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Background and Objectives: A rare type of nonsyndromic autosomal recessive hereditary hearing loss is caused by pathogenic mutations in the TRIOBP gene mostly involving exons 6 and 7. These mutations cause hearing loss originating from dysfunction of sensory inner ear hair cells. Of all the affected siblings, 2 brothers and 1 sister, part of an Afghan family, were referred to our clinic for diagnostic workup and candidacy selection for cochlear implantation (CI).Methods: Molecular analysis showed a homozygous c.1342C > T p. (Arg448*) pathogenic variant in exon 7 of the TRIOBP gene (reference sequence NM_001039141.2) in all 3 affected siblings. Clinical audiometry demonstrated profound sensorineural hearing loss in all 3 affected siblings (2 males and 1 female), and they were implanted unilaterally.Results: One month after activation, the pure-tone averages with the CI processor were between 30 and 23 dBHL. Ten months after the first activation of the implant, open-set speech audiometry test could be performed for the first time in the 2 younger CI recipients (S5 and S9), and they could identify up to a maximum 77% phonemes correctly. The oldest brother (S12) could not yet perform open-set speech audiometry at that moment.Conclusions: Implant outcomes are better with normal inner ear anatomy in general. The earlier congenital patients are implanted, the better their outcomes. Here, we demonstrate both statements are true in a homozygous c.1342C > T p. (Arg448*) pathogenic variant in the TRIOBP gene in all 3 affected siblings. [ABSTRACT FROM AUTHOR]

Purpose: To overcome the potential tension between clinical and ecological validity in speech audiometric assessment by creating a new set of sentence materials with high linguistic validity for the Dutch-speaking area. Methods: A linguistic “fingerprint” of modern spoken Dutch and Flemish served to generate a set of sentences recorded from 1 male and 1 female talker. The sentences were presented to 30 normal-hearing listeners in stationary speech noise at a signal-to-noise ratio (SNR) of –5 dB sound pressure level (SPL). A list design criterion was used to achieve perceptive homogeneity across the test lists, by scrambling lists of sentences of different syntactic types while controlling for linguistic complexity. The original set of test materials was narrowed down to 360 sentences, and list equivalency was evaluated at the audiological and linguistic levels. A psychometric curve was generated with a resolution of 2 dB based on a second group of 60 young normal-hearing native speakers of Dutch and Flemish. Results: Sentence understanding showed an average repetition accuracy of 63.40% (SD 1.01) across the lists at an SNR of –5 dB SPL. No significant differences were found between the lists at the level of the individual listener. At the linguistic level, the sentence lists showed an equal distribution of phonological, morphological, and syntactic features. Conclusion: LiCoS combines the clinical benefit of acoustic control at the list level with the high ecological validity of linguistically representative test items. The new speech audiometric test is particularly appropriate to assess sentence understanding in individuals who would otherwise exhibit near-ceiling performance when tested with linguistically more simplified test stimuli. In combination with pure tone audiometric assessment, LiCoS provides valuable complementary information with respect to the functional hearing of patients. [ABSTRACT FROM AUTHOR]

Objectives: Cochlear's new sound processor system (Nucleus(®) 6) features a new noise reduction algorithm called SNR-NR (signal-to-noise ratio), and an environmental classifier called SCAN, which activates the appropriate sound coding algorithms for a given listening environment. In addition, the sound processors (CP910 and CP920) have a data logging feature with data visually summarized using clinical programing software and come with two remote controls, CR210 and CR230. The objective of this clinical study was to conduct a field acceptance study comparing the user experience with the Nucleus(®) 6 to the Nucleus(®) 5 system and to evaluate the benefits of Nucleus(®) 6 in an adult population currently equipped with the previous generation Nucleus(®) 5 sound processor. Our primary objective was to compare speech recognition in speech-weighted noise using Nucleus(®) 6 with SCAN (activating SNR-NR) with the default Nucleus(®) 5 'Noise' Program. Secondary objectives included comparisons of speech recognition in quiet, subjective performance feedback via questionnaires and diaries, and recipient preference for device and program type.Methods: A prospective controlled trial was conducted with 30 adult Nucleus CI recipients using the Nucleus(®) 5 sound processor (condition A). The Nucleus(®) 6 sound processor (condition B) was evaluated in a within-subject ABBA design, with repeated speech in noise (S0N0, LIST sentence test), and speech in quiet testing (S0, NVA words). The remote controls were randomly given during the two B intervals. In addition, recipients had to complete questionnaires and diaries on the use of their current as well as new sound processors and remotes.Results: The group mean speech reception threshold in noise (SRT50) with Nucleus(®) 6 SCAN was significantly better (1.2 dB SNR) than with the Nucleus(®) 5 'Noise' Program. Mean speech recognition scores in quiet were not significantly different between the processors. Subjective performance feedback (APHAB) did not show a significant difference between Nucleus(®) 6 and Nucleus(®) 5 with high satisfaction scores being reported for both sound processors. Recipients preferred the SCAN program in noise and reported a clear overall preference for the Nucleus(®) 6 system. Clinicians were satisfied with the conversion process from Nucleus(®) 5 to Nucleus(®) 6.Discussion and Conclusion: SNR-NR provides a significant benefit in noise. Recipients were easily converted from Nucleus(®) 5 to Nucleus(®) 6 requiring little or no sound quality adjustment period. The Nucleus(®) 6 SCAN program was well accepted by the majority of recipients for use during their daily life. [ABSTRACT FROM AUTHOR]

Objectives Cochlear's new sound processor system (Nucleus® 6) features a new noise reduction algorithm called SNR-NR (signal-to-noise ratio), and an environmental classifier called SCAN, which activates the appropriate sound coding algorithms for a given listening environment. In addition, the sound processors (CP910 and CP920) have a data logging feature with data visually summarized using clinical programing software and come with two remote controls, CR210 and CR230. The objective of this clinical study was to conduct a field acceptance study comparing the user experience with the Nucleus® 6 to the Nucleus® 5 system and to evaluate the benefits of Nucleus® 6 in an adult population currently equipped with the previous generation Nucleus® 5 sound processor. Our primary objective was to compare speech recognition in speech-weighted noise using Nucleus® 6 with SCAN (activating SNR-NR) with the default Nucleus® 5 'Noise' Program. Secondary objectives included comparisons of speech recognition in quiet, subjective performance feedback via questionnaires and diaries, and recipient preference for device and program type. Methods A prospective controlled trial was conducted with 30 adult Nucleus CI recipients using the Nucleus® 5 sound processor (condition A). The Nucleus® 6 sound processor (condition B) was evaluated in a within-subject ABBA design, with repeated speech in noise (S0N0, LIST sentence test), and speech in quiet testing (S0, NVA words). The remote controls were randomly given during the two B intervals. In addition, recipients had to complete questionnaires and diaries on the use of their current as well as new sound processors and remotes. Results The group mean speech reception threshold in noise (SRT50) with Nucleus® 6 SCAN was significantly better (1.2 dB SNR) than with the Nucleus® 5 'Noise' Program. Mean speech recognition scores in quiet were not significantly different between the processors. Subjective performance feedback (APHAB) did not show a significant difference between Nucleus® 6 and Nucleus® 5 with high satisfaction scores being reported for both sound processors. Recipients preferred the SCAN program in noise and reported a clear overall preference for the Nucleus® 6 system. Clinicians were satisfied with the conversion process from Nucleus® 5 to Nucleus® 6. Discussion and conclusion SNR-NR provides a significant benefit in noise. Recipients were easily converted from Nucleus® 5 to Nucleus® 6 requiring little or no sound quality adjustment period. The Nucleus® 6 SCAN program was well accepted by the majority of recipients for use during their daily life. [ABSTRACT FROM AUTHOR]

Objectives To evaluate the possible impact of 'Fitting to Outcomes eXpert (FOX®)' on cochlear implant (CI) fitting in a clinic with extensive experience of fitting a range of CI systems, as a way to assess whether a software tool such as FOX is able to complement standard clinical procedures. Methods Ten adult post-lingually deafened and unilateral long-term users of the Advanced BionicsTM CI system (Clarion CII or HiRes 90KTM) underwent speech perception assessment with their current clinical program. One cycle 'iteration' of FOX optimization was performed and the program adjusted accordingly. After a month of using both clinical and FOX programs, a second iteration of FOX optimization was performed. Following this, the assessments were repeated without further acclimatization. Results FOX prescribed programming modifications in all subjects. Soundfield-aided thresholds were significantly lower for FOX than the clinical program. Group speech scores in noise were not significantly different between the two programs but three individual subjects had improved speech scores with the FOX MAP, two had worse speech scores, and five were the same. Conclusion FOX provided a standardized approach to fitting based on outcome measures rather than comfort alone. The results indicated that for this group of well-fitted patients, FOX improved outcomes in some individuals. There were significant changes, both better and worse, in individual speech perception scores but median scores remained unchanged. Soundfield-aided thresholds were significantly improved for the FOX group. [ABSTRACT FROM AUTHOR]

Objectives To evaluate the possible impact of 'Fitting to Outcomes eXpert (FOX((R)))' on cochlear implant (CI) fitting in a clinic with extensive experience of fitting a range of CI systems, as a way to assess whether a software tool such as FOX is able to complement standard clinical procedures. Methods Ten adult post-lingually deafened and unilateral long-term users of the Advanced Bionics(TM) CI system (Clarion CII or HiRes 90K(TM)) underwent speech perception assessment with their current clinical program. One cycle 'iteration' of FOX optimization was performed and the program adjusted accordingly. After a month of using both clinical and FOX programs, a second iteration of FOX optimization was performed. Following this, the assessments were repeated without further acclimatization. Results FOX prescribed programming modifications in all subjects. Soundfield-aided thresholds were significantly lower for FOX than the clinical program. Group speech scores in noise were not significantly different between the two programs but three individual subjects had improved speech scores with the FOX MAP, two had worse speech scores, and five were the same. Conclusion FOX provided a standardized approach to fitting based on outcome measures rather than comfort alone. The results indicated that for this group of well-fitted patients, FOX improved outcomes in some individuals. There were significant changes, both better and worse, in individual speech perception scores but median scores remained unchanged. Soundfield-aided thresholds were significantly improved for the FOX group. [ABSTRACT FROM AUTHOR]

Objective To assess the auditory performance of Digisonic((R)) cochlear implant users with electric stimulation (ES) and electro-acoustic stimulation (EAS) with special attention to the processing of low-frequency temporal fine structure. Method Six patients implanted with a Digisonic((R)) SP implant and showing low-frequency residual hearing were fitted with the Zebra((R)) speech processor providing both electric and acoustic stimulation. Assessment consisted of monosyllabic speech identification tests in quiet and in noise at different presentation levels, and a pitch discrimination task using harmonic and disharmonic intonating complex sounds ( Vaerenberg et al., 2011 ). These tests investigate place and time coding through pitch discrimination. All tasks were performed with ES only and with EAS. Results Speech results in noise showed significant improvement with EAS when compared to ES. Whereas EAS did not yield better results in the harmonic intonation test, the improvements in the disharmonic intonation test were remarkable, suggesting better coding of pitch cues requiring phase locking. Discussion These results suggest that patients with residual hearing in the low-frequency range still have good phase-locking capacities, allowing them to process fine temporal information. ES relies mainly on place coding but provides poor low-frequency temporal coding, whereas EAS also provides temporal coding in the low-frequency range. Patients with residual phase-locking capacities can make use of these cues. [ABSTRACT FROM AUTHOR]

Objective To assess the auditory performance of Digisonic® cochlear implant users with electric stimulation (ES) and electro-acoustic stimulation (EAS) with special attention to the processing of low-frequency temporal fine structure. Method Six patients implanted with a Digisonic® SP implant and showing low-frequency residual hearing were fitted with the Zebra® speech processor providing both electric and acoustic stimulation. Assessment consisted of monosyllabic speech identification tests in quiet and in noise at different presentation levels, and a pitch discrimination task using harmonic and disharmonic intonating complex sounds ( Vaerenberg et al., 2011). These tests investigate place and time coding through pitch discrimination. All tasks were performed with ES only and with EAS. Results Speech results in noise showed significant improvement with EAS when compared to ES. Whereas EAS did not yield better results in the harmonic intonation test, the improvements in the disharmonic intonation test were remarkable, suggesting better coding of pitch cues requiring phase locking. Discussion These results suggest that patients with residual hearing in the low-frequency range still have good phase-locking capacities, allowing them to process fine temporal information. ES relies mainly on place coding but provides poor low-frequency temporal coding, whereas EAS also provides temporal coding in the low-frequency range. Patients with residual phase-locking capacities can make use of these cues. [ABSTRACT FROM AUTHOR]

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The present paper reports on the implementation of a maternity based neonatal hearing-screening program in a private hospital. A retrospective analysis is performed on the test pass rate, the coverage and the number of children that become lost to follow-up. The data show a steady learning curve with a time course of several years. In the current screening practice, the test pass rate is at 99.0%, the coverage is at 96% (birth rate of 2000 per annum) and almost no babies get lost to follow-up. [ABSTRACT FROM AUTHOR]

Objective: The paper aims to demonstrate the feasibility of defining a substantial set of psychoacoustic outcome measures with preset targets and to adopt a systematic methodology for reaching these targets in a large group of subjects, by more than one clinical centre., Design: Retrospective data analysis., Setting: Multicentre with 14 participating centres., Patients: 255 adults and children using the Advanced Bionics HiRes90k cochlear implant., Intervention: Target driven fitting with the fitting to outcomes expert (FOX) system., Main Outcome Measures: For each patient, 66 measurable psychoacoustical outcomes were recorded several times after cochlear implantation: free field audiometry (6 measures) and speech audiometry (4), spectral discrimination (20), and loudness growth (36), defined from the A§E test battery. These outcomes were reduced to 22 summary variables. The initial results were compared with the latest results., Results: The state of the fitting process could be well monitored by means of the measured variables. The use of the FOX computer assisted CI-programming significantly improved the proportion of the 22 variables on target. When recipients used the automated MAPs provided at switch-on, more than half (57%) of the 22 targets were already achieved before any further optimisation took place. Once the FOX system was applied there was a significant 24% (P < 0.001) increase in the number of targets achieved., Conclusions: This study demonstrates that it is feasible to set targets and to report on the effectiveness of a fitting strategy in terms of these targets. FOX provides an effective tool for achieving a systematic approach to programming, allowing for better optimisation of recipients' MAPs. The setting of well-defined outcome targets allowed a range of different centres to successfully apply a systematic methodology to monitoring the quality of the programming provided.

The programming of CIs is essential for good performance. However, no Good Clinical Practice guidelines exist. This paper reports on the results of an inventory of the current practice worldwide. A questionnaire was distributed to 47 CI centers. They follow 47600 recipients in 17 countries and 5 continents. The results were discussed during a debate. Sixty-two percent of the results were verified through individual interviews during the following months. Most centers (72%) participated in a cross-sectional study logging 5 consecutive fitting sessions in 5 different recipients. Data indicate that general practice starts with a single switch-on session, followed by three monthly sessions, three quarterly sessions, and then annual sessions, all containing one hour of programming and testing. The main focus lies on setting maximum and, to a lesser extent, minimum current levels per electrode. These levels are often determined on a few electrodes and then extrapolated. They are mainly based on subjective loudness perception by the CI user and, to a lesser extent, on pure tone and speech audiometry. Objective measures play a small role as indication of the global MAP profile. Other MAP parameters are rarely modified. Measurable targets are only defined for pure tone audiometry. Huge variation exists between centers on all aspects of the fitting practice.