Objective:To summarize the clinical characteristics and surgical outcomes of endolymphatic sac tumor（ELST）, and improve the experience of diagnosis and treatment of this disease. Methods:A retrospective analysis was conducted on the clinical data of patients with ELST who underwent surgical treatment by the Department of Otolaryngology Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University from January 2015 to December 2024.The clinical and image features, perioperative management, surgical methods and follow-up results of the disease were summarized. Results:Of the 6 cases, 4 were male and 2 were female. The primary clinical characteristics were hearing loss（6 cases）, tinnitus（5 cases）, dizziness（2 cases）, facial paralysis（1 case）, and headache（1 case）. CT and MRI of temporal bone were performed in all cases. The manifestation of CT was a space occupying lesion centered on the region of endolymphatic sac, accompanied by bone destruction and intertumoral calcification. MRI showed tumor center isosignal and peripheral hypersignal in T1 and T2 sequences in 3 cases, and mixed hypersignal in T1 and T2 sequences in 3 cases. Enhancement was observed in all cases on the enhanced scan. 5 cases underwent DSA examination and showed the tumors were supplied by the occipital artery（2 cases）, posterior auricular artery（4 cases）, and the bunch of internal carotid artery（1 case）. Embolization of the feeding artery was performed in 3 patients. Five patients underwent tumor resection. Translabyrinthine approach were performed in 4 cases, and middle cranial fossa approach was performed in 1 case. All cases followed up for 24 to 70 months with no distant metastases or death. Two patients experienced twice recurrences and were treated with surgical operation. The tumors were found to be closely related to the internal auditory canal or dura during the surgery. Conclusion:The clinical manifestations of ELST are not typical, and hearing loss is the most common clinical symptom. CT and MRI exhibit typical imaging characteristics. ELST has a risk of recurrence, and the tumor should be carefully managed when invade to the internal auditory canal and dura to reduce postoperative recurrence.
Humans ; Endolymphatic Sac/surgery* ; Male ; Female ; Retrospective Studies ; Ear Neoplasms/diagnosis* ; Magnetic Resonance Imaging ; Tomography, X-Ray Computed ; Adult ; Middle Aged ; Treatment Outcome

Humans ; Facial Nerve/surgery* ; Endolymphatic Sac/surgery* ; Cranial Sinuses ; Postoperative Complications ; Neoplasms

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.
Female ; Humans ; Adult ; Endolymphatic Sac/surgery* ; Neoplasm Recurrence, Local/pathology* ; Labyrinth Diseases ; Tinnitus ; Ear Neoplasms/pathology* ; Bone Neoplasms ; Hemorrhage

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.
Adult ; Aged ; Drainage ; Endolymphatic Sac ; Female ; Follow-Up Studies ; Humans ; Male ; Mastoid ; Meniere Disease ; therapy ; Middle Aged ; Vertigo ; Young Adult

To investigate the postoperative anxiety and its relationship with life quality in patients with Ménière's diseases.
 Methods: A total of 68 patients with Ménière's disease, who received the treatment of endolymphatic sac decompression from 2010 to 2016, were enrolled. They finished two scales (the self-rating anxiety scale and the quality of life questionnaire for endolymphatic sac decompression of Ménière's disease) by snail mail. The patients were divided into different groups based on their preoperative course, postoperative follow-up time, clinic stage and quality of life after endolymphatic sac decompression surgery, and their anxiety was analyzed. In addition, 109 gender and age-matched patients with other diseases of otorhinolaryngology served as the control group, and their anxiety was also analyzed.
 Results: There was no statistical difference in the postoperative anxiety among the patients with preoperative course for less than 1 year, 1 to 5 years or more than 5 years (all P>0.05). There was no statistical difference in the patients with different duration of follow-up (P>0.05). There was no statistical difference in the patients with the different clinic stages (all P>0.05). There was no statistical difference in the patients with the improved life or non-improved life after surgery (P>0.05). The degree of the anxiety in the Ménière's group was more severe than that in the control group (P<0.01).
 Conclusion: It needs to keep in mind that a certain degree of postoperative anxiety still keeps in patients with Ménière's disease.
Anxiety ; Anxiety Disorders ; Case-Control Studies ; Decompression, Surgical ; methods ; Endolymphatic Sac ; surgery ; Humans ; Meniere Disease ; psychology ; surgery ; Postoperative Complications ; psychology ; Quality of Life

Inner ear is composed of cochlea, vestibule, and endolymphatic sac which are enclosed by thin layer of epithelial cells. The enclosed space is filled with fluid named as endolymph where the [K⁺] is high and [Na⁺] is low. This unique ion composition is very important in maintaining normal hearing and balance function by providing K⁺ ions into sensory hair cells, which finally depolarize hair cells to facilitate the transport of sound and acceleration stimulation to central nervous system. The ion composition of inner ear is maintained by various ion transport through ion channels, transporters, and exchangers in the inner ear sensory and extra-sensory epithelium. The disruption of normal endolymphatic ion composition by the deterioration of the function of those ion channels can cause dysfunction of sensory epithelium, which consequently results in hearing and balance disorders. One of the possible pathology from the disruption of inner ear ion homeostasis is endolymphatic hydrops which is a phenomenon of excessive fluid accumulation of inner ear. The dysfunction of ion channels in inner ear epithelium can be an etiology of Ménière's disease since endolymphatic hydrops is a main pathological finding of the disease. In this review, we discussed about the possible pathological mechanism of Ménière's disease as a perspective of channelopathy as well as the role of various ion channels in the regulation of inner ear fluid volume based on the findings revealed by electrophysiological studies.
Acceleration ; Central Nervous System ; Channelopathies ; Cochlea ; Ear, Inner ; Electrophysiology ; Endolymph ; Endolymphatic Hydrops ; Endolymphatic Sac ; Epithelial Cells ; Epithelium ; Hair ; Hearing ; Homeostasis ; Ion Channels ; Ion Transport ; Ions ; Meniere Disease ; Pathology

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.
Adult ; Aged ; Contrast Media ; administration & dosage ; Endolymphatic Hydrops ; diagnostic imaging ; pathology ; surgery ; Endolymphatic Sac ; diagnostic imaging ; pathology ; surgery ; Female ; Gadolinium ; administration & dosage ; Humans ; Imaging, Three-Dimensional ; Magnetic Resonance Imaging ; Male ; Meniere Disease ; diagnostic imaging ; pathology ; surgery ; Middle Aged

Cholesterol granuloma is rare and has occasionally been reported in uncommon sites of the head and neck, such as in the nasal sinus (frontal, ethmoid, sphenoid, or maxillary sinus), endolymphatic sac, and pterygoid process of the sphenoid sinus. The pathogenesis of thisnrare disease is not clear. We experienced one patient with cholesterol granuloma located in the nasal septum, which was managed successfully by endoscopic assisted excision.
Cholesterol* ; Endolymphatic Sac ; Granuloma* ; Head ; Humans ; Nasal Septum* ; Neck ; Sphenoid Sinus

OBJECTIVE: To compare the vertigo controlling situation between the endolymphatic sac decompression(ELSD) and semicircular canal occlusion (SCO) in stage 4 Ménière disease. METHOD: Fourteen patients who underwent endolymphatic sac decompression and 9 patients who underwent semicircular canal occlusion from 2009 to 2013 were followed. All patients has complete preoperative examination and postoperative follow-up. RESULT: The vetigo control of the patients underwent endolymphatic sac decompression: completely control 35.7%; basic control 28.6%; partly control 14.3%; the vetigo control of the patients underwent semicircular canal occlusion: completely control 88.9%; basic control 11.1%. CONCLUSION ELSD and SCO are alternative methods for the patients of stage 4. SCO has a much highter complete vertigo control rate. For the patients without practical listening, SCO is a better choice.
Endolymphatic Sac ; surgery ; Humans ; Meniere Disease ; surgery ; Otologic Surgical Procedures ; methods ; Semicircular Canals ; surgery ; Vertigo ; surgery

Endolymphatic hydrops is a representing pathologic finding of Ménière's disease. For the induction of endolymphatic hydrops in an animal model, surgical ablation of endolymphatic sac has been used. Although traditional model with the blockage of endolymphatic sac induced severe hydrops, it has several limitations for the study of pathophysiology of Ménière's disease. Recently, modified experimental models have been introduced, in which additional procedure was performed to induce the acute aggravation of hydrops after the surgical ablation. These new models could be helpful to elucidate the mechanism and develop a new treatment of Ménière's disease. In this review, we introduce the characteristics of animal models using surgical ablation of endolymphatic sac from the classical model to novel modified models.
Edema ; Endolymphatic Hydrops ; Endolymphatic Sac ; Meniere Disease ; Models, Animal ; Models, Theoretical