The purpose is to create a disease model, mimicking congenital cytomegalovirus infection of the inner ear. By culturing inner ear organoids with cytomegalovirus, we seek insights into childhood hearing loss, bridging the gap towards a potential cure.
Cytomegalovirus (CMV) is the leading non-genetic cause of congenital hearing loss, transmitted from mother to child during pregnancy. The mechanism behind its hearing damage is unclear, and no cure exists. Given its species-specific nature, animal models provide limited insights. Yet, human stem cells hold promise, as they can differentiate into inner ear organoids. This enables us to perform comprehensive CMV research, without causing distress on mother and child.
The Otobiology Leiden group has been able to develop inner ear organoids containing a diversity of inner ear cell types. Currently I am in my third PhD year. I’ve successfully infected inner ear organoids with CMV, uncovering affected inner ear cell types. The whole protocol covers 90 days, followed by months of analysis. Nonetheless, protocol and results submission is on our horizon.
The image shows the effect that CMV has on inner ear cells. Unaffected cells have concentrated DNA in the nucleus (green). Affected cells are surrounded by a cloud of CMV protein (red) and the DNA is fragmented by the virus (blue). It is evident why this virus is called the cytomegalovirus, as the affected cells are enlarged.
Lucia Grijpink, MD
Otobiology Leiden, Leiden University Medical Center