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.

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.

Post-implantation clinical cost analysis between transcutaneous and percutaneous bone conduction devices.

INTRODUCTION: Bone conduction devices (BCD) are effective for hearing rehabilitation in patients with conductive and mixed hearing loss or single-sided deafness. Transcutaneous bone conduction devices (tBCD) seem to lead to fewer soft tissue complications than percutaneous BCDs (pBCD) but have other drawbacks such as MRI incompatibility and higher costs. Previous cost analyses have shown a cost advantage of tBCDs. The purpose of this study is to compare long-term post-implantations costs between percutaneous and transcutaneous BCDs. MATERIALS AND METHODS: Retrospective data from 77 patients implanted in a tertiary referral centre with a pBCD (n = 34), tBCD (n = 43; passive (tpasBCD; n = 34) and active (tactBCD; n = 9) and a reference group who underwent cochlear implantation (CI; n = 34), were included in a clinical cost analysis. Post-implantation costs were determined as the sum of consultation (medical and audiological) and additional (all post-operative care) costs. Median (cumulative) costs per device incurred for the different cohorts were compared at 1, 3 and 5 years after implantation. RESULTS: After 5 years, the total post-implantation costs of the pBCD vs tpasBCD were not significantly different (€1550.7 [IQR 1174.6-2797.4] vs €2266.9 [IQR 1314.1-3535.3], p = 0.185), nor was there a significant difference between pBCD vs tactBCD (€1550.7 [1174.6-2797.4] vs €1428.8 [1277.3-1760.4], p = 0.550). Additional post-implantation costs were significantly highest in the tpasBCD cohort at all moments of follow-up. CONCLUSION: Total costs related to post-operative rehabilitation and treatments are comparable between percutaneous and transcutaneous BCDs up to 5 years after implantation. Complications related to passive transcutaneous bone conduction devices appeared significantly more expensive after implantation due to more frequent explantations.

Cochlear Implants or Hearing Aids: Speech Perception, Language, and Executive Function Outcomes.

OBJECTIVES: We aimed to determine whether children with severe hearing loss (HL) who use hearing aids (HAs) may experience added value in the perception of speech, language development, and executive function (EF) compared to children who are hard of hearing (HH) or children who are deaf and who use cochlear implants (CIs) and would benefit from CIs over HAs. The results contribute to the ongoing debate concerning CI criteria. We addressed the following research question to achieve this aim: Do children who are HH or deaf with CIs perform better than children with severe HL with HAs with respect to auditory speech perception, and receptive vocabulary and/or EF? DESIGN: We compared two groups of children with severe HL, profound HL or deafness, with CIs or HAs, matched for gender, test age (range, 8 to 15 years), socioeconomic status, and nonverbal intelligence quotient. Forty-three children had CIs (pure-tone average at 2000 and 4000 Hz >85 dB HL), and 27 children had HAs (mean pure-tone average: 69 dB HL). We measured speech perception at the conversational level (65 dB SPL) and the soft speech perception level (45 dB SPL). We established receptive vocabulary using the Peabody Picture Vocabulary Test-III-NL. We tested EF using the Delis Kaplan Executive Function System battery and the Dutch Rey Auditory Verbal Learning Test. We employed the Mann-Whitney U test to compare data between the CI and HA groups. We used Chi-square goodness of fit tests to contrast the CI and HA group distributions with the norm data of children who are typically developing (TD). We harnessed Kendall's Tau-b to investigate relationships between the study variables. RESULTS: Both groups of children, with CIs and Has, obtained ceiling scores for perception of speech on a conversational level. However, the HA group exhibited significantly lower perception on a soft speech level scores (68 %) than the CI group (87%). No difference was present between the receptive vocabulary distributions of the CI and HA groups. The median receptive vocabulary standard scores for both groups were well within the normal range (CI group: 93; HA group: 96). In addition, we did not find any difference in EF between the CI and HA groups. For planning and verbal memory, the distributions of observed scores for children with CIs were different from the expected distributions of children who are TD. In both groups, a large proportion of children obtained below-average scores for planning (CI: 44%; HA: 33%) and for long-term verbal memory (CI: 44%; HA: 35%). In the HA group, perception at a soft speech level was associated with receptive vocabulary and planning. In the CI group, we did not find any associations. CONCLUSIONS: Both groups of children with severe and profound HL with HAs exhibit less favorable auditory perception on the soft speech level, but not at a conversational level, compared to children who are HH or deaf with CIs. Both groups, children with CIs and HAs, only exhibit more problems in planning and verbal memory than the norm groups of children who are TD. The results indicate that to obtain age-appropriate levels of receptive vocabulary and EF, the perception at the soft speech level is a necessary but not sufficient prerequisite.

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.

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.

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.

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.

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.

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.

The evaluation of a slim perimodiolar electrode: surgical technique in relation to intracochlear position and cochlear implant outcomes.

PURPOSE: In cochlear implantation (CI), the two factors that are determined by the surgeon with a potential significant impact on the position of the electrode within the cochlea and the potential outcome, are the surgical technique and electrode type. The objective of this prospective study was to evaluate the position of the slim, perimodiolar electrode (SPE), and to study the influence of the SPE position on CI outcome. METHODS: Twenty-three consecutively implanted, adult SPE candidates were included in this prospective cohort study conducted between December 2016 and April 2019. Mean age at surgery was 59.5 years. Mean preoperative residual hearing was 92.2 dB. Intra-operative fluoroscopy and high-resolution computed tomography scans were performed to evaluate electrode position after insertion using a cochleostomy (CS) approach. Follow-up was 12 months after implantation; residual hearing (6-8 weeks) and speech perception (6-8 weeks and 12 months) were evaluated in relation to the intracochlear SPE position. RESULTS: In most patients in whom the SPE was positioned in the scala tympani residual hearing was preserved [mean absolute increase in PTA of 4.4 dB and 77.2% relative hearing preservation (RHP%)]. Translocation into the scala vestibuli occurred in 36% of the insertions, resulting in a mean absolute increase in PTA of 17.9 dB, and a RHP% of 19.2%. Participants with a translocation had poorer speech perception scores at 12-month follow-up. CONCLUSION: Given the incidence of CS-associated translocations with the SPE and the negative effect on outcome, it is advised to insert the SPE using the (extended) round window approach.

Impact of cochlear implantation on the function of the three semicircular canals.

OBJECTIVE: To assess the effect of cochlear implantation on the function of the semicircular canals (SCC) and on experienced vestibular symptoms. Second, to determine the relation between vestibular test results. DESIGN: Retrospective cohort study assessing absolute and categorised results of caloric irrigation test, video Head Impulse Test (vHIT) and Dizziness Handicap Inventory (DHI) before and after cochlear implantation.Study sample: 192 patients, aged ≥7 years old, without preoperative areflexia. RESULTS: Mean maximum slow phase velocity decreased with 3.1°/s and 4.7°/s for warm and cold caloric irrigation respectively. About 37.4% of the patients deteriorated one or more categories on caloric testing. Complete caloric postoperative areflexia was found in 6.2%. Mean vHIT gain decreased with 0.06, 0.04 and 0.05 for anterior, lateral and posterior SCC, respectively. Seven patients (7.7%) acquired an abnormal gain value for the anterior SCC. Only mean score on DHI's physical subdomain rose significantly (1.4 points). Overall, 9.0% of the patients deteriorated one or two categories on DHI. Only few weak correlations were found between caloric test, vHIT and DHI shifts. CONCLUSIONS: Although mean objective and subjective-physical vestibular deteriorations were significant, its clinical impact seems limited. However, 9% of patients experience vestibular deterioration, thus, advocate assessment. Vestibular test results show no or merely weak mutual correlations.

Expanding unilateral cochlear implantation criteria for adults with bilateral acquired severe sensorineural hearing loss.

OBJECTIVES: To report on a retrospective cohort study on the effects of expanding inclusion criteria for application of cochlear implants (CIs) on the performance 1-year post-implantation. METHODS: Based on pre-implantation audiometric thresholds and aided speech recognition scores, the data of 164 CI recipients were divided into a group of patients that fulfilled conservative criteria (mean hearing loss at 0.5, 1 and 2 kHz > 85 dB HL and phoneme scores with hearing aids < 30%), and the remaining group of patients that felt outside this conservative criterion. Speech recognition scores (in quiet) and quality of life (using the NCIQ) of both groups, measured at 1-year post-implantation, were compared. RESULTS: The group that felt outside the conservative criterion showed a higher phoneme score at 1-year post-implantation compared to the conservative group, suggesting that relaxed criteria have a positive influence on the speech recognition results with CI. With respect to quality of life, both groups significantly improved 1-year post-implantation. The conservative group showed a higher benefit on the advanced perception domain of the NCIQ. Based on their worse pre-implantation hearing, this was expected. CONCLUSIONS: The data suggest that relaxation of CI indication positively affects the speech recognition performance of patients with severe hearing loss. Both groups of patients showed a positive effect of CI on the quality of life. This benefit relates to communication skills and the subjective day-to-day functioning in society.

Auricular prostheses attached to osseointegrated implants: multidisciplinary work-up and clinical evaluation.

PURPOSE: Not long after the introduction of osseointegrated implants outside the oral cavity, auricular prostheses are retrained on osseointegrated implants. New insights have been gained with the next-generation percutaneous osseointegrated titanium implants for bone conduction hearing since its introduction in 2010. As a result, the same technology was introduced in the Vistafix® system (VXI implant) to retain auricular prostheses. The aim of this study is to evaluate the surgical procedure, clinical outcome, and satisfaction of the patient of osseointegration-retained auricular prosthesis using VXI implants. MATERIALS AND METHODS: 11 patients who received an auricular prosthesis using VXI implants between December 2012 and November 2017 were evaluated retrospectively. The patient's medical files were reviewed to assess clinical complications and the necessity for revision surgery. The subjective outcome was measured using the Glasgow benefit inventory (GBI). RESULTS: In total, 31 implants were placed in 11 patients. None of these implants were lost nor revision surgery needed. An adverse skin reaction was observed in 13.0% of the implants and in 27.2% of the patients, adequately treated with an antibiotic ointment. The average follow-up time was 2 years and 7 months. The GBI displayed a positive score in every patient. CONCLUSIONS: The VXI implants used are a safe and reliable treatment option for retaining auricular prostheses in patients with an absent auricle. Patients were satisfied with their auricular prosthesis and showed benefit in quality of life. Studies with larger numbers and preferably a prospective character are needed to draw statistically significant conclusions.

Audiological and clinical outcomes of a transcutaneous bone conduction hearing implant: Six-month results from a multicentre study.

OBJECTIVES: To compare the hearing performance of patients with conductive and mild mixed hearing loss and single-sided sensorineural deafness provided with a new transcutaneous bone conduction hearing implant (the Baha Attract System) with unaided hearing as well as aided with a sound processor on a softband. Furthermore, to evaluate safety and subjective benefit before and after implantation of the test device. PARTICIPANTS: Fifty-four adult patients in five participating centres were enrolled in this prospective study. Baseline data were collected during a pre-operative visit, and after a softband trial, all patients were implanted unilaterally. Follow-up visits were scheduled at 10 days, 4, 6, 12 weeks and 6 months. MAIN OUTCOME MEASURES: Free-field hearing thresholds pure-tone average (PTA4 in dB HL; mean threshold at 500, 1000, 2000, 4000 Hz; primary outcome measure). Individual free-field hearing thresholds, speech recognition in quiet and in noise, soft tissue status during follow-up and subjective benefit as measured with the Abbreviated Profile of Hearing Aid Benefit (APHAB), Speech, Spatial and Qualities of Hearing Scale (SSQ) and Health Utilities Index (HUI) questionnaires. RESULTS: Implantation of the Baha Attract System resulted in favourable audiological outcomes compared to unaided conditions. On the primary outcome parameter, a statistically significant improvement was observed compared to unaided hearing for the patients with conductive/mixed hearing loss (mean PTA4 difference -20.8 dB HL, SD 9.8; P < 0.0001) and for the patients with single-sided sensorineural deafness (SSD) (mean PTA4 difference -21.6 dB HL, SD 12.2; P < 0.0001). During all audiology tests, the non-test ear was blocked. Statistically significant improvements were also recorded in speech tests in quiet and noise compared to unaided hearing for the conductive/mixed hearing loss group and for speech in quiet in the SSD group. Compared to the pre-operative measurement with softband, no significant differences were recorded in the PTA4 free-field hearing threshold or the other audiological outcomes in either of the groups (P > 0.05). Soft tissue-related issues observed during follow-up included numbness, pain/discomfort at the implant site and to a lesser extent pressure-related skin complications. A declining trend was noted in the rate of these complications during follow-up. Approximately 20% of patients reported some degree of numbness and 38% (slight) pain/discomfort at final follow-up of 6 months. Good results on the subjective benefit questionnaires were observed, with statistically significant improvements on APHAB and SSQ questionnaires, and on the hearing attribute of HUI3. CONCLUSIONS: The Baha Attract System provided a significant improvement in hearing performance and subjective benefit compared to the pre-operative unaided condition (with the non-test ear blocked). Hearing performance of the Baha Attract was similar to a test situation with the same sound processor on a softband. A proportion of the patients reported numbness and pain/discomfort at the implant site during follow-up, especially during the first post-operative weeks. Based on the results of the current multicentre study, the Baha Attract can be considered as a treatment option for patients with the aforementioned hearing losses. Especially in the SSD patients, a careful selection procedure is warranted. Therefore, a pre-operative trial should be part of the decision-making process before fitting a patient with the Baha Attract System.

Risk factors for complications in cochlear implant surgery.

PURPOSE: The objective of this study was to achieve uniform reporting of complications and failures in cochlear implantation, to analyze complications and failures and to identify risk factors for complications in a series of over 1300 cochlear implantations. METHODS: In a retrospective chart review and observational study, data from all cochlear implantations from 1987 to 2015 were entered in a custom-made database. Complications were classified using the contracted form of the Clavien-Dindo system and risk factors were identified by statistical analysis. RESULTS: A complication rate of 18.4% and a device failure rate of 2.9% were found. There was a higher rate of hematoma in patients with a clotting disorder and when a subtotal petrosectomy was performed, a higher rate of wound infections in patients who were not vaccinated against Streptococcus pneumoniae and a higher rate of meningitis in patients with an inner ear malformation. CONCLUSIONS: The use of a strict definition of a medical complication and device failure-in combination with the Clavien-Dindo classification system-enables uniform and objective registration of adverse events and prevents any tendency to downgrade complications. Complication and failure rates in this series are comparable to those reported in the literature. These results stress the need for pneumococcal vaccination, which may prevent general wound infections, but is especially important for patients with inner ear malformation, who have an increased risk of (postoperative) meningitis.

Influence of hearing loss and cognitive abilities on language development in CHARGE Syndrome.

Hearing loss and cognitive delay are frequently occurring features in CHARGE syndrome that may contribute to impaired language development. However, not much is known about language development in patients with CHARGE syndrome. In this retrospective study, hearing loss, cognitive abilities, and language development are described in 50 patients with CHARGE syndrome. After informed consent was given, data were collected from local medical files. Most patients (38.3%; 18/47 patients) had moderate hearing loss (41-70 dB) and 58.5% (24/41 patients) had an IQ below 70. The mean language quotients of the receptive and expressive language were more than one standard deviation below the norm. Both hearing loss and cognitive delay had an influence on language development. Language and cognitive data were not available for all patients, which may have resulted in a pre-selection of patients with a delay. In conclusion, while hearing thresholds, cognitive abilities and language development vary widely in CHARGE syndrome, they are mostly below average. Hearing loss and cognitive delay have a significant influence on language development in children with CHARGE syndrome. To improve our knowledge about and the quality of care we can provide to CHARGE patients, hearing and developmental tests should be performed regularly in order to differentiate between the contributions of hearing loss and cognitive delay to delays in language development, and to provide adequate hearing amplification in the case of hearing loss. © 2016 Wiley Periodicals, Inc.