AI model for predicting adult cochlear implant candidacy using routine behavioral audiometry.

OBJECTIVE: To describe an AI model to facilitate adult cochlear implant candidacy prediction based on basic demographical data and standard behavioral audiometry. METHODS: A machine-learning approach using retrospective demographic and audiometric data to predict candidacy CNC word scores and AzBio sentence in quiet scores was performed at a tertiary academic center. Data for the model were derived from adults completing cochlear implant candidacy testing between January 2011 and March 2023. Comparison of the prediction model to other published prediction tools and benchmarks was performed. RESULTS: The final dataset included 770 adults, encompassing 1045 AzBio entries, and 1373 CNC entries. Isophoneme scores and word recognition scores exhibited strongest importance to both the CNC and AzBio prediction models, followed by standard pure tone average and low-frequency pure tone average. The mean absolute difference between the predicted and actual score was 15 percentage points for AzBio sentences in quiet and 13 percentage points for CNC word scores, approximating anticipated test-retest constraints inherent to the variables incorporated into the model. Our final combined model achieved an accuracy of 87 % (sensitivity: 90 %; precision: 80 %). CONCLUSION: We present an adaptive AI model that predicts adult cochlear implant candidacy based on routine behavioral audiometric and basic demographical data. Implementation efforts include a public-facing online prediction tool and accompanying smartphone program, an embedded notification flag in the electronic medical record to alert providers of potential candidates, and a program to retrospectively engage past patients who may be eligible for cochlear implantation based on audiogram results.

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

OBJECTIVES: 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. DESIGN: 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. RESULTS: 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. CONCLUSIONS: 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.

Implementation Strategy for Highly-Coordinated Cochlear Implant Care With Remote Programming: The Complete Cochlear Implant Care Model.

OBJECTIVE: To introduce and discuss implementation strategy for the Complete Cochlear Implant Care (CCIC) model, a highly-coordinated cochlear implant (CI) care delivery model requiring a single on-site visit for preoperative workup, surgery, and postoperative programming. STUDY DESIGN: Prospective, nonrandomized, two-arm clinical trial. SETTING: Tertiary referral CI center. PATIENTS: Adults who meet audiologic criteria for cochlear implantation. INTERVENTIONS: Cochlear implantation, coordinated care delivery, including remote programming. MAIN OUTCOME MEASURES: Care delivery model feasibility and process implementation. RESULTS: Patients determined to be likely CI candidates based on routine audiometry are eligible for enrollment. The CCIC model uses telemedicine and electronic educational materials to prepare patients for same-day on-site consultation with CI surgery, same or next-day activation, and postoperative remote programming for 12 months. Implementation challenges include overcoming inertia related to the implementation of a new clinical workflow, whereas scalability of the CCIC model is limited by current hardware requirements for remote programming technology. A dedicated CCIC process coordinator is critical for overcoming obstacles in implementation and process improvement through feedback and iterative changes. Team and patient-facing materials are included and should be tailored to fit each unique CI program looking to implement CCIC. CONCLUSION: The CCIC model has the potential to dramatically streamline hearing healthcare delivery. Implementation requires an adaptive approach, as obstacles may vary according to institutional infrastructure and policies.

Language and Audiological Outcomes Among Infants Implanted Before 9 and 12 Months of Age Versus Older Children: A Continuum of Benefit Associated With Cochlear Implantation at Successively Younger Ages.

OBJECTIVE: To compare language and audiological outcomes among infants (<9 and <12 mo) and older children receiving cochlear implantation (CI). STUDY DESIGN: Retrospective chart review. SETTING: Tertiary academic referral center. PATIENTS: Pediatric patients receiving CI between October 1995 and October 2019. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Most recent language and audiological assessment scores were evaluated by age group. RESULTS: A total of 118 children were studied, including 19 who were implanted <9 months of age, 19 implanted 9 to <12 months of age, and 80 implanted 12 to <36 months of age. The mean duration of follow-up was 7.4 ±â€Š5.0 years. Most recent REEL-3 receptive (88 ±â€Š12 vs. 73 ±â€Š15; p = 0.020) and expressive (95 ±â€Š13 vs. 79 ±â€Š12; p = 0.013) communication scores were significantly higher in the <9 months group compared to the 9 to <12 months group. PLS and OWLS auditory comprehension and oral expression scores were significantly higher in the <12 months group compared to the 12 to <36 months group. The difference in NU-CHIPS scores between <12 and 12 to <36 months was statistically significant (89% ±â€Š6 vs. 83% ±â€Š12; p = 0.009). LNT scores differed significantly between <9 and 9 to <12 months (94% ±â€Š4 vs. 86% ±â€Š10; p = 0.028). CONCLUSIONS: The recent FDA expansion of pediatric CI eligibility criteria to include infants as young as 9 months of age should not serve as a strict clinical cutoff. Rather, CI can be pursued in appropriately selected younger infants to optimize language and audiological outcomes.

Application of Kaizen Principles to a Large Cochlear Implant Practice: A Continuous Quality Improvement Initiative at Mayo Clinic.

OBJECTIVE: To remove barriers and improve access for patients seeking cochlear implantation. STUDY DESIGN: Prospective quality improvement study at a large tertiary academic care center. METHODS: A Kaizen quality improvement model was applied over the course of a year. Four weeklong meetings were used to identify areas for improvement and remediation. Data were collected at baseline, 90-days, and 1-year postcompletion of the project. Outcome measures included lead times, defined as the wait time between first contact with the clinic and the first appointment, and the wait time between surgery and activation, and cycle times defined as the total test time needed to determine cochlear implant candidacy, and total time needed to complete initial activation. The total inventory kept as clinic stock was also calculated RESULTS:: Kaizen team members collected data for each outcome measure. After the Kaizen principles were applied, the following outcomes were observed: median lead times between first contact with the clinic to candidacy testing, candidacy testing to surgery, and surgery to activation of the implant remained stable from baseline to 1-year follow-up. Median cycle time for candidacy testing decreased from 7.3 hours at baseline to 3.0 hours at 1-year follow-up. Cycle times for initial activation of the device did not change over time. The total inventory of clinic stock was reduced by 31%. CONCLUSIONS: Though outcomes for lead and cycle times varied, implementation of Kaizen principles was found to be an effective method for completing this quality improvement project at a large cochlear implant program overall. LEVEL OF EVIDENCE: 3a.

Cochlear implantation for single-sided deafness in children and adolescents.

OBJECTIVE: To evaluate outcomes in pediatric and adolescent patients with single-sided deafness (SSD) undergoing cochlear implantation. METHODS: A retrospective cohort design at two tertiary level academic cochlear implant centers. The subjects included nine children ages 1.5 to 15 years-old with single-sided deafness (SSD) who had undergone cochlear implantation in the affected ear. Objective outcome measures included were speech reception testing in quiet and noise, bimodal speech reception threshold testing in noise, tinnitus suppression, and device usage. RESULTS: Nine pediatric and adolescent patients with SSD were implanted between 2011 and 2017. The median age at implantation was 8.9 years (range, 1.5-15.1) and the children had a median duration of deafness 2.9 years (range, 0.8-9.5). There was variability in testing measures due to patient age. Median pre-operative aided word recognition scores on the affected side were <30% regardless of the testing paradigm used. Six patients had pre-operative word testing (4 CNC, median score 25%; 2 MLNT, 8% and 17%). Four patients had pre-operative sentence testing (3 AzBio, median score 44%; 1 HINT-C, 57%). Median post-implantation follow-up interval was 12.3 months (range, 3-27.6 months). Six subjects had post-operative word recognition testing (CNC median, 70%; MLNT 50%, 92%) with a median improvement of 45.5% points. Five subjects had post-operative sentence testing (AzBio, median 82%; HINT, median 76%), with a median improvement of 40.5% points. Eight patients are full time users of their device. Tinnitus and bimodal speech reception thresholds in noise were improved. CONCLUSION: Pediatric subjects with SSD benefit substantially from cochlear implantation. Objective speech outcome measures are improved in both quiet and noise, and bimodal speech reception thresholds in noise are greatly improved. There is a low rate of device non-use.

Cochlear Implantation in Adults With Asymmetric Hearing Loss: Speech Recognition in Quiet and in Noise, and Health Related Quality of Life.

OBJECTIVE: To examine the possible speech recognition and health related quality of life (HRQoL) benefits of cochlear implantation among adults with asymmetric sensorineural hearing loss. STUDY DESIGN: Retrospective chart review, single-subject design. METHODS: A total of 45 adult cochlear implant recipients with asymmetric sensorineural hearing loss where performance for the best-aided condition exceeded 60% correct open set sentence recognition in quiet, and the implanted ear met traditional candidacy criteria. End point testing of the implanted ear was evaluated with use of the Consonant-Vowel Nucleus-Consonant (CNC) word test and AzBio sentence test materials in quiet, and bimodally with the AzBio sentence test materials in noise at +5 dB signal-to-noise ratio (SNR). HRQoL was measured using the Nijmegen Cochlear Implant Questionnaire (NCIQ). RESULTS: Measured in quiet, with the non-implanted ear plugged, the average CNC word scores increased from 9.1% preoperatively to 55.7% (p < 0.01) at the 6-month post-activation test interval. Similarly, average AzBio sentence scores in quiet, with the non-implanted ear plugged, increased from 13.9% preoperatively to 73.4% (p < 0.01) at the 6-month post-activation test interval. Finally, in the bilateral/bimodal condition, the AzBio sentence score in +5 dB SNR improved from an average of 26.8% preoperatively to 52.4% (p < 0.01) at the 6-month test interval. Results of the NCIQ showed improved scores on all six subdomains. CONCLUSIONS: These data demonstrate significant benefit of cochlear implantation among a group of postlingually deafened adults whose preoperative hearing and aided speech recognition fell outside of the currently specified Food and Drug Administration candidacy guidelines. Results of this study support the evaluation of a candidate's speech recognition in noise in the best-aided condition to adequately assess candidacy for a cochlear implant.

Hearing Preservation in Pediatric Cochlear Implantation.

OBJECTIVE: Currently, there is a paucity of literature evaluating hearing preservation outcomes in children following cochlear implantation. The objective of the current study is to report pediatric hearing preservation results following cochlear implantation with conventional full-length electrodes. STUDY DESIGN: Retrospective review (2000-2016). SETTING: Tertiary referral center. PATIENTS: All pediatric patients with a ≤ 75 dB preoperative low-frequency pure tone average (LFPTA; 250-500 Hz average), who underwent cochlear implantation with a conventional length electrode. INTERVENTION(S): Cochlear implantation. MAIN OUTCOME MEASURE(S): Complete, partial, minimal, or no hearing preservation following cochlear implantation (Skarzynski et al., 2013); maintenance of functional low frequency hearing (≤85 dB LFPTA). RESULTS: A total of 43 ears, in 35 pediatric patients, met inclusion criteria. The mean age at time of implantation was 8.6 years (range, 1.4-17.8 yr), 20 (57.1%) patients were female, and 25 (58.1%) cases were left-sided.The mean preoperative ipsilateral low frequency PTA and conventional four-frequency PTA (500, 1000, 2000, 3000 Hz average) were 54.2 dB (range, 15-75 dB) and 82.2 dB (range, 25-102.5 dB), respectively. The mean low frequency PTA and conventional four-frequency PTA shifts comparing the pre- and first postoperative audiogram were Δ25.2 dB (range, -5 to 92.5 dB) and Δ18.3 dB (range, -8.8 to 100 dB), respectively. Overall, 17 (39.5%) ears demonstrated complete hearing preservation, 19 (44.2%) ears partial hearing preservation, 0 minimal hearing preservation, and 7 (16.3%) exhibited no measurable acoustic hearing after surgery. In total, 28 (65.1%) ears maintained functional low-frequency hearing (i.e., ≤85 dB LFPTA) based on the initial postoperative audiogram. There was no statistically significant difference in the initial low frequency PTA shift comparing lateral wall and perimodiolar electrodes (Δ22.2 versus Δ28.1 respectively; p = 0.44), cochleostomy and round window insertions (Δ25.2 vs. Δ24.7 respectively; p = 0.95), or statistically significant association between age at implantation and low frequency PTA shift (r = 0.174; p = 0.26).In total, 22 ears in 19 patients had serial audiometric data available for review. Over a mean duration of 43.8 months (range, 2.6-108.3 mo) following surgery, the mean low frequency PTA and conventional four-frequency PTA shift comparing the initial postoperative and most recent postoperative audiogram was Δ9.7 dB (range, -27.5 to 57.5 dB) and Δ8.1 dB (range, -18.8 to 31.9 dB), respectively. CONCLUSIONS: Varying levels of hearing preservation with conventional length electrodes can be achieved in most pediatric subjects. In the current study, 82% of patients maintained detectable hearing thresholds and 65% maintained functional low-frequency acoustic hearing. These data may be used to guide preoperative counseling in pediatric patients with residual acoustic hearing. Additionally, the favorable rates of hearing preservation achieved in children provide further evidence for the expansion of pediatric cochlear implant candidacy to include patients with greater degrees of residual hearing.

Bilateral Labyrinthine and Internal Auditory Canal Enhancement in an Infant With Severe Labyrinthine Dysplasia: A Previously Unreported Phenomenon.

OBJECTIVE: To describe a novel case of congenital profound bilateral sensorineural hearing loss in a patient with bilateral nodular internal auditory canal and labyrinthine enhancement and temporal bone dysplasia. PATIENTS: A 76-day-old female was referred to the authors' center for evaluation of congenital deafness. Behavioral observations and objective audiometric evaluation demonstrated bilateral profound sensorineural hearing loss and a comprehensive multidisciplinary evaluation identified compound heterozygous pathogenic variants in MYO7A, a gene associated with Usher Syndrome Type 1B or DFNB2. Computed tomography and contrast-enhanced magnetic resonance imaging studies demonstrated bilateral temporal bone anomalies with unique middle and inner ear malformations, as well as unique contrast enhancement in the membranous labyrinth, internal auditory canals, and cranial nerves, which have not been previously described with MYO7A variants. INTERVENTIONS: Given the potential risk for progressive bilateral labyrinthitis ossificans, bilateral simultaneous cochlear implantation was performed at 4 months of age. MAIN OUTCOME MEASURES: Subsequent audiologic follow up after implantation shows significantly improved access to auditory information and increased vocalizations. At last testing, speech and language skills for both receptive and expressive language abilities were found to be commensurate with her chronological age. CONCLUSION: We report a novel presentation and imaging findings of congenital bilateral profound sensorineural hearing loss in a patient with nodular internal auditory canal and labyrinthine enhancement and coexisting inner ear dysplasia. Despite the multiple radiologic abnormalities, the patient has demonstrated good benefit from cochlear implantation. Future study of rare variants of congenital deafness, such as this, is critical toward defining new disease processes and determining optimal treatment.

Early outcomes after cochlear implantation for adults and children with unilateral hearing loss.

OBJECTIVES/HYPOTHESIS: This study was designed to examine speech recognition and self-perceived health-related quality of life (HRQoL) received from cochlear implantation among a cohort of adults and children with a short duration of unilateral hearing loss greater than 6 months, but less than 2 years. STUDY DESIGN: Single-subject repeated measures prospective study. METHODS: This study assessed changes in speech recognition and self-perceived quality of life by prospectively analyzing data at the preoperative evaluation and at the 3-month and 6-month postactivation intervals. Measurement tools included Medical Outcomes Study Questionnaire Short Form 36, Nijmegen Cochlear Implant Questionnaire, Speech Spatial and Qualities of Hearing-Comparative, and speech recognition measures in quiet and in noise. RESULTS: Results indicated significant improvement in speech recognition, both in quiet and noise. Quality-of-life measures showed a significant increase in self-perceived benefit with disease-specific instruments, but remained constant with a generic HRQoL instrument. CONCLUSIONS: Cochlear implantation was a successful intervention for improved hearing in quiet and noise, and a self-perceived benefit for this group of adults and children with a short duration of unilateral hearing loss. LEVEL OF EVIDENCE: 4 Laryngoscope, 127:1683-1688, 2017.

Evidence for the expansion of pediatric cochlear implant candidacy.

OBJECTIVE: To test the hypothesis that children who are non-traditional cochlear implant candidates, but are not making progress with appropriately fitted hearing aids and intervention, will demonstrate significant benefit from cochlear implantation as defined by improvement in (1) speech perception, (2) auditory skills development, and/or (3) progress on standardized measures of receptive and expressive language. STUDY DESIGN: Retrospective case series. SETTING: Two tertiary academic cochlear implant centers. PATIENTS: All pediatric patients that underwent cochlear implantation were reviewed. Only those meeting one or both of the following criteria were included: (1) less severe hearing loss than specified in the current indications and (2) open-set word and/or sentence recognition scores greater than 30% for children who are able to participate in speech perception testing. Patients with auditory neuropathy were excluded. INTERVENTION(S): Cochlear implantation. MAIN OUTCOME MEASURES: Pre- and postoperative results of age appropriate speech recognition tests, auditory questionnaires, and standardized norm-referenced estimates of speech and language development. RESULTS: A total of 51 patients met study criteria. The mean age at time of surgery was 8.3 years and 24% underwent bilateral sequential implantation. Overall, the mean speech recognition improvement was 63 percentage points in the implanted ear (p < 0.001) and 40 percentage points in the bimodal condition (p < 0.001). Results of auditory and language development measures revealed significant improvement after implantation (p < 0.05). CONCLUSION: Non-traditional pediatric implant recipients derive significant benefit from cochlear implantation. A large-scale reassessment of pediatric cochlear implant candidacy, including less severe hearing losses and higher preoperative speech recognition, is warranted to allow more children access to the benefits of cochlear implantation.

Development and validation of the pediatric AzBio sentence lists.

OBJECTIVES: The goal of this study was to create and validate a new set of sentence lists that could be used to evaluate the speech-perception abilities of listeners with hearing loss in cases where adult materials are inappropriate due to difficulty level or content. The authors aimed to generate a large number of sentence lists with an equivalent level of difficulty for the evaluation of performance over time and across conditions. DESIGN: The original Pediatric AzBio sentence corpus included 450 sentences recorded from one female talker. All sentences included in the corpus were successfully repeated by kindergarten and first-grade students with normal hearing. The mean intelligibility of each sentence was estimated by processing each sentence through a cochlear implant simulation and calculating the mean percent correct score achieved by 15 normal-hearing listeners. After sorting sentences by mean percent correct scores, 320 sentences were assigned to 16 lists of equivalent difficulty. List equivalency was then validated by presenting all sentence lists, in a novel random order, to adults and children with hearing loss. A final-validation stage examined single-list comparisons from adult and pediatric listeners tested in research or clinical settings. RESULTS: The results of the simulation study allowed for the creation of 16 lists of 20 sentences. The average intelligibility of each list ranged from 78.4 to 78.7%. List equivalency was then validated, when the results of 16 adult cochlear implant users and 9 pediatric hearing aid and cochlear implant users revealed no significant differences across lists. The binomial distribution model was used to account for the inherent variability observed in the lists. This model was also used to generate 95% confidence intervals for one and two list comparisons. A retrospective analysis of 361 instances from 78 adult cochlear implant users and 48 instances from 36 pediatric cochlear implant users revealed that the 95% confidence intervals derived from the model captured 94% of all responses (385 of 409). CONCLUSIONS: The cochlear implant simulation was shown to be an effective method for estimating the intelligibility of individual sentences for use in the evaluation of cochlear implant users. Furthermore, the method used for constructing equivalent sentence lists and estimating the inherent variability of the materials has also been validated. Thus, the AzBio Pediatric Sentence Lists are equivalent and appropriate for the assessment of speech-understanding abilities of children with hearing loss as well as adults for whom performance on AzBio sentences is near the floor.

Cochlear implantation in children with auditory neuropathy spectrum disorder: long-term outcomes.

BACKGROUND: Best practices concerning the audiological management of the child diagnosed with auditory neuropathy spectrum disorder (ANSD) have not been definitively defined nor fully understood. One reason is that previous studies have demonstrated conflicting findings regarding the outcomes of cochlear implantation for children with ANSD. Thus, the question remains whether children with ANSD are able to achieve similar outcomes following cochlear implantation as those children with sensorineural hearing loss (SNHL). PURPOSE: To assess speech perception outcomes for children with cochlear implants who have a diagnosis of ANSD as well as their age-matched peers who have sensorineural hearing loss. RESEARCH DESIGN: Retrospective study STUDY SAMPLE: Thirty-five subject pairs (n = 70) ranging in age at implant activation from to 10 to 121 mo (mean 39.2 mo) were included in this retrospective study. Subjects were matched on variables including age at initial implant activation and months of implant use at postoperative test point. DATA COLLECTION AND ANALYSIS: Speech recognition scores for monosyllabic and multisyllabic stimuli were compared across the subject groups. For those not developmentally and/or linguistically ready for completion of open-set speech recognition testing with recorded stimuli, GASP (Glendonald Auditory Screening Procedure) word recognition and/or questionnaire data using either the LittlEARS or Meaningful Auditory Integration Scale were compared across the groups. Statistical analysis using a repeated-measures analysis of variance (ANOVA) evaluated the effects of etiology (ANSD or SNHL) on postoperative outcomes. RESULTS: The results of this study demonstrate that children with ANSD can clearly benefit from cochlear implantation and that their long-term outcomes are similar to matched peers with SNHL on measures of speech recognition. There were no significant differences across the ANSD and SNHL groups on any of the tested measures. CONCLUSION: Cochlear implantation is a viable treatment option for children with a diagnosis of ANSD who are not making auditory progress with hearing aids that have been fit using the Desired Sensation Level method (DSL v5.0). Expected outcomes of cochlear implantation for children with ANSD, excluding children with cochlear nerve deficiency, are no different than for children with non-ANSD SNHL. These results are important for counseling families on the expected outcomes and realistic expectations following cochlear implantation for children with ANSD who demonstrate no evidence of cochlear nerve deficiency.