Electrically evoked compound action potentials are associated with the site of intracochlear stimulation.

OBJECTIVES: Objective measurements to predict the position of a cochlear electrode during cochlear implantation surgery may serve to improve the surgical technique and postoperative speech outcome. There is evidence that electrically evoked compound action potentials (ECAP) are a suitable approach to provide information about the site of stimulation. This study aims to contribute to the knowledge about the association between the intraoperative intracochlear ECAP characteristics and the site of stimulation. METHODS: In a retrospective cohort study, patients undergoing cochlear implant surgery with flexible lateral wall electrode arrays (12 stimulating channels) between 2020 and 2022 were analyzed. The CDL was measured using a CT-based clinical planning software. ECAP were measured for all electrode contacts and associated to the CDL as well as to the site of stimulation in degree. RESULTS: Significant differences among the amplitudes and slopes for the individual stimulated electrode contacts at the stimulation sites of 90°, 180°, 270°, 360°, 450° and 540° were found. The values showed a trend for linearity among the single electrodes. CONCLUSIONS: ECAP characteristics correlate with the electrode's position inside the cochlea. In the future, ECAP may be applied to assess the intracochlear position inside the cochlea and support anatomy-based fitting.

Comparing linear and non-linear models to estimate the appropriate cochlear implant electrode array length-are current methods precise enough?

PURPOSE: In cochlear implantation with flexible lateral wall electrode arrays, a cochlear coverage (CC) range between 70% and 80% is considered ideal for optimal speech perception. To achieve this CC, the cochlear implant (CI) electrode array has to be chosen according to the individual cochlear duct length (CDL). Here, we mathematically analyzed the suitability of different flexible lateral wall electrode array lengths covering between 70% and 80% of the CDL. METHODS: In a retrospective cross-sectional study preoperative high-resolution computed tomography (HRCT) from patients undergoing cochlear implantation was investigated. The CDL was estimated using an otosurgical planning software and the CI electrode array lengths covering 70-80% of the CDL was calculated using (i) linear and (ii) non-linear models. RESULTS: The analysis of 120 HRCT data sets showed significantly different model-dependent CDL. Significant differences between the CC of 70% assessed from linear and non-linear models (mean difference: 2.5 mm, p < 0.001) and the CC of 80% assessed from linear and non-linear models (mean difference: 1.5 mm, p < 0.001) were found. In up to 25% of the patients none of the existing flexible lateral wall electrode arrays fit into this range. In 59 cases (49,2%) the models did not agree on the suitable electrode arrays. CONCLUSIONS: The CC varies depending on the underlying CDL approximation, which critically influences electrode array choice. Based on the literature, we hypothesize that the non-linear method systematically overestimates the CC and may lead to rather too short electrode array choices. Future studies need to assess the accuracy of the individual mathematical models.

Holistic assessment of cochlear implant outcomes using the international classification of functioning disability and health model: data analysis of a longitudinal prospective multicenter study.

PURPOSE: To study outcome after cochlear implantation using the Cochlear Implant (CI) outcome assessment protocol based on the International Classification of Functioning, Disability and Health (ICF) model (CI-ICF). METHODS: Raw data of a prospective, longitudinal, multicenter study was analyzed. Seventy-two CI candidates were assessed preoperatively and six months postoperatively using the CI-ICF protocol. Following tools were used: (1) Work Rehabilitation Questionnaire (WORQ), (2) Abbreviated Profile of Hearing Aid Benefit (APHAB), (3) Audio Processor Satisfaction Questionnaire (APSQ), (4) Speech, Spatial, and Qualities of Hearing Scale (SSQ12), (5) Hearing Implant Sound Quality Index (HISQUI19), (6) Nijmegen CI Questionnaire (NCIQ) (7) pure tone audiometry, (8) speech audiometry, (9) sound localization. RESULTS: There was a significant improvement of speech discrimination in quiet (p = 0.015; p < 0.001) and in noise (p = 0.041; p < 0.001), sound detection (p < 0.001), tinnitus (p = 0.026), listening (p < 0.001), communicating with-receiving-spoken messages (p < 0.001), conversation (p < 0.001), family relationships (p < 0.001), community life (p = 0.019), NCIQ total score and all subdomain scores (p < 0.001). Subjective sound localization significantly improved (p < 0.001), while psychometric sound localization did not. There was no significant subjective deterioration of vestibular functioning and no substantial change in sound aversiveness. CI users reported a high level of implant satisfaction postoperatively. CONCLUSION: This study highlights the positive impact of cochlear implantation on auditory performance, communication, and subjective well-being. The CI-ICF protocol provides a holistic and comprehensive view of the evolution of CI outcomes.

Dual-site transcranial direct current stimulation to treat tinnitus: a randomized controlled trial.

Transcranial direct current stimulation (tDCS) has been proposed as a potential intervention for subjective tinnitus, but supporting evidence remains limited. We aimed to investigate the effect of anodal high-definition tDCS of the left temporal area and right dorsolateral prefrontal cortex on tinnitus severity. This double-blind randomized controlled trial included 77 patients (age range 18-79, 43 male) with chronic subjective tinnitus as their primary complaint. Thirty-eight subjects received six consecutive sessions of dual-site sequential high-definition-tDCS with electrodes positioned over the left temporal area and right dorsolateral prefrontal cortex. Both areas were stimulated for 15 min per session, with total stimulation time amounting to 30 min. Thirty-nine subjects received sham stimulation. The primary outcome measure was the change in tinnitus severity, as evaluated by the Tinnitus Functional Index, from baseline to a follow-up visit at 8 ± 2 weeks after treatment completion. Secondary outcomes included changes in perceived tinnitus loudness, as measured with a visual analogue scale and a tinnitus matching procedure, as well as scores on the Hospital Anxiety and Depression Scale, and the Hyperacusis Questionnaire. No differences in Tinnitus Functional Index change scores were identified between the active treatment and sham control groups (linear regression: P = 0.86). The Tinnitus Functional Index scores decreased significantly over time in both groups (P = 0.0012), indicating the presence of a considerable placebo effect. These change scores were significantly influenced by sex (linear regression: P = 0.037) and baseline symptoms of anxiety (linear regression: P = 0.049) in both groups. In general, Tinnitus Functional Index scores decreased more profoundly in males and in subjects with a higher degree of anxiety at baseline. None of the included secondary measures differed significantly between experimental arms. Our results suggest that dual-site sequential high-definition-tDCS of the left temporal area and right dorsolateral prefrontal cortex does not alleviate tinnitus severity. Interestingly, in our study population, fluctuations in tinnitus severity were influenced by gender and concurrent mental condition. It is therefore important to take these factors into account when conducting or planning randomized controlled trials in tinnitus populations.

Robotic cochlear implantation in post-meningitis ossified cochlea.

AIM: To report the experience of an image-guided and navigation-based robot arm as an assistive surgical tool for cochlear implantation in a case with a labyrinthitis ossificans. PATIENT: A 55-years-old man with a history of childhood meningitis whose hearing deteriorated progressively to bilateral profound sensorineural hearing loss. INTERVENTION: Robotic Assisted Cochlear Implant Surgery (RACIS) with a straight flexible lateral wall electrode. PRIMARY OUTCOME MEASURES: Electrode cochlear insertion depth with RACIS with facial recess approach and autonomous inner ear access with full electrode insertion of a flexible straight cochlear implant array. CONCLUSIONS: Intra cochlear ossifications pose a challenge for entering the cochlea and full-length insertion of a cochlear implant. RACIS has shown that computations of radiological images combined with navigation-assisted robot arm drilling can provide efficient access to the inner ear.

Audiological outcomes of robot-assisted cochlear implant surgery.

PURPOSE: The main objective of this study is to evaluate the short-term and long-term audiological outcomes in patients who underwent cochlear implantation with a robot-assisted system to enable access to the cochlea, and to compare outcomes with a matched control group of patients who underwent cochlear implantation with conventional access to the cochlea. METHODS: In total, 23 patients were implanted by robot-assisted cochlear implant surgery (RACIS). To evaluate the effectiveness of robotic surgery in terms of audiological outcomes, a statistically balanced control group of conventionally implanted patients was created. Minimal outcome measures (MOM), consisting of pure-tone audiometry, speech understanding in quiet and speech understanding in noise were performed pre-operatively and at 3 months, 6 months, 12 months and 2 years post-activation of the audioprocessor. RESULTS: There was no statistically significant difference in pure-tone audiometry, speech perception in quiet and speech perception in noise between robotically implanted and conventionally implanted patients pre-operatively, 3 months, 6 months, 12 months and 2 years post-activation. A significant improvement in pure-tone hearing thresholds, speech understanding in quiet and speech understanding in noise with the cochlear implant has been quantified as of the first measurements at 3 months and this significant improvement remained stable over a time period of 2 years for HEARO implanted patients. CONCLUSION: Clinical outcomes in robot-assisted cochlear implant surgery are comparable to conventional cochlear implantation. CLINICALTRAILS. GOV TRAIL REGISTRATION NUMBERS: NCT03746613 (date of registration: 19/11/2018), NCT04102215 (date of registration: 25/09/2019).

Multimodal phenotypic labelling using drug-induced sleep endoscopy, awake nasendoscopy and computational fluid dynamics for the prediction of mandibular advancement device treatment outcome: a prospective study.

Mandibular advancement device (MAD) treatment outcome for obstructive sleep apnea (OSA) is variable and patient dependent. A global, clinically applicable predictive model is lacking. Our aim was to combine characteristics obtained during drug-induced sleep endoscopy (DISE), awake nasendoscopy, and computed tomography scan-based computational fluid dynamic (CFD) measurements in one multifactorial model, to explain MAD treatment outcome. A total of 100 patients with OSA were prospectively recruited and treated with a MAD at fixed 75% protrusion. In all, 72 underwent CFD analysis, DISE, and awake nasendoscopy at baseline in a blinded fashion and completed a 3-month follow-up polysomnography with a MAD. Treatment response was defined as a reduction in the apnea-hypopnea index (AHI) of ≥50% and deterioration as an increase of ≥10% during MAD treatment. To cope with missing data, multiple imputation with predictive mean matching was used. Multivariate logistic regression, adjusting for body mass index and baseline AHI, was used to combine all potential predictor variables. The strongest impact concerning odds ratios (ORs) was present for complete concentric palatal collapse (CCCp) during DISE on deterioration (OR 28.88, 95% confidence interval [CI] 1.18-704.35; p = 0.0391), followed by a C-shape versus an oval shape of the soft palate during wakefulness (OR 8.54, 95% CI 1.09-67.23; p = 0.0416) and tongue base collapse during DISE on response (OR 3.29, 95% CI 1.02-10.64; p = 0.0464). Both logistic regression models exhibited excellent and fair predictive accuracy. Our findings suggest DISE to be the most robust examination associated with MAD treatment outcome, with tongue base collapse as a predictor for successful MAD treatment and CCCp as an adverse DISE phenotype.

The Rapid Screening for Somatosensory Tinnitus Tool: a Data-Driven Decision Tree Based on Specific Diagnostic Criteria.

BACKGROUND: Somatosensory or somatic tinnitus (ST) is a type of tinnitus where changes in somatosensory afference from the cervical spine or temporomandibular area alter the tinnitus perception. Very recently, the diagnostic value of a set of 16 diagnostic criteria for ST was determined. The next step in the development of easily applicable diagnostic criteria is to provide an uncomplicated model, based on the existing criteria, which can easily be used in clinical practice. OBJECTIVES: This study aims to construct an accurate decision tree, combining several diagnostic criteria, to optimize both sensitivity and specificity of ST diagnosis. DESIGN: An online survey was launched on the online forum Tinnitus Talk, managed by Tinnitus Hub in a convenience sample of participants with tinnitus. The survey included 42 questions, both on the presence of diagnostic criteria for ST and on other potentially influencing factors. A decision tree was constructed to classify participants with and without ST using the rpart package in R. Tree depth was optimized during a five-fold cross-validation. Finally, model performance was evaluated on a subset containing 20% of the original dataset. RESULTS: Data of 7981 participants were used to construct a decision tree for ST diagnosis. Four criteria were included in the final decision tree: 'Tinnitus and neck/jaw pain increase/decrease simultaneously', 'Tension in suboccipital muscles', 'Somatic modulation', and 'Bruxism'. The presented model has an accuracy of 82.2%, a sensitivity of 82.5%, and a specificity of 79%. Receiver operator characteristic curves demonstrated an area under the curve of 0.88. CONCLUSIONS: Based on a 42-item survey, a decision tree was created that was able to detect ST patients with high accuracy (82.2%) using only 4 questions. The RaSST is therefore expected to be easily implementable in clinical practice.

Random Forest Classification to Predict Response to High-Definition Transcranial Direct Current Stimulation for Tinnitus Relief: A Preliminary Feasibility Study.

OBJECTIVES: Transcranial direct current stimulation (tDCS) of the right dorsolateral prefrontal cortex has been hypothesized to reduce tinnitus severity by modifying cortical activity in brain regions associated with the perception of tinnitus. However, individual response to tDCS has proven to be variable. We investigated the feasibility of using random forest classification to predict the response to high-definition (HD) tDCS for tinnitus relief. DESIGN: A retrospective analysis was performed on a dataset consisting of 99 patients with subjective tinnitus receiving six consecutive sessions of HD-tDCS at the Antwerp University Hospital. A baseline assessment consisted of pure-tone audiometry and a set of questionnaires including the Tinnitus Functional Index (TFI), Hospital Anxiety and Depression Scale, and Edinburgh Handedness Inventory. Random forest classification was applied to predict, based on baseline questionnaire scores and hearing levels, whether each individual responded positively to the treatment (defined as a decrease of at least 13 points on the TFI). Further testing of the model was performed on an independent cohort of 32 patients obtained from the tinnitus center at the University of Regensburg. RESULTS: Twenty-four participants responded positively to the HD-tDCS treatment. The random forest classifier predicted treatment response with an accuracy of 85.71% (100% sensitivity, 81.48% specificity), significantly outperforming a more traditional logistic regression approach. Performance of the classifier on an independent cohort was slightly but not significantly above chance level (71.88% accuracy, 66.67% sensitivity, 73.08% specificity). Feature importance analyses revealed that baseline tinnitus severity, co-occurrence of depressive symptoms and handedness were the most important predictors of treatment response. Baseline TFI scores were significantly higher in responders than in nonresponders. CONCLUSIONS: The proposed random forest classifier predicted treatment response with a high accuracy, significantly outperforming a more traditional statistical approach. Machine learning methods to predict treatment response might ultimately be used in a clinical setting to guide targeted treatment recommendations for individual tinnitus patients.

Endotypic Mechanisms of Successful Hypoglossal Nerve Stimulation for Obstructive Sleep Apnea.

Rationale: Approximately one-third of patients with obstructive sleep apnea (OSA) treated with hypoglossal nerve stimulation (HGNS) therapy are incomplete responders, despite careful patient selection based on baseline characteristics and drug-induced sleep endoscopy.Objectives: Here we use polysomnographic endotyping to assess the pathophysiological mechanisms underlying favorable versus incomplete responses to HGNS therapy.Methods: Baseline polysomnography data of the STAR (Stimulation Therapy for Apnea Reduction) trial were included. Raw baseline polysomnographic data from 91/126 patients were available for analysis. Traits-loop gain, arousal threshold, collapsibility, and muscle compensation-were calculated from the baseline polysomnography data according to Sands and colleagues (AJRCCM 2018, SLEEP 2018). Logistic regression assessed apnea-hypopnea index (AHI)-adjusted associations between HGNS response (>50% reduction in AHI to <10/h at 1 yr) and OSA traits.Measurements and Main Results: Overall, HGNS treatment reduced AHI from 30.7 (24.9-39.9) to 8.5 (4.0-19.5) events/h (P < 0.0001; median [quartiles 1-3]); N = 53/91 were responders. In adjusted analysis, a favorable response to therapy was independently associated with higher arousal threshold (odds ratio [95% confidence interval]: 6.76 [2.44-23.3], P = 0.001), greater compensation (odds ratio: 4.22 [1.70-12.55] per SD, P = 0.004), and lower loop gain (in milder collapsibility, per significant interaction, P = 0.003). The higher arousal threshold was evident in responders before adjusted analysis. Predicted responders had an approximately fourfold lower treatment AHI versus predicted nonresponders (4.9 [2.7-8.5] vs. 20.7 [10.9-29.7], P < 0.0001; median [quartiles 1-3]); differences remained significant after cross-validation.Conclusions: Favorable responses to HGNS therapy are associated with the pathophysiological traits causing OSA, particularly a higher arousal threshold. Along with established criteria, individuals with favorable traits could potentially be prioritized for precision HGNS therapy.This analysis was a secondary analysis of the STAR trial registered with clinicaltrials.gov (NCT01161420).

The smaller the frequency-to-place mismatch the better the hearing outcomes in cochlear implant recipients?

OBJECTIVE: To investigate the effect of frequency-to-place mismatch, i.e. the mismatch between the tonotopic frequency map in the cochlea and the frequency band that is assigned to an electrode contact of a cochlear implant (CI) at the same cochlear location on speech perception outcomes, using postoperative CT images. STUDY DESIGN: Retrospective observational single-centre study. METHODS: Retrospective pre- and postoperative clinical CT data of 39 CI recipients with normal cochlear anatomy were analysed in an otological surgical planning software. The tonotopic frequency at each electrode position was estimated using the Greenwood function. For each patient, frequency-to-place mismatch between the tonotopic frequency and the fitted centre frequency for each electrode contact was calculated. The influence of frequency-to-place mismatch on speech perception in noise at 6 and 12 months after CI activation was studied. RESULTS: A significant linear correlation was found between the frequency-to-place mismatch and speech perception in noise 6 months after cochlear implantation (p < 0.05). The smaller the frequency-to-place mismatch, the better the initial speech perception in noise results of the CI recipients. The significant effect disappeared after 12 months CI experience. CONCLUSION: The study findings support the idea of minimizing the frequency-to-place mismatch in CI recipients in order to pursue better initial speech perception in noise. Further research is needed to investigate the prospect of tonotopic fitting strategies based upon postoperative CT images of the exact locations of the electrode contacts.

The impact of cochlear implantation on health-related quality of life in older adults, measured with the Health Utilities Index Mark 2 and Mark 3.

PURPOSE: To determine the usefulness of the Health Utilities Index (HUI) in older cochlear implant (CI) recipients, the primary aims were: (1) to assess health-related quality of life (HRQoL), measured with HUI, in older CI candidates while comparing with age- and gender-matched normal-hearing controls; (2) to compare HRQoL after CI with the pre-operative situation, using HUI and the Nijmegen cochlear implant questionnaire (NCIQ). The difference between pre- and postoperative speech intelligibility in noise (SPIN) and in quiet (SPIQ) and the influence of pre-operative vestibular function on HRQoL in CI users were also studied. METHODS: Twenty CI users aged 55 years and older with bilateral severe-to-profound postlingual sensorineural hearing loss and an age- and gender-matched normal-hearing control group were included. HRQoL was assessed with HUI Mark 2 (HUI2), HUI Mark 3 (HUI3) and NCIQ. The CI recipients were evaluated pre-operatively and 12 months postoperatively. RESULTS: HUI3 Hearing (p = 0.02), SPIQ (p < 0.001), SPIN (p < 0.001) and NCIQ (p = 0.001) scores improved significantly comparing pre- and postoperative measurements in the CI group. No significant improvement was found comparing pre- and postoperative HUI3 Multi-Attribute scores (p = 0.07). The HUI3 Multi-Attribute score after CI remained significantly worse (p < 0.001) than those of the control group. Vestibular loss was significantly related to a decrease in HUI3 Multi-Attribute (p = 0.037) and HUI3 Emotion (p = 0.021) scores. CONCLUSION: The HUI is suitable to detect differences between normal-hearing controls and CI users, but might underestimate HRQoL changes after CI in CI users over 55.

Sound localization in patients with bilateral vestibulopathy.

PURPOSE: The goal of this study was to evaluate if bilaterally (partially) absent vestibular function during static sound localization testing, would have a negative impact on sound localization skills. Therefore, this study compared horizontal static sound localization skills of normal-hearing patients with bilateral vestibulopathy (BV) and healthy controls. METHODS: Thirteen normal-hearing patients with BV and thirteen age-matched healthy controls were included. Sound localization skills were tested using seven loudspeakers in a frontal semicircle, ranging from - 90° to + 90°. Sound location accuracy was analyzed using the root-mean-square error (RMSE) and the mean absolute error (MAE). To evaluate the severity of the BV symptoms, the following questionnaires were used: Dizziness Handicap Inventory (DHI), Oscillopsia severity questionnaire (OSQ), 12-item Spatial, Speech, and Qualities Questionnaire (SSQ12), and Health Utilities Index Mark 3 (HUI3). RESULTS: The RMSE and MAE were significantly larger (worse) in the BV group than in the healthy control group, with respective median RMSE of 4.6° and 0°, and a median MAE of 0.7° and 0°. The subjective reporting of speech perception, spatial hearing, and quality of life only demonstrated a moderate correlation between DHI (positive correlation) and HUI total score (negative correlation), and localization scores. CONCLUSION: Static sound localization skills of patients with BV were only mildly worse compared to healthy controls. However, this difference was very small and therefore most likely due to impaired cognitive function. The vestibular system does not seem to have a modulating role in sound localization during static conditions, and its impact is negligible in contrast to the impact of hearing impairment. Furthermore, the subjective reporting of speech perception, spatial hearing, and quality of life was not strongly correlated with localization scores.

Hyperacusis: demographic, audiological, and clinical characteristics of patients at the ENT department.

PURPOSE: To document whether patients with and without hyperacusis differ from each other on demographic, audiological, and clinical characteristics. METHODS: Based on the Hyperacusis Questionnaire's (HQ) cut-off (HQ > 28), a total of 2301 participants were divided into patients with and without hyperacusis. Demographic data, scores on self-reported questionnaires [Tinnitus Functional Index (TFI), Visual Analogue Scale of tinnitus loudness (VASloudness), Hospital Anxiety Depression Scale (HADS)], and audiological parameters were retrospectively analysed to determine differential factors between the two groups. RESULTS: In total, 10.9% of the patients was classified as hyperacusis patients (n = 251). They reported a significant, higher tinnitus severity (mean difference of 19 points on TFI) and mental distress (mean difference of 4 points on the HADS subscales) (p < 0.001) than patients without hyperacusis. Moreover, this group consisted of more women (45% % in hyperacusis group vs. 35% in non-hyperacusis group) and women scored significantly higher on the HQ (p < 0.001) and TFI (p < 0.01). CONCLUSION: Patients with hyperacusis have distinctive characteristics. The presence of hyperacusis in combination with tinnitus can indicate a higher need for psychoeducation. Patients that present themselves with hyperacusis without tinnitus complaints remain a minority, yet might be underdiagnosed. Hence, future studies should disentangle tinnitus from hyperacusis. In clinical practice, greater efforts are required to increase knowledge about hyperacusis as a primary or secondary complaint and to provide individualized treatment for these patients.

Long-term effects of a single psycho-educational session in chronic tinnitus patients.

PURPOSE: To evaluate the effects of a single psycho-educational session on tinnitus burden in chronic tinnitus patients. The session is organized at a tertiary referral center for otologic disorders at the University Hospital Antwerp as a group session (maximum of 10-15 patients a time) lasting for approximately 3-4 h. The session focusses on different aspects of tinnitus. METHODS: The current manuscript reports on 96 patients who completed the Tinnitus Functional Index (TFI), Visual Analogue Scale for mean loudness (VAS), Hyperacusis Questionnaire (HQ), and the Hospital Anxiety and Depression Scale (HADS) prior to treatment and at 6-month follow-up. The TFI was chosen as the primary outcome. Paired-samples T tests were performed to evaluate therapy effect at 6-month follow-up. In addition, a logistic regression model revealed baseline TFI/VAS scores and duration of tinnitus as contributing factors to a significant decrease of the TFI. RESULTS: The TFI total score showed a significant decrease (p < 0.001) at the 6-month follow-up time point. At follow-up, 75% of patients reported their tinnitus to be under control not requiring any additional treatment. The logistic regression model showed that patients with higher baseline TFI scores, lower baseline mean VAS loudness ratings, and shorter tinnitus duration were more likely to show clinically significant improvement on the TFI scale. CONCLUSIONS: Tinnitus Retraining Therapy or Cognitive Behavioural Therapy are effective, though very time-consuming and expensive treatments. A single psycho-educational group session was shown to be highly effective in decreasing the tinnitus burden, which increases feasibility and cost-effectiveness. TRIAL REGISTRATION: Not applicable as this is a retrospective reporting of tinnitus outcome in the daily clinical practice, not a clinical trial.

A New Pathogenic Variant in the TRIOBP Associated with Profound Deafness Is Remediable with Cochlear Implantation.

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.

Systematic Review of Quality of Life Assessments after Cochlear Implantation in Older Adults.

Cochlear implants (CI) have increasingly been adopted in older adults with severe to profound sensorineural hearing loss as a result of the growing and aging world population. Consequently, researchers have recently shown great interest in the cost-effectiveness of cochlear implantation and its effect on quality of life (QoL) in older CI users. Therefore, a systematic review and critical evaluation of the available literature on QoL in older adult CI users was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies were selected by searching MEDLINE (PubMed) and the Cochrane Library and by checking the reference lists of relevant articles. Inclusion criteria were as follows: (1) the study sample were adults aged 50 years and older with postlingual onset of bilateral severe to profound hearing loss, (2) all subjects received a multi-electrode CI, and (3) QoL was assessed before and after implantation. Out of 1,093 records, 18 articles were accepted for review. Several studies demonstrated significant positive effects of cochlear implantation on QoL in older adults, but high-level evidence-based medicine is lacking. An improvement of QoL was generally reported when using disease-specific instruments, which are designed to detect treatment-specific changes, whereas the outcomes of generic QoL questionnaires, assessing general health states, were rather ambiguous. However, only generic questionnaires would be able to provide calculations of the cost-effectiveness of CI and comparisons across patient populations, diseases, or interventions. Hence, generic and disease-specific QoL instruments are complementary rather than contradictory. In general, older CI users' QoL was assessed using a variety of methods and instruments, which complicated comparisons between studies. There is a need for a standardized, multidimensional, and comprehensive QoL study protocol including all relevant generic and disease-specific instruments to measure and compare QoL, utility, and/or daily life performance in CI users.

Cognitive Improvement After Cochlear Implantation in Older Adults With Severe or Profound Hearing Impairment: A Prospective, Longitudinal, Controlled, Multicenter Study.

OBJECTIVE: To compare the cognitive evolution of older adults with severe or profound hearing impairment after cochlear implantation with that of a matched group of older adults with severe hearing impairment who do not receive a cochlear implant (CI). DESIGN: In this prospective, longitudinal, controlled, and multicenter study, 24 older CI users were included in the intervention group and 24 adults without a CI in the control group. The control group matched the intervention group in terms of gender, age, formal education, cognitive functioning, and residual hearing. Assessments were made at baseline and 14 months later. Primary outcome measurements included the change in the total score on the Repeatable Battery for the Assessment of Neuropsychological Status for Hearing impaired individuals score and on its subdomain score to assess cognitive evolution in both groups. Secondary outcome measurements included self-reported changes in sound quality (Hearing Implant Sound Quality Index), self-perceived hearing disability (Speech, Spatial, and Qualities of Hearing Scale), states of anxiety and depression (Hospital Anxiety and Depression Scale), and level of negative affectivity and social inhibition (Type D questionnaire). RESULTS: Improvements of the overall cognitive functioning (p = 0.05) and the subdomain "Attention" (p = 0.02) were observed after cochlear implantation in the intervention group; their scores were compared to the corresponding scores in the control group. Significant positive effects of cochlear implantation on sound quality and self-perceived hearing outcomes were found in the intervention group. Notably, 20% fewer traits of Type D personalities were measured in the intervention group after cochlear implantation. In the control group, traits of Type D personalities increased by 13%. CONCLUSION: Intervention with a CI improved cognitive functioning (domain Attention in particular) in older adults with severe hearing impairment compared to that of the matched controls with hearing impairment without a CI. However, older CI users did not, in terms of cognition, bridge the performance gap with adults with normal hearing after 1 year of CI use. The fact that experienced, older CI users still present subnormal cognitive functioning may highlight the need for additional cognitive rehabilitation in the long term after implantation.

The Antwerp Vestibular Compensation Index (AVeCI): an index for vestibular compensation estimation, based on functional balance performance.

PURPOSE: To create an index that is a measure of the amount of vestibular compensation and for which only functional balance performance is needed. METHODS: The medical charts of 62 eligible peripheral vestibular dysfunction (PVD) patients were analyzed retrospectively. To be included, the following vestibulo-ocular reflex (VOR) and balance performance data had to be available: (1) caloric and sinusoidal harmonic acceleration test (SHA) and (2) standing balance sum-eyes closed (SBS-EC), Timed Up and Go Test and Dynamic Gait Index. Patients were divided into three groups: normal caloric- and SHA test (group 1), abnormal caloric- and normal SHA test (group 2, PVD compensated) and abnormal caloric- and SHA test (group 3, PVD uncompensated). Next to the use of non-parametric tests to study the VOR and balance variables, logistic regression was used to identify the balance measures that predict whether PVD patients were compensated or uncompensated. This resulted also in the construction of a continuous measure representing the degree of compensation. RESULTS: Logistic regression identified SBS-EC and age to classify uncompensated from compensated patients with sensitivity of 83.9% and specificity of 72.4%. Then an index was created, called the Antwerp Vestibular Compensation Index, AVeCI = - 50 + age × 0.486 + SBS-EC × 0.421. A patient belongs to the uncompensated group when AVeCI < 0 and to the compensated group when AVeCI > 0, with respective group means of - 5 and 5. CONCLUSION: AVeCI stages the degree of compensation of PVD patients and can serve to evaluate rehabilitation effects.

Two-phase survey on the frequency of use and safety of MRI for hearing implant recipients.

PURPOSE: Magnetic resonance imaging (MRI) is often used to visualize and diagnose soft tissues. Hearing implant (HI) recipients are likely to require at least one MRI scan during their lifetime. However, the MRI scanner can interact with the implant magnet, resulting in complications for the HI recipient. This survey, which was conducted in two phases, aimed to evaluate the safety and performance of MRI scans for individuals with a HI manufactured by MED-EL (MED-EL GmbH, Innsbruck, Austria). METHODS: A survey was developed and distributed in two phases to HEARRING clinics to obtain information about the use of MRI for recipients of MED-EL devices. Phase 1 focused on how often MRI is used in diagnostic imaging of the head region of the cochlear implant (CI) recipients. Phase 2 collected safety information about MRI scans performed on HI recipients. RESULTS: 106 of the 126 MRI scans reported in this survey were performed at a field strength of 1.5 T, on HI recipients who wore the SYNCHRONY CI or SYNCHRONY ABI. The head and spine were the most frequently imaged regions. 123 of the 126 scans were performed without any complications; two HI recipients experienced discomfort/pain. One recipient required reimplantation after an MRI was performed using a scanner that had not been approved for that implant. There was only one case that required surgical removal of the implant to reduce the imaging artefact. CONCLUSION: Individuals with either a SYNCHRONY CI or SYNCHRONY ABI from MED-EL can safely undergo a 1.5 T MRI when it is performed according to the manufacturer's safety policies and procedures.