Parents never want to be in the position of making life-or-death medical decisions for their children. But for parents of children in a new drug trial at the National Institutes of Health, there is an added complication. The medication being used in the trial has the ability to prolong life in the face of a fatal disease, but it also causes significant and permanent hearing loss.
A Dangerous Drug for a Deadly Disease
The disease at the heart of the NIH’s study is Niemann-Pick Type C, often shorthanded to NPC. It’s an incredibly rare genetic disorder that affects a person’s cholesterol metabolism. Worldwide, there are roughly 500 diagnosed cases. Sadly, the vast majority of individuals with NPC pass away before their 20th birthday, and many of them die before the age of 10.
Given the fatality rate of NPC, it’s easy to see the need for a medication to combat it. The drug that the NIH is currently testing is a form of cyclodextrin, a sugar-based compound which doctors hope will extend the lives of children with NPC. Unfortunately, with this drug the trade-off for a longer life is a loss of hearing. So far, participants in the trial have experienced some degree of hearing loss after nearly every round of cyclodextrin injection.
How Does Cyclodextrin Cause Hearing Loss?
Researchers have found that cyclodextrin actually causes hearing loss in two different ways. Initially, the medication damages the outer hair cells of the ears. These cells act as amplifiers, heightening and clarifying auditory information. Scientists have also seen damage to the inner hair cells of mice that have been exposed to high doses of cyclodextrin.
Unfortunately, hearing loss is already a naturally occurring symptom of NPC, and the cyclodextrin is only making matters worse. Further, once this type of hearing loss reaches a certain point, even hearing aids can’t do anything to help.
This kind of hearing loss is also a side effect of other drugs, such as cisplatin, which is used to fight cancer. Scientists are currently conducting research to find ways of combatting the loss of hearing while retaining the medication’s beneficial effects. Still, it may be years before researchers find a way to stave off drug-induced hearing loss.
In the meantime, the parents in the NIH’s cyclodextrin trial are faced with a nearly impossible choice: watch their child’s quality of life diminish or watch them die. It’s a choice that no parent should have to make.
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