Friedreich’s Ataxia and Auditory Processing Disorder

Teive, H.A., Iliadou, V.M. & Manto, M.; Cerebellum (2021). doi:10.1007/s12311-021-01238-7

The link between auditory deprivation (in this case of the form of APD) and cognition has long-term outcomes and should not be thought as a cross-sectional one [17, 18]. Thus, it is of outmost importance to longitudinally evaluate patients with ATX-FXN, document the cognitive deficits when identifying APD at an early stage, and provide appropriate management for these challenging patients. Could this improve patients’ quality of life? This remains to be confirmed. Furthermore, the issue of a possible use of these findings as a biomarker for longitudinal or therapeutic studies remains open and requires novel studies.

Auditory Phenotypic Variability in Friedreich’s Ataxia Patients

Koohi, N., Thomas-Black, G., Giunti, P., Doris-Eva Bamiou.; Cerebellum (2021). doi:10.1007/s12311-021-01236-9

To our knowledge, this is the first study to demonstrate an association between genotype and auditory spatial processing phenotype in patients with FRDA. Auditory temporal processing, neural sound conduction, spatial processing and speech perception were more severely affected in patients with GAA1 greater than 500 repeats. The results of our study may indicate that auditory deprivation plays a role in the development of mild cognitive impairment in FRDA patients.