Endolymphatic sac tumor (ELST) is very rare but its aggressive papillary neoplasm destructs the temporal bone widely and often involves the intracranial structures. The most effective treatment is complete surgical removal, but it is very difficult because the character of tumor is very aggressive and extensive to the intracranial region. This tumor is not yet reported in Korea but we have recently experienced one case of ELST which we treated surgically by the traslabyrinthine/petrosal combined approach. We report the clinical and histological aspects of this rare tumor with a review of the literatures.
Endolymphatic Sac* ; Korea ; Temporal Bone

Meningioma's account for around 15% of all primary brain tumors with some 10% of meningiomas arising in the posterior fossa. In rare cases, a meningioma can form around the endolymphatic sac. When formed in the posterior fossa, meningioma tumors can produce vague, non-specific vertiginous symptoms. Research has observed that a subset of these lesions could produce symptoms indistinguishable from those of Meniere's disease. Therefore, we described the clinical features of a case of posterior petrous meningioma with recurrent vertigo as well as the substantial resolution of symptoms after tumor removal via transmastoid approach.
Brain Neoplasms ; Endolymphatic Hydrops ; Endolymphatic Sac ; Meniere Disease ; Meningioma ; Vertigo

Ear Neoplasms ; diagnosis ; Endolymphatic Sac ; pathology ; Humans

Endolymphatic sac tumor (ELST) is a very rare tumor arising from the epithelium of the endolymphatic sac. Pathologically, ELST shows papillary growth patterns with extensive bony and soft tissue destruction. ELST commonly shows symptoms of endolymphatic hydrops such as hearing loss, vertigo and tinnitus by blocking the endolymphatic duct. As the tumor enlarges, patients experience various symptoms that include multiple cranial nerve palsy. Ten to thirty percents of ELST are related to Von Hippel Lindau (VHL) diseases. Also, bilateral tumors can develop when a patient has VHL-related ELST. The goal of treatment for ELST is total tumor eradication. The type of surgical approach is chosen by the surgeon in consideration of the tumor grade and the hearing status of the patient. In this report, we present a patient with ELST who was successfully treated with the modified translabyrinthine approach. We have also reviewed relevant literatures.
Cranial Nerve Diseases ; Endolymphatic Duct ; Endolymphatic Hydrops ; Endolymphatic Sac ; Epithelium ; Hearing ; Hearing Loss ; Humans ; Tinnitus ; Vertigo

Adenocarcinoma ; pathology ; Aged ; Ear Neoplasms ; pathology ; Endolymphatic Sac ; Humans ; Male

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.
Decompression ; Endolymphatic Hydrops ; Endolymphatic Sac ; Hearing Loss, Sensorineural ; Meniere Disease* ; Tinnitus ; Vertigo

Endolymphatic sac tumor is rare, locally aggressive hypervascular tumor of papillary structure, arising from the endolymphatic duct or sac in the posterior petrous bone. We present four cases with this tumor. Two patients were male and the other two were female. Age of each patient was 15, 52, 58, and 67 years. Three patients presented with progressive hearing loss and sustained vertigo for months to years and another one was referred for the tumor detected in routine medical check-up. Preoperative embolization was performed in 3 patients. Complete excision of the tumor was achieved in all patients using translabyrinthine or retrosigmoid approach. Herein, we describe the clinical and radiographic features, surgical treatment and pathologic findings with a review of the literature.
Endolymphatic Duct ; Endolymphatic Sac ; Female ; Hearing Loss ; Humans ; Male ; Petrous Bone ; Vertigo

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

OBJECTIVETo study the expression of aquaporin 1 (AQP1) in cochlea and endolymphatic sac of guinea pig of two types of animal model of endolymphatic hydrops.

METHODSThirty guinea pigs were divided into three groups at random: surgery group; aldosterone group and control group. Each group included 10 animals. Surgical endolymphatic sac obstruction or aldosterone intraperitoneal injection was used to build the animal models of endolymphatic hydrops. Immunohistochemistry and western blot were used to examine the expression of AQP1 in cochlea and endolymphatic sac of animal models. Image processing soft (Image Tool) was used to do the semiquantitative analysis.

RESULTSMiddle to high grade endolymphatic hydrops were found in surgery group. It was most significant in the apical coil, and lessened from the apical coil to the basal coil. Low to middle expression grade in endolymphatic hydrops was found in aldosterone group. Most of them were found in the basal coil. The positions of AQP1 expression in two animal models of endolymphatic hydrops were identical with the control group. In cochlea, no difference of AQP1 expression was found in the surgery and control group (t = 0.718 , P > 0.05) , but the expression of AQP1 was down regulated in the cochlea of aldosterone group (t = 6.609, P < 0.01) while the expressions of AQP1 in endolymphatic sac of aldosterone and control group were no difference between them (t = 0.998, P > 0.05). The quantization of AQP1 protein in the lateral wall of cochlea of aldosterone group was lower than that of control group (t = 13.626 , P < 0.01).

CONCLUSIONSThe expression of AQP1 is no change in surgery and control group, but decreased in aldosterone group. The expression of AQP1 may be regulated by the ionic concentration in inner ear of guinea pig.

Animals ; Aquaporin 1 ; metabolism ; Cochlea ; metabolism ; Disease Models, Animal ; Endolymphatic Hydrops ; metabolism ; Endolymphatic Sac ; metabolism ; Guinea Pigs

A large vestibular aqueduct, an isolated anomaly of temporal bone, is relatively rare and its association with sensorinerual hearing loss in childhood is known as the enlarged vestibular aqueduct syndrome. Using MRI, we found enlarged endolymphatic ducts and sacs in four ears of our two patients. Signal intensity of enlarged endolymphatic sac that is detected higher than that of CSF in Fluid Attenuated Inversion Recovery (FLAIR) image may indicate poor prognisis with respect to bone conduction threshold in pure tone audiogram.
Bone Conduction ; Ear ; Endolymphatic Duct ; Endolymphatic Sac ; Hearing Loss ; Humans ; Magnetic Resonance Imaging ; Temporal Bone ; Vestibular Aqueduct*