Radboud University Medical Center, Netherlands
Professor Hannie Kremer’s interest in biology was raised at high school when her biology teacher involved them in a program on how DNA guides the synthesis of proteins at the right time and place.
After high school, Hannie trained as a biologist at the Radboud University in Nijmegen, the Netherlands. Once she received a PhD on the molecular genetics of fruit fly spermatogenesis, she started her research in the field of human genetics.
More about Hannie’s work
Since the year 2000, Hannie’s research has focused on understanding hereditary hearing impairment and Usher syndrome with the goal to provide adequate (genetic) counseling to people with hearing loss and their families and as a starting point for the development of treatment.
Her research group importantly contributed to the current insights into the function of Usher proteins in multi-protein complexes in hair cells of the inner ear and in photoreceptor cells in the retina. In addition, they have identified a significant number of genes associated with non-syndromic and syndromic types of hearing loss.
Hannie’s team has contributed to the current insights into the types and variability of hearing loss caused by defects in the many different genes that are associated with hearing problems.
Hannie’s approaches to hearing research
For me, this is a difficult question to answer, but among the exciting breakthroughs is the CRISPR/Cas technology. This enables us to introduce genetic defects that cause hearing loss in humans into the DNA of mice or other animal models. These animal models will enable us to understand what happens in the inner ear of humans with such defects and they will facilitate the development of treatments.
One of the next big steps forward in hearing research will be treating people with inherited adult-onset hearing loss to prevent further progression or even the onset of the hearing loss. An additional big step will be to understand age-related hearing loss.
Hearing is very important to stay connected with family, friends and colleagues, to enjoy music and to hear sounds of the environment for example while walking in the woods. My motivation is that my research can contribute to prevent or cure hearing loss in the future and for today, that my research provides answers to questions of people with regard to the cause of their hearing loss or of the hearing loss of their child.
The inner ear is an amazing organ in its structural and functional complexity. Working in hearing research allowed me to combine genetics of human diseases and biology. At the time I started in hearing research, there was so much to discover on the genes and proteins that build the inner ear and make it function properly. Finding the defects that underlie hearing loss in humans and mice was important for our current understanding of the inner ear.