Elaine Wong

BSc, MPhil, PhD
Lead investigator – Stem cell and inner ear gene therapy
Senior Research Fellow, Ear Science Institute Australia
Adjunct Senior Research Fellow, Curtin University
Adjunct Senior Research Fellow, University of Western Australia

HEARING THERAPEUTICS

Our hope is we might be able to cure and restore hearing for patients. We are using stem cell and gene therapy for inner ear hair cell regeneration and protection.

Dr. Elaine Wong is internationally recognised for her expertise in human hearing loss and inner ear hair cell regeneration. She is a Senior Research Fellow at the Ear Science Institute Australia, Medical School at Curtin University and Ear Sciences Centre at University of Western Australia. Her research focus is on understanding the genetic, cellular and molecular factors underlying human deafness with the aim of developing novel therapies to restore hearing. Her lead research programme currently investigates the therapeutic potential of hair cell regeneration.

Dr Wong’s research has attracted over $2 million in research funding from the Australian and Hong Kong governments. She has filed a provisional patent application based on the ear organoid methodology she has developed. Another aspect of her work is the differentiation of patient-derived iPSCs into inner ear organoids to generate a human in vitro disease model of Usher syndrome, which causes both profound vision and hearing loss.

Dr Wong and her team at ESIA have developed a protocol to derive inner ear organoids from human induced pluripotent stem cell (iPSC), which contain hair cells, the key sensory cells of the auditory system. These organoids are being used to evaluate how specific gene mutations affect hair cell development and function, with the aim to improve ear organoid technology and identify therapeutic targets. The strength of her research is in the comparative analysis of primary human cell cultures with preclinical transgenic and knockout mouse models. These models have been instrumental in understanding the regulatory pathways required for hair cell development and function, and have provided key insights into the human situation.

Dr. Wong’s team is also focused on studying the role of the transcription factors in the regeneration of inner ear hair cells. The long-term goal is to develop therapies for the prevention of hearing loss and restoration of function in human deafness. Dr. Wong has existing active collaborations with investigators in Lions Eye Institute and Curtin University in the team demonstrated by joint Honours students, PhD students and research publication.

Areas of research
  • Stem cell technology
  • Gene delivery
  • Gene editing
Top publications

Zaw, K^., Wong, E.Y^., Zhang, X., Zhang, D., Chen, S.C., Thompson, J.A., Lamey, T., McLaren, T., De Roach, J.N. Wilton, S.D., Fletcher, S., Mitrpant, C., Atlas, M.D., Chen, F.K., McLenachan, S. (2021) Generation of three induced pluripotent stem cell lines from a patient with Usher syndrome caused by biallelic c.949C>A and c.1256G>T mutations in the USH2A gene. Stem Cell Research, 50, 102129 (^Co-1st author) https://doi.org/10.1016/j.scr.2020.102129

Whatley, M., Francis, A., Ng, Z.Y., Khoh, X.E., Atlas, M.D., Dilley, R.J., Wong, E.Y.* Usher syndrome: genetics and molecular links of hearing loss and directions for therapy. Frontiers in Genetics, 11, 565216. (*Corresponding author)
https://doi.org/10.3389/fgene.2020.565216

McLenachan, S.*, Wong, E.Y.*, Leith, F., Moon, S.Y., Zhang, D., Chen, S.C., Zhang, X., Thompson, J.A., McLaren, T., Lamey, T., De Roach, J.N., Atlas, M.D., Dilley, R.J. and Chen, F.K. (2019) Generation of two induced pluripotent stem cell lines from a patient with compound heterozygous mutations in the USH2A gene. Stem Cell Research, 36, 101420 (*Co-1st author)
https://doi.org/10.1016/j.scr.2019.101420

Wong, E.Y., Xu, C.Y., Brahmachary, M. and Xu, P.X. (2016) A novel ENU-induced mutation in Myo6 causes vestibular dysfunction and deafness. PLoS ONE, 11, e0154984.
https://doi.org/10.1371/journal.pone.0154984

Ahmed M, Wong EY, Xu J, Wang F and Xu PX (2012) Eya1-Six1 interaction is sufficient to induce hair cell fate in the cochlea by activating Atoh1 expression in cooperation with Sox2. Developmental Cell, 22, 377-390 (Recommended by FACULTY OF 1000).
https://doi.org/10.1016/j.devcel.2011.12.006

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