Cochlear Implant Recipients Research Articles

Navigating the Usher Syndrome Genetic Landscape: An Evaluation of the Associations between Specific Genes and Quality Categories of Cochlear Implant Outcomes.

Usher syndrome (US) is a clinically and genetically heterogeneous disorder that involves three main features: sensorineural hearing loss, retinitis pigmentosa (RP), and vestibular impairment. With a prevalence of 4-17/100,000, it is the most common cause of deaf-blindness worldwide. Genetic research has provided crucial insights into the complexity of US. Among nine confirmed causative genes, MYO7A and USH2A are major players in US types 1 and 2, respectively, whereas CRLN1 is the sole confirmed gene associated with type 3. Variants in these genes also contribute to isolated forms of hearing loss and RP, indicating intersecting molecular pathways. While hearing loss can be adequately managed with hearing aids or cochlear implants (CIs), approved RP treatment modalities are lacking. Gene replacement and editing, antisense oligonucleotides, and small-molecule drugs hold promise for halting RP progression and restoring vision, enhancing patients' quality of life. Massively parallel sequencing has identified gene variants (e.g., in PCDH15) that influence CI results. Accordingly, preoperative genetic examination appears valuable for predicting CI success. To explore genetic mutations in CI recipients and establish correlations between implant outcomes and involved genes, we comprehensively reviewed the literature to gather data covering a broad spectrum of CI outcomes across all known US-causative genes. Implant outcomes were categorized as excellent or very good, good, poor or fair, and very poor. Our review of 95 cochlear-implant patients with US, along with their CI outcomes, revealed the importance of presurgical genetic testing to elucidate potential challenges and provide tailored counseling to improve auditory outcomes. The multifaceted nature of US demands a comprehensive understanding and innovative interventions. Genetic insights drive therapeutic advancements, offering potential remedies for the retinal component of US. The synergy between genetics and therapeutics holds promise for individuals with US and may enhance their sensory experiences through customized interventions.

Listening-Related Fatigue in New and Experienced Adult Cochlear Implant Users.

Active listening in everyday settings is challenging and requires substantial mental effort, particularly in noisy settings. In some cases, effortful listening can lead to significant listening-related fatigue and negatively affect quality of life. However, our understanding of factors that affect the severity of fatigue is limited. Hearing aids and cochlear implants (CIs) can improve speech understanding and thus, potentially, reduce listening effort and fatigue. Some research supports this idea for adult hearing aid users with mild-to-moderate hearing loss, but similar work in CI users is very limited. This study examined (1) longitudinal changes in listening-related fatigue in new and established CI users, and (2) relationships between demographic and audiologic factors and preimplantation and postimplantation listening-related fatigue. Participants included an experimental group of 48 adult CI candidates receiving either a unilateral implant (n = 46) or simultaneous, bilateral implants (n = 2) and a control group of 96 experienced (>12 months experience) adult CI users (50 unilateral, 46 bilateral). Listening-related fatigue was evaluated using the 40-item version of the Vanderbilt Fatigue Scale for Adults. Experimental group ratings were obtained before implantation and again at 0.5-, 1-, 2-, 3-, 6-, and 12-month(s) postactivation. Control group participants completed the scale twice-upon study entry and approximately 3 months later. Additional measures, including a social isolation and disconnectedness questionnaire, hearing handicap inventory, and the Effort Assessment Scale, were also administered at multiple time points. The role of these measures and select demographic and audiologic factors on preimplant and postimplant fatigue ratings were examined. Adult CI candidates reported significantly more fatigue, greater self-perceived hearing handicap, greater listening effort, and more social isolation than experienced adult CI users. However, significant reductions in fatigue and effort were observed within 2 weeks postimplantation. By 3 months, there were no significant differences in fatigue, effort, hearing handicap, or social isolation between new CI recipients and experienced CI users. Secondary analyses revealed that age at onset of hearing loss (before or after 2 years of age) and subjective hearing handicap contributed significantly to the variance of preimplantation fatigue ratings (those with higher handicap reported higher fatigue). In contrast, variance in postimplantation fatigue ratings was not affected by age of hearing loss onset but was affected by gender (females reported more fatigue than males) and subjective ratings of effort, handicap, and isolation (those reporting more effort, handicap, and isolation reported more fatigue). Listening-related fatigue is a significant problem for many CI candidates, as well as for many experienced unilateral and bilateral CI users. Receipt of a CI significantly reduced listening-related fatigue (as well as listening effort, hearing handicap, and social isolation) as soon as 2 weeks post-CI activation. However, the magnitude of fatigue-related issues for both CI candidates and experienced CI users varies widely. Audiologic factors, such as hearing loss severity and aided speech recognition, were not predictive of individual differences in listening-related fatigue. In contrast, strong associations were observed between perceived hearing handicap and listening-related fatigue in all groups suggesting fatigue-related issues may be a component of perceived hearing handicap.

Do Different Types of Microphones Affect Listening Effort in Cochlear Implant Recipients? A Pupillometry Study.

It is known that subjects with a cochlear implant (CI) need to exert more listening effort to achieve adequate speech recognition compared to normal hearing subjects. One tool for assessing listening effort is pupillometry. The aim of this study is to evaluate the effectiveness of adaptive directional microphones in reducing listening effort for CI recipients. We evaluated listening in noise and listening effort degree (by pupillometry) in eight bimodal subjects with three types of CI microphones and in three sound configurations. We found a correlation only between sound configurations and listening in noise score (p-value 0.0095). The evaluation of the microphone types shows worse scores in listening in noise with Opti Omni (+3.15 dB SNR) microphone than with Split Dir (+1.89 dB SNR) and Speech Omni (+1.43 dB SNR). No correlation was found between microphones and sound configurations and within the pupillometric data. Different types of microphones have different effects on the listening of CI patients. The difference in the orientation of the sound source is a factor that has an impact on the listening effort results. However, the pupillometry measurements do not significantly correlate with the different microphone types.

Factors that influence performance in pediatric cochlear implant recipients with cochlear nerve deficiency

Objectives Children with cochlear nerve deficiency (CND) have wide variability in outcomes with cochlear implant (CI) use. The current study aims to report a large cohort of pediatric CI recipients with CND and to evaluate for factors that may predict improved performance. Methods The current study is a retrospective review of pediatric CI recipients with CND at a tertiary academic hospital. Variables including cochlear nerve status (hypoplasia vs aplasia), age at implantation, cochleovestibular malformation, bony cochlear nerve aperture, internal auditory canal aperture, and cognitive delay were evaluated for predictors of postoperative performance. A stepwise multinomial regression analysis was performed. Results Forty-seven CI recipients (54 ears) were included in the analysis. A majority (59%) showed auditory capabilities with their CI. Twenty percent of recipients achieved some level of open-set speech perception with their CI. The regression analysis identified cochlear nerve status and cognitive delay as predictors of performance. CI recipients with cochlear nerve hypoplasia had significantly improved performance compared to those with aplasia (p = 0.003). Recipients with cognitive delay had more limited benefit than those without cognitive delay (p = 0.033). Conclusions Children with CND can benefit from CI use, with outcomes spanning from non-use to development of spoken language. Predictive factors for improved performance include a lack of cognitive delay and cochlear hypoplasia rather than aplasia. These can be important considerations for parent counseling and decision making.

Computer-based Auditory Training by New Adult Cochlear Implant Recipients Is Associated With Durable Improvements in Cochlear Implant Quality of Life.

The process of adapting to communicate with a cochlear implant (CI) is complex. The use of auditory training after cochlear implantation may help to facilitate improvements in postoperative speech recognition and quality-of-life outcomes in new adult CI recipients. However, the effectiveness of auditory training remains uncertain and long-term effects have not been examined in a large sample of new adult CI users. As such, the objective of this study was to examine the influence of common forms of auditory training on speech recognition and CI-related quality-of-life (CI-related QOL) outcomes at 1 year after cochlear implantation. We hypothesized that patients who reported use of computer-based auditory training (CBAT) would show improved speech and CIQOL-35 Profile scores at 1 year after activation of their implant, compared with their peers. This study was designed as a prospective study and was undertaken at a tertiary academic CI center. Participants included 114 adults undergoing cochlear implantation for bilateral hearing loss. Patients serially self-reported use of the following types of post-CI auditory training over their first-year postactivation: (1) face-to-face training (e.g., speech-language pathologist), (2) passive home-based training (e.g., listening to audiobooks), and (3) CBAT (e.g., self-directed software). Outcomes measures for this study included change in Consonant-Nucleus-Consonant phoneme (CNCp), CNC word (CNCw), AzBio sentences in quiet, and CIQOL-35 Profile global and domain scores from pre-CI to 12-mo post-CI. Of 114 patients, 94 (82.5%) used one or more auditory training resources. Of these, 19.3% used face-to-face training, 67.5% passive home-based training, and 46.5% CBAT. Of 114 patients, 73 had complete CIQOL data. At 12 mo, only CBAT use was associated with significantly greater improvements in global and all domain-specific CIQOL scores ( d -range = 0.72-0.87), compared with those not using CBAT. Controlling for demographics and use of multiple training resources, CBAT remained the strongest positive predictor of CIQOL improvement, with significant associations with global score (ß = 12.019[4.127,19.9]) and all domain scores at 12-mo post-CI: communication (ß = 11.937[2.456,21.318), emotional (ß = 12.293[1.827,22.759), entertainment (ß = 17.014[5.434,28.774), environment (ß = 13.771[1.814,25.727]), listening effort (ß = 12.523[2.798,22.248]), and social (ß = 18.114[7.403,28.826]). No significant benefits were noted with use of CBAT or any other form of auditory training and speech recognition scores at 12-mo post-CI ( d -range = -0.12-0.22). Auditory training with CBAT was associated with improved CI-related QOL outcomes at 12-mo post-CI. Given its availability and low cost, this study provides evidence to support using CBAT to improve real-world functional abilities in new adult CI recipients.

Cochlear Implant Qualification in Noise Versus Quiet: Do Patients Demonstrate Similar Postoperative Benefits?

To assess patient factors, audiometric performance, and patient-reported outcomes in cochlear implant (CI) patients who would not have qualified with in-quiet testing alone. Retrospective chart review. Tertiary referral center. Adult CI recipients implanted between 2012 and 2022 were identified. Patients with preoperative AzBio Quiet > 60% in the implanted ear, requiring multitalker babble to qualify, comprised the in-noise qualifying (NQ) group. NQ postoperative performance was compared with the in-quiet qualifying (QQ) group using CNC, AzBio Quiet, and AzBio +5 dB signal-to-noise ratio. Speech, Spatialand Qualities of Hearing Scale (SSQ), Cochlear Implant Quality of Life scale (CIQOL-10), and daily device usage were also compared between the groups. The QQ group (n = 771) and NQ group (n = 67) were similar in age and hearing loss duration. NQ had higher average preoperative and postoperative speech recognition scores. A larger proportion of QQ saw significant improvement in CNC and AzBio Quiet scores in the CI-only listening condition (eg, CI-only AzBio Quiet: 88% QQ vs 51% NQ, P < .001). Improvement in CI-only AzBio +5 dB and in all open set testing in the best-aided binaural listening condition was similar between groups (eg, Binaural AzBio Quiet 73% QQ vs 59% NQ, P = .345). Postoperative SSQ ratings, CIQOL scores, and device usage were also equivalent between both groups. Patients who require in-noise testing to meet CI candidacy demonstrate similar improvements in best-aided speech perception and patient-reported outcomes as in-QQ, supporting the use of in-noise testing to determine CI qualification for borderline CI candidates.

A Comparison of Montreal Cognitive Assessment Scores among Individuals with Normal Hearing and Cochlear Implants.

The Montreal Cognitive Assessment (MoCA) is a cognitive screening tool that has 4 of 10 test items heavily dependent on auditory input, potentially leaving hearing-impaired (HI) individuals at a disadvantage. Previous work found that HI individuals scored lower than normal-hearing (NH) individuals on the MoCA, potentially attributed to the degraded auditory signals negatively impacting the ability to commit auditory information to memory. However, there is no research comparing how cochlear implant (CI) recipients perform on the MoCA relative to NH and HI individuals. This study aimed to (1) examine the effect of implementing three different hearing-adjusted scoring methods for a group of age-matched CI recipients and NH individuals, (2) determine if there is a difference between the two groups in overall scores and hearing-adjusted scores, and (3) compare scores across our CI and NH data to the published HI data for all scoring methods. We hypothesized that (1) scores for CI recipients would improve with implementation of the hearing-adjusted scoring methods over the original method, (2) CI recipients would score lower than NH participants for both original and adjusted scoring methods, and (3) the difference in scores between NH and CI listeners for both adjusted and unadjusted scores would be greater than that reported in the literature between NH and HI individuals due to the greater severity of hearing loss and relatively poor spectral resolution of CIs. A total of 94 adults with CIs and 105 adults with NH were initially enrolled. After age-matching the two groups and excluding those who self-identified as NH but failed a hearing screening, a total of 75 CI participants (mean age 61.2 y) and 74 NH participants (mean age 58.8 y) were administered the MoCA. Scores were compared between the NH and CI groups, as well as to published HI data, using the original MoCA scoring method and three alternative scoring methods that excluded various auditory-dependent test items. MoCA scores improved for all groups when two of the three alternative scoring methods were used, with no significant interaction between scoring method and group. Scores for CI recipients were significantly poorer than those for age-matched NH participants for all scoring methods. CI recipients scored better than the published data for HI individuals; however, the HI group was not age matched to the CI and NH groups. MoCA scores are only partly affected by the potentially greater cognitive processing required to interpret degraded auditory signals. Even with the removal of the auditory-dependent items, CI recipients still did not perform as well as the age-matched NH group. Importantly, removing auditory-dependent items significantly and fundamentally alters the test, thereby reducing its sensitivity. This has important limitations for administration and interpretation of the MoCA for people with hearing loss.

Patient and Device Factors Contributing to Electrically Evoked Stapedial Reflex Thresholds in Cochlear Implanted Adults

Introduction: Optimal cochlear implant (CI) outcomes are due to, at least in part, appropriate device programming. Objective measures, such as electrically evoked stapedial reflex thresholds (ESRTs), can be used to more accurately set programming levels. However, underlying factors that contribute to ESRT levels are not well understood. The objective of the current study was to analyze how demographic variables of patient sex and age, along with CI electrode location, influence ESRTs in adult CI recipients. Methods: A single institution retrospective review was performed. Electronic medical records, CI programming records, and clinic database of postoperative computerized tomography were reviewed to gather information regarding patient demographics, ESRTs, and electrode array metrics including medial-lateral distance and scalar location. Linear mixed models were constructed to determine how demographic variables and electrode position influence ESRTs recorded in 138 adult CI recipients. Results: ESRTs were significantly affected by recipient age, with older listeners demonstrating higher ESRT levels. On average, males had higher ESRT levels when compared to females. In a subset of the study sample, ESRT levels increased with increasing medial-lateral distance; however, there was not a statistically significant effect of electrode type (lateral/straight arrays compared to perimodiolar arrays). ESRTs were not affected by scalar location. Discussion/Conclusions: The results suggest that key demographic and electrode position characteristics influence the level of ESRTs in adult CI recipients. While ESRTs are widely used to assist with CI programming, underlying factors are not well understood. The significant factors of aging and sex could be due to middle ear mechanics or neural health differences. However, further data are needed to better understand these associations.

Outcome Prediction of Speech Perception in Quiet and in Noise for Cochlear Implant Candidates Based on Pre-Operative Measures.

(1) Background: The fitting of cochlear implants (CI) is an established treatment, even in cases with considerable residual hearing but insufficient speech perception. The aim of this study was to evaluate a prediction model for speech in quiet and to provide reference data and a predictive model for postoperative speech perception in noise (SPiN) after CI provision. (2) Methods: CI candidates with substantial residual hearing (either in hearing threshold or in word recognition scores) were included in a retrospective analysis (n = 87). Speech perception scores in quiet 12 months post-surgery were compared with the predicted scores. A generalized linear model was fitted to speech reception thresholds (SRTs) after CI fitting to identify predictive variables for SPiN. (3) Results: About two-thirds of the recipients achieved the expected outcome in quiet or were better than expected. The mean absolute error of the prediction was 13.5 percentage points. Age at implantation was the only predictive factor for SPiN showing a significant correlation (r = 0.354; p = 0.007). (4) Conclusions: Outcome prediction accuracy for speech in quiet was comparable to previous studies. For CI recipients in the included study population, the SPiN outcome could be predicted only based on the factor age.

Intraoperative Handheld Digital X-ray for Assessment of Intracochlear Positioning of Electrode Arrays in Recipients of Cochlear Implants.

Objectives: This study aims to evaluate the practicality of handheld digital X-ray in determining the position of the electrode array following Cochlear implantation (CI). Methods: A retrospective study was conducted involving 11 patients (12 ears) who underwent intraoperative imaging via handheld X-ray (MINE ALNU®, OTOM, Gwangju, South Korea) post-CI between December 2021 and January 2023. Immediate confirmation of the correct electrode array placement in the cochlea was achieved, with subsequent comparisons made to C-arm image and postoperative transorbital view X-ray. Results: Rapid intraoperative imaging was achieved in all instances. The electrode types used included 9 Nucleus slim modiolar electrodes, 1 Nucleus contour electrode, and 2 Medel flex26 electrodes. A malpositioned electrode array was detected in one patient. The handheld digital X-ray also adeptly visualized the electrodes implanted in pediatric patients. Conclusions: The use of intraoperative handheld digital X-ray using MINE ALNU® proves to be a safe, efficient, straightforward, and reliable method for immediate identification of an inserted electrode array. It has potential to replace the traditional C-arm X-ray for verifying electrode positioning in the operating room.

Speech Understanding and Subjective Listening Effort in Noise With Different OTEs and Sound Processing Technologies.

To determine speech reception thresholds (SRTs) in noise and subjective listening effort (LE) in cochlear implant (CI) recipients for application of three sound processing (SP) technologies with two off-the-ear (OTE) CI sound processors, a fixed moderately directional microphone (Standard), an adaptive directional microphone (Beam), and the spatial noise-reduction setting ForwardFocus, with the Kanso 2 (OTE2), and Beam with the Kanso (OTE1). Prospective repeated measures, within-subject design. Single tertiary-referral center. Twenty CI recipients with bilateral severe-to-profound sensorineural hearing loss. SRTs were assessed in two spatial configurations with frontal speech and noise from 90-180-270 degrees (S0N90-180-270) or from the CI side (S0NCI). SRTs were obtained for sentences of the Oldenburg sentence test presented in International Collegium of Rehabilitative Audiology (ICRA) noise ICRA5-250. LE for speech understanding in noise was evaluated in S0N90-180-270 and assessed in effort scale categorical units (ESCUs) by using Adaptive Categorical Listening Effort Scaling (ACALES). LEs at 5-dB signal-to-noise ratio (SNR) were calculated from fitted psychometric curves. With OTE2 in S0N90-180-270, SRT with ForwardFocus (-4.28 dB SNR) was better than with Beam (-3.13 dB SNR) and Standard (0.43 dB SNR). ForwardFocus showed lower LE5dB (2.61 ESCU) compared with Beam (4.60 ESCU) and Standard (5.32 ESCU). In a comparison of both OTEs in S0N90-180-270 regarding best-performing SP technology, ForwardFocus with OTE2 yielded a better SRT and better LE5dB than Beam with OTE1 (SRT: -1.70 dB SNR; LE5dB: 4.00 ESCU). With OTE2 in S0NCI, SRT was improved with ForwardFocus (-2.78 dB SNR) compared with Beam (-1.23 dB SNR) and Standard (1.83 dB SNR). With respect to SP technology and OTE, CI recipients experience best SRT and lowest LE in S0N90-180-270 when using ForwardFocus with OTE2. ACALES is feasible for assessing subjective LE in CI recipients.

Advancing Cochlear Implant Programming: X-ray Guided Anatomy-Based Fitting.

Anatomy-based fitting (ABF) is a new research area in the field of cochlear implants (CIs). Despite the reported benefits and acceptable levels of ABF among CI recipients, some limitations remain, like the postoperative computed tomography (CT) scan, which is preferred for confirming electrode array insertion. This study aimed to investigate the feasibility of using plain film radiography (X-ray) for postoperative electrode detection and for building ABF as an alternative to CT. A total of 53 ears with CI were studied. All cases had routine post-insertion X-rays in the cochlear view and additionally underwent postoperative CT. The insertion angles and center frequencies measured by two independent observers were compared for each imaging modality. The angular insertion depth and center frequencies resulting from the X-ray and CT scans were then compared. No significant differences were observed between the X-ray- and CT-measured angles for the electrode contacts. Radiographic measurements between the two readers showed an almost perfect (≥0.8) or substantial (0.71) intraclass correlation coefficient along the electrode contacts. X-ray images showed a mean difference of 4.7 degrees from CT. The mean semitone deviation of the central frequency between the CT and X-ray images was 0.6. X-ray imaging provides a valid and easy-to-perform alternative to CT imaging, with less radiation exposure and lower costs. The radiographs showed excellent concordance with the CT-measured angular insertion depth and consequently with the central frequency for most electrode contacts. Therefore, plain X-ray could be a viable alternative in building ABF for the CI recipients.

A Single Intratympanic Triamcinolone Acetonide Administration Elicits Long-Term Reduction in Impedances Following Cochlear Implantation.

Intracochlear fibrosis and inflammation remain important limitations in cochlear implantation (CI). Glucocorticoids are routinely used to ameliorate the inflammatory response following CI. This study investigates the long-term effects of an intratympanically-applied triamcinolone-acetonide suspension on intracochlear impedance changes in CI recipients and investigates differences in drug concentrations and timepoints of injection. A total of 87 patients were included in the study, of whom 39 received an intratympanically-applied triamcinolone-acetonide suspension at either 10 or 40 mg/ml, 1 hour or 24 hours prior to cochlear implantation, while 48 patients served as an untreated control group. Electrode impedances were measured and compared over a period of 3 years following cochlear implantation. The preoperative intratympanic application of a triamcinolone-acetonide suspension resulted in significantly lower mean impedances following cochlear implantation compared with an untreated control group at first fitting (4.66 ± 1.3 kΩ to 5.90 ± 1.4 kΩ, P = .0001), with sustained reduction observed over 3 months. A sustained reduction was observed after spatial grouping of the electrode contacts, with significant improvements in both the middle cochlear region over a 24 month period (from 3.97 ± 1.3 kΩ to 5.85 ± 1.3 kΩ, P = .049) and the basal region over a 6 month period (from 5.02 ± 1.3 kΩ to 5.85 ± 1.3 kΩ, P = .008). The injection of 10 mg/ml of triamcinolone-acetonide 1 hour prior to cochlear implantation resulted in higher impedances compared with 40 mg/ml and 24 hour time interval until surgery. A single preoperative intratympanic injection of triamcinolone-acetonide significantly reduces electrode impedances across the entire cochlea. This effect is sustained for up to 2 years, after which impedances gradually equalize between the groups. A preoperative triamcinolone-acetonide injection could therefore be a favorable approach to attenuate the immediate tissue response following cochlear implantation.

Pathophysiology of Facial Nerve Stimulation and Its Implications for Electrical Stimulation in Cochlear Implants.

A small number of cochlear implant (CI) users experience facial nerve stimulation (FNS), which can manifest as facial twitching. In some patients, this can be resolved by adjusting the electrical stimulation parameters. However, for others, facial stimulation can significantly impair CI outcomes or even prevent its use. The exact mechanisms underlying FNS are unclear and may vary among patients. Transimpedance measurements were used to assess lateral and longitudinal spread of current within 15 cochlea of nucleus CI recipients with FNS (13 unilateral recipients and 1 bilateral recipient). We compared the transimpedance measurements with programming parameters from clinical visits and pre- and postoperative temporal bone computed tomography (CT) scans to identify factors that may contribute to FNS in each CI ear. In nine ears, transimpedance curves showed inflection, which suggests a localized current sink within the cochlea. This indicates a low-impedance pathway through which current exits the cochlea and stimulates the labyrinthine segment of the facial nerve canal. Electrodes near this current sink were disabled or underfit to minimize facial stimulation. In the other seven ears, current flow peaked toward the basal end of the cochlea, suggesting that current exits through the round window or other structures near the basal end of the cochlea, stimulating the tympanic segment of the facial nerve. Objective transimpedance measurements can be used to elucidate the mechanisms of FNS and to develop strategies for optimizing electrical stimulation parameters and speech coding to minimize or eliminate FNS in a small subset of CI users.

Correlation of Scalar Cochlear Volume and Hearing Preservation in Cochlear Implant Recipients with Residual Hearing.

Preservation of residual hearing is one of the main goals in cochlear implantation. There are many factors that can influence hearing preservation after cochlear implantation. The purpose of the present study was to develop an algorithm for validated preoperative cochlear volume analysis and to elucidate the role of cochlear volume in preservation of residual hearing preservation after atraumatic cochlear implantation. Retrospective analysis. Tertiary referral center. A total of 166 cochlear implant recipients were analyzed. All patients were implanted with either a MED-EL (Innsbruck, Austria) FLEXSOFT (n = 3), FLEX28 (n = 72), FLEX26 (n = 1), FLEX24 (n = 41), FLEX20 (n = 38), or FLEX16 (n = 11, custom made device) electrode array through a round window approach. Main outcome measures: Cochlear volume as assessed after manual segmentation of cochlear cross-sections in cone beam computed tomography, and preservation of residual hearing 6 months after implantation were analyzed. The association between residual hearing preservation and cochlear volume was then assessed statistically. Rapid and valid cochlear volume analysis was possible using the individual cross-sections and a newly developed and validated algorithm. Cochlear volume had the tendency to be larger in patients with hearing preservation than in those with hearing loss. Significant correlations with hearing preservation could be observed for the basal width and length of the basal turn. Preservation of residual hearing after cochlear implantation may depend on cochlear volume but appears to be influenced more strongly by other cochlear dimensions.

Outcomes of Cochlear Implant Recipient With ANSD: Intra and Post Operative Findings, Progress in Audition and Speech Skills.

Auditory neuropathy spectrum disorder (ANSD) is a distinct type of SNHL that is characterized by the presence of otoacoustic emissions and/or cochlear microphonics. Cochlear implantation was initially not recommended for ANSD children, later studies showed variable outcomes among ANSD. CI is currently the intervention option of choice for many children with ANSD who are unable to obtain benefit from conventional amplification. To review experiences with some of the preoperative and postoperative findings in a child who was diagnosed with auditory neuropathy and provided with cochlear implant. To describe changes in auditory function, which enabled to have significant improvement in hearing and communication skills through auditory verbal therapy (AVT) and regular follow ups. Pre and postoperative, findings in cochlear implant recipient who was diagnosed with ANSD. Child received complete medical examinations, including related consultations in audiology, otorhinolaryngology, paediatrics, neurology, psychology, speech language pathology, and radiology. A 3-year-old-female have brought to the hospital with a C/o not responding to sounds, name call and unable to speak. Medical and Audiological evaluations were initiated. The hearing assessments of thechild included appropriate behavioural audiometric techniques, objective measures of middle ear function, acoustic reflex studies, transient evoked (TEOAE), distortion product (DPOAE) otoacoustic emissions and auditory brainstem responses (ABR). Implanted with (HiRes Ultra CI HiFocus SlimJ Electrode), and objective measures were recorded intraoperatively electrode impedances and neural response telemetry (NRT) to assess the outcomes technically. These intraoperative objective measures were used to help program the speech processor for the child. Postoperatively, child has had regular follow-up with otorhinolaryngologist to assure complete healing of the surgical incision, to assess their general medical conditions, and audiologist for switch-on (speech processor) followed by mapping. The hearing and communication skills have been assessed, also continued Auditory Verbal Therapy (AVT) on a regular basis. Postoperatively, objective measures were recorded in regular intervals and monitored with therapy outcomes. The child has shown significant improvement in sound detection, speech perception abilities, communication skills and shown evidence of progression of good NRT results, which were recorded and had no postoperative complications. Experience with cochlear implantation for child diagnosed with ANSD that effectively received and benefited from CI. A detailed and careful evaluations, audiological follow-ups and tailored rehabilitation plans, can be considered as a beneficial management approach for CI, especially who diagnosed with ANSD. The regular use of cochlear implant in this diagnosis can lead to a clear increase in speech comprehension, development and overall progress in quality of life. Success or lack of success with a CI appears to be somewhat dependent on the specific site of lesion (pre- or post-synaptic). Currently there are no clinical measures available to diagnose the specific site of lesion. Indeed, CI appears to be an effective rehabilitation modality for ANSD patients. This may be explained by the fact that the implanted electrode delivers synchronized electrical impulses directly to the auditory nerve, bypassing the presynaptic IHCs and its synapse involved in the unsynchronized firing of the auditory nerve described in ANSD. However, genetic studies that have proven to be essential in the knowledge of underlying mechanisms of ANSD represent a promising therapeutic approach in the management of ANSD.

Systematic Review of Auditory Training Outcomes in Adult Cochlear Implant Recipients and Meta-Analysis of Outcomes.

Objective: to review evidence on the efficacy of auditory training in adult cochlear implant recipients. Data Sources: PRISMA guidelines for a systematic review of the literature were followed. PubMed, Scopus, and CINAHL databases were queried on 29 June 2023 for terms involving cochlear implantation and auditory training. Studies were limited to the English language and adult patient populations. Study Selection: Three authors independently reviewed publications for inclusion in the review based on a priori inclusion and exclusion criteria. Inclusion criteria encompassed adult cochlear implant populations, an analysis of clinician- or patient-directed auditory training, and an analysis of one or more measures of speech recognition and/or patient-reported outcome. Exclusion criteria included studies with only pediatric implant populations, music or localization training in isolation, and single-sample case studies. Data Extraction: The data were collected regarding study design, patient population, auditory training modality, auditory training timing, speech outcomes, and data on the durability of outcomes. A quality assessment of the literature was performed using a quality metric adapted from the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) Working Group guidelines. Data Synthesis and Meta-Analysis: Data were qualitatively summarized for 23 studies. All but four studies demonstrated significant improvement in at least one measured or patient-reported outcome measure with training. For 11 studies with sufficient data reporting, pre-intervention and post-intervention pooled means of different outcome measures were compared for 132 patients using meta-analysis. Patient-direct training was associated with significant improvement in vowel-phoneme recognition and speech recognition in noise (p < 0.05 and p < 0.001, respectively), and clinician-directed training showed significant improvement in sentence recognition in noise (p < 0.001). Conclusions: The literature on auditory training for adult cochlear implant recipients is limited and heterogeneous, including a small number of studies with limited levels of evidence and external validity. However, the current evidence suggests that auditory training can improve speech recognition in adult cochlear implant recipients.

Localization of Low- and High-Frequency Sounds in Cochlear Implant Recipients Using a Contralateral Hearing Aid.

The ability to localize sounds is partly recovered in patients using a cochlear implant (CI) in one ear and a hearing aid (HA) on the contralateral side. Binaural processing seems effective at least to some extent, despite the difference between electric and acoustic stimulation in each ear. To obtain further insights into the mechanisms of binaural hearing in these listeners, localization of low- and high-frequency sounds was tested. The study used a within-subject design, where participants were tasked with localizing sound sources in the horizontal plane. The experiment was conducted in an anechoic chamber, where an array of seven loudspeakers was mounted along the 24 azimuthal angle span from -90° to +90°. Stimuli were applied with different frequencies: broadband noise and high- and low-frequency noise. Ten CI recipients participated in the study. All had an asymmetric hearing loss with a CI in the poorer ear and an HA on the contralateral side. Accuracy of sound localization in terms of angular error and percentage of correct localization scores. The median angular error was 40° in bimodal conditions for both broadband noise and high-frequency noise stimuli. The angular error increased to 47° for low-frequency noise stimuli. In the unilaterally aided condition with an HA, only a median angular error of 78° was observed. Irrespective of the frequency composition of the stimuli, this group of bimodal listeners showed some ability to localize sounds. Angular errors were larger than those reported in the literature for bilateral CI users or single-sided deaf listeners with a CI. In the unilateral listening condition with HA, only localization of sounds was not possible for most subjects.

Audiologists' attitudes and practice toward referring for psychosocial intervention with cochlear implant patients.

Hearing loss is associated with a range of poor psychosocial outcomes. Cochlear implants (CI) are an available treatment option for significant hearing loss and have been linked to improved quality of life in patients. Evidence suggests that audiologists lack the skills to appropriately detect, address, and refer for psychosocial needs among patients with hearing loss. The objective of this study is to examine the attitudes and practice patterns related to psychosocial care among audiologists who work with CI users. A cross-sectional survey was administered to clinical audiologists who work with CI recipients in the United States. The survey evaluated participants' attitudes toward psychosocial services and factors that contribute to their abilities to address the psychosocial needs of their patients. Additionally, participants were surveyed about their practice patterns including the use of psychosocial screeners, clinical protocols regarding psychosocial care, and referral patterns for coordinated psychosocial services. Descriptive statistics were used to summarize survey responses. Sixty-eight audiologists completed the survey. Of these audiologists, a majority (73.6%) held the attitude that most or all CI patients would benefit from psychosocial intervention. Despite clinicians' recognition of psychosocial needs in this population, over 90% of participants reported never screening for psychosocial symptoms. Additionally, a majority of respondents indicated that they seldom refer their patients for psychosocial services, with referrals occurring less than half the time (58%) or never (27%). Additionally, few audiologists reported utilizing protocols or resources for guiding psychosocial practices. Audiologists indicated that the primary factors that influence their psychosocial practices include time available to spend with the patient and their comfort level in counseling. Audiologists working with CI patients recognize the potential benefit of psychosocial intervention in this population. Nevertheless, audiologists encounter barriers in clinical practice which limit their ability to identify and address the psychosocial needs of their patients. Strategies designed to enhance audiologists' capacity to recognize the psychosocial needs of CI users, in addition to improved interprofessional practice on CI teams, implies significant opportunities to improve the provision of patient-centered hearing care.

Can Mandarin-speaking prelingual deaf adults benefit from cochlear implant?

Background With advances in cochlear implant (CI) technology, prelingual deaf adults may experience improved speech perception and quality of life (QoL). It is still a challenge for Mandarin-speaking CI user with tone recognition due to CI technology focused on intonation language. Objectives To evaluate the long-term post-CI auditory performance and social-emotional benefits in prelingual deaf Mandarin-speaking adults and the difference between them and post-lingual deaf adults. Material and Methods Fifty-five adult implanted ears were included (forty-six postlingual deaf group; nine prelingual deaf group). Post-CI long-term outcomes were using vowels, consonants, disyllabic words, Mandarin monosyllable words, categories of audiology performance, speech intelligibility rating, subjective social-emotional questionnaires. Results Post-CI auditory performance and speech intelligibility of prelingual deafness adults was significantly inferior to that of those with postlingual deafness. However, both groups presented improved social-emotional benefits, with no significant difference between both groups. Conclusions Adult CI recipients who deaf before the age of 4 can experience benefits in social-emotional life functioning, regardless of their limited auditory performance and speech intelligibility. Therefore, prelingual Mandarin-speaking deaf adults, especially those using oral communication, can be considered as relative indications for cochlear implantation. Significance To clarify and validate the benefits among Mandarin-speaking prelingual deaf adult recipients.