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Publications 2019-2021

Edward C. and Amy H. Sewall Professor in the School of Medicine, Professor of Otolaryngology - Head & Neck Surgery (OHNS) and, by courtesy, of Pediatrics

Hair Cell Development

Jan, Taha A., Yasmin Eltawil, Angela H. Ling, Leon Chen, Daniel C. Ellwanger, Stefan Heller, and Alan G. Cheng. “Spatiotemporal Dynamics of Inner Ear Sensory and Non-Sensory Cells Revealed by Single-Cell Transcriptomics.” Cell Reports 36, no. 2 (July 13, 2021): 109358. https://doi.org/10.1016/j.celrep.2021.109358.

Jansson, Lina, Michael Ebeid, Jessica W. Shen, Tara E. Mokhtari, Lee A. Quiruz, David M. Ornitz, Sung-Ho Huh, and Alan G. Cheng. “β-Catenin Is Required for Radial Cell Patterning and Identity in the Developing Mouse Cochlea.” Proceedings of the National Academy of Sciences of the United States of America 116, no. 42 (October 15, 2019): 21054–60. https://doi.org/10.1073/pnas.1910223116.

Najarro, Elvis Huarcaya, Jennifer Huang, Adrian Jacobo, Lee A. Quiruz, Nicolas Grillet, and Alan G. Cheng. “Dual Regulation of Planar Polarization by Secreted Wnts and Vangl2 in the Developing Mouse Cochlea.” Development (Cambridge, England) 147, no. 19 (October 5, 2020): dev191981. https://doi.org/10.1242/dev.191981.

Sun, Haiying, Tian Wang, Patrick J. Atkinson, Sara E. Billings, Wuxing Dong, and Alan G. Cheng. “Gpr125 Marks Distinct Cochlear Cell Types and Is Dispensable for Cochlear Development and Hearing.” Frontiers in Cell and Developmental Biology 9 (2021): 690955. https://doi.org/10.3389/fcell.2021.690955.

Human Hair Cell Biology

Aaron, Ksenia A., Davood K. Hosseini, Yona Vaisbuch, Mirko Scheibinger, Nicolas Grillet, Stefan Heller, Tian Wang, and Alan G. Cheng. “Selection Criteria Optimal for Recovery of Inner Ear Tissues from Deceased Organ Donors.” Otology & Neurotology: Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, February 3, 2022. https://doi.org/10.1097/MAO.0000000000003496.

Hair Cell Degeneration & Regeneration

Galjaard, H., R. Willemsen, A. T. Hoogeveen, G. M. Mancini, S. Palmeri, F. W. Verheijen, and A. D’Azzo. “Molecular Heterogeneity in Human Beta-Galactosidase and Neuraminidase Deficiency.” Enzyme 38, no. 1–4 (1987): 132–43. https://doi.org/10.1159/000469199.

Jan, Taha A., Yasmin Eltawil, Angela H. Ling, Leon Chen, Daniel C. Ellwanger, Stefan Heller, and Alan G. Cheng. “Spatiotemporal Dynamics of Inner Ear Sensory and Non-Sensory Cells Revealed by Single-Cell Transcriptomics.” Cell Reports 36, no. 2 (July 13, 2021): 109358. https://doi.org/10.1016/j.celrep.2021.109358.


Assistant Professor of Otolaryngology - Head & Neck Surgery (OHNS)

Mechanotransduction

Miller, Katharine K., Patrick Atkinson, Kyssia Ruth Mendoza, Dáibhid Ó Maoiléidigh, and Nicolas Grillet. “Dimensions of a Living Cochlear Hair Bundle.” Frontiers in Cell and Developmental Biology 9 (2021): 742529. https://doi.org/10.3389/fcell.2021.742529.

Trouillet, Alix, Katharine K. Miller, Shefin Sam George, Pei Wang, Noor-E.-Seher Ali, Anthony Ricci, and Nicolas Grillet. “Loxhd1 Mutations Cause Mechanotransduction Defects in Cochlear Hair Cells.” The Journal of Neuroscience: The Official Journal of the Society for Neuroscience 41, no. 15 (April 14, 2021): 3331–43. https://doi.org/10.1523/JNEUROSCI.0975-20.2021.

Inner Ear Imaging

Aaron, Ksenia A., Davood K. Hosseini, Yona Vaisbuch, Mirko Scheibinger, Nicolas Grillet, Stefan Heller, Tian Wang, and Alan G. Cheng. “Selection Criteria Optimal for Recovery of Inner Ear Tissues from Deceased Organ Donors.” Otology & Neurotology: Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, February 3, 2022. https://doi.org/10.1097/MAO.0000000000003496.

Miller, Katharine K., Patrick Atkinson, Kyssia Ruth Mendoza, Dáibhid Ó Maoiléidigh, and Nicolas Grillet. “Dimensions of a Living Cochlear Hair Bundle.” Frontiers in Cell and Developmental Biology 9 (2021): 742529. https://doi.org/10.3389/fcell.2021.742529.

Genetics of Hearing Loss

Lezirovitz, Karina, Gleiciele A. Vieira-Silva, Ana C. Batissoco, Débora Levy, Joao P. Kitajima, Alix Trouillet, Ellen Ouyang, et al. “A Rare Genomic Duplication in 2p14 Underlies Autosomal Dominant Hearing Loss DFNA58.” Human Molecular Genetics 29, no. 9 (June 3, 2020): 1520–36. https://doi.org/10.1093/hmg/ddaa075.

Trouillet, Alix, Katharine K. Miller, Shefin Sam George, Pei Wang, Noor-E.-Seher Ali, Anthony Ricci, and Nicolas Grillet. “Loxhd1 Mutations Cause Mechanotransduction Defects in Cochlear Hair Cells.” The Journal of Neuroscience: The Official Journal of the Society for Neuroscience 41, no. 15 (April 14, 2021): 3331–43. https://doi.org/10.1523/JNEUROSCI.0975-20.2021.

Inner Ear Development

Najarro, Elvis Huarcaya, Jennifer Huang, Adrian Jacobo, Lee A. Quiruz, Nicolas Grillet, and Alan G. Cheng. “Dual Regulation of Planar Polarization by Secreted Wnts and Vangl2 in the Developing Mouse Cochlea.” Development (Cambridge, England) 147, no. 19 (October 5, 2020): dev191981. https://doi.org/10.1242/dev.191981.


Edward C. and Amy H. Sewall Professor in the School of Medicine and Professor of Otolaryngology - Head & Neck Surgery (OHNS)

Hair Cell Degeneration & Regeneration

Benkafadar, Nesrine, Amanda Janesick, Mirko Scheibinger, Angela H. Ling, Taha A. Jan, and Stefan Heller. “Transcriptomic Characterization of Dying Hair Cells in the Avian Cochlea.” Cell Reports 34, no. 12 (March 23, 2021): 108902. https://doi.org/10.1016/j.celrep.2021.108902.

Janesick, Amanda S., and Stefan Heller. “Stem Cells and the Bird Cochlea-Where Is Everybody?” Cold Spring Harbor Perspectives in Medicine 9, no. 4 (April 1, 2019): a033183. https://doi.org/10.1101/cshperspect.a033183.

Roccio, Marta, Pascal Senn, and Stefan Heller. “Novel Insights into Inner Ear Development and Regeneration for Targeted Hearing Loss Therapies.” Hearing Research 397 (November 2020): 107859. https://doi.org/10.1016/j.heares.2019.107859.

Senn, Pascal, Amir Mina, Stefan Volkenstein, Veronika Kranebitter, Kazuo Oshima, and Stefan Heller. “Progenitor Cells from the Adult Human Inner Ear.” Anatomical Record (Hoboken, N.J.: 2007) 303, no. 3 (March 2020): 461–70. https://doi.org/10.1002/ar.24228.

Hair Cell Development

Ellwanger, Daniel C., Mirko Scheibinger, Rachel A. Dumont, Peter G. Barr-Gillespie, and Stefan Heller. “Transcriptional Dynamics of Hair-Bundle Morphogenesis Revealed with CellTrails.” Cell Reports 23, no. 10 (June 5, 2018): 2901-2914.e13. https://doi.org/10.1016/j.celrep.2018.05.002.

Janesick, Amanda, Mirko Scheibinger, Nesrine Benkafadar, Sakin Kirti, Daniel C. Ellwanger, and Stefan Heller. “Cell-Type Identity of the Avian Cochlea.” Cell Reports 34, no. 12 (March 23, 2021): 108900. https://doi.org/10.1016/j.celrep.2021.108900.

Kubota, Marie, Mirko Scheibinger, Taha A. Jan, and Stefan Heller. “Greater Epithelial Ridge Cells Are the Principal Organoid-Forming Progenitors of the Mouse Cochlea.” Cell Reports 34, no. 3 (January 19, 2021): 108646. https://doi.org/10.1016/j.celrep.2020.108646.


Edward C. and Amy H. Sewall Professor

Hair Cell Structure, Mechanics, & Function

Holt, Jeffrey R., Mélanie Tobin, Johannes Elferich, Eric Gouaux, Angela Ballesteros, Zhiqiang Yan, Zubair M. Ahmed, and Teresa Nicolson. “Putting the Pieces Together: The Hair Cell Transduction Complex.” Journal of the Association for Research in Otolaryngology: JARO 22, no. 6 (December 2021): 601–8. https://doi.org/10.1007/s10162-021-00808-0.

Smith, Eliot T., Itallia Pacentine, Anna Shipman, Matthew Hill, and Teresa Nicolson. “Disruption of Tmc1/2a/2b Genes in Zebrafish Reveals Subunit Requirements in Subtypes of Inner Ear Hair Cells.” The Journal of Neuroscience: The Official Journal of the Society for Neuroscience 40, no. 23 (June 3, 2020): 4457–68. https://doi.org/10.1523/JNEUROSCI.0163-20.2020.

Animal Models for Human Deafness

Erickson, Timothy, Itallia V. Pacentine, Alexandra Venuto, Rachel Clemens, and Teresa Nicolson. “The Lhfpl5 Ohnologs Lhfpl5a and Lhfpl5b Are Required for Mechanotransduction in Distinct Populations of Sensory Hair Cells in Zebrafish.” Frontiers in Molecular Neuroscience 12 (2019): 320. https://doi.org/10.3389/fnmol.2019.00320.

Gao, Yan, and Teresa Nicolson. “Temporal Vestibular Deficits in Synaptojanin 1 (Synj1) Mutants.” Frontiers in Molecular Neuroscience 13 (2020): 604189. https://doi.org/10.3389/fnmol.2020.604189.

Nicolson, Teresa. “Navigating Hereditary Hearing Loss: Pathology of the Inner Ear.” Frontiers in Cellular Neuroscience 15 (2021): 660812. https://doi.org/10.3389/fncel.2021.660812.

Pacentine, Itallia V., and Teresa Nicolson. “Subunits of the Mechano-Electrical Transduction Channel, Tmc1/2b, Require Tmie to Localize in Zebrafish Sensory Hair Cells.” PLoS Genetics 15, no. 2 (February 2019): e1007635. https://doi.org/10.1371/journal.pgen.1007635.


Assistant Professor of Otolaryngology - Head & Neck Surgery (OHNS)

Hair Cell Structure, Mechanics, & Function

Ó Maoiléidigh, Dáibhid, and Anthony J. Ricci. “A Bundle of Mechanisms: Inner-Ear Hair-Cell Mechanotransduction.” Trends in Neurosciences 42, no. 3 (March 2019): 221–36. https://doi.org/10.1016/j.tins.2018.12.006.

Miller, Katharine K., Patrick Atkinson, Kyssia Ruth Mendoza, Dáibhid Ó Maoiléidigh, and Nicolas Grillet. “Dimensions of a Living Cochlear Hair Bundle.” Frontiers in Cell and Developmental Biology 9 (2021): 742529. https://doi.org/10.3389/fcell.2021.742529.


Edward C. and Amy H. Sewall Professor in the School of Medicine and Professor of Otolaryngology - Head & Neck Surgery (OHNS) and, by courtesy of Molecular and Cellular Physiology

Hair Cell Structure, Mechanics, & Function

George, Shefin S., Charles R. Steele, and Anthony J. Ricci. “A Two-Photon FRAP Protocol to Measure the Stereociliary Membrane Diffusivity in Rat Cochlear Hair Cells.” STAR Protocols 2, no. 3 (September 17, 2021): 100637. https://doi.org/10.1016/j.xpro.2021.100637.

Niwa, Mamiko, Eric D. Young, Elisabeth Glowatzki, and Anthony J. Ricci. “Functional Subgroups of Cochlear Inner Hair Cell Ribbon Synapses Differently Modulate Their EPSC Properties in Response to Stimulation.” Journal of Neurophysiology 125, no. 6 (June 1, 2021): 2461–79. https://doi.org/10.1152/jn.00452.2020.

Qian, Z. Jason, and Anthony J. Ricci. “Effects of Cochlear Hair Cell Ablation on Spatial Learning/Memory.” Scientific Reports 10, no. 1 (November 26, 2020): 20687. https://doi.org/10.1038/s41598-020-77803-7.

Wang, Yanli, Charles R. Steele, Sunil Puria, and Anthony J. Ricci. “In Situ Motions of Individual Inner-Hair-Cell Stereocilia from Stapes Stimulation in Adult Mice.” Communications Biology 4, no. 1 (August 11, 2021): 958. https://doi.org/10.1038/s42003-021-02459-6.


Bertarelli Foundation Professor and Professor of Otolaryngology - Head & Neck Surgery (OHNS) and, by courtesy, of Neurosurgery

Human Hair Cell Biology

Iyer, Janani S., Richard Seist, In Seok Moon, and Konstantina M. Stankovic. “Two Photon Fluorescence Microscopy of the Unstained Human Cochlea Reveals Organ of Corti Cytoarchitecture.” Frontiers in Cellular Neuroscience 15 (2021): 690953. https://doi.org/10.3389/fncel.2021.690953.

Jeong, Minjin, Karen E. Ocwieja, Dongjun Han, P. Ashley Wackym, Yichen Zhang, Alyssa Brown, Cynthia Moncada, et al. “Direct SARS-CoV-2 Infection of the Human Inner Ear May Underlie COVID-19-Associated Audiovestibular Dysfunction.” Communications Medicine 1, no. 1 (2021): 44. https://doi.org/10.1038/s43856-021-00044-w.

Hair Cell Degeneration & Regeneration

Stojkovic, Miodrag, Dongjun Han, Minjin Jeong, Petra Stojkovic, and Konstantina M. Stankovic. “Human Induced Pluripotent Stem Cells and CRISPR/Cas-Mediated Targeted Genome Editing: Platforms to Tackle Sensorineural Hearing Loss.” Stem Cells (Dayton, Ohio) 39, no. 6 (June 2021): 673–96. https://doi.org/10.1002/stem.3353.

Auditory Nerve Biology & Dysfunction

Seist, Richard, Mingjie Tong, Lukas D. Landegger, Sasa Vasilijic, Hiroshi Hyakusoku, Sachiyo Katsumi, Charles E. McKenna, Albert S. B. Edge, and Konstantina M. Stankovic. “Regeneration of Cochlear Synapses by Systemic Administration of a Bisphosphonate.” Frontiers in Molecular Neuroscience 13 (2020): 87. https://doi.org/10.3389/fnmol.2020.00087.

Wu, Chen-Chi, Aurore Brugeaud, Richard Seist, Hsiao-Chun Lin, Wei-Hsi Yeh, Marco Petrillo, Giovanni Coppola, Albert S. B. Edge, and Konstantina M. Stankovic. “Altered Expression of Genes Regulating Inflammation and Synaptogenesis during Regrowth of Afferent Neurons to Cochlear Hair Cells.” PloS One 15, no. 10 (2020): e0238578. https://doi.org/10.1371/journal.pone.0238578.

Noise Induced Hearing Loss

Landegger, Lukas D., Sasa Vasilijic, Takeshi Fujita, Vitor Y. Soares, Richard Seist, Lei Xu, and Konstantina M. Stankovic. “Cytokine Levels in Inner Ear Fluid of Young and Aged Mice as Molecular Biomarkers of Noise-Induced Hearing Loss.” Frontiers in Neurology 10 (2019): 977. https://doi.org/10.3389/fneur.2019.00977.


Associate Professor of Otolaryngology - Head & Neck Surgery (OHNS)

Prevention of Middle Ear Hearing Loss

Santa Maria, Peter L., Adam C. Kaufman, Brian Bacacao, Anthony Thai, Xiaohua Chen, Anping Xia, Zhixin Cao, Ayman Fouad, and Laurent A. Bekale. “Topical Therapy Failure in Chronic Suppurative Otitis Media Is Due to Persister Cells in Biofilms.” Otology & Neurotology: Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 42, no. 9 (October 1, 2021): e1263–72. https://doi.org/10.1097/MAO.0000000000003222.

Santa Maria, Peter Luke. “A Design-Thinking Approach to Therapeutic Translation: Tympanic Regeneration.” Current Opinion in Otolaryngology & Head and Neck Surgery 28, no. 5 (October 2020): 274–80. https://doi.org/10.1097/MOO.0000000000000650.

Vaisbuch, Yona, Anthony Thai, Sarah L. Pirko, and Peter L. Santa Maria. “Sensorineural Hearing Loss in the Nonimplanted Ear Following Cochlear Implantation in a Patient With Bilateral Enlarged Vestibular Aqueducts.” Otology & Neurotology: Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 40, no. 8 (September 2019): e782–86. https://doi.org/10.1097/MAO.0000000000002319.

Auditory Nerve Biology & Dysfunction

Santa Maria, Peter L., Yangyang Shi, Richard K. Gurgel, C. Eduardo Corrales, Scott G. Soltys, Chloe Santa Maria, Kevin Murray, et al. “Long-Term Hearing Outcomes Following Stereotactic Radiosurgery in Vestibular Schwannoma Patients-A Retrospective Cohort Study.” Neurosurgery 85, no. 4 (October 1, 2019): 550–59. https://doi.org/10.1093/neuros/nyy407.

    

Hair Cell Degeneration & Regeneration

Prevention of Hair Cell Death

Prevention of Middle Ear Hearing Loss

Human Hair Cell Biology

Animal Models for Human Deafness

Noise Induced Hearing Loss

Hair Cell Development

Hair Cell Structure, Mechanics, & Function

Auditory Nerve Biology & Dysfunction

Noise Induced Hearing Loss