The basic science of Meniere??s disease and endolymphatic hydrops

Semaan, Maroun T.; Alagramam, Kumar N.; Megerian, Cliff A.

doi:10.1097/01.moo.0000186335.44206.1c

Cited by 59 publications

(56 citation statements)

References 48 publications

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“…Histologically, Meniere's disease is characterized by endolymphatic hydrops and swelling of the endolymphatic space of the inner ear, 15 but the etiology and pathophysiology of endolymphatic hydrops are unknown. 16 Whether the swelling affects or injures the vestibular end organs is also unknown, but many patients with Meniere's disease have vestibular deficits as assessed by electronystagmography. Despite the possibility of altered or injured vestibular end organs in the Meniere's patients, we found that the vestibular epithelia were viable, could be maintained in culture and could be transfected with adenovirus.…”

Section: Cell Viability and Availabilitymentioning

confidence: 99%

An in vitro model system to study gene therapy in the human inner ear

et al. 2007

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The confined fluid-filled labyrinth of the human inner ear presents an opportunity for introduction of gene therapy reagents designed to treat hearing and balance dysfunction. Here we present a novel model system derived from the sensory epithelia of human vestibular organs and show that the tissue can survive up to 5 days in vitro. We generated organotypic cultures from 26 human sensory epithelia excised at the time of labyrinthectomy for intractable Meniere's disease or vestibular schwannoma. We applied multiply deleted adenoviral vectors at titers between 10 5 and 10 8 viral particles/ml directly to the cultures for 4-24 h and examined the tissue 12-96 h post-transfection. We noted robust expression of the exogenous transgene, green fluorescent protein (GFP), in hair cells and supporting cells suggesting both were targets of adenoviral transfection. We also transfected cultures with a vector that carried the genes for GFP and KCNQ4, a potassium channel subunit that causes dominant-progressive hearing loss when mutated. We noted a positive correlation between GFP fluorescence and KCNQ4 immunolocalization. We conclude that our in vitro model system presents a novel and effective experimental paradigm for evaluation of gene therapy reagents designed to restore cellular function in patients who suffer from inner ear disorders.

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Section: Cell Viability and Availabilitymentioning

confidence: 99%

An in vitro model system to study gene therapy in the human inner ear

et al. 2007

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“…Although the most common pathological correlate of Meniere's disease is endolymphatic hydrops, the cause remains unclear and the hydrops may be an epiphenomenon (Merchant et al, 2005). Recent theories, including genetics, autoimmune disorders, perturbation in fluid dynamics and oxidative stress have been implicated in the development of endolymphatic hydrops (Semaan et al, 2005;Calabrese et al, 2010).…”

Section: Discussionmentioning

confidence: 99%

Immunocytochemical Localization of the Translocase of the Outer Mitochondrial Membrane (Tom20) in the Human Cochlea

Balaker

Ishiyama

López

et al. 2012

The Anatomical Record

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Mitochondrial degeneration in the inner ear is likely a contributing factor in age-related hearing loss and other otopathologies such as Meniere's disease. Most mitochondrial proteins are synthesized in the cytosol and imported through the mitochondrial membranes by translocators. The translocase of the outer membrane (Tom) is the universal entry gate for all proteins that are imported into mitochondria. Altered function of the translocator could alter protein transport into the mitochondria, and disrupt function. In this study, we determined the immunolocalization of Tom20, a major mitochondrial protein import receptor, in microdissected human cochlea frozen sections obtained from postmortem autopsy and celloidin-embedded archival specimens. We used affinity purified rabbit polyclonal antibodies against Tom20. We also determined the Tom20 immunolocalization in the mouse inner ear. In the human and mouse cochlea, Tom20 was ubiquitously distributed in the organ of Corti, allowing well-delineated visualization of inner and outer hair cells. Tom20 immunoreactivity localized in the cytoplasm of spiral ganglia neurons. In the inner ear of aged subjects with Meniere's disease, there was decreased expression of Tom20. These results suggest that Tom20 can be used in the inner ear as a marker for mitochondrial protein import. Anat Rec,

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“…Болезнь Меньера может протекать с преобладанием вестибулярных или слуховых расстройств, в зависимости от чего возникают различные варианты ее течения [3,12,15,16]. Наиболее сложен для диагностики вариант, когда внача-ле болезнь проявляется только приступами вестибулярного головокружения, к которым позднее (обычно в течение го-да) присоединяются нарушения слуха.…”

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“…Болезнь Меньера харак-теризуется хроническим прогрессирующим течением. Одна-ко у 50-70% пациентов в течение первых 2-8 лет заболева-ния могут возникать спонтанные ремиссии [3,12,15,16].…”

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Diagnosis, treatment, and rehabilitation in patients with dizziness and cognitive impairment

Антоненко

2017

RJTAO

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The basic science of Meniere??s disease and endolymphatic hydrops

Abstract: These recent advances have expanded our understanding and will allow for the development of targeted therapeutic interventions aimed at preventing the progression oochleovestibular deterioration.

Cited by 59 publications

References 48 publications

An in vitro model system to study gene therapy in the human inner ear

An in vitro model system to study gene therapy in the human inner ear

Immunocytochemical Localization of the Translocase of the Outer Mitochondrial Membrane (Tom20) in the Human Cochlea

Diagnosis, treatment, and rehabilitation in patients with dizziness and cognitive impairment

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