Measuring abnormalities in the neurological circuits the brain uses to make sense of sound could be a useful way to track the severity of Friedreich’s ataxia in people with the disease as well as in laboratory models, a new study reports.
The study, “Auditory neuropathy in mice and humans with Friedreich ataxia,” was published in the journal Annals of Clinical and Translational Neurology.
Hearing problems are a common symptom of Friedreich’s ataxia. The disease generally doesn’t affect the cells in the ear that are needed to detect sounds — but, it can cause problems with the nerve circuits that the brain normally uses to make sense of the sounds a person is hearing. For example, many people with Friedreich’s ataxia have difficulty carrying on a conversation in settings with a lot of background noise.
