Symptoms Of Friedreich's Ataxia

Hearing Loss

Friedreich's ataxia patients will begin to have trouble understanding others when communicating verbally and they will also have delayed reactions to sounds that change rapidly. Selective nerve degeneration occurs in the temporal lobe of the brain where signals are exchanged between the brain and the auditory components. This causes inevitable interruption of nerve synchronization that results in the deterioration of normal temporal resolution or the capability to discern quick changes of auditory signals over time. In other words, the hearing loss caused by Friedreich's ataxia is not due to the inability to physically hear sounds, but instead lies in the functionality of the nerve communication responsible for how the brain perceives and interprets such auditory sounds. An individual with Friedreich's ataxia will not be able to interpret the rapidly changing speech phonemes present in running speech fast enough for their temporal lobe to correctly interpret what that speech is. Because the loss of hearing caused by Friedreich's ataxia cannot be remedied with a hearing aid, often there are negative impacts on socialization, education, and general communication with others.

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