Introduction: Friedreich’s ataxia (FRDA) is an inherited ataxia with a range of progressive features including axonal degeneration of sensory nerves. The aim of this study was to investigate auditory perception in affected individuals. Methods: Fourteen subjects with genetically defined FRDA participated. Two control groups, one consisting of healthy, normally hearing individuals and another comprised of subjects with sensorineural hearing loss, were also assessed. Auditory processing was evaluated using structured tasks designed to reveal the listeners’ ability to perceive temporal and spectral cues. Findings were then correlated with open-set speech understanding. Results: Nine of 14 individuals with FRDA showed evidence of auditory processing disorder. Gap and amplitude modulation detection levels in these subjects were significantly elevated, indicating impaired encoding of rapid signal changes. Electrophysiologic findings (auditory brainstem response, ABR) also reflected disrupted neural activity. Speech understanding was significantly affected in these listeners and the degree of disruption was related to temporal processing ability. Speech analyses indicated that timing cues (notably consonant voice onset time and vowel duration) were most affected. Conclusion: The results suggest that auditory pathway abnormality is a relatively common consequence of FRDA. Regular auditory evaluation should therefore be part of the management regime for all affected individuals. This assessment should include both ABR testing, which can provide insights into the degree to which auditory neural activity is disrupted, and some functional measure of hearing capacity such as speech perception assessment, which can quantify the disorder and provide a basis for intervention.

Peripheral neuropathy is a major consequence of diabetes mellitus with up to 50 % of patients showing clinically significant neural injury during the disease course. Hearing loss (as defined by impaired sound detection thresholds) is a recognized symptom of DM, but the possibility of auditory neuropathy (AN) has not been explored in this population. This pilot study investigated peripheral auditory function, auditory processing and speech perception in individuals with Type 1 diabetes mellitus (T1DM) and compared the findings with measures of vestibular function, ocular pathology/visual acuity and overall neurologic profile. Ten adults with T1DM and ten matched controls underwent a battery of tests which included: audiometry, otoacoustic emissions, auditory brainstem responses, temporal processing measures and speech perception. Six of the ten T1DM participants showed electrophysiologic evidence of AN and impaired functional hearing. Furthermore, auditory capacity was correlated with both visual acuity and degree of somatic peripheral neuropathy. This pilot investigation revealed functional-hearing deficits severe enough to impact upon everyday communication. Should the findings be confirmed by larger studies, auditory evaluation may form an important part of the management regimen for individuals with T1DM. This may be especially important for those with DM-related eye conditions, as deficits across multiple sensory modalities can have multiplicative detrimental effects on quality-of-life.

High levels of stress and anxiety are common in children with Autism Spectrum Disorder (ASD). Within this study of school-aged children (20 male, 6 female) we hypothesised that functional hearing deficits (also pervasive in ASD) could be ameliorated by auditory interventions and that, as a consequence, stress levels would be reduced. The use of Ear-Level Remote Microphone devices and Classroom Amplification systems resulted in significantly improved listening, communication and social interaction and a reduction in physiologic stress levels (salivary cortisol) in both one-on-one and group listening situations.

Hearing deficits severe enough to restrict communication and threaten academic progress were common on our group of school-aged children with Type 1 diabetes. Evaluation of both cochlear and auditory neural function may form an important part of the standard management regime for children with diabetes.

Objective: The high energy demand of the auditory and visual pathways render these sensory systems prone to diseases that impair mitochondrial function. Primary open-angle glaucoma, a neurodegenerative disease of the optic nerve, has recently been associated with a spectrum of mitochondrial abnormalities. This study sought to investigate auditory processing in individuals with open-angle glaucoma. Design/Study sample: Twenty-seven subjects with open-angle glaucoma underwent electrophysiologic (auditory brainstem response), auditory temporal processing (amplitude modulation detection), and speech perception (monosyllabic words in quiet and background noise) assessment in each ear. A cohort of age, gender and hearing level matched control subjects was also tested. Results: While the majority of glaucoma subjects in this study demonstrated normal auditory function, there were a signifi cant number (6/27 subjects, 22%) who showed abnormal auditory brainstem responses and impaired auditory perception in one or both ears. Conclusions:The fi nding that a signifi cant proportion of subjects with open-angle glaucoma presented with auditory dysfunction provides evidence of systemic neuronal susceptibility. Affected individuals may suffer signifi cant communication diffi culties in everyday listening situations.