There is documented research indicating that hearing loss is sometimes associated with
Friedreich's ataxia. It is an unusual form of hearing loss that is best examined
physiologically rather than with the standard electronic tests. Standard earphone
tests often proclaim FRDA patients to have normal hearing when in fact they cannot hear
well in noisy situations or open classrooms or theaters. A better test is a
combination of four procedures: Otoacoustic Emissions, Tympanometry and Middle Ear
Muscle Reflexes and the Audutory Brainstem Response Test (ABR). If the ABR is abnormal but
the emissions are present, the patient will have trouble in noise but will NOT benefit
from hearing aids. They might benefit from something called an FM system.
As a matter of fact, few if any FRDA patients really benefit from hearing aids
regardless of their audiograms. So, if you or a loved one with FRDA complains
of hearing problems or seems to hear poorly especially in noisy situations, ask for a full
test including all of the above to get a solid picture of what can and can't be done.
With the progression of this type of hearing loss, loud noisy places such as
restaurants/bars or crowded places aren't the place to go if you want to have a
conversation, because it is difficult to discern the conversation from background noise,
such as music, other people talking, dishes rattling, chairs moving, etc. However, if you
are watching tv, talking on a phone or having a conversation and there is no background
noise, hearing is usually fine.
Contributed by Bronya Keats, PhD; Louisiana State University (LSU) Health Sciences
Center
Scientific Advisory Board, Friedreich's Ataxia Research Alliance (FARA)
Charles Berlin, PhD; LSU Health Sciences Center
Raychel Bartek, FARA
