Friedreich ataxia (FRDA) is a neurodegenerative disease affecting both motor and sensory systems through mutations of the FXN gene. Electrophysiologic changes consistent with axonopathy in the cochlear nerve and auditory brainstem have been reported in a high proportion ([90 %) of affected individuals, as have functional effects including disruption of temporal-resolution and speech perception [1, 2]. We present longitudinal findings for two males with FRDA in whom auditory deterioration mirrored overall disease progress. Patient 1 reported walking difficulties evident from his late teens. Molecular diagnosis confirmed homozygosity for GAA expansions in intron 1 of the FXN gene (repeat lengths: 527/1058). When last examined (at 32 years old), he could walk independently but required a motorisedscooter for community mobility. He also had mild dysarthria, upper limb dysmetria, lower limb areflexia and scoliosis. Patient 2 also reported walking problems in adolescence (15 years). Molecular diagnosis confirmed homozygosity for GAA expansions in intron 1 of the FXN gene (repeat lengths: 569/884). At diagnosis he was independently ambulant, but his physical difficulties progressed rapidly, and at 22 years old he required a walker for short distances and wheelchair for community access. He also had upper limb dysmetria, dysarthria, lower limb areflexia, scoliosis and hypertrophic cardiomyopathy. Both individuals underwent annual auditory brainstem response (ABR) and binaural speech-processing (Listening in Spacialized Noise [LiSN-S]) evaluations over a 3 year period. Patient 1 was 29–32 years and Patient 2, 18–21 years old at assessment. Overall disease severity was equivalent in the two subjects at initial assessment with both achieving scores of about 65 on the Friedreich Ataxia Rating Scale (FARS) [3]. Disability levels for Patient 1 remained stable, but Patient 2 had significant functional deterioration across the study period (Fig. 1). Both subjects showed evidence of cochlear nerve axonopathy despite maintaining normal sound detection. Patient 1 presented with ABRs of normal latency and low amplitude at each test point (Fig. 2a), suggesting normal transmission efficiency but reduced neural populations in the central auditory pathways [1]. Patient 2 showed normal ABRs at first assessment, which decreased in amplitude over the study period and became unidentifiable in the final year (Fig. 2b). ‘‘Spatial listening’’ is the ability to localize sound sources (and hence improve perception) through the integration of subtle timing and level differences in the acoustic-signal reaching the two ears [4]. These interaural cues are initially processed in the lower brainstem, where neural impulses from left and right nerves meet at the G. Rance (&) Department of Audiology and Speech Pathology, The University of Melbourne, 550 Swanston Street, Parkville, Melbourne, VIC 3010, Australia e-mail: grance@unimelb.edu.au