High Resolution Three-Dimensional Delayed Contrast MRI Detects Endolymphatic Hydrops in Patients With Vertigo and Vestibular Schwannoma

Moayer, Roxana; Ishiyama, Gail; Karnezis, Stellios; Sepahdari, Ali R.; Ishiyama, Akira

doi:10.1097/mao.0000000000001627

Cited by 7 publications

(10 citation statements)

References 15 publications

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“…However, previous HTB histopathological studies demonstrate a high incidence of EH in the saccule, consistent with theories that MD and DEH begin in the saccule (5). Additionally, the current methodology of MRI acquisition in our practice has demonstrated isolated involvement of other endolymphatic compartments (19,20). One of the strengths of the present study are the detailed clinical histories before and after the MRI and audiovestibular testing results available for nearly all patients.…”

Section: Discussionsupporting

confidence: 84%

“…In a recent case report, a patient with a small inferior vestibular nerve schwannoma who presented with recurrent vertigo spells and fluctuating hearing loss, clinically identical to MD, had MRI evidence of isolated saccular hydrops. Notably, the recurrent vertigo spells and aural fullness were responsive to acetazolamide (20). In our experience, idiopathic saccular EH has been uniformly associated with MD or DEH.…”

Section: Discussionsupporting

confidence: 50%

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Isolated Saccular Hydrops on High-resolution MRI Is Associated With Full Spectrum Menière's Disease

Maxwell

Ishiyama²,

Karnezis

et al. 2021

Otology &Amp; Neurotology

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Objective:To describe the clinical presentation of patients with isolated saccular endolymphathic hydrops (EH) detected.Study Design:Clinical case series.Setting:University-based tertiary referral center.Patients:All subjects presenting with vertigo or hearing loss who had isolated saccular EH detected.Intervention:High-resolution delayed-contrast magnetic resonance imaging (MRI) conducted between November 2015 and November 2016.Main Outcome Measures:Audiovestibular testing results and analysis of clinical histories.Results:Isolated saccular EH was detected in 18 subjects. Sixteen met criteria for definite Menière's disease (MD, n = 12) or delayed endolymphatic hydrops (DEH, n = 4). One had a history of sudden sensorineural hearing loss (SSNHL) and 3 years after MRI developed recurrent vertigo characteristic of DEH. One patient had a history of atypical DEH (Tumarkin falls without vertigo following SSNHL). Four patients had Tumarkin falls. Most (83%) demonstrated mild-to-severe low-frequency fluctuating loss, and six (33.3%) had a history of ipsilateral sudden profound SNHL. Nine of the 17 (53%) patients tested had an ipsilateral caloric paresis ranging from 26 to 67%. Ipsilateral vestibular-evoked myogenic potentials showed reduced or absent responses in 5 of the 17 tested (29%).Conclusions:The full spectrum of MD may be associated with saccular hydrops. We propose that MD and DEH often begin in the saccule, and MRI may provide clues to the pathophysiology of MD. Saccular hydrops was present in one patient with SSNHL who did not develop vertigo spells until 3 years after MRI, indicating that saccular hydrops may be the first manifestation of MD or DEH.

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Section: Discussionsupporting

confidence: 84%

Section: Discussionsupporting

confidence: 50%

Isolated Saccular Hydrops on High-resolution MRI Is Associated With Full Spectrum Menière's Disease

Maxwell

Ishiyama²,

Karnezis

et al. 2021

Otology &Amp; Neurotology

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“…The current study concurs with previous reports that EH may occur in the setting of VS (6,7). Evaluation of patient symptoms related to EH is limited in this study because clinical charts did not all contain complete information about vestibular symptoms, fluctuating hearing and tinnitus.…”

Section: Discussionsupporting

confidence: 93%

Endolymphatic Hydrops in the Setting of Vestibular Schwannoma: A Temporal Bone Study

Wong¹,

López²,

Wichova³

et al. 2023

Otol Neurotol

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HypothesisVestibular schwannoma (VS) may be associated with endolymphatic hydrops (EH). EH may account for symptomatology in a subset of patients with VS.BackgroundPresenting symptoms of VS and EH overlap, and MRI evaluation of the membranous labyrinth in some patients with VS demonstrates EH. The aim of the current study is to evaluate whether EH is present in temporal bones of patients with VS.MethodsThe NIDCD and House Temporal Bone Laboratory at UCLA Eccles database was queried for the diagnosis of “acoustic neuroma.” Exclusion criteria included concomitant ear disease and surgery. Temporal bones were analyzed for EH of the basal, middle, and apical turns and vestibule. Premortem audiometric and clinical data were gathered.ResultsOf 43 human temporal bones with VS, 6 met inclusion criteria. All temporal bones demonstrated VS that was undisturbed by surgery. Three of six demonstrated EH of at least one cochlear turn as well as vestibular hydrops. Three patients had severe to profound hearing loss. One patient carried a diagnosis of Menière’s disease.ConclusionsEH is demonstrated in the setting of VS in human temporal bones. EH may be one mechanism of hearing loss and dizziness in patients with VS.Professional Practice Gap and Educational NeedThe underlying mechanisms of symptoms of VS may be multifactorial. The association of EH in some patients with VS would modify our clinical approach to management.Learning ObjectiveTo discover if EH may be associated with VS.Desired ResultTo broaden understanding of pathophysiologic mechanisms in patients with VS.Level of EvidenceLevel IVIRB Approved: UCLA IRB No. 10-001449

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“…In the past years, intratympanic gadolinium injection has arisen as a new tool in the diagnosis of EH (40). Recently, delayed intravenous gadolinium-enhanced high-resolution MRI of the inner ear has been shown to provide resolution adequate for accurate detection of EH (41, 42). In addition, heavily weighted T2 sequences are useful to evaluate the cochlear fluids in patients with VS.…”

Section: Biological Markersmentioning

confidence: 99%

Biomarkers in Vestibular Schwannoma–Associated Hearing Loss

et al. 2019

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Vestibular schwannomas (VSs) are benign tumors composed of differentiated neoplastic Schwann cells. They can be classified into two groups: sporadic VS and those associated with neurofibromatosis type 2 (NF2). VSs usually grow slowly, initially causing unilateral sensorineural hearing loss (HL) and tinnitus. These tumors cause HL both due to compression of the auditory nerve or the labyrinthine artery and due to the secretion of different substances potentially toxic to the inner ear or the cochlear nerve. As more and more patients are diagnosed and need to be managed, we are more than ever in need of searching for biomarkers associated with these tumors. Owing to an unknown toxic substance generated by the tumor, HL in VS may be linked to a high protein amount of perilymph. Previous studies have identified perilymph proteins correlated with tumor-associated HL, including μ-Crystallin (CRYM), low density lipoprotein receptor-related protein 2 (LRP2), immunoglobulin (Ig) γ-4 chain C region, Ig κ-chain C region, complement C3, and immunoglobulin heavy constant γ 3. Besides, the presence of specific subtypes of heat shock protein 70 has been suggested to be associated with preservation of residual hearing. It has been recently demonstrated that chemokine receptor-4 (CXCR4) is overexpressed in sporadic VS as well as in NF2 tumors and that hearing disability and CXCR4 expression may be correlated. Further, the genetic profile of VS and its relationship with poor hearing has also been studied, including DNA methylation, deregulated genes, growth factors, and NF2 gene mutations. The knowledge of biomarkers associated with VS would be of significant value to maximize outcomes of hearing preservation in these patients.

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High Resolution Three-Dimensional Delayed Contrast MRI Detects Endolymphatic Hydrops in Patients With Vertigo and Vestibular Schwannoma

Cited by 7 publications

References 15 publications

Isolated Saccular Hydrops on High-resolution MRI Is Associated With Full Spectrum Menière's Disease

Isolated Saccular Hydrops on High-resolution MRI Is Associated With Full Spectrum Menière's Disease

Endolymphatic Hydrops in the Setting of Vestibular Schwannoma: A Temporal Bone Study

Biomarkers in Vestibular Schwannoma–Associated Hearing Loss

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