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Tumors of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Endolymphatic sac cystadenoma of the ear: Located in the temporal bone.5% of patients with VHL.May be bilateral.Often present with vertigo and hearing loss.
Acute vertigo
Published in S. Musheer Hussain, Paul White, Kim W Ah-See, Patrick Spielmann, Mary-Louise Montague, ENT Head & Neck Emergencies, 2018
Rahul Kanegaonkar, Max Whittaker
If first-line treatments fail to control the attacks, surgery may be appropriate. If functional hearing exists in the affected ear, grommet insertion in isolation or combined with the Meniett device may be appropriate. The evidence base is poor for both these procedures. The device has been approved by the National Institute for Health and Care Excellence (NICE) for monitored usage. The use of steroid injection into the middle ear has been gaining momentum, but equally the evidence base is sparse. Endolymphatic sac decompression has been used in the management of Ménière’s disease for many years. This is achieved via a cortical mastoidectomy, exposure of the posterior fossa dura and incision of the sac and/or insertion of a silastic tube. This has been a controversial procedure but has proponents, and indeed patients, who find it helpful.
Tumours of the Temporal Bone
Published in John C Watkinson, Raymond W Clarke, Christopher P Aldren, Doris-Eva Bamiou, Raymond W Clarke, Richard M Irving, Haytham Kubba, Shakeel R Saeed, Paediatrics, The Ear, Skull Base, 2018
Marcus Atlas, Noweed Ahmad, Peter O’Sullivan
In 1988, Gaffey et al. 18 proposed the term aggressive papillary middle ear tumour (APMET) for adenomatous middle ear tumours that have a primarily papillary architecture. As distinct from the middle ear adenoma described above, these tumours were extremely aggressive locally invading bone and possibly extending intracranially. In Gaffey’s series, brain invasion was seen in one patient and no patient had evidence of distant metastases. All of these patients were treated with surgery and, in some, adjuvant radiotherapy. In 1989, Heffner proposed that the middle ear tumour previously described as the papillary middle ear tumour was in fact derived from the endolymphatic sac.21 Li et al. 22 confirmed their findings in 1993 using similar histochemical and histopathological techniques. Terms such as Heffner tumour, low grade adenocarcinoma of endolymphatic sac origin, and aggressive papillary middle ear tumour are now obsolete and it should be described as endolymphatic sac tumour (ELST) according to the more recently published World Health Organization tumour classification.23
Intraoperative auditory brainstem response monitoring during semicircular canal plugging surgery in treatment of Meniere’s disease
Published in Acta Oto-Laryngologica, 2021
Xiaofei Li, Yafeng Lv, Ruijie Wang, Xiuhua Chao, Zhaomin Fan, Haibo Wang, Daogong Zhang
Meniere’s disease (MD) is one of the most common causes of vertigo in humans, which is characterized by intermittent episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural pressure. Its prevalence ranges from 2 to 513 per 100,000 [1]. Because its definitive pathogenesis has not been completely clarified, there is currently no cure for this disorder. The primary histopathological correlate is endolymphatic hydrops, which is considered as the imbalance of the homeostasis of the endolymph at the level of the endolymphatic sac. The role of endolymphatic sac in MD has been debated. Some believed that endolymphatic sac plays an important role in the absorption of the endolymphatic fluid [2]. However, some argued that endolymphatic sac could secrete auto-antigens or trophic factors, cause endolymphatic sac cells hyperactivity and lead to hydrops [3,4].
The pharmacological management of vertigo in Meniere disease
Published in Expert Opinion on Pharmacotherapy, 2020
Juan Manuel Espinosa-Sanchez, José A. Lopez-Escamez
The accumulation of endolymph in the membranous labyrinth, leading to an increased pressure in the cochlear duct, is termed endolymphatic hydrops, and it is considered the pathological substrate of the syndrome, but it can be also found in other conditions or even in healthy subjects [6]. In any case, the endolymphatic hydrops would be the common denominator of a series of inflammatory processes in different parts of the cochlea (lateral wall, endolymphatic sac) leading to cochlear damage with autoinflammation or autoimmunity responses resulting from multiple causes (allergy, viral infection, genetic variants) that affect the inner ear homeostasis, lead to the accumulation of endolymph and manifest with a common core phenotype (episodic vertigo with fluctuating auditory symptoms) that defines a clinically heterogeneous syndrome. Classically, when this set of symptoms cannot be attributed to a specifically identified cause, the syndrome is considered idiopathic, and then it is referred to as MD.
Current state of evidence for endolymphatic sac surgery in Menière’s disease: a systematic review
Published in Acta Oto-Laryngologica, 2019
Louise Devantier, Jesper Hvass Schmidt, Bjarki Ditlev Djurhuus, Dan Dupont Hougaard, Mina Nicole Händel, Frank Liviu-Adelin Guldfred, Henriette Edemann-Callesen
Menière’s disease is characterized by spontaneous episodes of vertigo combined with tinnitus, aural fullness and fluctuating low frequency sensorineural hearing loss. It is a chronic inner ear disease where both hearing loss and vestibular deficits generally progress regardless of treatment. Even though the disease was first described more than 150 years ago, the etiology remains uncertain [1]. Endolymphatic hydrops due to endolymphatic malabsorption in the labyrinth’s endolymphatic sac is considered as a hallmark of Menière’s disease [1]. Within recent years, it has become possible to visualize endolymphatic hydrops with gadolinium magnetic resonance imaging (MRI). However, this has not been globally implemented and it is not a requirement in the newest set of diagnostic criteria from the Barany society [2]. As of now, there is no cure for Menière’s disease. Wide ranges of different treatment modalities exist including dietary salt restriction as well as treatment with diuretics in an attempt to influence the endolymphatic pressure imbalance. Severely disabled patients with Menière disease, who have failed to respond to other available treatment modalities, may be offered endolymphatic sac surgery. Endolymphatic sac surgery is performed using different surgical procedures such as endolymphatic sac decompression and duct blockage. Both methods tend to regulate the endolymphatic flow using different approaches.