Hearing loss has a significant impact on quality of life and society in general
Hearing loss is the most frequent sensory impairment and affects 360 million people worldwide. Hearing loss also increases the chance of cognitive decline and dementia. Hearing rehabilitation is therefore a way to slow down cognitive decline in older adults. It was therefore listed by the World Health Organization (WHO) as a priority disease for research into therapeutic interventions.
DFNA9 is a dominant inherited condition that leads to severe hearing loss and loss of balance
It is caused by heterozygous mutations in the COCH gene, which progressively leads to bilateral deafness and loss of balance at the age of 50-70 years. Currently there is no treatment available that can prevent hearing loss in DFNA9 patients. Local gene therapy to restore hearing or prevention of hearing loss has been extensively studied in recent years via neonatal mouse models. Currently, there is a clinical trial in which adult patients with severe perceptive hearing loss are injected with virus-based vectors carrying gene therapy to repair hair cells. This injection is done directly in the cochlea.
Development of gene therapy for the inner ear as prevention against hearing loss
By using adeno-associated viral vectors, CRISPR-Cpf1 genome correcting technology in the cochlea will identify and remove the mutated (and normal) Coch in the genomic DNA. This in a safe and efficient way to block the expression of the mutant (and normal) Coch protein in the presymptomatic phase. In this way we expect to reduce the severity of the hearing loss or to prevent the occurrence of hearing loss in DFNA9.
Mapping the impact of hearing and balance on dementia
We do this by evaluating cognition, both spatially and non-spatially, with different forms of hearing loss, a loss of balance or both. We are specifically interested in the effect on neurogenesis in the hippocampus.
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Statute & functions
Zelfstandig academisch pers.
- assistant professor