REGENERATIVE WEAKNESS OF THE INNER EAR AND BIOENGINEERED HEARING RESTORATION: A BRIEF PROBLEM OVERVIEW DOI
Volodymyr V. Medvediev

OTORHINOLARYNGOLOGY, Journal Year: 2023, Volume and Issue: unknown, P. 91 - 100

Published: Jan. 1, 2023

The structure of the hearing organ combines simplicity and depth, remaining in many issues subject active scientific research. ontogenesis this exquisite mechanism is regulated by complex still incompletely understood mechanisms. technological tools modern biomedical science allow not only to study, but also work at cellular molecular level. In article, we review data on realism restoring population human auditory receptors promising ways solve problem. addition, offer an answer a fundamental question – why, with small number relatively simply arranged receptor cells inner ear, their self-restored. Key words: spiral organ, stem cells, progenitors, impairments, regeneration, bioprosthetics, bioengineering.

Language: Английский

Modeling, applications and challenges of inner ear organoid DOI Creative Commons
Jieyu Qi, Liyan Zhang, Xiaohan Wang

et al.

Smart Medicine, Journal Year: 2024, Volume and Issue: 3(1)

Published: Jan. 16, 2024

More than 6% of the world's population is suffering from hearing loss and balance disorders. The inner ear organ that senses sound balance. Although disorders are common, there limited ways to intervene restore its sensory functions. development establishment biologically therapeutic interventions for auditory require clarification basics signaling pathways control endogenous or exogenous cell-based methods. In vitro models ear, such as organoid systems, can help identify new protective regenerative drugs, develop gene therapies, be considered potential tools future clinical applications. Advances in stem cell technology culture offer unique opportunities modeling diseases developing personalized therapies loss. Here, we review discuss mechanisms applications organoids.

Language: Английский

Citations

6

Human pluripotent stem cell-derived inner ear organoids recapitulate otic development in vitro DOI Creative Commons
Daniela Doda,

Sara Alonso Jimenez,

Hubert Rehrauer

et al.

Development, Journal Year: 2023, Volume and Issue: 150(19)

Published: Aug. 10, 2023

ABSTRACT Our molecular understanding of the early stages human inner ear development has been limited by difficulty in accessing fetal samples at gestational stages. As an alternative, previous studies have shown that morphogenesis can be partially recapitulated using induced pluripotent stem cells directed to differentiate into organoids (IEOs). Once validated and benchmarked, these systems could represent unique tools complement refine our otic differentiation model developmental defects. Here, we provide first direct comparisons embryonic otocyst sensory organs with IEOs. We use multiplexed immunostaining single-cell RNA-sequencing characterize IEOs three key steps, providing a new signature vitro-derived placode, epithelium, neuroblasts epithelia. In parallel, evaluate expression localization crucial markers equivalent embryos. Together, data indicate current state-of-the-art protocol enables specification bona fide tissue, supporting further application inform biology disease.

Language: Английский

Citations

15

Inner Ear Organoids: Strengths and Limitations DOI Creative Commons
Giulia Pianigiani, Marta Roccio

Journal of the Association for Research in Otolaryngology, Journal Year: 2024, Volume and Issue: 25(1), P. 5 - 11

Published: Feb. 9, 2024

Abstract Inner ear organoids derived from differentiation of human pluripotent stem cells have recently gained momentum as tools to study inner development and developmental defects. An additional exciting aspect about this technology is represented by its translational potential, specifically, the use validate therapeutics for hearing balance restoration on human/patient-specific cells. This latter will be briefly discussed here including opportunities current limitations.

Language: Английский

Citations

4

The Reconstruction of Peripheral Auditory Circuit: Recent Advances and Future Challenges DOI Creative Commons
Zhe Li, Ying Zhang, Zhenyu Xiao

et al.

Advanced Science, Journal Year: 2025, Volume and Issue: unknown

Published: March 24, 2025

Abstract The auditory circuit primarily consists of peripheral organs and neural pathways. Hearing loss (HL), as reported by the World Health Organization, affects more than one in eight people worldwide, often leading to severe dysfunction that detrimentally impacts patients' quality life. Therefore, reconstruction has emerged a persistent research hotspot challenge within biomedical field. Traditional methods for reconstruction, such drug therapy, hearing aids, cochlear implants (CIs), so on. To certain extent, it can help patients with HL improve their status. However, they possess inherent advantages limitations. Recent advancements biomaterials, gene editing, stem cells, organoids, other technologies have ushered new prospects treatment impairment. This review focuses on potential innovative editing recovery. It reviews current status inner ear hair cell (HC) regeneration, spiral ganglion neuron (SGN) organoid construction loop. Furthermore, discusses challenges associated these approaches explores future directions, aiming furnish comprehensive reference both clinical applications domain restoration.

Language: Английский

Citations

0

Mapping oto-pharyngeal development in a human inner ear organoid model DOI Creative Commons
Matthew R. Steinhart, Wouter H. van der Valk, Daniel Osorio

et al.

Development, Journal Year: 2023, Volume and Issue: 150(19)

Published: Oct. 1, 2023

ABSTRACT Inner ear development requires the coordination of cell types from distinct epithelial, mesenchymal and neuronal lineages. Although we have learned much animal models, many details about human inner remain elusive. We recently developed an in vitro model organogenesis using pluripotent stem cells a 3D culture, fostering growth sensorineural circuit, including hair neurons. Despite previously characterizing some types, undefined. This study aimed to chart timeline organoid understand mechanisms at play. Using single-cell RNA sequencing ten stages during first 36 days differentiation, tracked evolution pluripotency various after exposure specific signaling modulators. Our findings showcase gene expression that influences identifying plethora ectodermal types. also discern aspects consistent with vivo development, while highlighting potential discrepancies. establishes Ear Organoid Developmental Atlas (IODA), offering deeper insights into biology improving tissue differentiation.

Language: Английский

Citations

8

Construction of organ of Corti organoid to study the effects of berberine sulfate on damaged auditory cells DOI
Junming Zhang, Li Liu, Rong Shen

et al.

Journal of Biomedical Materials Research Part B Applied Biomaterials, Journal Year: 2024, Volume and Issue: 112(7)

Published: June 24, 2024

Sensorineural hearing loss (SNHL) is mainly caused by injury or of hair cells (HCs) and associated spiral ganglion neurons (SGNs) in the inner ear. At present, there still no effective treatment for SNHL clinic. Recently, advances organoid bring a promising prospect research SNHL. Meanwhile, three-dimensional (3D) printing provides tremendous opportunity to construct versatile organoids tissue engineering regenerative medicine. In this study, gelatin (Gel), sodium alginate (SA), polyvinyl alcohol (PVA) were used fabricate biomimetic scaffold through 3D printing. The organ Corti derived from neonatal mice ear was seeded on PVA/Gel/SA organoid. Then, study potential protective effects berberine sulfate neomycin-juried auditory HCs SGNs. results showed that scaffolds had good cytocompatibilities mechanical properties. constructed could maintain activity well vitro. addition, intervention significantly inhibit neomycin-induced HC SGN damage. This suggests fabricated highly Corti, which may provide an model drug development, cell gene therapy

Language: Английский

Citations

2

The Role of Pericytes in Inner Ear Disorders: A Comprehensive Review DOI Creative Commons
Antonino Maniaci, Marilena Briglia,

Fabio Allia

et al.

Biology, Journal Year: 2024, Volume and Issue: 13(10), P. 802 - 802

Published: Oct. 8, 2024

Inner ear disorders, including sensorineural hearing loss, Meniere’s disease, and vestibular neuritis, are prevalent conditions that significantly impact the quality of life. Despite their high incidence, underlying pathophysiology these disorders remains elusive, current treatment options often inadequate. Emerging evidence suggests pericytes, a type vascular mural cell specialized to maintain integrity function microvasculature, may play crucial role in development progression inner disorders. The pericytes present microvasculature both cochlea system, where they regulate blood flow, blood–labyrinth barrier, facilitate angiogenesis, provide trophic support neurons. Understanding valuable insights into lead novel diagnostic therapeutic strategies, improving standard living. This comprehensive review aims detailed overview highlighting anatomy physiology analyzing mechanisms contribute Furthermore, we explore potential pericyte-targeted therapies, antioxidant, anti-inflammatory, angiogenic approaches, as well gene therapy strategies.

Language: Английский

Citations

2

Hair cell toxicology: With the help of a little fish DOI Creative Commons
Alejandro Barrallo‐Gimeno, Jordi Llorens

Frontiers in Cell and Developmental Biology, Journal Year: 2022, Volume and Issue: 10

Published: Dec. 13, 2022

Hearing or balance loss are disabling conditions that have a serious impact in those suffering them, especially when they appear children. Their ultimate cause is frequently the of function mechanosensory hair cells inner ear. Hair can be damaged by environmental insults, like noise chemical agents, known as ototoxins. Two most common ototoxins life-saving medications: cisplatin against solid tumors, and aminoglycoside antibiotics to treat infections. However, due their localization inside temporal bone, difficult study mammals. As an alternative animal model, zebrafish larvae similar mammals, some which located fish specific organ on surface skin, lateral line. This makes them easy observe

Language: Английский

Citations

10

Cellular diversity of human inner ear organoids revealed by single-cell transcriptomics DOI
Mireia Rumbo, Berta Alsina

Development, Journal Year: 2024, Volume and Issue: 151(23)

Published: Nov. 29, 2024

ABSTRACT Human inner ear organoids are three-dimensional tissular structures grown in vitro that recapitulate some aspects of the fetal and allow differentiation cell types. These offer a system which to study human development, mutations causing hearing loss vertigo, new therapeutic drugs. However, extent such mimic vivo development cellular composition remains unclear. Several recent studies have performed single-cell transcriptomics on interrogate heterogeneity, reveal developmental trajectories sensory lineages compare organoid-derived vesicles developing ear. Here, we discuss insights provided by these analyses help define paths investigation understand development.

Language: Английский

Citations

1

Shared features in ear and kidney development – implications for oto-renal syndromes DOI Creative Commons

Scarlet Xiaoyan Wang,

Andrea Streit

Disease Models & Mechanisms, Journal Year: 2024, Volume and Issue: 17(2)

Published: Feb. 1, 2024

ABSTRACT The association between ear and kidney anomalies has long been recognized. However, little is known about the underlying mechanisms. In last two decades, embryonic development of inner studied extensively. Here, we describe developmental pathways shared both organs with particular emphasis on genes that regulate signalling cross talk specification progenitor cells specialised cell types. We relate this to clinical features oto-renal syndromes explore links

Language: Английский

Citations

0