Long-Term Outcomes of Cochlear Implantation in Usher Syndrome.

OBJECTIVES: Usher syndrome (USH), characterized by bilateral sensorineural hearing loss (SNHL) and retinitis pigmentosa (RP), prompts increased reliance on hearing due to progressive visual deterioration. It can be categorized into three subtypes: USH type 1 (USH1), characterized by severe to profound congenital SNHL, childhood-onset RP, and vestibular areflexia; USH type 2 (USH2), presenting with moderate to severe progressive SNHL and RP onset in the second decade, with or without vestibular dysfunction; and USH type 3 (USH3), featuring variable progressive SNHL beginning in childhood, variable RP onset, and diverse vestibular function. Previous studies evaluating cochlear implant (CI) outcomes in individuals with USH used varying or short follow-up durations, while others did not evaluate outcomes for each subtype separately. This study evaluates long-term CI performance in subjects with USH, at both short-term and long-term, considering each subtype separately. DESIGN: This retrospective, observational cohort study identified 36 CI recipients (53 ears) who were categorized into four different groups: early-implanted USH1 (first CI at ≤7 years of age), late-implanted USH1 (first CI at ≥8 years of age), USH2 and USH3. Phoneme scores at 65 dB SPL with CI were evaluated at 1 year, ≥2 years (mid-term), and ≥5 years postimplantation (long-term). Each subtype was analyzed separately due to the significant variability in phenotype observed among the three subtypes. RESULTS: Early-implanted USH1-subjects (N = 23 ears) achieved excellent long-term phoneme scores (100% [interquartile ranges {IQR} = 95 to 100]), with younger age at implantation significantly correlating with better CI outcomes. Simultaneously implanted subjects had significantly better outcomes than sequentially implanted subjects (p = 0.028). Late-implanted USH1 subjects (N = 3 ears) used CI solely for sound detection and showed a mean phoneme discrimination score of 12% (IQR = 0 to 12), while still expressing satisfaction with ambient sound detection. In the USH2 group (N = 23 ears), a long-term mean phoneme score of 85% (IQR = 81 to 95) was found. Better outcomes were associated with younger age at implantation and higher preimplantation speech perception scores. USH3-subjects (N = 7 ears) achieved a mean postimplantation phoneme score of 71% (IQR = 45 to 91). CONCLUSIONS: This study is currently one of the largest and most comprehensive studies evaluating CI outcomes in individuals with USH, demonstrating that overall, individuals with USH benefit from CI at both short- and long-term follow-up. Due to the considerable variability in phenotype observed among the three subtypes, each subtype was analyzed separately, resulting in smaller sample sizes. For USH1 subjects, optimal CI outcomes are expected with early simultaneous bilateral implantation. Late implantation in USH1 provides signaling function, but achieved speech recognition is insufficient for oral communication. In USH2 and USH3, favorable CI outcomes are expected, especially if individuals exhibit sufficient speech recognition with hearing aids and receive ample auditory stimulation preimplantation. Early implantation is recommended for USH2, given the progressive nature of hearing loss and concomitant severe visual impairment. In comparison with USH2, predicting outcomes in USH3 remains challenging due to the variability found. Counseling for USH2 and USH3 should highlight early implantation benefits and encourage hearing aid use.

Patient-Led Research to Develop a Training Programme for Restoring Musical Joy in Cochlear Implant Recipients: A Reflexive Process Evaluation.

BACKGROUND: The role of patients in healthcare research is slowly evolving, although patient roles in the research process are limited. This paper reports on a patient-led research project aiming to develop a musical hearing training programme for patients with a cochlear implant (CI): the Musi-CI programme. A CI is an inner ear prosthesis that allows people with severe hearing loss to hear. However, while speech can be understood, CI users cannot fully enjoy music or feel aversion to it. The Musi-CI programme aims to reduce this music aversion to ultimately improve music enjoyment and social participation. The development of the Musi-CI programme was supported by a consortium of professionals in CI rehabilitation and research. The aim of this paper is to describe and evaluate the Musi-CI programme development process and its impact on professional CI rehabilitation and research. METHODS: Programme development was described using a 3-layered process model of action research, distinguishing the CI user process, the healthcare professional process and the research process. To evaluate perceptions on the programme development process, consortium partners provided written comments and participated in a reflexive evaluation session that was video-recorded. Reflexive evaluation aims for collective learning and strengthening collaboration among participants. Written comments and video data were analysed using template analysis. RESULTS: The involvement of an expert by experience was perceived as challenging but rewarding for all consortium partners, opening up new perspectives on CI-rehabilitation practice and research. Data analysis revealed two themes on the programme development process, professional space and acknowledgement, and two themes on the outcomes on CI rehabilitation and research: critical reflection and paradigm shift. CONCLUSION: Experts by experience represent a different knowledge domain that may contribute to change in rehabilitation and research. PATIENT OR PUBLIC CONTRIBUTION: The development of the programme was initiated by a professional musician and CI user who organized the funding, had a leading role throughout the research process, including the write-up of the results, and co-authored this paper.

Remote Cochlear Implant Assessments: Validity and Stability in Self-Administered Smartphone-Based Testing.

OBJECTIVES: The stability of remote testing in cochlear implant care was studied by testing the influence of time-of-day, listener fatigue, and motivation on the outcomes of the aided threshold test (ATT) and digit triplets test (DTT) in cochlear implant (CI) recipients using self-tests at-home on a smartphone or tablet. DESIGN: A single-center repeated measures cohort study design (n = 50 adult CI recipients). The ATT and DTT were tested at-home ten times, with nine of these sessions planned within a period of eight days. Outcomes were modeled as a function of time-of-day, momentary motivation, listeners' task-related fatigue, and chronotype (i.e., someone's preference for morning or evening due to the sleep-wake cycle) using linear mixed models. Additional factors included aided monosyllabic word recognition in quiet, daily-life fatigue, age, and CI experience. RESULTS: Out of 500 planned measurements, 407 ATTs and 476 DTTs were completed. The ATT determined thresholds and impedances were stable across sessions. The factors in the DTT model explained 75% of the total variance. Forty-nine percent of the total variance was explained by individual differences in the participants' DTT performance. For each 10% increase in word recognition in quiet, the DTT speech reception threshold improved by an average of 1.6 dB. DTT speech reception threshold improved, on average by 0.1 dB per repeated session and correlated with the number of successful DTTs per participant. There was no significant time-of-day effect on auditory performance in at-home administered tests. CONCLUSIONS: This study is one of the first to report on the validity and stability of remote assessments in CI recipients and reveals relevant factors. CI recipients can be self-tested at any waking hour to monitor performance via smartphone or tablet. Motivation, task-related fatigue, and chronotype did not affect the outcomes of ATT or DTT in the studied cohort. Word recognition in quiet is a good predictor for deciding whether the DTT should be included in an individual's remote test battery. At-home testing is reliable for cochlear implant recipients and offers an opportunity to provide care in a virtual hearing clinic setting.

Human Histology after Structure Preservation Cochlear Implantation via Round Window Insertion.

OBJECTIVES: Current surgical techniques aim to preserve intracochlear structures during cochlear implant (CI) insertion to maintain residual cochlear function. The optimal technique to minimize damage, however, is still under debate. The aim of this study is to histologically compare insertional trauma and intracochlear tissue formation in humans with a CI implanted via different insertion techniques. METHODS: One recent temporal bone from a donor who underwent implantation of a full-length CI (576°) via round window (RW) insertion was compared with nine cases implanted via cochleostomy (CO) or extended round window (ERW) approach. Insertional trauma was assessed on H&E-stained histological sections. 3D reconstructions were generated and virtually re-sectioned to measure intracochlear volumes of fibrosis and neo-ossification. RESULTS: The RW insertion case showed electrode translocation via the spiral ligament. 2/9 CO/ERW cases showed no insertional trauma. The total volume of the cochlea occupied by fibro-osseous tissue was 10.8% in the RW case compared with a mean of 30.6% (range 8.7%-44.8%, N = 9) in the CO/ERW cases. The difference in tissue formation in the basal 5 mm of scala tympani, however, was even more pronounced when the RW case (12.3%) was compared with the cases with a CO/ERW approach (mean of 93.8%, range 81% to 100%, N = 9). CONCLUSIONS: Full-length CI insertions via the RW can be minimally traumatic at the cochlear base without inducing extensive fibro-osseous tissue formation locally. The current study further supports the hypothesis that drilling of the cochleostomy with damage to the endosteum incites a local tissue reaction. LEVEL OF EVIDENCE: 4: Case-control study Laryngoscope, 134:945-953, 2024.

The relation between cochlear implant programming levels and speech perception performance in post-lingually deafened adults: a data-driven approach.

PURPOSE: Programming a cochlear implant (fitting) is an essential part of a user's post-implantation journey, defining how sound will be translated into electrical stimulation and aiming to provide optimal speech perception outcomes. Currently, there are no established, evidence-based guidelines for fitting cochlear implant users, leading to a high degree of variability in fitting practices, users' parameters, and probably outcomes. In this study a data-driven approach is used to retrospectively investigate the relation between cochlear implant fitting parameters and speech perception outcomes in post-lingually deafened adults. METHODS: 298 data points corresponding to fitting parameters and speech audiometry test results for the same number of adult, post-lingually deafened, experienced CI users were analyzed. Correlation analysis was performed, after which parameters from the top-scoring and bottom-scoring tertiles were compared via the Mann-Whitney-Wilcoxon u test. RESULTS: Weak correlations between dynamic range and speech audiometry outcomes were identified, having p values lower than (albeit close to) 0.05. A significant (p < 0.05) difference in electrical dynamic range (the difference between the minimum and maximum amount of current which may be delivered by each electrode) was found, with top-scoring subjects having on average a wider dynamic range. CONCLUSION: The association between dynamic range and speech perception outcomes shown in this retrospective study highlights the need for deeper investigation into evidence-driven fitting. It might be a first step in the direction of evidence-based fitting, minimizing variability in outcomes for cochlear implant users and helping mitigate the issue of unexplained low performance.

Phoneme Training for Adult Cochlear Implant Users: A Review of the Literature and Study Protocol.

PURPOSE: This study describes a protocol for a novel individualized phoneme training program for adult cochlear implant (CI) users, based on individual phoneme confusion errors. The protocol is underpinned by a literature review on phoneme training and a focus group with adult CI users. METHOD: After a literature search, five studies were included for review and evaluation of quality and level of evidence. A focus group with experienced adult CI users (n = 7) was then conducted to gain insights into their experiences of auditory training post-implantation and recommendations for future training programs. The knowledge gained from the literature review and focus group was used as the foundation for a novel, individualized phoneme training program for adult CI users, for which the protocol is described in this study. RESULTS: A review of the literature shows that phoneme training in adult CI users has variable outcomes for on-task and off-task measures. Overall, the concept of individualized training relates to adaptive difficulty within training tasks and not to tailoring training content to participants' individual needs, as indicated by clinical outcomes. The focus group revealed that participants want to be able to track their training progress, have training content tailored to their individual needs, and expressed a preference for shorter training sessions. CONCLUSIONS: Using learnings from a literature review and focus group, this study describes a protocol for a novel, individualized phoneme training program for adult CI users. Study findings from this phoneme training program will be disseminated when available. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.24392863.

Variability in clinicians' prediction accuracy for outcomes of adult cochlear implant users.

OBJECTIVE: The variability in outcomes among adult cochlear implant (CI) users poses challenges for clinicians in accurately predicting the benefits of the implant for individual candidates. This study aimed to investigate the accuracy and confidence of clinicians in predicting speech perception outcomes for adult CI users one-year post-implantation. DESIGN: Participants were presented with comprehensive information on pre-implantation, one-month post-implantation, and six-month post-implantation data for 10 case studies. The cases encompassed a range of one-year post-implantation phoneme scores, from low performers (27%) to high performers (92%). Participants were tasked with predicting the speech perception outcomes for these cases one year after implantation. STUDY SAMPLE: Forty-one clinicians completed the full outcome prediction survey. RESULTS: Our findings revealed a significant over-prediction of low performance by clinicians. Interestingly, clinicians tended to predict average performance (73-76% phoneme score) even when provided with information suggesting lower-than-average performance. Most clinicians expressed confidence in their predictions, irrespective of their accuracy. CONCLUSIONS: Identifying signs of low performance, particularly in the early post-implantation period, can enable clinicians to implement early interventions. Further research into accurate outcome prediction is essential for managing expectations, providing counselling, increasing CI adoption, and optimising clinical care for both high and low performers.

Development of the Musi-CI Training, A Musical Listening Training for Cochlear Implant Users: A Participatory Action Research Approach.

A cochlear implant (CI) is a prosthesis that allows people with severe to profound hearing loss to understand speech in quiet settings. However, listening to music presents a challenge to most CI users; they often do not enjoy music or avoid it altogether. The Musi-CI training course was developed for CI users with the goal of reducing music aversion and improving music enjoyment. A consortium was established consisting of a professional musician with CI, CI rehabilitation professionals and researchers. Participatory action research (PAR) was applied to develop and evaluate the training experiences, collaborating with 37 CI users during three cycles of eight training sessions, each held over a period of 3 months. Input and feedback were collected after each training session using questionnaires, observations and focus group interviews. Almost all participants (86%) completed the training. After completing the training a large majority of participants reported increased music appreciation, increased social participation in musical settings and a positive impact on general auditory perception. The resulting Musi-CI training programme focuses on music listening skills, self-efficacy, and self-motivation. It consists of exercises intended to strengthen attention and working memory, to improve beat and rhythm perception (with online rhythm exercises) and exercises to distinguish timbre of instruments and emotion in music. A Melody Game was developed to improve pitch and melodic contour discrimination.

Changes on clinical and participatory outcomes in people with severe-to-profound hearing loss after cochlear implantation: protocol of a multicentre prospective observational cohort study - Societal Merit of Intervention on Hearing Loss Evaluation (SMILE).

INTRODUCTION: Cochlear implantation (CI) is a (cost-)effective intervention for people with severe or profound hearing loss. Since its introduction experience increased and the technology evolved, leading to better results and relaxation of CI eligibility criteria. Meanwhile, with national healthcare costs increasing there is a need for evidence of healthcare technology's value. This protocol describes a study to investigate clinical and participatory outcomes after CI for the currently (expanded) eligible hearing impaired population. The study adds to the current evidence base through its multicentre design, long-term follow-up and use of participatory outcomes alongside standard clinical outcomes. METHODS: This multicentre prospective observational cohort study will include at least 156 adult patients with severe-to-profound hearing loss, approximately evenly divided into two groups (1, ages 18-65 years and 2, age >65 years). The measurements consist of audiometry, cognition tests, listening effort tests and multiple generic and disease specific questionnaires. Questionnaires will be administered twice before CI, soon after inclusion at CI referral and shortly before CI surgery, with an annual follow-up of 3 years after CI. The Impact on Participation and Autonomy questionnaire will be used to assess participation. Generalised models (linear, logistic, Poisson) will be used. Mixed effects models will be used to investigate changes over time while exploring differences in subgroups and the influence of covariates. ETHICS AND DISSEMINATION: The study has received ethical approval from the Medical Ethical Committee of all participating centres. The results could provide valuable insights into changes in participatory outcomes of people with severe-to-profound hearing loss after CI. Results will be disseminated through peer-reviewed journals, scientific conferences and professional and patient organisation meetings. TRIAL REGISTRATION NUMBER: NCT05525221.

Exploring neurocognitive factors and brain activation in adult cochlear implant recipients associated with speech perception outcomes-A scoping review.

Background: Cochlear implants (CIs) are considered an effective treatment for severe-to-profound sensorineural hearing loss. However, speech perception outcomes are highly variable among adult CI recipients. Top-down neurocognitive factors have been hypothesized to contribute to this variation that is currently only partly explained by biological and audiological factors. Studies investigating this, use varying methods and observe varying outcomes, and their relevance has yet to be evaluated in a review. Gathering and structuring this evidence in this scoping review provides a clear overview of where this research line currently stands, with the aim of guiding future research. Objective: To understand to which extent different neurocognitive factors influence speech perception in adult CI users with a postlingual onset of hearing loss, by systematically reviewing the literature. Methods: A systematic scoping review was performed according to the PRISMA guidelines. Studies investigating the influence of one or more neurocognitive factors on speech perception post-implantation were included. Word and sentence perception in quiet and noise were included as speech perception outcome metrics and six key neurocognitive domains, as defined by the DSM-5, were covered during the literature search (Protocol in open science registries: 10.17605/OSF.IO/Z3G7W of searches in June 2020, April 2022). Results: From 5,668 retrieved articles, 54 articles were included and grouped into three categories using different measures to relate to speech perception outcomes: (1) Nineteen studies investigating brain activation, (2) Thirty-one investigating performance on cognitive tests, and (3) Eighteen investigating linguistic skills. Conclusion: The use of cognitive functions, recruiting the frontal cortex, the use of visual cues, recruiting the occipital cortex, and the temporal cortex still available for language processing, are beneficial for adult CI users. Cognitive assessments indicate that performance on non-verbal intelligence tasks positively correlated with speech perception outcomes. Performance on auditory or visual working memory, learning, memory and vocabulary tasks were unrelated to speech perception outcomes and performance on the Stroop task not to word perception in quiet. However, there are still many uncertainties regarding the explanation of inconsistent results between papers and more comprehensive studies are needed e.g., including different assessment times, or combining neuroimaging and behavioral measures. Systematic review registration: https://doi.org/10.17605/OSF.IO/Z3G7W.

Impact of Expanding Eligibility Criteria for Cochlear Implantation - Dynamic Modeling Study.

OBJECTIVES: Eligibility criteria for cochlear implantation (CI) are shifting due to technological and surgical improvements. The aim of this study was to explore the impact of further expanding unilateral CI criteria in those with severe hearing loss (HL) (61-80 dBHL) in terms of number of CI recipients, costs, quality of life, and cost-effectiveness. METHODS: A dynamic population-based Markov model was constructed mimicking the Dutch population in three age categories over a period of 20 years. Health states included severe HL (61-80 dBHL), profound HL (>81 dBHL), CI recipients, and no-CI recipients. Model parameters were based on published literature, (national) databases, expert opinion, and model calibration. RESULTS: If persons with severe HL would qualify and opt for CI similar to those with profound HL now, this would lead to a 6-7 times increase of new CI recipients and an associated increase in costs (€550 million) and QALYs (54.000) over a 20-year period (incremental cost utility ratio: 10.771 euros/QALY [2.5-97.5 percentiles: 1.252-23.171]). One-way-sensitivity analysis indicated that model outcomes were most sensitive to regaining employment, utility associated with having a CI, and costs of surgery and testing. CONCLUSION: Our findings suggest that expanding eligibility for CI to persons with severe HL could be a cost-effective use of resources. Clearly, however, it would require a significant increase in diagnostic, operative, and rehabilitative capacity. Our quantitative estimates can serve as a basis for a wider societal deliberation on the question whether such an increase can and should be pursued. LEVEL OF EVIDENCE: NA Laryngoscope, 133:924-932, 2023.

Intracochlear electrode array position and cochlear implant outcomes using the nucleus slim modiolar electrode and the extended round window approach: a follow-up study.

PURPOSE: The aim of this study was to evaluate the intracochlear position of the Slim Modiolar Electrode (SME) after insertion via the extended Round Window (eRW) approach, and to correlate this with residual hearing preservation and speech perception outcomes. METHODS: Twenty-three adult participants, consecutively implanted with the SME via the eRW approach, were included in this prospective, single-center, observational study. Electrode position was evaluated intra-operatively using X-ray fluoroscopy and TIM measurement, and post-operatively using ultra-high resolution CT. Residual hearing [threshold shift in PTA between pre- and post-operative measurement, relative hearing preservation (RHP%)] and speech perception were evaluated at 2 and 12 months after surgery. RESULTS: In each of the 23 participants, complete scala tympani positioning of the electrode array could be achieved. In one participant, an initial tip fold-over was corrected during surgery. Average age at implantation was 63.3 years (SD 13.3, range 28-76) and mean preoperative residual hearing was 81.5 dB. The average post-operative PTA threshold shift was 16.2 dB (SD 10.8) at 2 months post-operatively, corresponding with a RHP% score of 44% (SD 34.9). At 12 months, the average RHP% score decreased to 37%. Postoperative phoneme scores improved from 27.1% preoperatively, to 72.1% and 82.1% at 2 and 12 months after surgery, respectively. CONCLUSION: Use of the eRW approach results in an increased likelihood of complete scala tympani insertion when inserting the SME, with subsequent excellent levels of speech perception. However, residual hearing preservation was found to be moderate, possibly as a result of the extended round window approach, emphasizing that it is not an all-purpose approach for inserting this particular electrode array.

Ultra-High-Resolution CT to Detect Intracochlear New Bone Formation after Cochlear Implantation.

Background Histopathologic studies reported that cochlear implantation, a well-established means to treat severe-to-profound sensorineural hearing loss, may induce inflammation, fibrosis, and new bone formation (NBF) with possible impact on loss of residual hearing and hearing outcome. Purpose To assess NBF in vivo after cochlear implantation with ultra-high-spatial-resolution (UHSR) CT and its implication on long-term residual hearing outcome. Materials and Methods In a secondary analysis of a prospective single-center cross-sectional study, conducted between December 2016 and January 2018, patients with at least 1 year of cochlear implantation experience underwent temporal bone UHSR CT and residual hearing assessment. Two observers evaluated the presence and location of NBF independently, and tetrachoric correlations were used to assess interobserver reliability. In addition, the scalar location of each electrode was assessed. After consensus agreement, participants were classified into two groups: those with NBF (n = 83) and those without NBF (n = 40). The association between NBF and clinical parameters, including electrode design, surgical approach, and long-term residual hearing loss, was tested using the χ2 and Student t tests. Results A total of 123 participants (mean age ± standard deviation, 63 years ± 13; 63 women) were enrolled. NBF was found in 83 of the 123 participants (68%) at 466 of 2706 electrode contacts (17%). Most NBFs (428 of 466, 92%) were found around the 10 most basal contacts, with an interobserver agreement of 86% (2297 of 2683 contacts). Associations between electrode types and surgical approaches were significant (58 of 79 participants with NBF and a precurved electrode vs 24 of 43 with NBF and a straight electrode, P = .04; 64 of 88 participants with NBF and a cochleostomy approach vs 18 of 34 with NBF and a round window approach, P = .03). NBF was least often seen in full scala tympani insertions, but there was no significant association between scalar position and NBF (P = .15). Long-term residual hearing loss was significantly larger in the group with NBF compared with the group without NBF (mean, 22.9 dB ± 14 vs 8.6 dB ± 18, respectively; P = .04). Conclusion In vivo detection of new bone formation (NBF) after cochlear implantation is possible by using ultra-high-spatial-resolution CT. Most cochlear implant recipients develop NBF, predominately located at the base of the cochlea. NBF adversely affects long-term residual hearing preservation. © RSNA, 2021 An earlier incorrect version appeared online. This article was corrected on December 8, 2021.

Comparison Between Transimpedance Matrix (TIM) Measurement and X-ray Fluoroscopy for Intraoperative Electrode Array Tip Fold-Over Detection.

OBJECTIVE: The aim of this study was to compare Transimpedance Matrix (TIM-) measurement and X-ray fluoroscopy for the intraoperative detection of electrode array tip fold-over in cochlear implant recipients. STUDY DESIGN: Retrospective agreement study. SETTING: Tertiary referral hospital. PATIENTS: Forty-two patients (47 ears) consecutively implanted with the Slim Modiolar Electrode. INTERVENTIONS: Five raters, with different levels of clinical experience, individually retrospectively evaluated the TIM-heatmaps and X-ray fluoroscopy images of all patients included in this study for electrode array tip fold-over. MAIN OUTCOME MEASURES: Agreement between raters' individual evaluation and the diagnosis given during clinical intraoperative evaluation for both modalities, as well as the inter-method agreement between TIM-measurement and fluoroscopy, and the inter-rater agreement for both modalities. RESULTS: A tip fold-over was found in three of the forty-seven implantations (6.4%) included in this study. The average agreement between raters' evaluation and the intraoperative evaluation was 88% (Cohens κ = 0.378) for fluoroscopy and 99% (Cohens κ = 0.915) for TIM-measurement. Two raters misdiagnosed at least one tip fold-over as being correctly positioned when evaluating the fluoroscopy images (1/3 and 3/3, respectively). Each of the raters correctly detected all three tip fold-overs using the TIM-heatmaps. The inter-rater agreement for fluoroscopy was classified as "fair" (Fleiss' κ = 0.286), while the inter-rater agreement for TIM-measurement was classified as "near-perfect" (Fleiss' κ = 0.850). CONCLUSIONS: TIM-measurement has a high potential to replace X-ray fluoroscopy for intraoperatively detecting electrode array tip fold-over in cochlear implantation, especially in patients implanted with flexible, precurved arrays.

Computational Audiology: New Approaches to Advance Hearing Health Care in the Digital Age.

The global digital transformation enables computational audiology for advanced clinical applications that can reduce the global burden of hearing loss. In this article, we describe emerging hearing-related artificial intelligence applications and argue for their potential to improve access, precision, and efficiency of hearing health care services. Also, we raise awareness of risks that must be addressed to enable a safe digital transformation in audiology. We envision a future where computational audiology is implemented via interoperable systems using shared data and where health care providers adopt expanded roles within a network of distributed expertise. This effort should take place in a health care system where privacy, responsibility of each stakeholder, and patients' safety and autonomy are all guarded by design.

Factors Influencing Speech Perception in Adults With a Cochlear Implant.

OBJECTIVES: The primary objective of this study is to identify the biographic, audiologic, and electrode position factors that influence speech perception performance in adult cochlear implant (CI) recipients implanted with a device from a single manufacturer. The secondary objective is to investigate the independent association of the type of electrode (precurved or straight) with speech perception. DESIGN: In a cross-sectional study design, speech perception measures and ultrahigh-resolution computed tomography scans were performed in 129 experienced CI recipients with a postlingual onset of hearing loss. Data were collected between December 2016 and January 2018 in the Radboud University Medical Center, Nijmegen, the Netherlands. The participants received either a precurved electrode (N = 85) or a straight electrode (N = 44), all from the same manufacturer. The biographic variables evaluated were age at implantation, level of education, and years of hearing loss. The audiometric factors explored were preoperative and postoperative pure-tone average residual hearing and preoperative speech perception score. The electrode position factors analyzed, as measured from images obtained with the ultrahigh-resolution computed tomography scan, were the scalar location, angular insertion depth of the basal and apical electrode contacts, and the wrapping factor (i.e., electrode-to-modiolus distance), as well as the type of electrode used. These 11 variables were tested for their effect on three speech perception outcomes: consonant-vowel-consonant words in quiet tests at 50 dB SPL (CVC50) and 65 dB SPL (CVC65), and the digits-in-noise test. RESULTS: A lower age at implantation was correlated with a higher CVC50 phoneme score in the straight electrode group. Other biographic variables did not correlate with speech perception. Furthermore, participants implanted with a precurved electrode and who had poor preoperative hearing thresholds performed better in all speech perception outcomes than the participants implanted with a straight electrode and relatively better preoperative hearing thresholds. After correcting for biographic factors, audiometric variables, and scalar location, we showed that the precurved electrode led to an 11.8 percentage points (95% confidence interval: 1.4-20.4%; p = 0.03) higher perception score for the CVC50 phonemes compared with the straight electrode. Furthermore, contrary to our initial expectations, the preservation of residual hearing with the straight electrode was poor, as the median preoperative and the postoperative residual hearing thresholds for the straight electrode were 88 and 122 dB, respectively. CONCLUSIONS: Cochlear implantation with a precurved electrode results in a significantly higher speech perception outcome, independent of biographic factors, audiometric factors, and scalar location.

Force and pressure measurements in temporal bones.

PURPOSE: Some cochlear implant (CI) patients lose their residual hearing during surgery. Two factors that might play a role in residual hearing loss are the change in intracochlear hydraulic pressure and force on the cochlear wall during electrode insertion. The aim of this study is to investigate whether a difference in peak hydraulic pressure and peak force on the cochlear wall exists during a CI electrode insertion with different insertion techniques. MATERIALS AND METHODS: Twenty fresh frozen temporal bones were used. Hydraulic pressure and force on the cochlear wall were recorded during straight electrode insertions with 1) slow versus fast insertion speed, 2) manual versus automatic insertion method and 3) round window approach (RWA) versus extended RWA (ERWA). RESULTS: When inserting with a slow compared to a fast insertion speed, the peak hydraulic pressure is 239% (95% CI: 130-399%) higher with a RWA and 58% (95% CI: 6-137%) higher with an ERWA. However, the peak force on the cochlear wall is a factor 29% less (95% CI: 13-43%) with a slow insertion speed. No effect was found of opening and insertion method. CONCLUSIONS: As contradictory findings were found for hydraulic pressure and force on the cochlear wall on insertion speed, it remains unclear which insertion speed (slow versus fast) is less traumatic to inner ear structure.

The effect of cochlear implantation on autonomy, participation and work in postlingually deafened adults: a scoping review.

PURPOSE: This scoping review examines the available evidence on the effect of unilateral cochlear implantation (CI) in adults with postlingual bilateral hearing loss on societal-related outcomes in terms of work, autonomy and participation. METHODS: Five databases were searched (Pubmed, Web of Science, Embase, PsycINFO and Cochrane Library). Publications were screened in three steps on inclusion criteria. Of the 4230 screened publications, 110 met the inclusion criteria and were assessed for data extraction regarding outcomes "work", "autonomy", "participation". Study characteristics and key findings are presented and narratively described. RESULTS: Twenty-seven publications were included and categorized into retrospective (n = 3), cross-sectional (n = 18) or prospective (n = 6) study designs. Measurement or identification of number of outcomes (no) were related to work (no = 20), participation (no = 9) and autonomy or independency (no = 10). Most studies indicated benefits of CI on these outcomes. However, some studies did not or indicated additional barriers for benefits. Eleven publications primarily aimed to study one or more of our primary outcomes. CONCLUSION: In this literature search, scientific databases are reviewed. The results indicate that there is a relatively small body of evidence regarding the effect of CI on the outcomes "work", "autonomy" and "participation". Even though there are some limitations of the current study including some overlap in outcome definitions, most included studies indicate a beneficial effect of CI on work, autonomy and participation. The lack of consensus in definitions and the small body of evidence indicates a need for additional prospective studies investigating the societal outcomes of CI in postlingually deafened adults.

Transimpedance Matrix (TIM) Measurement for the Detection of Intraoperative Electrode Tip Foldover Using the Slim Modiolar Electrode: A Proof of Concept Study.

OBJECTIVES: The aim of this study is to report on our preliminary experience with Transimpedance Matrix (TIM)-measurement for the detection of cochlear implant electrode tip foldovers compared with intraoperative imaging in patients implanted with the slim modiolar electrode (SME). STUDY DESIGN: Proof of concept study. SETTING: Tertiary university referral center. PATIENTS: Twenty five ears (in 22 patients) implanted consecutively with the SME. INTERVENTIONS: Following cochlear implantation, intraoperative TIM-measurement and fluoroscopy were performed. One week postoperatively, the electrode position was evaluated using Computed Tomography (CT)-imaging. MAIN OUTCOME MEASURES: Electrode array tip foldover. RESULTS: Electrode array tip foldover occurred in three of the 25 cochlear implantations performed (12%). In each case, the foldover was detected by both TIM and fluoroscopy, leading to reposition and correct intracochlear placement of the array. CONCLUSIONS: TIM-measurement is a promising method for the intraoperative detection of an electrode array tip foldover. The TIM-tool with intuitive heatmap display is easy to use, fast, and readily available to clinics using TIM-software in the operating theatre.