Exploring the support needs of Australian parents of young children with Usher syndrome: a qualitative thematic analysis.

BACKGROUND: Advancements in genetic testing have led to Usher syndrome now being diagnosed at a much earlier age than in the past, enabling the provision of early intervention and support to children and families. Despite these developments, anecdotal reports suggest there are substantial gaps in the services and supports provided to parents of children with Usher syndrome. The current study investigated the support needs of parents of children with Usher syndrome Type 1 when their child was aged 0 to 5 years. METHOD: Purposive sampling was used, and six semi-structured interviews were conducted with Australian parents of children with Usher syndrome, Type 1. Data was analysed using modified reflexive thematic analysis. RESULTS: Four key themes were identified as being central to the support needs of parents of children with Usher syndrome aged 0 to 5 years. (1) Social Needs referred to parents' need for various sources of social support, (2) Informational Needs described the lack of information parents received regarding Usher syndrome from treating professionals, (3) Practical Needs included supports needed to assist parents in managing the day-to-day tasks of caring for a child with a disability, and (4) Emotional Needs represented the emotional support (both formal and informal) that parents needed to be a positive support to their child. CONCLUSIONS: Findings provide rich information for relevant support groups, policy makers, individual healthcare professionals, and professional governing bodies regarding the education of stakeholders and the development and implementation of best-practice treatment guidelines.

Generalization of tactile perceptual skills to new context following tactile-alone word recognition training with the Tickle Talker.

The Tickle Talker is an electrotactile speech perception device. Subjects were evaluated using the device in various tactile-alone and tactile-visual contexts to assess the generalization to other contexts of tactile-alone perceptual skills. The subjects were from a group of six normally hearing subjects who had previously received 12 to 33 h of tactile-alone word recognition training and had learned an average vocabulary of 50 words [Galvin et al., J. Acoust. Soc. Am. 106, 1084-1089 (1999)]. The tactile-alone evaluation contexts were sentences, unfamiliar talkers, and untrained words. The tactile-visual evaluation contexts were closed-set words, open-set words, and open-set sentences. Tactile-alone perceptual skills were generalized to unfamiliar speakers, sentences, and untrained words, though scores indicated that generalization was not complete. In contrast, the generalization of skills to tactile-visual contexts was minimal or absent. The potential value of tactile-alone training for hearing-impaired users of the Tickle Talker is discussed.

A comparison of Tactaid II+ and Tactaid 7 use by adults with a profound hearing impairment.

OBJECTIVE: To evaluate and compare use of the Tactaid II+ and the Tactaid 7, in terms of speech perception, by adults with a hearing impairment. DESIGN: Eight adults used one device daily for approximately 10 wk and attended seven training sessions. Performance was measured with tests of phonetic contrast perception, closed-set vowel and consonant identification, word and phoneme recognition in monosyllabic word lists, word recognition in sentences and speechtracking rate. A questionnaire was also administered. The protocol was repeated with the alternative device. RESULTS: With each device, the group discriminated most phonetic contrasts at better-than-chance levels and demonstrated somewhat enhanced visual or auditory-visual perception when measured in terms of vowel identification, monosyllabic word recognition and speechtracking rate. An increase in speechtracking rate was also demonstrated for some individuals. Subjects generally reported little subjective improvement in speech perception and production, but were satisfied with the physical attributes of each device. Five of six subjects preferred the Tactaid 7. CONCLUSIONS: The Tactaid II+ and the Tactaid 7 provided suprasegmental and segmental information, enabling the group to discriminate phonetic contrasts and improve their perception of some speech materials. No consistent advantage was found for either device, thought most subjects preferred the Tactaid 7. Alternatives likely to provide a greater benefit to communication should be considered before a Tactaid fitting.

Acquisition of a tactile-alone vocabulary by normally hearing users of the Tickle Talker.

Tactile-alone word recognition training was provided to six normally hearing users of the Tickle Talker, an electrotactile speech perception device. A mean group tactile-alone vocabulary of 31 words was learned in 12 h of training. These results were comparable to, or superior to, those reported for other tactile devices and Tadoma. With increased training the group became faster at learning tactually new words, which were introduced in small training sets. However, as their tactile-alone vocabulary grew, subjects required more training time to reach the pass criterion when evaluated on their recognition of their whole vocabulary list. A maximum possible vocabulary size was not established. The application of tactile-alone training with hearing-impaired users of the device is discussed.

Speech perception results for children with implants with different levels of preoperative residual hearing.

OBJECTIVE: Many reports have established that hearing-impaired children using the Nucleus 22-channel cochlear implant may show both significant benefits to lipreading and significant scores on open-set words and sentences using electrical stimulation only. These findings have raised questions about whether severely or severely-to-profoundly deaf children should be candidates for cochlear implants. To study this question, postoperative results for implanted children with different levels of preoperative residual hearing were evaluated in terms of speech perception benefits. STUDY DESIGN/SETTING: A retrospective study of the first 117 children, sequentially, to undergo implantation in the Melbourne and Sydney Cochlear Implant Clinics was undertaken. All children had been assessed by and received their implants in a tertiary referral centre. MAIN OUTCOME MEASURES: To assess aided residual hearing, the children were grouped into four categories of hearing on the basis of their aided residual hearing thresholds measured preoperatively. To assess benefits, the scores of children on standard speech perception tests were reviewed. As different tests were used for children with different ages and language skills, children were grouped into categories according to the level of postoperative speech perception benefit. RESULTS: The results showed that children in the higher categories of aided preoperative residual hearing showed significant scores on open-set word and sentence perception tests using the implant alone. For children in lower categories of aided residual hearing, results were variable within the groups. More than 90% of children with implants with aided residual hearing thresholds in the speech range above 1 kHz achieved open-set understanding of words and sentences. CONCLUSION: While the results of this preliminary study confirm previous findings of differential outcomes for children with different levels of preoperative residual hearing, they suggest that children with severe to profound hearing impairments should be considered for cochlear implantation.

Speech perception in children using the advanced Speak speech-processing strategy.

The Speak speech-processing strategy, developed by the University of Melbourne and commercialized by Cochlear Pty Limited for use in the new Spectra 22 speech processor, has been shown to provide improved speech perception for adults in both quiet and noisy situations. The present study evaluated the ability of children experienced in the use of the Multipeak (Mpeak) speech-processing strategy (implemented in the Nucleus Minisystem-22 cochlear implant) to adapt to and benefit from the advanced Speak speech-processing strategy (implemented in the Nucleus Spectra 22 speech processor). Twelve children were assessed using Mpeak and Speak over a period of 8 months. All of the children had over 1 year's previous experience with Mpeak, and all were able to score significantly on open-set word and sentence tests using the cochlear implant alone. Children were assessed with both live-voice and recorded speech materials, including Consonant-Nucleus-Consonant monosyllabic words and Speech Intelligibility Test sentences. Assessments were made in both quiet and in noise. Assessments were made at 3-week intervals to investigate the ability of the children to adapt to the new speech-processing strategy. For most of the children, a significant advantage was evident when using the Speak strategy as compared with Mpeak. For 4 of the children, there was no decrement in speech perception scores immediately following fitting with Speak. Eight of the children showed a small (10% to 20%) decrement in speech perception scores for between 3 and 6 weeks following the changeover to Speak. After 24 weeks' experience with Speak, 11 of the children had shown a steady increase in speech perception scores, with final Speak scores higher than for Mpeak. Only 1 child showed a significant decrement in speech perception with Speak, which did not recover to original Mpeak levels.

Improved electrotactile speech processor: Tickle Talker.

The Tickle Talker, an eight-channel electrotactile speech processor, has been developed from continuing research at the University of Melbourne. The development of the device has focused on production of reliable speech-processing hardware, design of cosmetically and ergonometrically acceptable electrode transducers, implementation of acute and chronic biomedical studies demonstrating device safety, design and testing of alternative speech-encoding strategies to provide benefit to speech perception and production, and design and testing of appropriate training methods for optimizing benefits. The Tickle Talker has been shown to provide benefits in supplementing lipreading or aided residual hearing for hearing-impaired adults and children. Improvements in speech processing have resulted in an increase in benefits to speech perception, and open the way for more flexible approaches to encoding speech input. Continuing development of the electrode circuitry has now produced a device that is robust and has an extended battery life. Safety studies have clearly demonstrated that there are no long-term contraindications to device use. The results suggest that the device has a role to play in rehabilitation programs for severely and profoundly hearing-impaired adults and children.

Articulation accuracy of children using an electrotactile speech processor.

OBJECTIVE: Use of wearable tactile speech perception devices is suggested to help overcome the difficulties in speech production resulting from severe and profound hearing impairment in children. This suggestion is based on the assumption that subjects can use tactile input in isolation, or in combination with information from residual aided hearing, to monitor and modify their speech. The present study evaluated the benefits to articulation provided through use of a multichannel electrotactile device ("Tickle Talker"). DESIGN: Six profoundly hearing-impaired children were videotaped speaking with the Tickle Talker on and with the Tickle Talker off during conversations with their audiologist. Five of the subjects also wore their binaural hearing aids during all recorded conversations. The number of vowels, consonants, and overall phonemes correctly articulated by each child in the two conditions were compared. RESULTS: One subject improved articulation of initial consonants and initial phonemes; one subject improved articulation of total vowels, total consonants, initial consonants, total phonemes, and initial phonemes; and a third subject improved articulation of total vowels and medial phonemes. CONCLUSIONS: Use of on-line tactile feedback from the Tickle Talker may benefit the articulation accuracy of some children, and the device may therefore be suitable to use with children who have not responded to more traditional speech training techniques.

Cochlear implants for congenitally deaf adolescents: is open-set speech perception a realistic expectation?

The prognosis for benefit from use of cochlear implants in congenitally deaf adolescents, who have a long duration of profound deafness prior to implantation, has typically been low. Speech perception results for two congenitally deaf patients implanted as adolescents at the University of Melbourne/Royal Victorian Eye and Ear Hospital Clinic show that, after 12 months of experience, both patients had significant open-set speech discrimination scores without lipreading. These results suggest that although benefits may in general be low for congenitally deaf adolescents, individuals may attain significant benefits to speech perception after a short period of experience. Prospective patients from this group should therefore be considered on an individual basis with regard to prognosis for benefit from cochlear implantation.

Factors in the development of a training program for use with tactile devices.

A review of the literature suggests that, in order to maximize the benefits available through a tactile device, it must be accompanied by an effective and adaptive training program. There are a number of factors to consider in the design of such a training program, including the type of tasks and response formats to include, the amount of training, subject motivation and device use, the characteristics of the potential user population, the specific device to be used and the type of information it provides, and the evaluation procedures to be followed. The type and saliency of the information provided by a particular tactile device are highlighted as the most important yet neglected consideration in designing a training program. The training program used with the University of Melbourne's multiple-channel electrotactile device is presented to show how these important factors may be addressed, to indicate the flexibility required in a training program, and to provide a general framework on which researchers may base the development of programs for other tactile devices.

The effect of handedness in tactile speech perception.

This study examined differential performance of normally hearing subjects using a tactile device on the dominant versus non-dominant hand. The study evaluated whether tactual sensitivity for non-speech stimuli was greater for the dominant hand as compared with the non-dominant hand, and secondly, whether there was an advantage for speech presented tactually to the dominant hand, resulting from a preferential pathway to the language processing area in the left cerebral hemisphere. Evaluations of threshold pulse width, dynamic ranges, paired electrode identification, and a closed-set tactual pattern discrimination test battery showed no difference in tactual sensitivity measures between the two hands. Speech perception was assessed with closed sets of vowels and consonants and with open-set Harvey Gardner (HG) words and Arthur Boothroyd (AB) words. Group mean scores were higher in each of the tactually aided conditions as compared with the unaided conditions for speech tests, with the exception of AB words in the tactile plus lip-reading plus audition/lip-reading plus audition condition on the right hand. Overall mean scores on the closed-set vowel test and on open-set HG and AB words were significantly higher for the tactually aided condition as compared with the unaided condition. Comparison of performance between the dominant and non-dominant hand showed a significant advantage for the dominant hand on the closed-set vowel test only. No significant differences between hands in either tactually aided or unaided conditions were evident for any of the other speech perception tests. Factors influencing this result could have been variations in degree of difficulty of the tests, the amount of training subjects received, or the training strategy employed. Although an advantage to presenting speech through the dominant hand may exist, it is unlikely to be great enough to outweigh possible restrictions on everyday use.

Use of a multichannel electrotactile speech processor by profoundly hearing-impaired children in a total communication environment.

As part of a larger subject group, four profoundly hearing-impaired children enrolled in a total communication educational program were fitted with the University of Melbourne's multichannel electrotactile speech processor (Tickle Talker). Sound detection thresholds for pure tones were at lower levels with the tactile device than with hearing aids, especially for high frequency sounds above 2 kHz. Two of the children also detected all speech sounds of the Ling five-sound test at normal conversational levels using only the Tickle Talker. The children were able to use tactile input to achieve higher scores on three speech feature subtests of the PLOTT test when using the Tickle Talker plus hearing aids as compared to hearing aids alone. Mean improvements were 22.4 percent on vowel length, 28 percent on vowel identification, and 35 percent on consonant manner. Improvements were also shown by individual children on the closed-set WIPI and open-set PBK word tests, and on the open-set BKB sentence test, when the Tickle Talker was combined with hearing aids, and with hearing aids and lipreading. Comparisons of these results with those of children using the Tickle Talker in other educational settings show that children in a total communication environment can potentially benefit to a similar degree from use of tactual input. Anecdotal reports from the children and school staff members indicated that daily use of the Tickle Talker did not interfere with the signing aspects of total communication.

Role of a multichannel electrotactile speech processor in a cochlear implant program for profoundly hearing-impaired adults.

Four profoundly hearing-impaired adults who did not meet current selection criteria for implantation at the University of Melbourne were each fitted with a wearable multichannel electrotactile speech processor (Tickle Talker). The subjects were evaluated with a test battery of speech discrimination tests subsequent to training in use of the device. Thresholds for detection of pure tones were lower for the Tickle Talker than for hearing aids across the frequency range 250 to 4000 Hz. Mean speech detection thresholds for the Ling 5-sound test showed that all sounds were detected by users of the electrotactile device at normal conversational speech intensity levels. Mean speech discrimination scores were significantly higher (p less than 0.05) in the tactually aided condition as compared with the tactually unaided for identification of vowels and consonants, on open-set words, open-set sentences, and on connected discourse tracking. Mean scores increased by 20% for vowels, 19% for consonants, 30% for open-set words, and 25% for open-set sentences when the Tickle Talker was used in a multimodal combination with lipreading or lipreading and hearing aids. Speechtracking rates for three subjects showed increases of from 18 to 28 wpm when the tactile device was used. Comparison of tactually aided versus unaided tracking rates for two subjects with long-term experience shows continuing improvement with additional experience with the device. These results demonstrate that hearing-impaired adults not meeting selection criteria for cochlear implantation may benefit from use of an electrotactile speech processor, and highlight the potential benefits from integration of such devices into cochlear implant programs for profoundly hearing-impaired patients.

Perception of multiple electrode stimulus patterns: implications for design of an electrotactile speech processor.

Identification of multiple-electrode stimulus patterns was evaluated in nine adult subjects, to assess the feasibility of providing additional speech information through the tactual display of an electrotactile speech processor. Absolute identification scores decreased from 97.8% for single electrodes, to 61.9% for electrode pairs, and, to 31.8% for electrode triplets. Although input information increased with paired-and triple-electrode stimuli, information transmission scores were not significantly increased for either electrode pairs (2.99 bits) or triplets (2.84 bits) as compared with single electrodes (2.84 bits). These results suggest that speech coding strategies using stimulus patterns of electrode pairs or triplets would provide little improvement beyond that found for the present single-electrode scheme. However, higher absolute identification scores (73.6%), and an increase in information transmission to 3.88 bits, were recorded for test stimuli containing all combinations of paired and single electrodes. Based on this finding, two stimulus sets using a restricted number of combinations of paired and single electrodes were evaluated. The two stimulus sets simulated the spatial patterns of paired and single electrodes arising from use of alternative speech coding schemes to increase consonant voicing information. Results for the two stimulus sets showed higher electrode identification scores (79.7% and 90.4%), as compared with paired-electrode stimuli. Although electrode identification score was not as high as for single electrodes, information transmission was increased to 3.31 bits for the VF2 stimulus set. Analysis of the responses also showed that scores for identification of simulated voicing information conveyed by the two stimulus sets were 99.4 and 90.4% correct.(ABSTRACT TRUNCATED AT 250 WORDS)

Perception of amplitude envelope variations of pulsatile electrotactile stimuli.

Gross variations of the speech amplitude envelope, such as the duration of different segments and the gaps between them, carry information about prosody and some segmental features of vowels and consonants. The amplitude envelope is one parameter encoded by the Tickle Talker, an electrotactile speech processor for the hearing impaired which stimulates the digital nerve bundles with a pulsatile electric current. Psychophysical experiments measuring the duration discrimination and identification, gap detection, and integration times for pulsatile electrical stimulation are described and compared with similar auditory measures for normal and impaired hearing and electrical stimulation via a cochlear implant. The tactile duration limen of 15% for a 300-ms standard was similar to auditory measures. Tactile gap detection thresholds of 9 to 20 ms were larger than for normal-hearing but shorter than for some hearing-impaired listeners and cochlear implant users. The electrotactile integration time of about 250 ms was shorter than previously measured tactile values but longer than auditory integration times. The results indicate that the gross amplitude envelope variations should be conveyed well by the Tickle Talker. Short bursts of low amplitude are the features most likely to be poorly perceived.

Perception of sentences, words, and speech features by profoundly hearing-impaired children using a multichannel electrotactile speech processor.

Fourteen prelinguistically profoundly hearing-impaired children were fitted with the multichannel electrotactile speech processor (Tickle Talker) developed by Cochlear Pty. Ltd. and the University of Melbourne. Each child participated in an ongoing training and evaluation program, which included measures of speech perception and production. Results of speech perception testing demonstrate clear benefits for children fitted with the device. Thresholds for detection of pure tones were lower for the Tickle Talker than for hearing aids across the frequency range 250-4000 Hz, with the greatest tactual advantage in the high-frequency consonant range (above 2000 Hz). Individual and mean speech detection thresholds for the Ling 5-sound test confirmed that speech sounds were detected by the electrotactile device at levels consistent with normal conversational speech. Results for three speech feature tests showed significant improvement when the Tickle Talker was used in combination with hearing aids (TA) as compared with hearing aids along (A). Mean scores in the TA condition increased by 11% for vowel duration, 20% for vowel formant, and 25% for consonant manner as compared with hearing aids alone. Mean TA score on a closed-set word test (WIPI) was 48%, as compared with 32% for hearing aids alone. Similarly, mean WIPI score for the combination of Tickle Talker, lipreading, and hearing aids (TLA) increased by 6% as compared with combined lipreading and hearing aid (LA) scores. Mean scores on open-set sentences (BKB) showed a significant increase of 21% for the tactually aided condition (TLA) as compared with unaided (LA). These results indicate that, given sufficient training, children can utilize speech feature information provided through the Tickle Talker to improve discrimination of words and sentences. These results indicate that, given sufficient training, children can utilize speech feature information provided through the Tickle Talker to improve discrimination of words and sentences. These results are consistent with improvement in speech discrimination previously reported for normally hearing and hearing-impaired adults using the device. Anecdotal evidence also indicates some improvements in speech production for children fitted with the Tickle Talker.