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A mouse model for benign paroxysmal positional vertigo with genetic predisposition for displaced otoconia.

ABSTRACT: Abnormal formation of otoconia, the biominerals of the inner ear, results in balance disorders. The inertial mass of otoconia activates the underlying mechanosensory hair cells in response to change in head position primarily during linear and rotational acceleration. Otoconia associate exclusively with the two gravity receptors, the utricle and saccule. The cristae sensory epithelium is associated with an extracellular gelatinous matrix known as cupula, equivalent to otoconia. During head rotation, the inertia of endolymphatic fluids within the semicircular canals deflects the cupula of the corresponding crista and activates the underlying mechanosensory hair cells. It is believed that detached free-floating otoconia particles travel ectopically to the semicircular canal and cristae and are the culprit for benign paroxysmal positional vertigo (BPPV). The Slc26a4 mouse mutant harbors a missense mutation in pendrin. This mutation leads to impaired transport activity of pendrin and to defects in otoconia composition and distribution. All Slc26a4 loop/loop homozygous mutant mice are profoundly deaf but show inconsistent vestibular deficiency. A panel of behavioral tests was utilized in order to generate a scoring method for vestibular function. A pathological finding of displaced otoconia was identified consistently in the inner ears of mutant mice with severe vestibular dysfunction. In this work, we present a mouse model with a genetic predisposition for ectopic otoconia with a clinical correlation to BPPV. This unique mouse model can serve as a platform for further investigation of BPPV pathophysiology, and for developing novel treatment approaches in a live animal model.

SUBMITTER: Dror AA

PROVIDER: S-EPMC7286769 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature

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A mouse model for benign paroxysmal positional vertigo with genetic predisposition for displaced otoconia.

Dror Amiel A AA Taiber Shahar S Sela Eyal E Handzel Ophir O Avraham Karen B KB

Genes, brain, and behavior 20200116 5

Abnormal formation of otoconia, the biominerals of the inner ear, results in balance disorders. The inertial mass of otoconia activates the underlying mechanosensory hair cells in response to change in head position primarily during linear and rotational acceleration. Otoconia associate exclusively with the two gravity receptors, the utricle and saccule. The cristae sensory epithelium is associated with an extracellular gelatinous matrix known as cupula, equivalent to otoconia. During head rotat ...[more]

PMID: 31898392

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Project description:BackgroundBenign paroxysmal positional vertigo is the most common disease in which vertigo is the main clinical manifestation, and it has become a global medical problem, affecting a wide range of areas and seriously affecting the quality of human life.ObjectiveThis article presents an analysis of the current characteristics of BPPV-related research and summarizes the current hot topics and trends, with the goal of inspiring future research into the prevention and treatment of BPPV, thereby improving the differential diagnosis and prevention of peripheral vertigo.MethodsA bibliometric approach was used to collect 1,219 eligible studies on BPPV from four databases-PubMed, Embase, Scopus, and Web of Science-published between 1974 and 2022. The characteristics and status of the accumulated scientific output were processed using R and VOSviewer so that we could visualize any trends or hotspots.ResultsThe results showed a significant increase in the annual number of publications, with an average annual growth rate of 21.58%. A possible reason for the especially pronounced peak in 2021 was an increase in the prevalence of BPPV as a result of COVID-19. The new coronavirus became a focus of research in 2021. A total of 3,876 authors (of whom 1,097 were first authors) published articles in 307 different journals; 15.7% of the articles were published in Acta Oto-Larygologica, Otology and Neurotology, and Frontiers in Neurology. Acta Oto-Laryngologica was well ahead of the other journals in terms of growth rate and number of articles published. American scholars generated the largest number of articles overall, and the USA was involved in the greatest number of international collaborations, followed by Italy and China. The themes of the research centered around three topics, namely the treatment of BPPV, its influencing factors, and diagnosis.ConclusionsThere has been a major increase in BPPV-related research over the last 50 years, leading to an increase in related articles and rapid development of the field. Key directions for future research include the improvement of individualized treatment for residual symptoms after initial treatment of BPPV among the elderly; effective control of comorbidities such as osteoporosis; and secondary inner ear disease, such as Ménière's disease.

Dataset Information

A mouse model for benign paroxysmal positional vertigo with genetic predisposition for displaced otoconia.

Publications

A mouse model for benign paroxysmal positional vertigo with genetic predisposition for displaced otoconia.

Similar Datasets

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