Cochlear Implantation in Patients With Osteolytic Labyrinthitis: A Case Series.

OBJECTIVE: To describe the rare process of osteolytic labyrinthitis, previously referred to as labyrinthine sequestrum, which involves progressive obliteration of the bony and membranous labyrinth with eventual supplantation with soft tissue and, in some cases, bony sequestrum. PATIENTS: Three patients with diverse presentations of osteolytic labyrinthitis from two tertiary care academic medical centers. INTERVENTIONS: Case series report analyzing the relevant clinical, radiologic, pathologic, and surgical data on our patients with osteolytic labyrinthitis and comparing these index cases to the existing literature. MAIN OUTCOME MEASURES: We describe the varying image findings seen in osteolytic labyrinthitis on computed tomography and magnetic resonance imaging. Also, we report successful surgical intervention and hearing rehabilitation with cochlear implantation in patients with osteolytic labyrinthitis. RESULTS: Our three patients presented with profound sudden sensorineural hearing loss and vertigo consistent with labyrinthitis. None of the three patients had a history of chronic otitis media. Imaging workup revealed varying degrees of erosion to the otic capsule bone demonstrating the spectrum of disease seen in osteolytic labyrinthitis. Although two cases showed osteolytic changes to the semicircular canals and vestibule, the first case revealed frank bony sequestrum within the obliterated labyrinth. The three cases were taken for surgical debridement and cochlear implantation. CONCLUSIONS: We propose the new term, osteolytic labyrinthitis-previously referred to as labyrinthine sequestrum-to describe the rare spectrum of disease characterized by destruction of the osseous and membranous labyrinth and potential supplantation with bony sequestrum. Cochlear implantation is a viable option in selected patients with osteolytic labyrinthitis.

Current practices and opinions on auditory training in adult cochlear implant recipients.

OBJECTIVE: To examine current practices and opinions of cochlear implant (CI) providers with respect to post-implantation auditory training. METHODS: A survey was submitted to the American Cochlear Implant Alliance membership that reviewed current practice and opinions with respect to post-implantation auditory training for adult CI recipients. MAIN OUTCOME MEASURES: Review of respondent practice, center volume, role on CI team, and current usage and opinions surrounding auditory training, including resources used and schedule of use. RESULTS: Most (79 %) of the 79 CI providers surveyed reported working at academic centers, 34 % at high-volume centers (>150 CIs/year), and 38 % were surgeons. Nearly all (99 %) respondents recommend auditory training for new adult CI recipients. Just over half (52 %) provide auditory training resources to the patient in the form of a broad list of patient-directed exercises from which a patient could select. A specific training resource, generally a computer-based auditory training program (e.g., AngelSound™), is recommended to patients by 30 % of the respondents. Regarding timing of rehabilitation, median preferred start time was 0 months (interquartile range [IQR] 0-1) post-activation. Sessions were preferably performed for a median of 3 h per week (IQR 2-4) and continued for a median of 12 months (IQR 6-12). Recommendations for auditory training were fairly consistent between surgeon and non-surgeon providers and by center volume. Non-surgeons more often had specific recommendations on training resources, benefits of music, and training condition (e.g., contralateral ear plugged). CONCLUSIONS: Despite a lack of clinical guidelines for adult post-implantation auditory training, a cross-sectional survey of providers' current practices and opinions demonstrates that these services are widely recommended and regarded as valuable. Training is almost universally patient-directed and believed to be most beneficial if started soon after activation. Interestingly, specific recommendations for which training approaches to use are not common, suggesting a gap in provider knowledge of which resources are most efficacious.

Tensor Tympani and Stapedial Tendon Reconstruction for Postoperative Hyperacusis after Tendon Lysis for Middle Ear Myoclonus.

OBJECTIVE: To present a method for repair of the stapedial and tensor tympani tendons in a patient with hyperacusis after a tendon lysis procedure. PATIENTS: A 71-year-old professional musician who presented to clinic with debilitating hyperacusis following a tensor tympani and stapedial tendon lysis procedure to treat middle ear myoclonus. INTERVENTIONS: A novel procedure for reapproximation of the tensor tympani and stapedial tendons into their native insertion points using periosteal grafts and nitinol wire. MAIN OUTCOMES MEASURES: Stapedial reflex measurements, uncomfortable loudness level, and subjective patient experience. RESULTS: Postoperatively, the patient had objective improvement in hyperacusis with return of acoustic reflexes in the affected ear and durable improvements in their frequency-specific uncomfortable loudness levels. CONCLUSIONS: This case describes the debilitating complication of hyperacusis following tendon lysis and highlights the importance of maximizing behavioral and medical measures prior to undergoing surgical intervention for middle ear myoclonus.

Coordinated Same- or Next-Day Radiosurgery and Cochlear Implantation for Vestibular Schwannoma.

OBJECTIVE: To describe the experience and results from coordinated and closely scheduled radiosurgery and cochlear implantation (CI) in a vestibular schwannoma (VS) cohort. PATIENTS: Patients with VS who underwent radiosurgery followed by CI on the same or next day. INTERVENTIONS: Interventions included sequential radiosurgery and CI. MAIN OUTCOME MEASURES: Tumor control defined by tumor growth on posttreatment surveillance and audiometric outcomes including consonant-nucleus-consonant words and AzBio sentences in quiet. RESULTS: In total, six patients were identified that met the inclusion criteria, with an age range of 38 to 69 years and tumor sizes ranging from 2.0 to 16.3 mm. All patients successfully underwent radiosurgery and CI on the same or immediately successive day. Postoperatively, all patients obtained open-set speech recognition. Consonant-nucleus-consonant word scores ranged from 40 to 88% correct, and AzBio scores ranged from 44 to 94% correct. During posttreatment magnetic resonance imaging surveillance, which ranged from 12 to 68 months, all tumors were noted to be adequately visualized, and no tumor progression was noted. CONCLUSION: Coordinated radiosurgery and CI can be safely performed in patients with VS on the same or next day, serving to decrease burden on patients and increase access to this vital rehabilitative strategy.

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

OBJECTIVE: 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. DESIGN: 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. RESULTS: 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). CONCLUSIONS: 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.

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.

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.

Anatomic Step-by-Step Dissection of Complex Skull Base Approaches for Trainees: Surgical Anatomy of the Infratemporal Fossa Approach to the Jugular Foramen.

BACKGROUND AND OBJECTIVES: The infratemporal fossa (ITF) is a complex region bounded by the temporal bone, maxilla, sphenoid, pterygoid plates, and mandibular ramus. Containing a high density of neurovascular and musculoskeletal structures, the ITF can house a number of pathologies, and access is challenging. The ITF approach and its variations can be challenging due to complex anatomy and unfamiliarity by many surgeons. The objective of this study was to present a step-by-step 3-dimensional anatomic dissection for the classic Fisch Type A and modified ITF approach from the surgeon's perspective. METHODS: Six sides of 3 formalin-fixed latex-injected specimens were dissected under microscopic magnification (JRD and AMN). Standard Fisch Type A and modified ITF approaches were performed on contralateral sides of each specimen. Representative high-quality 3-dimensional photography was performed for each key step. RESULTS: The ITF approach affords excellent access to the posterior ITF and jugular foramen. Modifications to this approach include preservation of the ear canal and limiting facial nerve transposition, thus limiting morbidity while generally still providing sufficient access to key anatomic structures. CONCLUSION: The ITF approach provides access to the lateral skull base for jugular foramen paraganglioma and other lesions. Modifications of the classic Fisch Type A technique can be used to access pathologies in this region without sacrificing conductive hearing or facial nerve function. Three dimensional operatively oriented neuroanatomy dissections provide surgeons with a valuable resource for learning this complex surgical approach.

Risk of Monopolar Electrosurgery in Cochlear Implant Recipients is Nominal: Evidence to Guide Clinical Practice.

OBJECTIVE: Comprehensively assess the prevalence of monopolar electrosurgery-related device complications among cochlear implant (CI) recipients. STUDY DESIGN: Multifaceted retrospective review and survey. SETTING: Tertiary medical center. METHODS: Multifaceted approach including: (i) review of the current literature; (ii) historical review of institutional data from an academic, tertiary CI center; (iii) review of industry data provided by 3 Food and Drug Administration-approved CI manufacturers; and (iv) survey of high-volume CI centers. RESULTS: Literature review identified 9 human studies, detailing 84 devices with 199 episodes of device-cautery exposure. From studies reporting on patients records, no implant showed evidence of damage after exposure. One cadaveric study using dental cautery reported 1 episode of device damage. Review of institutional records did not identify any CI damage in 84 instances of exposure. Data from the 3 major implant manufacturers showed a single report of damage that could be reasonably linked to monopolar electrosurgery, out of a possible 689,426 CIs. Last, a survey of 8 high-volume CI centers did not identify any adverse events associated with monopolar cautery. CONCLUSION: These data estimate the risk of adverse device-related events or tissue injury to be extraordinarily low. Short of operating in immediate proximity to the CI (ie, the ipsilateral temporoparietal scalp), these data indicate that monopolar electrosurgery can be used in the body and the head-and-neck of CI recipients with nominal risk. These findings may guide decision-making in cases that are optimally or preferably performed with monopolar electrocautery and can be used to counsel CI patients following inadvertent exposures.

Third window lesions of the inner ear: A pictorial review.

PURPOSE: Radiographic review of pathologies that associate with third window syndrome. METHODS: Case series and literature review. RESULTS: Eight unique third window conditions are described and illustrated, including superior, lateral, and posterior semicircular canal dehiscence; carotid-cochlear, facial-cochlear, and internal auditory canal-cochlear dehiscence, labyrinthine erosion from endolymphatic sac tumor, and enlarged vestibular aqueduct. CONCLUSION: The present study highlights the characteristic imaging features and symptoms to differentiate third window pathologies for expedient diagnosis and management planning.

Comparing Speech Recognition Outcomes Between Cochlear Implants and Auditory Brainstem Implants in Patients With NF2-Related Schwannomatosis.

OBJECTIVE: To compare cochlear implant (CI) and auditory brainstem implant (ABI) performance in patients with NF2-related schwannomatosis (NF2). STUDY DESIGN: Historical cohort. SETTING: Tertiary academic center. PATIENTS: A total of 58 devices among 48 patients were studied, including 27 ABIs implanted from 1997 to 2022 and 31 CIs implanted from 2003 to 2022. Three patients had bilateral ABIs, three had bilateral CIs, three had an ABI on one side and a CI on the other, one had a CI that was later replaced with an ipsilateral ABI, and one had an ABI and CI concurrently on the same side. INTERVENTIONS: CI or ABI ipsilateral to vestibular schwannoma. MAIN OUTCOME MEASURES: Open-set speech perception, consonant-nucleus-consonant word scores, and AzBio sentence in quiet scores. RESULTS: Among all patients, 27 (47%) achieved open-set speech perception, with 35 (61%) daily users at a median of 24 months (interquartile range [IQR], 12-87 mo) after implantation. Comparing outcomes, CIs significantly outperformed ABIs; 24 (77%) CIs achieved open-set speech perception compared with 3 (12%) ABIs, with median consonant-nucleus-consonant and AzBio scores of 31% (IQR, 0-52%) and 57% (IQR, 5-83%), respectively, for CIs, compared with 0% (IQR, 0-0%) and 0% (IQR, 0-0%), respectively, for ABIs. Patients with ABIs were younger at diagnosis and at implantation, had larger tumors, and were more likely to have postoperative facial paresis. CONCLUSION: Many patients with NF2-associated vestibular schwannoma achieved auditory benefit with either a CI or an ABI; however, outcomes were significantly better in those patients who were able to receive a CI. When disease and anatomy permit, hearing rehabilitation with a CI should be considered over an ABI in these patients. Tumor management strategies that increase the ability to successfully use CIs should be strongly considered given the high risk of losing bilateral functional acoustic hearing in this population.

Rationale for the Development of a Novel Clinical Grading Scale for Postoperative Facial Nerve Function: Results of a Multidisciplinary International Working Group.

OBJECTIVE: The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. STUDY DESIGN: Survey. SETTING: A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. RESULTS: House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. CONCLUSIONS: Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.

Preliminary Evidence to Support a De-Escalated Cochlear Implant Programming Paradigm for New Adult Recipients: A Systematic Review.

Background: No standard schedule for cochlear implant (CI) programming has been developed, and common practices may have CI recipients seen in excess of what is necessary. The objective of this study was to review evidence for a de-escalated, evidence-based schedule for adult CI programming. Methods: Systematic review was undertaken in March 2023 of PubMed, Scopus, and CINAHL databases using the Preferred Reporting Items for Systemic Reviews and Meta-analyses (PRISMA) guidelines. Studies were included if (1) they evaluated an evidence-based programming/follow-up schedule in new adult CI patients or (2) they evaluated programming or outcomes in a longitudinal fashion such that they could inform CI follow-up strategies. Level of evidence was evaluated using the LEGEND evidence assessment tool. Results: Our review identified 940 studies. After screening with a priori inclusion criteria, 18 studies were ultimately included in this review. Of these, 2 demonstrated feasibility of de-escalated approaches to new adult CI programming. The remainder presented longitudinal speech and programming parameter data that demonstrated relative stability of both categories by 3 to 6 months post-activation. Conclusions: Overall, there is a paucity of literature evaluating any form of evidence-based CI programming or follow-up. Most applicable data derive from longitudinal outcomes featured in studies of other CI features, with only a handful of studies directly evaluating CI programming strategies over time. However, stability in outcomes and programming detailed in the available data supports consideration of a de-escalated programming paradigm that could primarily limit programming to the very early post-activation period (before 3 to 6 months) to enhance patient care and reduce operational strains on cochlear implant programs.

Initial Experiences with the Envoy Acclaim® Fully Implanted Cochlear Implant.

Introduction: Cochlear implantation has become the standard of care for the treatment of moderate-to-profound bilateral sensorineural hearing loss. However, current technologies, all of which rely on an external sound processor, have intrinsic limitations that prevent certain activities and diagnostics, thus hampering full integration into a patient's lifestyle. The Envoy Medical (White Bear Lake, MN, USA) Acclaim® fully implanted cochlear implant is a new device currently undergoing testing that has been designed to alleviate many of the current constraints by housing all components within the patient, thus allowing for near-constant use in many environments that are not conducive to a traditional cochlear implant. Methods: As part of an Early Feasibility Study, three adult implant candidates were implanted with the Acclaim® cochlear implant. Surgical video and photography were taken, and initial observations were recorded. Implantation with the Acclaim® device is largely similar to a traditional cochlear implant, with modifications to allow room for the implanted sensor as well as the implantation of a battery in the subcutaneous tissues of the chest. Results: This study demonstrates a step-by-step overview of implanting the Acclaim® and discusses initial insight and experiences with the first three implantations with this new device. Conclusions: All three surgeries proceeded without complication, and at activation, all three patients were hearing through their devices. Surgery is more technically challenging compared to a standard cochlear implant, but the skills needed can all be mastered by a dedicated otologic surgeon.

Anatomic Variants in the Anterior Inferior Cerebellar Artery Encountered During Resection of Vestibular Schwannomas.

BACKGROUND AND OBJECTIVES: Vestibular schwannomas (VS) are often phenotypically benign lesions that may be technically challenging to resect because of involvement of neurovascular structures. The anterior inferior cerebellar artery (AICA) is commonly identified near VS, with variable position in relation to the tumor; however, little published literature describes anatomic and pathologic variants of AICA observed during VS resection. METHODS: A prospectively maintained cohort of surgically managed VS with available operative reports and clinical/radiographic follow-up was queried and reviewed for noted aberrations. RESULTS: We identified 66 cases with noted AICA abnormalities among 880 reviewer cases, including 20 loops extending into the internal auditory canal (2.3%), 18 arteries embedded in dura (2.0%), 15 AICA branches directly within VS (1.7%), 8 main trunk arteries coursing between cranial nerves 7 and 8 (0.9%), 3 arteries embedded in temporal bone (0.2%), 1 aneurysm (0.1%), and 1 artery bifurcating cranial nerve 6 (0.1%). The median age of AICA-variant patients was 55 years (range 19-74), and 29 were female (45%). Compared with the other AICA variants, tumors embedded with AICA tended to be larger lesions on maximal axial diameter (2.9 vs 1.6 cm; P = .006), they more commonly underwent less than total resection (73% vs 28%; P = .0001), and they had higher rates postoperative House-Brackmann scores >2 (47% vs 20%; P = .005). Two patients had radiographic and symptomatic postoperative cerebral ischemia or hemorrhage-1 from a bone-encased AICA and 1 from a dural embedded variant. CONCLUSION: Anatomic variants of AICA occur in approximately 7% of VS operations. Most aberrations do not affect surgical or clinical outcomes, and the rate of major vascular injury was low. However, certain types variably add operative time and in the case of AICA encasement in the tumor, likely indicate a more aggressive tumor phenotype with lower rates of gross total resection and high incidences of facial nerve weakness.

Cochlear Implant Outcomes between Patients with Sporadic and Neurofibromatosis Type 2-Associated Vestibular Schwannoma.

OBJECTIVE: Compare cochlear implant (CI) performance between patients with ipsilateral sporadic vestibular schwannoma (VS) and NF2-related schwannomatosis (NF2). Compare CI performance according to VS management modality. STUDY DESIGN: Historical cohort. SETTING: Tertiary academic center. PATIENTS: Forty-nine patients (52 ears) undergoing cochlear implantation in the setting of ipsilateral sporadic (n = 21) or NF2-associated VS (n = 28). INTERVENTIONS: CI ipsilateral to VS. MAIN OUTCOME MEASURES: Auditory thresholds, consonant-nucleus-consonant (CNC) word scores, and AzBio sentences in quiet scores. RESULTS: Among all patients, median post-CI pure tone average was 28 dB HL (interquartile range [IQR], 21-38), CNC word score was 39% (IQR, 6-62), and AzBio sentences in quiet score was 60% (IQR, 11-83) at a median of 12.5 months postimplantation. Despite the NF2 cohort having larger tumors, when comparing patients with sporadic versus NF2-associated VS, there were no statistically significant differences in CNC word (49% [30-70] vs. 31% [0-52]) or AzBio sentences in quiet (66% [28-80] vs. 57% [5-83]) scores. Regardless of NF2 status, all patients managed with observation, and radiosurgery achieved open-set speech. In patients who underwent microsurgery, 6 (46%) of 13 with NF2 achieved open-set speech recognition compared with 4 (67%) of 6 with sporadic disease. CONCLUSION: Select patients with VS achieve successful hearing rehabilitation with a CI. In this cohort, tumor management strategy significantly influenced CI performance, whereas differences in NF2 status exhibited less effect. Specifically, all patients managed with observation or radiosurgery achieved open-set speech perception, whereas approximately half of people with NF2-related VS and two-thirds of people with sporadic VS achieved this outcome after tumor microsurgery. When disease permits, observation and radiosurgery should be considered in patients who may later pursue a CI.

Natural history of facial paraganglioma with 2 decades of follow-up: A case report and literature review.

OBJECTIVE: To report 20 years of natural history data for a facial paraganglioma and provide a comprehensive review of the existing literature. PATIENT: 81-year-old female with a remote history of cardiac arrest while under anesthesia who elected to observe her facial paraganglioma for 20 years. INTERVENTIONS: Observation, clinical documentation, radiographic surveillance. MAIN OUTCOME MEASURES: Tumor progression, patient symptomatology, and review of management options. RESULTS: The initial presentation of the facial paraganglioma was facial spasm. Over the course of observation, symptoms progressed to include complete facial nerve paralysis, pulsatile tinnitus, and otalgia on the affected side. Radiologic surveillance demonstrated incremental growth and erosion of surrounding structures, including the posterior external auditory canal, stylomastoid foramen, and lateral semicircular canal with near-dehiscence. Twenty-four cases of facial paraganglioma were identified in the extended literature search and are summarized herein. CONCLUSIONS: This unique case contributes to the scarce literature surrounding facial paragangliomas by reporting the extended natural history of this disease.

Patient-Related Factors Do Not Predict Use of Computer-Based Auditory Training by New Adult Cochlear Implant Recipients.

OBJECTIVE: The use of computer-based auditory training (CBAT) after cochlear implantation is associated with improved speech recognition and real-world functional abilities. However, patient-related factors associated with CBAT use remain unknown. This study seeks to identify such factors and recognize those at risk for not implementing CBAT. STUDY DESIGN: Prospective natural experiment. SETTING: Tertiary academic center. PATIENTS: A total of 117 new adult cochlear implant (CI) recipients with bilateral moderate-to-profound hearing loss. INTERVENTIONS/MAIN OUTCOME MEASURES: Patient demographic and lifestyle information, preimplantation aided speech recognition scores, Cochlear Implant Quality of Life (CIQOL) domain and global scores, CIQOL-Expectations scores, and CBAT use in the first 3 months after activation. Patient-related variables included age, sex, race, duration of hearing loss before implantation, hours of CI use per day, hearing-aid use before implantation, living arrangements/marital status, annual household income, employment, technology use, and education. RESULTS: Overall, 33 new CI users (28.2%) used CBAT in the first 3 months after activation. On bivariate analysis of the pre-CI CIQOL scores, CIQOL-Expectations score, aided speech recognition scores, and demographic/lifestyle factors examined, regular use of smartphone, tablet, or computer technology was significantly associated with an increased likelihood of CBAT use (odds ratio, 9.354 [1.198-73.020]), whereas higher CIQOL-Expectations emotional domain scores were associated with a lower likelihood of CBAT use (d = -0.69 [-1.34 to -0.05]). However, using multivariable analysis to control for potential confounding factors revealed no significant associations between CBAT use in the first 3 months after cochlear implantation and any examined factor. CONCLUSIONS: No associations between patient demographic, lifestyle, or pre-CI speech recognition and patient-reported outcome measures and CBAT use were identified. Therefore, discussions with all patients after implantation on the availability of CBAT and its potential benefits are warranted. In addition, given the limited overall use of CBAT and its association with improved CI outcomes, future studies are needed to investigate facilitators and barriers to CBAT use.

Using Clinical Audiologic Measures to Determine Cochlear Implant Candidacy.

INTRODUCTION: Only a small percentage (6-10%) of patients who are candidates receive cochlear implants (CIs). One potential reason contributing to low usage rates may be confusion regarding which patients to refer for CI evaluation. The extent to which information provided by standard clinical audiologic assessments is sufficient for selecting appropriate CI evaluation referrals is uncertain. The objective of this study is to evaluate the capacity of standard clinical audiologic measures to differentiate CI candidates from noncandidates. METHOD: The study design is a retrospective review of a prospectively maintained CI database from a university-based tertiary medical center of 518 patients undergoing CI evaluations from 2012 to 2020. Each ear of each patient was treated as an independent value. Receiver operating characteristic (ROCs) curves were constructed using aided AzBio sentence recognition scores in quiet and aided AzBio +10 dB signal-to-noise ratio scores <60% as binary classifiers for CI candidacy. For each ROC, we examined the capacity of multiple pure-tone thresholds, pure-tone average (PTA), and CNC word recognition scores (WRSs) measured under earphones to determine CI candidacy. Area under the curve ROC (AUC-ROC) values were calculated to demonstrate the capacity of each model to differentiate CI candidates from noncandidates. RESULTS: Variables with the greatest capacity to accurately differentiate CI candidates from noncandidates using aided AzBio in quiet scores were earphone CNC WRS, earphone pure-tone threshold at 1,000 Hz, and earphone PTA (AUC-ROC values = 0.86-0.88). Using aided AzBio +10 scores as the measure for candidacy, only CNC word recognition had a fair capacity to identify candidates (AUC-ROC value = 0.73). Based on the ROCs, a 1,000 Hz pure-tone threshold >50 dB HL, PTA >57 dB HL, and a monosyllabic WRS <60% can each serve as individual indicators for referral for CI evaluations. CONCLUSION: The current study provides initial indicators for referral and a first step at developing evidence-based criteria for CI evaluation referral using standard audiologic assessments.

Use of Auditory Training and Its Influence on Early Cochlear Implant Outcomes in Adults.

OBJECTIVE: Assess associations between postcochlear implant (CI) auditory training and early outcomes related to speech recognition and CI quality of life (CIQOL). STUDY DESIGN: Longitudinal, prospective cohort. SETTING: Tertiary academic center. PATIENTS: Seventy-two adults undergoing cochlear implantation for bilateral severe-to-profound hearing loss. INTERVENTIONS: Self-reported use of three categories of auditory training post-CI activation: (1) face-to-face training (e.g., speech pathologist), (2) passive home-based training (e.g., listening to audiobooks), and (3) computer-based training (e.g., interactive software). MAIN OUTCOME MEASURES: 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 3-month post-CI. RESULTS: Of 72 patients, 52 (72.2%) used an auditory training resource. Of all patients, 18.4% used face-to-face training, 58.3% passive home-based training, and 33.3% computer-based training. At 3 months post-CI, use of any training was associated with greater improvement in speech recognition (d-range = 0.57-0.85) and global and domain-specific CIQOL scores, except entertainment (d-range = -0.33 to 0.77). Use of computer-based training demonstrated the greatest effect, with larger improvements in speech recognition (CNCp: d = 0.69[0.03,1.35]; CNCw: d = 0.80[0.14,1.46]; AzBio: d = 1.11[0.44,1.77]) and global and all domain-specific CIQOL scores (d-range = 0.05-1.35). Controlling for age, sex, household income, and use of multiple training resources, computer-based training remained the strongest positive predictor of speech recognition and CIQOL improvement, with significant associations with CNCp (ß = 33.07[1,43,64.719]), AzBio (ß = 33.03[5.71,60.35]), and CIQOL-global (ß = 10.92[1.15,20.70]) score improvement. CONCLUSIONS: Our findings provide preliminary evidence-based recommendations for use of specific auditory training resources for new adult CI recipients. Auditory training, especially self-directed computer software, resulted in improved speech recognition and CIQOL outcomes after 3 months and are widely available for CI users.