Stapedotomy Versus Cochlear Implantation for Far Advanced Otosclerosis: Insights From a Patient With Matched Preoperative and Postoperative Function.

OBJECTIVE: To examine patient preference after stapedotomy versus cochlear implantation in a unique case of a patient with symmetrical profound mixed hearing loss and similar postoperative speech perception improvement. PATIENTS: An adult patient with bilateral symmetrical far advanced otosclerosis, with profound mixed hearing loss. INTERVENTION: Stapedotomy in the left ear, cochlear implantation in the right ear. MAIN OUTCOME MEASURE: Performance on behavioral audiometry, and subjective report of hearing and intervention preference. RESULTS: A patient successfully underwent left stapedotomy and subsequent cochlear implantation on the right side, per patient preference. Preoperative audiometric characteristics were similar between ears (pure-tone average [PTA] [R: 114; L: 113 dB]; word recognition score [WRS]: 22%). Postprocedural audiometry demonstrated significant improvement after stapedotomy (PTA: 59 dB, WRS: 75%) and from cochlear implant (PTA: 20 dB, WRS: 60%). The patient subjectively reported a preference for the cochlear implant ear despite having substantial gains from stapedotomy. A nuanced discussion highlighting potentially overlooked benefits of cochlear implants in far advanced otosclerosis is conducted. CONCLUSION: In comparison with stapedotomy and hearing aids, cochlear implantation generally permits greater access to sound among patients with far advanced otosclerosis. Though the cochlear implant literature mainly focuses on speech perception outcomes, an underappreciated benefit of cochlear implantation is the high likelihood of achieving "normal" sound levels across the audiogram.

Repeat Radiosurgery for Sporadic Vestibular Schwannoma After Primary Radiosurgical Failure: An International Multi-institutional Investigation.

OBJECTIVE: To describe outcomes of patients with sporadic vestibular schwannoma (VS) who underwent repeat stereotactic radiosurgery (SRS) after primary SRS failure. STUDY DESIGN: Multi-institutional historical cohort study. SETTING: Five tertiary care referral centers. PATIENTS: Adults ≥18 years old with sporadic VS. INTERVENTION: Primary and repeat treatment with SRS. MAIN OUTCOME MEASURE: Microsurgery-free survival after repeat SRS. RESULTS: Across institutions, 32 patients underwent repeat SRS after primary SRS. Most patients (74%) had tumors with cerebellopontine angle extension at primary SRS (median size, 13.5 mm [interquartile range, 7.5-18.8] mm). After primary SRS, patients underwent repeat SRS at a median of 4.8 years (interquartile range, 3.2-5.7 yr). For treatment modality, 30 (94%) patients received gamma knife for primary treatment and 31 (97%) patients received gamma knife as their repeat treatment. Median tumor volume increased from 0.970 cm3 at primary SRS to 2.200 cm3 at repeat SRS. Facial nerve function worsened in two patients after primary SRS and in two patients after repeat SRS. There were no instances of intracranial complications after repeat SRS. Microsurgery-free survival rates (95% confidence interval; number still at risk) at 1, 3, and 5 years after repeat SRS were 97% (90-100%, 24), 84% (71-100%, 13), and 68% (48-96%, 6), respectively. There was one occurrence of malignancy diagnosed after repeat radiosurgery. CONCLUSION: Overall, repeat SRS for sporadic VS has comparable risk profile, but lower rates of tumor control, compared with primary SRS.

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.

Salvage Microsurgery Following Failed Primary Radiosurgery in Sporadic Vestibular Schwannoma.

Importance: Management of sporadic vestibular schwannoma with radiosurgery is becoming increasingly common globally; however, limited data currently characterize patient outcomes in the setting of microsurgical salvage for radiosurgical failure. Objective: To describe the clinical outcomes of salvage microsurgery following failed primary stereotactic radiosurgery (SRS) or fractionated stereotactic radiotherapy (FSRT) among patients with sporadic vestibular schwannoma. Design, Setting, and Participants: This was a cohort study of adults (≥18 years old) with sporadic vestibular schwannoma who underwent salvage microsurgery following failed primary SRS/FSRT in 7 vestibular schwannoma treatment centers across the US and Norway. Data collection was performed between July 2022 and January 2023, with data analysis performed between January and July 2023. Exposure: Salvage microsurgical tumor resection. Main Outcomes and Measures: Composite outcome of undergoing less than gross total resection (GTR) or experiencing long-term facial paresis. Results: Among 126 patients, the median (IQR) age at time of salvage microsurgery was 62 (53-70) years, 69 (55%) were female, and 113 of 117 (97%) had tumors that extended into the cerebellopontine angle at time of salvage. Of 125 patients, 96 (76%) underwent primary gamma knife SRS, while 24 (19%) underwent linear accelerator-based SRS; the remaining patients underwent FSRT using other modalities. Postoperative cerebrospinal fluid leak was seen in 15 of 126 patients (12%), hydrocephalus in 8 (6%), symptomatic stroke in 7 (6%), and meningitis in 2 (2%). Each 1-mm increase in cerebellopontine angle tumor size was associated with a 13% increased likelihood of foregoing GTR (64 of 102 patients [63%]) or long-term postoperative House-Brackmann grade higher than I (48 of 102 patients [47%]) (odds ratio, 1.13; 95% CI, 1.04-1.23). Following salvage microsurgery, tumor growth-free survival rates at 1, 3, and 5 years were 97% (95% CI, 94%-100%), 93% (95% CI, 87%-99%), and 91% (95% CI, 84%-98%), respectively. Conclusions: In this cohort study, more than half of patients who received salvage microsurgery following primary SRS/FSRT underwent less than GTR or experienced some degree of facial paresis long term. These data suggest that the cumulative risk of developing facial paresis following primary SRS/FSRT by the end of the patient's journey with treatment approximates 2.5% to 7.5% when using published primary SRS/FSRT long-term tumor control rates.

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.

Neuroimaging Characteristics of Hearing Loss in the Mayo Clinic Study of Aging.

OBJECTIVE: To investigate the association between standard pure tone and speech audiometry with neuroimaging characteristics reflective of aging and dementia in older adults. STUDY DESIGN: Prospective population-based study. SETTING: Single tertiary care referral center. METHODS: Participants from the Mayo Clinic Study of aging 60 years old or older with normal cognition or mild cognitive impairment, baseline neuroimaging, and a behavioral audiogram associated with neuroimaging were eligible for study. Imaging modalities included structural MRI (sMRI) and fluid-attenuated inversion recovery MRI (FLAIR-MRI; N = 605), diffusion tensor imaging MRI (DTI-MRI; N = 444), and fluorodeoxyglucose-positron emission tomography (FDG-PET; N = 413). Multivariable logistic and linear regression models were used to evaluate associations with neuroimaging outcomes. RESULTS: Mean (SD) pure tone average (PTA) was 33 (15) dB HL and mean (SD) word recognition score (WRS) was 91% (14). There were no significant associations between audiometric performance and cortical thinning assessed by sMRI. Each 10-dB increase in PTA was associated with increased likelihood of abnormal white-matter hyperintensity (WMH) from FLAIR-MRI (odds ratio 1.26, P = .02). From DTI-MRI, participants with <100% WRSs had significantly lower fractional anisotropy in the genu of the corpus callosum (parameter estimate [PE] -0.012, P = .008) compared to those with perfect WRSs. From FDG-PET, each 10% decrease in WRSs was associated with decreased uptake in the anterior cingulate cortex (PE -0.013, P = .001). CONCLUSION: Poorer audiometric performance was not significantly associated with cortical thinning but was associated with white matter damage relevant to cerebrovascular disease (increased abnormal WMH, decreased corpus callosum diffusion). These neuroimaging results suggest a pathophysiologic link between hearing loss and cerebrovascular disease.

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.

Natural History of Serviceable Hearing During Active Surveillance of Nongrowing Sporadic Vestibular Schwannoma Supports Consideration of Initial Wait-and-Scan Management.

OBJECTIVE: The treatment paradigm of vestibular schwannoma (VS) focuses on preservation of neurologic function, with small tumors increasingly managed with active surveillance. Often, tumor size and hearing outcomes are poorly correlated. The aim of the current work was to describe the natural history of hearing among patients with nongrowing VS during observational management. STUDY DESIGN: Historical cohort study. PATIENTS: Adults with sporadic VS. INTERVENTION: Wait-and-scan management. MAIN OUTCOME MEASURE: Maintenance of serviceable hearing (SH) after diagnosis. RESULTS: Among 228 patients with nongrowing VS, 157 patients had SH at diagnosis. Rates of maintaining SH (95% CI; number still at risk) at 1, 3, and 5 years after diagnosis were 94% (89-98; 118), 81% (74-89; 65), and 78% (71-87; 42), respectively. Poorer hearing at diagnosis (hazard ratio [HR] per 10 dB hearing level increase in pure-tone average of 2.51, p < 0.001; HR per 10% decrease in word recognition score of 1.70, p = 0.001) was associated with increased likelihood of developing non-SH during observation. When controlling for baseline hearing status, tumors measuring 5 mm or greater in the internal auditory canal or with cerebellopontine angle extension were associated with significantly increased risk of developing non-SH (HR, 4.87; p = 0.03). At 5 years after diagnosis, 95% of patients with nongrowing intracanalicular VS measuring less than 5 mm maintained SH. CONCLUSIONS: Hearing worsens during periods of nongrowth in sporadic VS. Patients with small (<5 mm) intracanalicular tumors demonstrate robust maintenance of SH over time, reinforcing the consideration of initial observation in this patient subset.

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.

Teaching module for obesity bias education: incorporating comprehensive competencies and innovative techniques.

BACKGROUND: The majority of the United States population is overweight or obese, and obesity bias is frequently reported by patients. Obesity bias is associated with adverse health outcomes, even independent of body weight. Primary care residents are often sources of obesity bias towards patients with weight, yet education regarding obesity bias is significantly lacking in most family medicine residency teaching curricula. The aim of this study is to describe an innovative web-based module on obesity bias and discuss its impact in family medicine residents. METHODS: The e-module was developed by an interprofessional team of health care students and faculty. It consisted of a 15-minute video containing five clinical vignettes that depicted instances of explicit and implicit obesity bias in a patient-centered medical home (PCMH) model. Family medicine residents viewed the e-module as part of a dedicated one-hour didactic on obesity bias. Surveys were administered prior to and following the viewing of the e-module. They assessed previous education on obesity care, comfort in working with patients with obesity, residents' understanding of their own biases in working with this population, and the anticipated impact of the module on future patient care. RESULTS: A total of 83 residents from three family medicine residency programs viewed the e-module and 56 completed both the pre and post survey. There was a significant improvement in residents' comfort in working with patients with obesity as well as their understanding of their own biases. CONCLUSION: This teaching e-module is a short, interactive, web-based educational intervention that is free and open-sourced. The first-person patient perspective allows learners to better understand the patient's point of view and its PCMH setting illustrates interactions with a variety of healthcare professionals. It was engaging and well received by family medicine residents. This module can begin the conversation around obesity bias, leading to improved patient care.

Age, ASA-status, and Changes in NSQIP Comorbidity Indices Reporting in Facial Fracture Repair.

OBJECTIVE: To evaluate the impact of age and the American Society of Anesthesiologists (ASA) classification on post operative outcomes as well as the changes in the National Surgical Quality Improvement Program (NSQIP) database reporting of comorbidity index variables in patients with facial fractures. METHODS: The NSQIP database was queried for facial fracture repair CPT codes between 2012 and 2019 and for modified Frailty Index (mFI) and modified Charlson Comorbidity Index (mCCI) variables between years 2006 and 2018. The predominant question analyzed two preoperative risk factors: patient and ASA classification. Chi-square analysis, Kruskal-Wallis, Mann-Whitney, Spearman correlation, and multivariable logistic regression were used to evaluate age and ASA classification with wound dehiscence, superficial surgical site infection (SSSI), deep wound infection (DWI), readmission status, and return to the OR. The reporting of indices variables was evaluated with descriptive statistics. CONCLUSION: In this large database with univariate analysis, patients with a higher ASA classification and older patients experience significantly increased risks of readmission, return to the OR, and longer hospital stays. On multivariate analyses, ASA classes II, III, and IV are independently associated with increased risk of readmission and return to the OR, while controlling for patient age. The reporting of all mFI and mCCI variables were consistent from 2006 to 2010, but after 2011, there has been inconsistent or absent reporting of variables, therefore, conclusions on the impact of comorbidities on facial fracture repair are unreliable. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:2572-2577, 2023.

Hearing Preservation in Observed Sporadic Vestibular Schwannoma: A Systematic Review.

OBJECTIVE: To characterize the natural history of hearing loss for patients presenting with serviceable hearing (SH) who undergo a wait-and-scan approach for sporadic vestibular schwannoma (VS) using aggregate time-to-event survival analysis. STUDY DESIGN: Systematic review. SETTING: Published international English literature, January 1, 2000 to May 31, 2020. PATIENTS: Patients with sporadic VS entering a wait-and-scan approach with SH at diagnosis. INTERVENTIONS: Observation with serial MRI and audiometry. RESULTS: In total, 3,652 patients from 26 studies were included for analysis. Mean age at diagnosis was 58.8 years (SD, 4.1). Mean follow-up was 49.2 months (SD, 26.5). In total, 755 patients (21%) failed conservative treatment and underwent radiosurgery or microsurgery at the time of last follow-up. The average loss to follow-up was 6.9% (SD, 11.1). A total of 1,674 patients had SH at the time of diagnosis. Survival rates for maintaining SH were 96% at 1 year, 77% at 3 years, 62% at 5 years, and 42% at 10 years following diagnosis. CONCLUSION: In this systematic review, aggregate data from 3,652 patients across 26 studies show consistent patterns in progression of hearing loss during observation for patients with sporadic VS as a function of time. As an easy-toremember conservative benchmark for those presenting with SH at diagnosis: approximately 75% retain SH at 3 years, 60% at 5 years, and 40% at 10 years.

Intraoperative Use of Electrical Stapedius Reflex Testing for Cochlear Nerve Monitoring During Simultaneous Translabyrinthine Resection of Vestibular Schwannoma and Cochlear Implantation.

OBJECTIVE: To report the novel use of intraoperative electrically evoked stapedial reflex (eSR) for cochlear nerve monitoring during simultaneous translabyrinthine resection of vestibular schwannoma (VS) and cochlear implantation. STUDY DESIGN: Clinical capsule report with video demonstration. SETTING: Tertiary academic referral center. PATIENT: A 58-year-old female presented with a small right intracanalicular VS with associated asymmetrical right moderate to severe sensorineural hearing loss, poor word recognition, tinnitus, and disequilibrium. Based on patient symptomatology and goals, simultaneous CI with translabyrinthine resection of the VS was performed. INTERVENTION: Cochlear implantation before the tumor was resected facilitated intraoperative eSRs by delivering repeated single-electrode stimulations through the cochlear implant (CI) electrode during tumor resection. A pulse duration of 50-us and a current amplitude of 200-CL or 648-us was used to elicit eSRs visible through the facial recess. Intraoperative eSR was monitored in conjunction with electrically evoked compound action potentials via neural response telemetry and electrical auditory brainstem response. RESULTS: Despite the transient evoked compound action potentials amplitude and electrical auditory brainstem response latency changes, the visually observed eSR was preserved and remained robust throughout tumor dissection, indicating an intact cochlear nerve. Four weeks postoperatively, the patient exhibited open-set speech capacity (14% CNC and 36% AzBio in quiet). CONCLUSION: The current study demonstrates the feasibility of using intraoperative eSR via a CI electrode to monitor cochlear nerve integrity during VS resection, which may indicate successful CI outcomes. These preliminary findings require further substantiation in a larger study.

Intraventricular Septation in the Context of Dilated Cardiomyopathy Associated With TTN Mutation.

A 6-month-old infant boy presented with symptomatic heart failure. Dilated cardiomyopathy was found in association with a mutation in TTN. Structural heart disease included novel septation of the left ventricle with a fenestrated membrane resulting from aberrant congenital mitral valve apparatus formation. (Level of Difficulty: Advanced.).