Edward S. A. van Beelen

Cytomegalovirus host receptor expression in the human fetal inner ear

Lucia C. M. Grijpink, Wouter H. van der Valk, Edward S. A. van Beelen, John C.M.J. de Groot et autres

Fetal infection with human cytomegalovirus (hCMV) can cause sensorineural hearing loss and vestibular impairment, yet its pathogenesis remains unclear. This study aims to identify potential target cell types of hCMV in the human fetal inner ear. Viral particles use several envelope glycoproteins …

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A single-cell level comparison of human inner ear organoids with the human cochlea and vestibular organs

Wouter H. van der Valk, Edward S. A. van Beelen, Matthew R. Steinhart, Carl A. Nist-Lund et autres

(Cell Reports 42, 112623; June 27, 2023) The authors acknowledge the presence of the following errors identified in the STAR Methods section of the article. The errors have now been corrected online. •Original: Mouse monoclonal anti-WRHN (1:50)•Correction: Mouse monoclonal anti-WHRN (1:50)•Original: Mouse …

A single-cell level comparison of human inner ear organoids with the human cochlea and vestibular organs

Wouter H. van der Valk, Edward S. A. van Beelen, Matthew R. Steinhart, Carl A. Nist-Lund et autres

Inner ear disorders are among the most common congenital abnormalities; however, current tissue culture models lack the cell type diversity to study these disorders and normal otic development. Here, we demonstrate the robustness of human pluripotent stem cell-derived inner ear organoids (IEOs) …

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A Single-Cell Level Comparison of Human Inner Ear Organoids and the Human Cochlea and Vestibular Organs

Wouter H. van der Valk, Edward S. A. van Beelen, Matthew R. Steinhart, Carl A. Nist-Lund et autres

Summary Genetic inner ear disorders are among the most common congenital abnormalities and lead to hearing loss and balance disorders. Ideally, tissue culture models of the inner ear should contain a functional unit combining otic sensory and nonsensory cell types to recapitulate …

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Efficient Viral Transduction in Fetal and Adult Human Inner Ear Explants with AAV9-PHP.B Vectors

Edward S. A. van Beelen, Wouter H. van der Valk, Thijs O. Verhagen, John C.M.J. de Groot et autres

Numerous studies have shown the recovery of auditory function in mouse models of genetic hearing loss following AAV gene therapy, yet translation to the clinic has not yet been demonstrated. One limitation has been the lack of human inner ear cell lines …

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Hearing loss, cleft palate, and congenital hip dysplasia in female carriers of an intragenic deletion of AMMECR1

Saskia Koene, Jeroen Knijnenburg, Mariëtte J.V. Hoffer, Fleur Zwanenburg et autres

Previously, mutations in the AMMECR1 gene have been described in six males with developmental delay, sensorineural hearing loss (SNHL) and/or congenital abnormalities, including fetal nuchal edema, fetal pericardial effusion, talipes, congenital hip dysplasia, elliptocytosis and cleft palate. In this report, we present …

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Variants in USP48 encoding ubiquitin hydrolase are associated with autosomal dominant non-syndromic hereditary hearing loss

Sissy Bassani, Edward S. A. van Beelen, Mireille Rossel, Norine Voisin et autres

Non-Syndromic Hereditary Hearing Loss (NSHHL) is a genetically heterogeneous sensory disorder with about 120 genes already associated. Through exome sequencing (ES) and data aggregation, we identified a family with six affected individuals and one unrelated NSHHL patient with predicted-to-be deleterious missense variants …

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Migration and fate of vestibular melanocytes during the development of the human inner ear

Edward S. A. van Beelen, Wouter H. van der Valk, John C.M.J. de Groot, Erik Frans Hensen et autres

Abstract Melanocytes are present in various parts of the inner ear, including the stria vascularis in the cochlea and the dark cell areas in the vestibular organs, where they contribute to endolymph homeostasis. Developmental studies describing the distribution of vestibular melanocytes are …

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Low-dose oncolytic adenovirus therapy overcomes tumor-induced immune suppression and sensitizes intracranial gliomas to anti-PD-1 therapy

Zineb Belcaid, Cor Berrevoets, John Hyunkuk Choi, Edward S. A. van Beelen et autres

Abstract Background The tumor-selective human adenovirus Delta24-RGD is currently under investigation in phase II clinical trials for patients with recurrent glioblastoma (GBM). To improve treatments for patients with GBM, we explored the potential of combining Delta24-RGD with antibodies targeting immune checkpoints. Methods …

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