Research Topics

at Yonsei Hearing Research Laboratory

Hereditary and Acquired Hearing Loss

Hearing plays a fundamental role in human life, enabling communication, supporting emotional well-being, and facilitating meaningful social connections. When hearing is compromised, the consequences extend far beyond simple auditory deficits. Hearing loss significantly diminishes quality of life and is associated with increased risks of social isolation, falls, depression, anxiety, and cognitive decline, including dementia. While genetic factors and ototoxic agents—including certain drugs, noise exposure, and aging—are known causes of hearing loss, the underlying pathological mechanisms remain poorly understood. We investigate these mechanisms to develop therapeutic strategies that protect existing hearing and restore hearing loss.

Jang et al. (2025) Antisense oligonucleotide therapy mitigates autosomal dominant progressive hearing loss in a murine model of human DFNA2. Mol Ther In Press
Joo et al. (2025) Bi-allelic variants of SEMA3F are associated with non-syndromic hearing loss. Mol Cells 48(3): 100190
Jung et al. (2024) MYH1 deficiency disrupts the outer hair cell electromotility, resulting in hearing loss. Exp Mol Med 56(11): 2423-2435.
Ma et al. (2022) Therapeutic effect of NLRP3 inhibition on hearing loss induced by systemic inflammation in a CAPS-associated mouse model. EBioMedicine 82:104148.
Moon et al. (2020) Dysregulation of sonic hedgehog signaling causes hearing loss in ciliopathy mouse models. eLife 9:e5655
Kim et al. (2019) Gene therapy for hereditary hearing loss by SLC26A4 mutations in mice reveals distinct functional roles of pendrin in normal hearing function. Theranostics 9(24):7184-7199.
Lee et al. (2018). Exocyst Complex Member EXOC5 Is Required for Survival of Hair Cells and Spiral Ganglion Neurons and Maintenance of Hearing. Mol Neurobiol 55(8): 6518-6532.

Tonotopic Organization

The auditory system's remarkable ability to discriminate sound frequencies originates in the cochlea, where hair cells are spatially organized according to their frequency sensitivity. Hair cells in the cochlear base respond to high frequencies, while those in the apex detect low frequencies. This precise spatial-frequency mapping, termed tonotopic organization, forms the foundation of auditory processing. We investigate the developmental mechanisms that establish this critical organizational pattern during cochlear formation.

Koo et al. (2023) Follistatin regulates the specification of the apical cochlea responsible for low-frequency hearing in mammals. Proc Natl Acad Sci U S A. 120(1):e2213099120.
Koo et al. (2021) Position Specific Alternative Splicing and Gene Expression Profiles Along the Tonotopic Axis of Chick Cochlea. Front Mol Biosci 8:726976
Son et al. (2015) Conserved role of Sonic Hedgehog in tonotopic organization of the avian basilar papilla and mammalian cochlea. Proc Natl Acad Sci U S A 112(12): 3746-51.
Son et al. (2012) Developmental gene expression profiling along the tonotopic axis of the mouse cochlea. PLoS ONE 7(7): e40735.

Stereociliary Bundle Architecture

Effective sound transduction relies on the precise architecture of stereociliary bundles of auditory hair cells. These bundles possess specific geometry with stereocilia arranged in three-row staircase patterns, forming linear shapes in inner hair cells and V-shapes in outer hair cells. Individual stereocilia are connected by specialized extracellular links, enabling coherent responses to sound waves for mechanotransduction. We are investigating the developmental mechanisms establishing this bundle architecture and their functional significance to understand how defects lead to hearing impairment and identify therapeutic targets.

Choi et al. (2025) SHANK2 establishes auditory hair bundle architecture essential for mammalian hearing. Proc Natl Acad Sci U S A. 122 (28) e2426646122
Youn et al. (2022). Microtubule-associated protein 1 A and tubby act independently in regulating the localization of stereocilin to the tips of inner ear hair cell stereocilia. Mol Brain 15(1): 80
Han et al. (2020) Distinct roles of stereociliary links in the nonlinear sound processing and noise resistance of cochlear outer hair cells. Proc Natl Acad Sci U S A 117(20):11109-11117.
Moon et al. (2020) Dysregulation of sonic hedgehog signaling causes hearing loss in ciliopathy mouse models. eLife 9:e5655

Molecular Landscape of Cochlear Development

The cochlea develops from a simple otocyst into a complex spiral structure containing specialized cells that process and transmit auditory information. Next-generation sequencing technologies have revolutionized our ability to decode the molecular mechanisms underlying this remarkable transformation. We investigate the regulatory networks controlling cochlear development to understand how this intricate sensory organ is formed.

Kim et al. (2023) Alternative splicing in shaping the molecular landscape of the cochlea. Front Cell Dev Biol 11: 1143428.
Koo et al. (2021) Position Specific Alternative Splicing and Gene Expression Profiles Along the Tonotopic Axis of Chick Cochlea. Front Mol Biosci 8:726976
Hwang et al. (2020) rMAPS2: an update of the RNA map analysis and plotting server for alternative splicing regulation. Nucleic Acids Res 48(W1):W300-W306.

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