Development of a Predictive Model for Individualized Hearing Aid Benefit.

OBJECTIVES: To develop a model to predict individualized hearing aid benefit. To provide interpretations of model predictions on global and individual levels. METHODS: We compiled a data set of patients with hearing loss who trialed hearing aids and completed the Client Oriented Scale of Improvement (COSI) questionnaire, a validated patient-reported outcome measure of hearing aid benefit. Features included demographic, medical, and audiological measures. The outcome was the COSI score for change in listening ability with hearing aids, scaled from 1 to 5. Model development was performed using fivefold cross-validation repeated three times with hyperparameter tuning. Model performance was assessed using the root mean squared error (RMSE) of the COSI scores. Model interpretation was performed using Shapley Additive Explanations. RESULTS: The data set comprised 1,286 patients across 3,523 listening situations. The best performing model was random forest with an RMSE of 0.80, found to be significantly better than the next best model (eXtreme gradient boosting with RMSE of 0.85, p < 0.01). The most important features in predicting hearing aid benefit were shorter duration of hearing aid use, higher pure-tone average in the better hearing ear, and younger age. CONCLUSION: We have developed a predictive model for hearing aid benefit that can also provide individualized explanations of model predictions. Predictive modeling could be a useful tool in assessing a patient's candidacy and predicted benefit from hearing aids.

Voluntary Field Recall of Advanced Bionics HiRes Cochlear Implants: A Single-Institution Experience.

OBJECTIVES: In 2020, Advanced Bionics (AB) announced a recall of two cochlear implant (CI) models, the "HiRes Ultra" and "HiRes Ultra 3D", because of reports of hearing degradation. The present study examines clinical parameters and patient features in cases of device failure and evaluates outcomes after reimplantation. MATERIALS AND METHODS: A series of 52 patients implanted with the recalled devices experienced suspected device failure and subsequently underwent revision CI placement at a tertiary academic medical center between December 2019 and November 2021. RESULTS: Consonant-nucleus-consonant scores and individual phonemes increased significantly between patients' preoperative evaluation and primary cochlear implantation. Performance declined significantly before revision and recovered after revision CI placement. Similarly, pure-tone average thresholds improved between preoperative and primary CI, fell before revision surgery, and were corrected with revision implantation. As a group, patients reached their peak hearing performance significantly faster after revision CI (mean ± standard deviation, 53.4 ± 51.8 d) compared with their primary CI (mean ± standard deviation, 260.6 ± 245.9 d). Electrical field imaging performed by AB and device impedance measurements were found to be abnormal in the basally positioned electrodes (electrodes 9-16). CONCLUSION: Hearing performance degradation is significant in AB Ultra device failures and seems to be linked to the basal-most electrodes in the array. Revision outcomes have been robust, necessitating continued monitoring of affected patients and support for reimplantation procedures. LEVEL OF EVIDENCE: IV.

Fractionated Proton Radiation Therapy and Hearing Preservation for Vestibular Schwannoma: Preliminary Analysis of a Prospective Phase 2 Clinical Trial.

BACKGROUND: Local management for vestibular schwannoma (VS) is associated with excellent local control with focus on preserving long-term serviceable hearing. Fractionated proton radiation therapy (FPRT) may be associated with greater hearing preservation because of unique dosimetric properties of proton radiotherapy. OBJECTIVE: To investigate hearing preservation rates of FPRT in adults with VS and secondarily assess local control and treatment-related toxicity. METHODS: A prospective, single-arm, phase 2 clinical trial was conducted of patients with VS from 2010 to 2019. All patients had serviceable hearing at baseline and received FPRT to a total dose of 50.4 to 54 Gy relative biological effectiveness (RBE) over 28 to 30 fractions. Serviceable hearing preservation was defined as a Gardner-Robertson score of 1 to 2, measured by a pure tone average (PTA) of ≤50 dB and a word recognition score (WRS) of ≥50%. RESULTS: Twenty patients had a median follow-up of 4.0 years (range 1.0-5.0 years). Local control at 4 years was 100%. Serviceable hearing preservation at 1 year was 53% (95% CI 29%-76%), and primary end point was not yet reached. Median PTA and median WRS both worsened 1 year after FPRT (P < .0001). WRS plateaued after 6 months, whereas PTA continued to worsen up to 1 year after FPRT. Median cochlea D90 was lower in patients with serviceable hearing at 1 year (40.6 Gy [RBE] vs 46.9 Gy [RBE]), trending toward Wilcoxon rank-sum test statistical significance (P = .0863). Treatment was well-tolerated, with one grade 1 cranial nerve V dysfunction and no grade 2+ cranial nerve dysfunction. CONCLUSION: FPRT for VS did not meet the goal of serviceable hearing preservation. Higher cochlea doses trended to worsening hearing preservation, suggesting that dose to cochlea correlates with hearing preservation independent of treatment modality.

Implementation of Mobile Audiometry During the COVID-19 Pandemic.

During the COVID-19 pandemic, the utility of portable audiometry became more apparent as elective procedures were deferred in an effort to limit exposure to health care providers. Herein, we retrospectively evaluated mobile-based audiometry in the emergency department and outpatient otology and audiology clinics. Air conduction thresholds with mobile audiometry were within 5 dB in 66% of tests (95% CI, 62.8%-69.09%) and within 10 dB in 84% of tests (95% CI, 81.4%-86.2%) as compared with conventional audiometry. No significant differences were noted between mobile-based and conventional audiometry at any frequencies, except 8 kHz (P < .05). The sensitivity and specificity for screening for hearing loss were 94.3% (95% CI, 91.9%-96.83%) and 92.3% (95% CI, 90.1%-94.4%), respectively. While automated threshold audiometry does not replace conventional audiometry, mobile audiometry is a promising screening tool when conventional audiometry is not available.

Patient Report of Hearing in Neurofibromatosis Type 2: Recommendations for Clinical Trials.

OBJECTIVE: To systematically evaluate published patient-reported outcome measures for the assessment of hearing function and hearing-related quality of life (QoL) and recommend measures selected by the Response Evaluation in Neurofibromatosis and Schwannomatosis International Collaboration (REiNS) as endpoints for clinical trials in neurofibromatosis type 2 (NF2). METHODS: The REiNS Patient-Reported Outcomes Working Group systematically evaluated published patient-reported outcome measures of (1) hearing function and (2) hearing-related QoL for individuals with hearing loss of various etiologies using previously published REiNS rating procedures. Ten measures of hearing functioning and 11 measures of hearing-related QoL were reviewed. Measures were numerically scored and compared primarily on their participant characteristics (including participant age range and availability of normative data), item content, psychometric properties, and feasibility for use in clinical trials. RESULTS: The Self-Assessment of Communication and the Self-Assessment of Communication-Adolescent were identified as most useful for adult and pediatric populations with NF2, respectively, for the measurement of both hearing function and hearing-related QoL. Measures were selected for their strengths in participant characteristics, item content, psychometric properties, and feasibility for use in clinical trials. CONCLUSIONS: REiNS recommends the Self-Assessment of Communication adult and adolescent forms for the assessment of patient-reported hearing function and hearing-related QoL for NF2 clinical trials. Further work is needed to demonstrate the utility of these measures in evaluating pharmacologic or behavioral interventions.

Predicting Depression From Hearing Loss Using Machine Learning.

OBJECTIVES: Hearing loss is the most common sensory loss in humans and carries an enhanced risk of depression. No prior studies have attempted a contemporary machine learning approach to predict depression using subjective and objective hearing loss predictors. The objective was to deploy supervised machine learning to predict scores on a validated depression scale using subjective and objective audiometric variables and other health determinant predictors. DESIGN: A large predictor set of health determinants from the National Health and Nutrition Examination Survey 2015-2016 database was used to predict adults' scores on a validated instrument to screen for the presence and severity of depression (Patient Health Questionnaire-9 [PHQ-9]). After model training, the relative influence of individual predictors on depression scores was stratified and analyzed. Model prediction performance was determined by prediction error metrics. RESULTS: The test set mean absolute error was 3.03 (95% confidence interval: 2.91 to 3.14) and 2.55 (95% confidence interval: 2.48 to 2.62) on datasets with audiology-only predictors and all predictors, respectively, on the PHQ-9's 27-point scale. Participants' self-reported frustration when talking to members of family or friends due to hearing loss was the fifth-most influential of all predictors. Of the top 10 most influential audiometric predictors, five were related to social contexts, two for significant noise exposure, two objective audiometric parameters, and one presence of bothersome tinnitus. CONCLUSIONS: Machine learning algorithms can accurately predict PHQ-9 depression scale scores from National Health and Nutrition Examination Survey data. The most influential audiometric predictors of higher scores on a validated depression scale were social dynamics of hearing loss and not objective audiometric testing. Such models could be useful in predicting depression scale scores at the point-of-care in conjunction with a standard audiologic assessment.

American Neurotology Society, American Otological Society, and American Academy of Otolaryngology - Head and Neck Foundation Guide to Enhance Otologic and Neurotologic Care During the COVID-19 Pandemic.

: This combined American Neurotology Society, American Otological Society, and American Academy of Otolaryngology - Head and Neck Surgery Foundation document aims to provide guidance during the coronavirus disease of 2019 (COVID-19) on 1) "priority" of care for otologic and neurotologic patients in the office and operating room, and 2) optimal utilization of personal protective equipment. Given the paucity of evidence to inform otologic and neurotologic best practices during COVID-19, the recommendations herein are based on relevant peer-reviewed articles, the Centers for Disease Control and Prevention COVID-19 guidelines, United States and international hospital policies, and expert opinion. The suggestions presented here are not meant to be definitive, and best practices will undoubtedly change with increasing knowledge and high-quality data related to COVID-19. Interpretation of this guidance document is dependent on local factors including prevalence of COVID-19 in the surgeons' local community. This is not intended to set a standard of care, and should not supersede the clinician's best judgement when managing specific clinical concerns and/or regional conditions.Access to otologic and neurotologic care during and after the COVID-19 pandemic is dependent upon adequate protection of physicians, audiologists, and ancillary support staff. Otolaryngologists and associated staff are at high risk for COVID-19 disease transmission based on close contact with mucosal surfaces of the upper aerodigestive tract during diagnostic evaluation and therapeutic procedures. While many otologic and neurotologic conditions are not imminently life threatening, they have a major impact on communication, daily functioning, and quality of life. In addition, progression of disease and delay in treatment can result in cranial nerve deficits, intracranial and life-threatening complications, and/or irreversible consequences. In this regard, many otologic and neurotologic conditions should rightfully be considered "urgent," and almost all require timely attention to permit optimal outcomes. It is reasonable to proceed with otologic and neurotologic clinic visits and operative cases based on input from expert opinion of otologic care providers, clinic/hospital administration, infection prevention and control specialists, and local and state public health leaders. Significant regional variations in COVID-19 prevalence exist; therefore, physicians working with local municipalities are best suited to make determinations on the appropriateness and timing of otologic and neurotologic care.

Hearing Vital Signs: Mobile Audiometry in the Emergency Department for Evaluation of Sudden Hearing Loss.

Emergency departments (EDs) are a common location for patients to present with sudden hearing loss (SHL). Unfortunately, high-quality, rapid quantitative measurement of hearing loss is challenging. Herein, we aim to evaluate the accuracy of tablet-based audiometry in patients complaining of SHL. Prospective tablet-based testing was completed in the ED in patients complaining of SHL. Air conduction thresholds (ACTs) obtained via tablet-based audiometry were compared to same-day measurements with a clinical-grade audiometer. Hearing loss (HL) was defined as >20 dB ACT for any frequency. In participant-level analysis, 30+ dB HL in 3 consecutive frequencies was used to define SHL. In the ED, mobile audiogram ACTs were within 5 dB (77%) and 10 dB (89.6%) of those determined by conventional audiometry. The sensitivity and specificity for mobile audiometry to detect 3 or more consecutive thresholds with 30+ dB HL were 100% and 62.5%, respectively. Findings have implications for increasing access to high-quality audiometry.

Effect of anesthesia on evoked auditory responses in pediatric auditory brainstem implant surgery.

OBJECTIVE: Electrically evoked auditory brainstem responses (EABR) guide placement of the multichannel auditory brainstem implant (ABI) array during surgery. EABRs are also recorded under anesthesia in nontumor pediatric ABI recipients prior to device activation to confirm placement and guide device programming. We examine the influence of anesthesia on evoked response morphology in pediatric ABI users by comparing intraoperative with postoperative EABR recordings. STUDY DESIGN: Retrospective review. METHODS: Seven children underwent ABI surgery by way of retrosigmoid craniotomy. General anesthesia included inhaled sevoflurane induction and propofol maintenance during which EABRs were recorded to confirm accurate positioning of the ABI. A mean of 7.7 ± 2.8 weeks following surgery, the ABI was activated under general anesthesia or sedation (dexmedetomidine) and EABR recordings were made. A qualitative analysis of intraoperative and postoperative waveform morphology was performed. RESULTS: Seven subjects (mean age 20.6 months) underwent nine ABI surgeries (seven primary, two revisions) and nine activations. EABRs were observed in eight of nine postoperative recordings. In three cases, intraoperative EABRs during general anesthesia were similar to postoperative EABRs with sedation. In one case, sevoflurane and propofol were used for intra- and postoperative recordings, and waveforms were also similar. In four cases, amplitude and latency changes were observed for intraoperative versus postoperative EABRs. CONCLUSION: Similarity of EABR morphology in the anesthetized versus sedated condition suggests that anesthesia does not have a large effect on far-field evoked potentials. Changes in EABR waveform morphology observed postoperatively may be influenced by other factors such as movements of the surface array. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:507-513, 2020.

The natural progression of low-frequency hearing loss in patients who meet hybrid implant system candidacy criteria.

OBJECTIVES/HYPOTHESIS: To investigate the natural progression of hearing loss in patients with high-frequency hearing loss whose audiograms met the criteria for a hybrid cochlear implant (CI). STUDY DESIGN: Retrospective database review. METHODS: We retrospectively identified patients who met the criteria for a hybrid CI from our audiometric database. We also recorded the etiology of hearing loss in each patient and excluded patients with retrocochlear etiologies, those age <6 years, duration of observation <1 year, or those with a mixed or conductive hearing loss. We calculated the pre-CI progression of residual low-frequency (LF) hearing level in decibels per year and investigated the risk factor of the progression of hearing loss. RESULTS: A total of 1,083 ears of 944 patients were included in this study. The average rate of hearing loss progression for all etiologies was 1.70 ± 0.10 dB/yr at an average of 250 Hz, 500 Hz, and 1 kHz in the affected ear(s). The progression of hearing loss was 5.0 ± 0.93 dB/yr in patients with Meniere's disease and 3.5 ± 0.76 dB/yr in those with autoimmune disease, both of which were significantly faster than progression rates in other etiologies. In patients with idiopathic, genetic/hereditary, and congenital hearing loss, the progression of hearing loss was 1.46 ± 0.10 dB/yr. Patients with steeply sloping hearing loss experienced significantly faster progression in the ipsilateral ear compared to patients with another audiometric type. CONCLUSIONS: It is suggested that Meniere's disease, autoimmune-mediated inner ear disease, and steeply sloping hearing loss in the ipsilateral ear are risk factors for faster progression of residual LF hearing level. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:1299-1303, 2020.

Frequency and electrode discrimination in children with cochlear implants.

The objective of this study was to develop reliable pediatric psychophysical methodologies in order to address the limits of frequency and electrode discrimination in children with cochlear implants. Discrimination was measured with a two-alternative, adaptive, forced choice design using a video game graphical user interface. Implanted children were compared to normal-hearing children in the same age ranges. Twenty-nine implanted children and 68 children with normal-hearing performed frequency discrimination studies at varying frequencies. Electrode discrimination was assessed in thirty-four implanted children at varying electrode locations and stimulation intensities. Older children had better frequency discrimination than younger children, both for implanted and hearing subjects. Implanted children had worse frequency discrimination overall and exhibited learning effects at older ages than hearing children. Frequency discrimination Weber fractions were smallest in low frequencies. Electrode discrimination improved with stimulus intensity level for older but not younger children at all electrode locations. These results support the premise that developmental changes in signal processing contribute to discrimination of simple acoustic stimuli. For implanted children, auditory discrimination improved at lower frequencies and with electrodes at higher intensity. These findings imply that spatial separation may not be the key determinant in creating discriminable electrical stimuli for this population.

Pediatric cochlear implantation - II: postoperative follow-up.

A child with a cochlear implant is expected to achieve the successful outcome of facilitated perception of sound and more oral communication. To achieve this goal, ongoing intervention from a variety of professionals is required. These professionals may represent the disciplines of medicine, audiology, social work, education, and speech / language pathology. In India, cochlear implantation is available in only a few large cities. Here, the otolaryngologist will direct the cochlear implant program. Besides determination of medical candidacy, device implantation and medical management, the otolaryngologist is responsible to ensure that other aspects of cochlear implant management are implemented. This paper, the second of two that describe the multidisciplinary, team approach of the Pediatric Cochlear Implant Program of The Children's Hospital of Philadelphia (CHOP), in Pennsylvania, USA, discusses the non-medical aspects of cochlear implant post-implantation follow-up. The first article, previously published, discussed cochlear implant candidacy. The various speech tests used at CHOP for assessment are based on the English language. They may be translated into the regional Indian languages where the assessment and training can be carried out accordingly.

Routine use of the crystal device integrity testing system in pediatric patients.

Crystal Device Integrity Testing System (CITS), the first commercially available testing system of its type, allows rapid assessment of cochlear implant function by measuring averaged electrode voltages-the scalp-recorded fields generated by electrode currents. We describe our experience performing routine integrity tests on 44 pediatric cochlear implant patients using the CITS. We present our findings focusing on the monopolar and common ground scans to provide a framework from which CITS scans can be evaluated in the future. We also describe selected cases in which abnormal results using the CITS influenced clinical treatment, demonstrating the utility of performing routine integrity tests.

Electrode interaction in pediatric cochlear implant subjects.

Multielectrode cochlear implants rely on differential stimulation of the cochlear nerve for presenting the brain with the spectral and timing information required to understand speech. In implant patients, the degree of overlap among cochlear nerve fibers stimulated by the different electrodes constitutes the electrode interaction. Electrode interaction degrades the spectral resolution of the implant's stimulus. We sought to define electrode interaction in a cohort of pediatric cochlear implant subjects as a function of both stimulus intensity and electrode location along the array. The 27 pediatric subjects that completed the study were implanted with either the Clarion Hi-Focus array with or without positioner, the Nucleus 24 Contour array, or the Nucleus 24 Straight array. All but two of the patients had congenital hearing loss, and none of the patients had meningitis prior to the onset of deafness. The cochlear nerve response was measured with the electrically evoked compound action potential (ECAP). A forward masking protocol was used such that a probe stimulus electrode remained fixed while a preceding masker was moved across the array. Electrode interaction was estimated by measuring the unmasked probe response minus the masked probe response. Three probe locations and three probe intensities were examined for each subject. At all probe locations, electrode interaction increased as probe intensity increased (p < 0.05). Interaction at the basal probe was less than that at either the middle or apical probe locations (p < 0.05), and significant correlation found between probe distance from the basal end of the array and electrode interaction (p < 0.001). These results demonstrate that in this cohort of pediatric subjects, electrode interaction depended on both stimulus intensity and probe location. Implications of these findings on future implant array design and current implant fitting strategies are discussed. The impact of electrode interaction on implant performance is yet to be elucidated.

Electrically evoked compound action potential amplitude growth functions and HiResolution programming levels in pediatric CII implant subjects.

OBJECTIVE: To characterize the amplitude growth functions of the electrically evoked compound action potential (ECAP) in pediatric subjects implanted with the Clarion HiFocus electrode array with respect to electrode position and the presence or absence of a Silastic positioner. Electrophysiologic growth function data are compared with HiResolution (HiRes) psychophysical programming levels. DESIGN: ECAP growth functions were measured for all electrodes along the implant's array in 16 pediatric subjects. Nine of the patients were implanted with a Silastic positioner, whereas seven had no positioner. ECAP thresholds and growth function slopes were calculated. Fifteen of the 16 patients had psychophysical threshold and maximum comfort levels available. Programming levels and ECAP thresholds were compared within and among the subjects. RESULTS: ECAP thresholds showed variability among patients, ranging from 178 to 920 nA at 32 musec pulse width. ECAP thresholds did not depend on electrode position along the cochlea but were lower in the presence of the Silastic positioner (p < 0.001). Thresholds determined with the masker-probe versus the alternating polarity paradigms revealed moderate (r = 0.76) correlation. Growth function slopes also showed considerable variation among patients. Unlike thresholds, slopes decreased from apical to basal cochlear locations (p < 0.001) but showed no difference between the absence and presence of the positioner. Programming levels in HiRes were correlated with ECAP threshold levels. When ECAP thresholds were adjusted for each patient by the difference between M level and ECAP threshold at electrode 9, however, overall correlation between the two measurements was excellent (r = 0.98, N = 224). CONCLUSIONS: In pediatric subjects with the Clarion HiFocus electrode, ECAP growth function thresholds appear to decrease with the presence of the Silastic positioner but are unaffected by electrode position along the array. Growth function slope, however, depends on electrode position along the array but not on the presence of the positioner. ECAP programming levels can reliably predict stimulus intensities within the patients' dynamic ranges, but considerable variability is seen between ECAP thresholds and HiRes programming levels.

Averaged electrode voltage testing to diagnose an unusual cochlear implant internal device failure.

This paper describes an unusual Clarion 1.2 cochlear implant failure in order to demonstrate the need for the clinical availability of electrode voltage testing. The case report describes a patient with a Clarion 1.2 cochlear implant who exhibited poor auditory progress in spite of grossly normal impedances and electric field testing, and an appropriate educational and psychosocial environment. For a subset of electrodes, however, electrode voltage testing was abnormal. At explantation it was found that the epoxy that protects the connection between the electrode leads of the array and the output contacts on the internal controller had fractured. This failure was not associated with reported head trauma. This case report demonstrates the utility of and clinical need for electrode voltage testing as a helpful tool to diagnose internal device failure.

Pediatric cochlear implantation-I : Candidacy.

The cochlear implant is a successful means of intervention to facilitate the perception of sound and oral communication for children with severe to profound sensorineural hearing loss. Often, the cochlear implantation process is viewed as being limited to a surgical procedure, rather than an entire process of medical, audiological, psychosocial, educational and therapeutic, management. In India, cochlear implantation is available in only a few large cities. Here, the otolaryngologist will direct the cochlear implant programme. Besides determination of medical candidacy, device implantation and medical management, the otolaryngologist is responsible to ensure that other aspects of cochlear implant management are implemented. This paper, the first of two that describe the multidisciplinary, team approach of the Pediatric Cochlear Implant Program of The Children's Hospital of Philadelphia (CHOP), in Pennsylvania, USA, discusses the non-medical aspects of cochlear implant candidacy. A second article will follow that covers post-implantation follow- up. The various speech tests used at CHOP for assessment are based on the English language. They may be translated into the regional Indian languages where the assessment and training can be carried out accordingly.