Endolymphatic hydrops in superior canal dehiscence and large vestibular aqueduct syndromes

Sone, Michihiko; Yoshida, Tadao; Morimoto, Kazushige; Teranishi, Masaaki; Nakashima, Tsutomu; Naganawa, Shinji

doi:10.1002/lary.25747

Cited by 59 publications

(39 citation statements)

References 21 publications

(42 reference statements)

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“…There was, however, one patient with underlying semicircular canal dehiscence having no evidence of EH. Patients with large vestibular aqueduct syndrome had no specific relationship between severity of their syndrome and that of EH, but these patients had overall more severe EH compared to those with semicircular canal dehiscence (33). Findings of this study support SEH being a result of third window syndromes in affected patients, however, the paucity of other studies on this topic certainly requires further investigation of this topic.…”

Section: Resultsmentioning

confidence: 91%

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Secondary Endolymphatic Hydrops

Ferster

Cüreoğlu

Keskin

et al. 2017

Otology &Amp; Neurotology

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Hypothesis A review of the most recent literature will provide clinicians with an update of secondary endolymphatic hydrops, aiding in diagnosis and treatment of affected patients. Background Secondary endolymphatic hydrops is a pathologic finding of the inner ear resulting in episodic vertigo and intermittent hearing loss. It is a finding for which extensive research is being performed. Methods A review of the most recent literature on secondary endolymphatic hydrops was performed using PubMed literature search. Results Recent investigation of secondary endolymphatic hydrops has brought attention to traumatic and inflammatory insults as causes for secondary endolymphatic hydrops. Such etiologies, including post-surgical effects of cochlear implantation and endolymphatic sac ablation; otosclerosis and its operative intervention(s); acoustic and mechanical trauma; medications; and systemic inflammatory processes, have been determined as causes of secondary lymphatic hydrops. Histopathological slides for many of the etiologies of secondary endolymphatic hydrops are presented. Conclusions Through an understanding of the pathophysiology and etiologies of secondary endolymphatic hydrops, clinicians will gain a better understanding of this complex disease process, which will aid in treatment of patients with this disease process.

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Section: Resultsmentioning

confidence: 91%

“…A study by Sone et al has recently been published, revealing varying degrees of endolymphatic hydrops as a result of underlying semicircular canal dehiscence (33.) There was, however, one patient with underlying semicircular canal dehiscence having no evidence of EH.…”

Section: Resultsmentioning

confidence: 99%

Secondary Endolymphatic Hydrops

Ferster

Cüreoğlu

Keskin

et al. 2017

Otology &Amp; Neurotology

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“…For diagnosis, there are many available tests that appear to represent the abnormal pressure transmission associated with a third mobile window (ocular and cervical VEMPs, pure tone audiometry, and ECoG), nevertheless, improved CT imaging techniques such as cone beam or flat panel CT may improve diagnostic accuracy. A recent study observed that some patients with SCDS may also have endolymphatic hydrops as determined by MRI with intratympanic gadolinium (114). This observation needs to be confirmed in a larger, well-defined population, for if confirmed, it may have implications for etiology.…”

Section: Future Directionsmentioning

confidence: 99%

Superior Canal Dehiscence Syndrome: Lessons from the First 20 Years

2017

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Superior semicircular canal dehiscence syndrome was first reported by Lloyd Minor and colleagues in 1998. Patients with a dehiscence in the bone overlying the superior semicircular canal experience symptoms of pressure or sound-induced vertigo, bone conduction hyperacusis, and pulsatile tinnitus. The initial series of patients were diagnosed based on common symptoms, a physical examination finding of eye movements in the plane of the superior semicircular canal when ear canal pressure or loud tones were applied to the ear, and high-resolution computed tomography imaging demonstrating a dehiscence in the bone over the superior semicircular canal. Research productivity directed at understanding better methods for diagnosing and treating this condition has substantially increased over the last two decades. We now have a sound understanding of the pathophysiology of third mobile window syndromes, higher resolution imaging protocols, and several sensitive and specific diagnostic tests. Furthermore, we have a treatment (surgical occlusion of the superior semicircular canal) that has demonstrated efficacy. This review will highlight some of the fundamental insights gained in SCDS, propose diagnostic criteria, and discuss future research directions.

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“…A study from Naganawa et al showed that EH can be visualized using 3-T MRI performed 4 hours after intravenous injection of gadolinium [38]. Recently, Sone et al investigated the presence of EH in subjects with EVA syndrome using 3T MRI and correlated imaging data concerning the degree of EH in the cochlea and the vestibule with clinical symptoms and hearing levels in 9 patients [39]. In this case, patient was studied using a 1.5T MRI that, due to its resolution, was unable to confirm EH; therefore, diagnosis was based on clinical and anamnestic data.…”

Section: Discussionmentioning

confidence: 99%

“…As expected, the larger benefits in hearing restoration following corticosteroid treatment were seen in the ear contralateral to EVA. One of the first reports regarding the effects of intratympanic treatment of steroids for EH showed an 80% improvement in vertigo [39]. Afterwards, several studies on intratympanic treatment for EH have been published [23, 25, 40] showing different results on hearing and vertigo: the choice of steroids, the variability of their concentration, and the outcome measurements could explain the variability of the published results.…”

Section: Discussionmentioning

confidence: 99%

Unilateral Enlarged Vestibular Aqueduct Syndrome and Bilateral Endolymphatic Hydrops

Ralli

Nola²,

Sparvoli³

et al. 2017

Case Reports in Otolaryngology

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Enlarged vestibular aqueduct (EVA) syndrome is a common congenital inner ear malformation characterized by a vestibular aqueduct with a diameter larger than 1.5 mm, mixed or sensorineural hearing loss that ranges from mild to profound, and vestibular disorders that may be present with a range from mild imbalance to episodic objective vertigo. In our study, we present the case of a patient with unilateral enlarged vestibular aqueduct and bilateral endolymphatic hydrops (EH). EH was confirmed through anamnestic history and audiological exams; EVA was diagnosed using high-resolution CT scans and MRI images. Therapy included intratympanic infusion of corticosteroids with a significant hearing improvement, more evident in the ear contralateral to EVA. Although most probably unrelated, EVA and EH may present with similar symptoms and therefore the diagnostic workup should always include the proper steps to perform a correct diagnosis. Association between progression of hearing loss and head trauma in patients with a diagnosis of EVA syndrome is still uncertain; however, these individuals should be advised to avoid activities that increase intracranial pressure to prevent further hearing deterioration. Intratympanic treatment with steroids is a safe and well-tolerated procedure that has demonstrated its efficacy in hearing, tinnitus, and vertigo control in EH.

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Endolymphatic hydrops in superior canal dehiscence and large vestibular aqueduct syndromes

Abstract: 4 Laryngoscope, 126:1446-1450, 2016.

Cited by 59 publications

References 21 publications

Secondary Endolymphatic Hydrops

Secondary Endolymphatic Hydrops

Superior Canal Dehiscence Syndrome: Lessons from the First 20 Years

Unilateral Enlarged Vestibular Aqueduct Syndrome and Bilateral Endolymphatic Hydrops

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