Altered prosody has been described in symptomatic Huntington’s Disease (HD) individuals, however, the extent to which acoustic analysis of speech is sensitive to gene positive pre-manifest (PreHD) individuals is unknown. Speech samples were acquired from 30 individuals carrying the mutant HTT gene (13 PreHD, 17 early stage HD) and 15 matched controls. Participants read a passage, produced a monologue, counted from 1-20 and said the days of the week. Data were analysed acoustically for measures of timing, frequency and intensity. Tasks were compared to determine their relative sensitivity to disease state. Tasks with greater cognitive demand appeared more suitable for detecting Huntington’s disease compared to tasks with high levels of automaticity.

The effect of 24 hours of sustained wakefulness on the speech of healthy adults is poorly documented. Therefore, speech samples were systematically acquired (e.g., every four hours) from 18 healthy adults over 24 hours. Stimuli included automated and extemporaneous tasks, sustained vowel and a read passage. Measures of timing and frequency were derived acoustically using Praat and significant changes were observed on all tasks. The effect of fatigue on speech was found to be strongest just before dawn (after 22 hours). Key features of timing (e.g., mean pause length), frequency (e.g., F4 variation) and power (alpha ratio) changed as a function of increasing levels of fatigue.

Dysphagia in Friedreich ataxia (FRDA) and its impact on quality of life is not adequately understood. The objective of this study was to characterise dysphagia in FRDA and to determine the impact of swallowing dysfunction on activities, participation, and sense of well-being. Thirty-six individuals with a confirmed diagnosis of FRDA were assessed via a clinical bedside examination (CBE), the Royal Brisbane Hospital outcome measure for swallowing, an oral-motor examination and the Australian therapy outcome measures for speech and swallowing (AusTOMS). Data on swallowing function, diet modification and swallowing strategies were collated. Thirty-three (91.67 %) participants exhibited clinical signs of dysphagia according to the CBE, and all participants received ratings indicating swallowing difficulties on at least one other measure. Dysphagia in FRDA is characterised by oral and pharyngeal stage impairment relating to incoordination, weakness and spasticity. A significant positive correlation was found between the severity of impairment, activity, participation and distress/well-being on the AusTOMS, suggesting that swallowing function decreases with overall reductions in quality of life. A significant correlation was found between activity on the AusTOMS and disease duration (r = −0.283, p = 0.012). No significant correlations were found between dysphagia severity and GAA repeat length, age of onset or disease severity. Participants employing diet modification and swallowing strategies demonstrated higher dysphagia severity, activity limitations and participation restrictions. These data advocate a holistic approach to dysphagia management in FRDA. Early detection of swallowing impairment and consideration of the potential impact dysphagia has on quality of life should be key aspects in disease management.

This study aimed to determine if adolescents/young adults gained additional perceptual benefit from sequential bilateral cochlear implants within 12 months, and to document adaptation to the second implant. Assessments comprised a pediatric version of The Speech, Spatial and Qualities of Hearing Scale (SSQ), anecdotal reports of device use and daily listening, and the Adaptive Spondee Discrimination Test (AdSpon). All nine participants achieved full-time use of, a preference for, and superior daily listening with, bilateral implants. Eight participants were comfortable using the second implant alone, and two achieved similar daily listening with either implant alone. SSQ ratings were higher post-operatively for the majority of participants. AdSpon performance was superior bilaterally for five participants with noise ipsilateral to the first implant, but not contralateral. Unilateral performance with either implant was similar for one participant. A second implant may provide additional benefit up to 19 years of age, even with congenital hearing loss and >16 years between implants. Families and clinicians should understand the aspects of second-implant candidacy and post-operative use that are unique to adolescents/young adults.

OBJECTIVES: To compare the listening effort expended by adolescents and young adults using implants versus their peers with normal hearing when these two groups are achieving similar speech perception scores. The study also aimed to compare listening effort expended by adolescents and young adults with bilateral cochlear implants when using two implants versus one. METHODS: Eight participants with bilateral cochlear implants and eight with normal hearing aged 10-22 years were included. Using a dual-task paradigm, participants repeated consonant-nucleus-consonant (CNC) words presented in noise and performed a visual matching task. Signal-to-noise ratios were set individually to ensure the word perception task was challenging but manageable for all. Reduced performance on the visual task in the dual-task condition relative to the single-task condition was indicative of the effort expended on the listening task. RESULTS: The cochlear implant group, when using bilateral implants, expended similar levels of listening effort to the normal hearing group when the two groups were achieving similar speech perception scores. For three individuals with cochlear implants, and the group, listening effort was significantly reduced with bilateral compared to unilateral implants. DISCUSSION: The similar amount of listening effort expended by the two groups indicated that a higher signal-to-noise ratio overcame limitations in the auditory information received or processed by the participants with implants. This study is the first to objectively compare listening effort using two versus one cochlear implant. The results provide objective evidence that reduced listening effort is a benefit that some individuals gain from bilateral cochlear implants.

Subjective assessment of hearing ability in everyday life complements more objective forms of evaluation. A broad evaluation of the additional benefit provided to children by a second bilateral cochlear implant required such an assessment. As no paediatric tool provided detailed evaluation of performance in the areas of daily listening in which benefit was likely to be demonstrated, an adult questionnaire was adapted. Items of the Speech, Spatial and Qualities of Hearing Scale (SSQ) focused mainly, although not exclusively, on hearing functions requiring the binaural system. The adapted child, parent, and teacher versions of the SSQ retained the structure of rating listening performance in everyday scenarios across the domains of speech perception, spatial hearing, and other qualities of hearing. Modifications were minimized, although deletion of some items and wording changes were required, and some subdomains could not be included. Observation periods were introduced so that parents and teachers observe performance prior to providing ratings. The suggested minimum age is 11 years for the child version and 5 years for the parent and teacher versions. Instructions indicate interview-style administration in which interpretation of the described listening scenarios can be clarified and use of the ruler-style response format demonstrated. Researchers applying the SSQ for parents have reported higher performance ratings for bilateral over unilateral cochlear implants, particularly in the spatial hearing domain. Further research should provide evidence for the target age range, compare child and parent responses, and evaluate modifications for use with younger children.

Proceedings of the Audiology Australia National Conference

OBJECTIVES: Although it has been established that bilateral cochlear implants (CIs) offer additional speech perception and localization benefits to many children with severe to profound hearing loss, whether these improved perceptual abilities facilitate significantly better language development has not yet been clearly established. The aims of this study were to compare language abilities of children having unilateral and bilateral CIs to quantify the rate of any improvement in language attributable to bilateral CIs and to document other predictors of language development in children with CIs. DESIGN: The receptive vocabulary and language development of 91 children was assessed when they were aged either 5 or 8 years old by using the Peabody Picture Vocabulary Test (fourth edition), and either the Preschool Language Scales (fourth edition) or the Clinical Evaluation of Language Fundamentals (fourth edition), respectively. Cognitive ability, parent involvement in children's intervention or education programs, and family reading habits were also evaluated. Language outcomes were examined by using linear regression analyses. The influence of elements of parenting style, child characteristics, and family background as predictors of outcomes were examined. RESULTS: Children using bilateral CIs achieved significantly better vocabulary outcomes and significantly higher scores on the Core and Expressive Language subscales of the Clinical Evaluation of Language Fundamentals (fourth edition) than did comparable children with unilateral CIs. Scores on the Preschool Language Scales (fourth edition) did not differ significantly between children with unilateral and bilateral CIs. Bilateral CI use was found to predict significantly faster rates of vocabulary and language development than unilateral CI use; the magnitude of this effect was moderated by child age at activation of the bilateral CI. In terms of parenting style, high levels of parental involvement, low amounts of screen time, and more time spent by adults reading to children facilitated significantly better vocabulary and language outcomes. In terms of child characteristics, higher cognitive ability and female sex were predictive of significantly better language outcomes. When family background factors were examined, having tertiary-educated primary caregivers and a family history of hearing loss were significantly predictive of better outcomes. Birth order was also found to have a significant negative effect on both vocabulary and language outcomes, with each older sibling predicting a 5 to 10% decrease in scores. CONCLUSIONS: Children with bilateral CIs achieved significantly better vocabulary outcomes, and 8-year-old children with bilateral CIs had significantly better language outcomes than did children with unilateral CIs. These improvements were moderated by children's ages at both first and second CIs. The outcomes were also significantly predicted by a number of factors related to parenting, child characteristics, and family background. Fifty-one percent of the variance in vocabulary outcomes and between 59 to 69% of the variance in language outcomes was predicted by the regression models.