Graeme Clark Collection

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    Clinical findings for a group of infants and young children with auditory neuropathy
    RANCE, GARY ; Beer, David E. ; Cone-Wesson, Barbara ; Shepherd, Robert K. ; Dowell, Richard C. ; King, Alison M. ; Rickards, Field W. ; Clark, Graeme M. ( 1999)
    Objective: To examine the prevalence of auditory neuropathy in a group of infants at risk for hearing impairment and to present an overview of the clinical findings for affected children. Design: Results for 20 subjects who showed repeatable cochlear microphonic potentials in the absence of click-evoked auditory brain stem responses are included in this study. Behavioral and steady state evoked potential thresholds were established in each case. Where possible, otoacoustic emission and speech perception results (unaided and aided) also were obtained. Results: One in 433 (0.23%) of the children in our series had evidence of auditory neuropathy. The audiometric findings for these subjects varied significantly, with behavioural thresholds ranging from normal to profound levels. Discrimination skills were also variable. Approximately half of the subjects showed little understanding, or even awareness, of speech inputs in both the unaided and aided conditions. There were, however, a number of children who could score at significant levels on speech discrimination tasks and who benefited from the provision of amplification. Conclusion: The results suggest that auditory neuropathy is more common in the infant population than previously suspected. The effects of neuropathy on auditory function appear to be idiosyncratic, producing significant variations in both the detection and discrimination of auditory signals. As such, the management of children with this disorder must allow for individual differences.
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    Steady-state evoked potential and behavioural hearing thresholds in a group of children with absent click-evoked auditory brain stem response
    RANCE, GARY ; Dowell, Richard C. ; Rickards, Field W. ; Beer, David E. ; Clark, Graeme M. ( 1998)
    Objective: 1) To examine the distribution of behavioural hearing thresholds in a group of children who had shown no click-evoked auditory brain stem response (ABR) at maximum presentation levels. 2) To describe the relationship between the 90 Hz steady-state evoked potential (SSEP) and behavioural thresholds in these subjects. Design: A retrospective study based on clinical findings obtained from 108 infants and young children. Each of these children had shown no recordable ABR to clicks presented at maximum levels (100 dB nHL). SSEP audiograms were obtained using AM/FM tones at the octave frequencies 250 to 4000 Hz. The results of these evoked potential assessments were compared with hearing thresholds established behaviourally. Results: Click-ABR assessment could not differentiate between the subjects in our sample with total hearing losses and those with useful residual hearing. Although some of the ears were anacusic, more than a quarter showed residual hearing ate each of the audiometric frequencies. Furthermore, at least 10% of the behavioural thresholds at each frequency fell within the moderate/severe hearing loss range. A far closer relationship was observed between SSEP and hearing thresholds. On occasions where the SSEP was absent at maximum levels, 99.5% of the ears showed either a total loss or a behavioural threshold within 10 dB of that level. When an SSEP was obtained, the hearing threshold was typically within 5 dB of the SSEP threshold. Conclusion: The results suggested that in our group of selected subjects, the SSEP technique was able to assess ears with only minimal amounts of residual hearing. Where the brevity of the acoustic click limits both its frequency specificity and its presentation level, the modulated tones used for SSEP testing allow accurate, frequency-specific assessment at high presentation levels.
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    The progress of children using the multichannel cochlear implant in Melbourne
    Cowan, R. S. C. ; Dowell, R. C. ; Hollow, R. ; Dettman, S. J. ; Rance, G. ; Barker, E. J. ; Sarant, J. Z. ; Galvin, K. L. ; Webb, R. C. ; Pyman, B. C. ; Cousins, V. C. ; Clark, Graeme M. ( 1995)
    Multi-channel cochlear implantation in children began in Australia in 1985 and there are now close to 4000 profoundly deaf children and adolescents using the Australian implant system around the world. The aim of the implant procedure is to provide adequate hearing for speech and language development through auditory input. This contrasts with the situation for adults with acquired deafness where the cochlear implant aims to restore hearing for someone with well-developed auditory processing and language skills. As with adults, results vary over a wide range for children using the Multi-channel implant. Many factors have been suggested that may contribute to differences in speech perception for implanted children. In an attempt to better understand these factors, the speech perception results for children implanted in Melbourne were reviewed and subjected to statistical analysis. This has indicated that the amount of experience with the implant and the length of sensory deprivation are strongly correlated with perceptual results. This means that younger children are likely to perform better with an implant and that a number of years of experience are required for children to reach their full potential. The results have also indicated that educational placement and management play a crucial role in children reaching their potential. Overall, 60% of the children and adolescents in the study have reached a level of open-set speech understanding using the cochlear implant without lipreading.