17 years, all of whom showed varying degrees of sensorineural hearing loss (SNHL). The imaging observations included the midpoint measurement (MP) and the operculum measurement(OP) of the temporal bone HRCT, the long signal area cross-sectional area (ESL), the short signal area cross-sectional area (ESS), as well as the largest total signal area cross-sectional area (EST) of the endolymphatic sac(ES) of the cochlear MRI. The audiological observations included collecting detailed medical history and subjective and/or objective audiological examinations to determine the character and degree of hearing loss. According to the progress and changes of hearing loss, they were divided into hearing stability group (86 ears) and fluctuation/progression group (42 ears). SPSS22.0 statistical software was used to carry out statistical analysis of the imaging measurement results.@*Results@#The sizes of MP and OP were smaller in the stable group than those in the fluctuating/progressive group, and the difference was statistically significant (P<0.05). For ESL, ESS or EST, there was no significant difference between the stable group and the fluctuating/progressive group (P>0.05). For the 13 patients with asymmetric hearing loss, the sizes of MP, OP, ESL, ESS or EST were not significantly different between the mild side and the serious side (P>0.05). For the ears whose ESL/ESS was>2 or the ESS/ESL was>2, the proportion was not significantly different between the stable group and the fluctuating/progressive group (P>0.05).@*Conclusions@#For patients with EVA,the smaller the enlarged vestibular aqueduct is,the more likely it is congenital severe sensorineural hearing loss. Patients with significantly enlarged vestibular aqueduct often exhibit volatility/progressive hearing loss. The degree of enlargement of the endolymphatic vessels and endolymphatic sac is not related to the degree of hearing loss. For EVA patients with asymmetric hearing loss, the mild or serious side of hearing loss is random, which is not affected by the degree of enlargement of the vestibular aqueduct and endolymphatic sac. The degree of hearing loss in patients with EVA is not related to the ratio of ESL/ESS.

Long-term results of endolymphatic sac mastoid drainage for Ménière disease / 临床耳鼻咽喉头颈外科杂志

To investigate the long-term efficacy of endolymphatic sac mastoid drainage for Ménière disease.Data from 26 patients diagnosed with MD strictly meeting the criteria issued by " Guideline of diagnosis and treatment of Meniere disease(2017)" from 2006 to 2015 were analyzed in this study.Endolymphatic sac mastoid shunt surgery was performed for each patient.The therapeutic effect was evaluated against the " Guideline of diagnosis and treatment of Meniere disease(2017)".Vertigo control and auditory function were measured after at least two years follow up.In 26 cases,16 cases were male and 10 cases were femaleThe age ranged from 24 to 71 years old,with an average of 52.04 years.The disease duration ranged from 1 to 32 years.22 cases were diagnosed as unilateral Ménière disease,and bilateral involvement was identified in 4 cases,thus a total of 30 ears were included.According to the preoperative staging of hearing,there were 0 cases in stage one,5 cases in stage two,16 in stage three and 9 cases in stage four.15 cases(57.7%)achieved class A vertigo conrol(complete control),9 cases(34.6%)class B(substantial control)and 2 cases(7.7%)class D(no control).The severity of vertigo and its impact on daily life were improved in 24 cases(92.3%)with a score of 0 point,and 2 cases(7.7%)scored 2 points.Post-operative hearing was improved in 3 cases(11.5%),unchanged in 16 cases(61.6%)and worsened in 7 cases(26.9%).After operation,tinnitus disappeared in 5 cases(19.2%),reduced in 13 case(50%)and unchanged in 8 cases(30.8%).Endolymphatic sac mastoid drainage was an effective and safe management for intractable Ménière disease patients with pre-operative residual hearing.The occurrence of complication was unsual.The patients who are in stage four could gain benifits from the surgery.

Endolymphatic Sac Surgery for Ménière's Disease - Current Opinion and Literature Review

Abstract Introduction The endolymphatic sac is thought to maintain the hydrostatic pressure and endolymph homeostasis for the inner ear, and its dysfunction may contribute to the pathophysiology of Ménière's disease. Throughout the years, different surgical procedures for intractable vertigo secondary to Ménière's disease have been described, and though many authors consider these procedures as effective, there are some who question its long-term efficacy and even those who think that vertigo control is achieved more due to a placebo effect than because of the procedure itself. Objective To review the different surgical procedures performed in the endolymphatic sac for the treatment of Ménière's disease. Data Sources PubMed, MD consult and Ovid-SP databases. Data Synthesis We focus on describing the different surgical procedures performed in the endolymphatic sac, such as endolymphatic sac decompression, endolymphatic sac enhancement, endolymphatic sac shunting and endolymphatic duct blockage, their pitfalls and advantages, their results in vertigo control and the complication rates. The senior author also describes his experience after 30 years of performing endolymphatic sac surgery. Conclusions The endolymphatic sac surgery, with all its variants, is a good option for patients with incapacitating endolymphatic hydrops, providing a high percentage of vertigo control and hearing preservation.

Aggressive papillary tumor of endolymphatic sac: case report of a rare neoplasia / Tumor papilar agressivo do saco endolinfático: relato de caso de uma rara neoplasia

ABSTRACT Aggressive papillary endolymphatic sac tumor (ELST) is a rare neoplasm, occasionally related to von Hippel-Lindau disease, characterized by locally aggressive growth with temporal bone destruction. The authors report a case of ELST in a female patient exhibiting fifth through eighth cranial nerve paralysis. Computed tomography (CT) revealed a large lytic process involving the right temporal bone. The patient underwent surgical resection. At microscopy, a neoplastic process was identified exhibiting monomorphic columnar cells with mild atypias, arranged in a papillary pattern. The lesion exhibited positivity for A31/AE3, epithelial membrane antigen (EMA), and vimentin; and negativity for synaptophysin, glial fibrillary acidic protein (GFAP), neuron-specific enolase (NSE), thyroglobulin, transthyretin, chromogranin, thyroid transcription factor 1 (TTF-1), trans-acting T-cell specific transcription factor GATA-3, and intestinal transcription factor CDX-2. The diagnosis of ELST was then established. Six years after surgical resection, lesion recurrence was observed.

RESUMO O tumor papilar agressivo do saco endolinfático (TPASE) é uma neoplasia rara, ocasionalmente relacionada com a doença de von Hippel-Lindau, que se caracteriza pelo crescimento agressivo local com destruição do osso temporal. Os autores relatam um caso de TPASE em paciente do sexo feminino, exibindo paralisia do quinto ao oitavo par craniano. A tomografia computadorizada (TC) revelou grande processo lítico comprometendo o osso temporal direito. A paciente foi submetida a ressecção cirúrgica. À microscopia, identificou-se processo neoplásico que exibiu células cilíndricas monomórficas com atipias leves, dispostas em padrão papilar. A lesão apresentou positividade para AE1/ AE3, antígeno da membrana epitelial (EMA) e vimentina; e negatividade para sinaptofisina, proteína ácida fibrilar glial (GFAP), enolase específica do neurônio (NSE), tireoglobulina, transtirretina, cromogranina, fator de transcrição da tireoide 1 (TTF-1), fator de transcrição de ação trans específico de células GATA-3 e fator de transcrição intestinal CDX-2. O diagnóstico de TPASE foi então estabelecido. Após seis anos da ressecção cirúrgica, foi identificada recorrência da lesão.

Changes in endolymphatic hydrops visualized by magnetic resonance imaging after sac surgery / 华中科技大学学报（医学）（英德文版）

The purpose of the study was to observe changes in endolymphatic hydrops by using intratympanic injection of gadolinium and magnetic resonance imaging (MRI) before and after endolymphatic sac surgery in patients with unilateral Meniere's disease. Thirteen patients with unilateral Meniere's disease undergoing endolymphatic sac surgery were retrospectively and prospectively analyzed. Three-dimensional fluid-attenuated inversion recovery or three-dimensional real inversion recovery MRI was performed 24 h after an intratympanic injection of gadolinium to grade the presence of endolymphatic hydrops. Among the 13 patients with hydrops confirmed by preoperative MRI, vestibular hydrops had no significant change in all patients; cochlear hydrops became negative in 2 patients, and remained unchanged in the other 11 patients after surgery. Definite vertigo attacks were substantially controlled in one patient and completely controlled in 12 patients during a follow-up period of 8-34 months after surgery. The hearing levels were improved in 3 patients, remained unchanged in 7 patients, and decreased in 3 patients. In conclusion, endolymphatic sac surgery does not always alleviate endolymphatic hydrops in patients with Meniere's disease. Relief from vertigo cannot always be attributed to the remission of hydrops. A change in hearing levels cannot be explained by hydrops status alone.

Semicircular Canal Plugging Based on Endolymphatic Sac Decompression:The Surgical Technique Discussion for Intractable Meniere's Disease / 听力学及言语疾病杂志

Objective To study the effects of endolymphatic sac decompression and on semicircular canal plugging in treating intractable Meniere's disease.Methods A total of 14 cases of intractable Meniere's disease were included in this study.The age was from 33~67 years old and the history was from 1 to 40 years.The hearing level of bone was from 35 to 65 dB for the speech frequencies.All cases received the treatments according to the pre-op-erative design.The endolymphatic sac was decompressed and the three semicircular canal bones were drilled to cre-ate a fenestra followed by soft tissue plugging into the canal.The vertigo,hearing level and tinnitus were included in the following up.Results All cases had no facial palsy,no cerebrospinal leak,and no vertical after surgery.In the period of 3 to 18 months following up,the attack of Meniere's disease was completely controlled for 13 cases excep-tion of one lost case.For five cases,the hearing level descended 10 to 15 dB compared to pre-operation.Conclusion Although the endolymphatic sac decompression can relieve endolymphatic pressure,this may not adequately pro-tect the hearing caused by the semicircular canal plugging.The surgical technique is reliable and safe;however fur-ther clinical data should be gathered.

Tumarkin Otolithic Crisis Controlled by Endolymphatic Sac Surgery / 대한평형의학회지

Turmarkin otolithic crisis is a rare feature of Meniere's disease. It shows sudden falling to the ground with no warning sign. It is an otologic emergency because of the risk of falling, and it has traditionally been treated with labyrinthectomy or vestibular neurectomy. We experienced a 49-year-old male suffering from recurrent drop attack, and found that he had hearing loss, tinnitus or recurrent vertigo on his left ear, and could make a diagnosis him as Tumarkin otolithic crisis. We have performed the endolymphatic sac decompression, considering the hearing preservation and therapeutic opinion of patients. Two years after surgery, he showed intermittent, mild dizzy symptoms, without further drop attack. Therefore, we report our clinical experience with a brief review of literature.

Hemangioma of Endolymphatic Sac in Von Hippel-Lindau Patient / 대한이비인후과학회지

The present study reports a rare case of capillary hemangioma of endolymphatic sac. A 23-year-old male who underwent von Hippel-Lindau disease presented with recurrent sudden sensorineural hearing loss. Magnetic resonance imaging revealed a heterogenous enhanced mass in the right endolymphatic sac, which was hyperintense on the enhanced T1-weighted images and inhomogenous on the T2-weighted images. Pre-operatively, this tumor was believed to be an endolymphatic sac tumor because of the history of von Hippel-Lindau disease. During the surgery, vascular tumor was removed by transmastoid approach. A histopathological examination indicated that the tumor was a capillary hemangioma. To the best of our knowledge, the present study is the second case of hemangioma in the endolymphatic sac and first case of von Hippel-Lindau disease.

A Case of Endolymphatic Sac Decompression of Intractable Meniere's Disease in Children / 대한이비인후과학회지

Meniere's disease in children is characterised with a hard and long-term diagnostic course, for which there is no accurate classification and treatment plan. A patient may undergo severe discomfort and long-term contraction of the disease. We experienced a 13-year-old female suffering from recurrent dizziness, and found that she had hearing loss, and tinnitus on her right ear. We diagnosed it as possible meniere's disease, but the disease progressed despite a trial of medication. The patient then underwent an endolymphatic sac decompression. During 6 months after the operation, she has shown only mild, intermittent dizzy symptoms, without severe vertigo. We report this clinical experience with a brief review of literature.

The Anatomic Differences of Endolymphatic Sac among Common Laboratory Rodents / 听力学及言语疾病杂志

Objective To compare the differences of laboratory rodents endolymphatic sac (ES) in histology , and to provide an anatomic basis for the clinical research of ES -related diseases .Methods The temporal bones of guinea pigs ,SD rats and KM mice were fixed by heart perfusion ,sectioned by paraffin at the horizontal position and stained by hematoxylin -eosin (HE) .Results All 3 rodents ES contained sac ,epithelium ,sub-mucosal tissue and saccal stroma .The cells were bulky ,stained deep and protruded into the cavity to form a large number of nipples and wrinkles in the middle ES epithelial of guinea pigs .But the nipples or wrinkles was rarely observed in the middle ES epithelial in rat and mouse .Conclusion The histology of ES has significant difference among guinea pig ,mouse and rat .While the difference betweeen mouse and rat is not obvious .

Current evidence for endolymphatic sac surgery in the treatment of Meniere's disease: a systematic review

This paper aims to identify emerging evidence for endolymphatic sac surgery (ESS) in the treatment of Meniere's disease since the landmark study by Thomsen et al, published in 1998 (conducted from 1981 to 1989). Using the MEDLINE database (PubMed), a systematic review of the literature published from January 1990 to June 2014 was performed. We included all English-language, peer-reviewed randomised controlled trials (RCTs) and controlled studies. Single-arm cohort studies were included if the sample size was ≥ 90 with a response rate > 60%. Altogether, 11 studies fulfilled our inclusion criteria; one was an RCT, two were controlled trials and eight were single-arm cohort studies. There currently exists a low level of evidence for the use of ESS in the treatment of Meniere's disease. Further studies, in particular RCTs and/or controlled studies, are required to fully evaluate this modality. However, there are difficulties in designing a valid placebo and achieving adequate blinding of observers and investigators.

Profound Hearing Loss after Endolymphatic Sac Decompression in Intractable Meniere's Disease / 대한평형의학회지

Endolymphatic sac decompression (ESD) is indicated in intractable Meniere's disease patients with serviceable hearing. A 43-year-old man presented with recurrent vertigo and fluctuating right hearing loss that had been intractable to medical treatment. ESD was performed for the purpose of vertigo control with hearing preservation. Positional vertigo with profound hearing loss developed immediate after surgery and positional vertigo was resolved within days. Following paralytic vestibulopathy with positive sign on head thrust test also resolved after 2 weeks, while sensorineural hearing loss was not recovered to preoperative level during 1 year of follow up. Recurrent vertigo attacks were developed again 1 year after the operation. Hearing preservation was not always guaranteed in ESD. Furthermore, chance of hearing loss should be included in informed consent though the procedure is purposed for hearing preservation.

A Case of Post-Traumatic Meniere's Disease

There are only a few reports of post-traumatic Meniere's disease and there is few literature that contains detailed data associated with the disease. We report a case of post-traumatic Meniere's disease. He suffered from tinnitus, fluctuating sensorineural hearing loss, and recurrent vertigo. Symptomatic medical treatment was not helpful and neither was soft tissue plugging around the oval and round windows during exploratory tympanotomy. Three months after soft tissue plugging, endolymphatic sac decompression surgery was performed. The patient's symptoms improved markedly thereafter. The clinical significance of post-traumatic Meniere's disease is described and we present a brief review of the literature.