BACKGROUND AND OBJECTIVES: Complete removal of large vestibular schwannomas (VS) poses a significant risk of facial nerve [cranial nerve VII (CNVII)] paralysis. However, less-than-total removal may be associated with an increased risk of regrowth, which could require further treatment with additional risks. The objective was to assess long-term outcomes of postoperative CNVII function in relation to the extent of resection in patients who underwent removal of large VS. METHODS: A multicenter, prospective, nonrandomized cohort study of patients with large VS (≥2.5 cm) who underwent gross total resection (GTR), near-total resection (NTR), or subtotal resection (STR). GTR was defined as no visible remnant, NTR as a remnant <0.5 cm 3 on postoperative MRI, and STR as any larger remnant. CNVII function was evaluated using binary variables: House-Brackmann grades I-II represented excellent-to-good function, whereas House-Brackmann grades III-VI represented fair-to-poor function. RESULTS: A total of 126 patients were included with a mean (SD) follow-up of 60 (±31) months. The mean preoperative tumor diameter was 3.3 ± 0.7 cm. Thirty-five received GTR, 39 received NTR, and 52 received STR based on postoperative MRI. Smaller preoperative tumor size ( P = .03) and volume ( P = .03) were associated with good immediate CNVII function. Neither immediate nor late facial nerve function was related to the extent of resection. STR was associated with a 3-fold higher surgical failure rate compared with GTR ( P = .02), and patients who experienced surgical failure requiring stereotactic radiosurgery or revision surgery had increased odds of fair-to-poor long-term CNVII function (odds ratio 4.6, 95% CI, 1.7-12.5, P = .002). CONCLUSION: The degree of resection did not predict immediate or late good CNVII function. Surgical failure was associated with worse long-term function. GTR or NTR should be attempted whenever feasible to reduce the likelihood of repeat surgery and poor facial nerve outcomes.
BACKGROUND AND OBJECTIVES: Subtotal resection (STR) of large vestibular schwannomas (VS) may achieve better facial nerve outcomes. However, this approach, involving less-than-total tumor removal, may also increase the risk of regrowth. The objective was to examine differences in the regrowth rate based on the extent of resection in patients with large VS. METHODS: A multicenter, prospective, nonrandomized cohort study was conducted on patients with large VS (≥2.5 cm) who underwent gross total resection (GTR), near-total resection (NTR), or STR. GTR was defined as no visible remnant, NTR as a remnant <0.5 cm 3 on postoperative MRI or 5 × 5 × 2 mm during surgery, and STR as any larger remnant. A Cox proportional hazard model assessed the risk of tumor regrowth. RESULTS: Among 126 patients (mean follow-up: 60 ± 31 months; mean tumor diameter: 3.3 ± 0.7 cm), 35 underwent GTR, 39 NTR, and 52 STR. Tumor regrowth occurred in 22 cases (17%). STR patients (27%) had a 3-fold higher regrowth risk compared with GTR patients (8%) ( P = .02). The median regrowth time for STR (24 months) was significantly shorter than for NTR or GTR (48 months, P = .03). A greater residual tumor volume percentage (TV%) was associated with regrowth (hazard ratio 1.73, 95% CI, 1.07-2.81, P = .02). A residual tumor volume of 0.7 cm 3 and a TV% of 12% were identified as optimal cutoff points for predicting regrowth in NTR. CONCLUSION: STR is associated with a significantly higher likelihood of tumor regrowth. The percentage of the residual tumor remnant is positively correlated with the risk of regrowth. A residual tumor volume of 0.7 cm 3 and a TV% of 12% were identified as optimal cutoffs for NTR. When feasible, GTR or NTR should be attempted to minimize the chances of tumor regrowth.
BACKGROUND AND OBJECTIVES: Management of large vestibular schwannomas (VS) increasingly includes partial resection strategies, yet the impact of extent of resection on perioperative and long-term symptom outcomes remains unclear. This study evaluates how the extent of resection influences perioperative measures, complications, and balance performance. METHODS: A prospective, multicenter cohort study was conducted from 2004 to 2019. A total of 126 patients with VS measuring at least 2.5 cm were enrolled. Perioperative outcomes included operation duration, length of stay, and complications such as cerebrospinal fluid leak, wound infection, and meningitis. Long-term outcomes included headache severity, Dizziness Handicap Inventory scores, and standardized balance testing. Analyses accounted for demographic variables, tumor characteristics, and surgical approach. RESULTS: The mean patient age was 48 ± 14 years, and the mean cerebellopontine angle tumor diameter was 3.3 ± 0.7 cm. Based on postoperative MRI, 35 patients underwent gross total resection, 39 underwent near-total resection, and 52 underwent subtotal resection (STR). Operation duration, length of stay, and overall complication rates did not differ by extent of resection. Younger age was associated with cerebrospinal fluid leak ( P = .024), and meningitis was more frequent after translabyrinthine approach ( P = .03). At 1 year, patients who underwent gross total resection or near-total resection exhibited significant improvement in headache severity ( P = .015 and P = .012), whereas STR patients did not. Residual tumor volume showed a significant positive association with Dizziness Handicap Inventory score at 1 year (r = 0.55, P = .03). STR was also associated with poorer postoperative balance performance. CONCLUSION: The extent of resection did not influence perioperative measures or early complication rates in large VS surgery. However, subtotal resection was associated with persistent headaches, greater dizziness handicap, and worse balance outcomes at 1 year. These findings indicate that partial resection does not confer perioperative benefit and may compromise long-term symptom recovery compared with more complete resection.
Purpose: The diagnosis of idiopathic intracranial hypertension (IIH) is often challenging in patients who do not present with classic symptoms. Brain MRI can play a pivotal role, as several imaging findings, such as an empty sella appearance (ESA), have been shown to be associated with IIH. Yet, none of the MRI signs have been shown to have a high sensitivity and specificity. In this study, we tested the hypothesis that presence of a geniculate ganglion diverticulum (GGD) is a potential imaging marker for the detection of IIH. Materials and methods: This is an IRB-approved, single-institution, retrospective, observational study. Brain MRI examinations of patients referred to Radiology by Otology clinic over a period of 10 years were reviewed. 244 MRI exams fulfilling inclusion and exclusion criteria were independently screened for the presence of GGD and ESA by two Neuroradiology fellows. Electronic medical records (EMR) of patients in this study were reviewed for presence of clinical manifestations of IIH. Receiver operator characteristic (ROC) curves were generated to estimate the accuracy of each covariate in diagnosing IIH. The area under each ROC curve (AUC) was calculated to identify an accurate prognostic covariate. Statistical analysis was done using R programming language V 4.2.2. Results: GGD was identified in MRI exams of 51 patients. A 2:1 propensity score (PS) matching for age, gender, and Body Mass Index (BMI) was used to select non-GGD control group for comparison with the GGD group. There was strong agreement between the 2 reviewers (kappa = 0.81, agreement = 95 %). Twelve patients in this study were diagnosed with IIH. There was a high incidence of GGD (OR = 12.19, 95 % CI (2.56, 58.10)) and ESA (OR = 4.97, 95 % CI (1.47, 16.74)) in IIH patients. The AUC observed in GGD for predicting IIH was 0.771 (0.655-0.888), specificity = 0.709 (0.638-0.780), and sensitivity = 0.833 (0.583-1). The AUC observed for ESA in predicting IIH was 0.682 (0.532-0.831), specificity = 0.780 (0.709-0.844), and sensitivity = 0.583 (0.333-0.833). Conclusion: GGD is potentially a novel imaging marker of IIH with sensitivity higher than and specificity comparable to that of ESA.
Recent advances in generative artificial intelligence (AI) have enabled remarkable capabilities in generating images, audio, and videos from textual descriptions. Tools like Midjourney and DALL-E 3 can produce striking visualizations from simple prompts, while services like Kaiber.ai and RunwayML Gen-2 can generate short video clips. These technologies offer intriguing possibilities for clinical and educational applications in otolaryngology. Visualizing symptoms like vertigo or tinnitus could bolster patient-provider understanding, especially for those with communication challenges. One can envision patients selecting images to complement chief complaints, with AI-generated differential diagnoses. However, inaccuracies and biases necessitate caution. Images must serve to enrich, not replace, clinical judgment. While not a substitute for healthcare professionals, text-to-image and text-to-video generation could become valuable complementary diagnostic tools. Harnessed judiciously, generative AI offers new ways to enhance clinical dialogues. However, education on proper, equitable usage is paramount as these rapidly evolving technologies make their way into medicine.
OBJECTIVES:Distinguishing between sporadic and germline/mosaic NF2-related schwannomatosis is important to ensure that patients have appropriate long-term care. With this report, we describe a unique case of a patient with 4 ipsilateral schwannomas and identify a combination of sequencing modalities that can accurately diagnose mosaic NF2-related schwannomatosis. METHODS:We present a 32-year-old woman with a familial history of vestibular schwannoma in her father and right-sided schwannomas involving the apical and basal turns of cochlea, lateral semicircular canal, and internal auditory canal (IAC). Genetic analysis of blood and frozen tissue from 2 tumors (intralabyrinthine and IAC tumors) was performed using next-generation sequencing (NGS), multiplex ligation-dependent probe amplification (MLPA), and optical genome mapping (OGM). RESULTS:Germline testing for NF2, LZTR1, and SMARCB1 was negative. Tumor genetic testing revealed a shared NF2 pathogenic variant between the 2 tumors ("first hit") but distinct "second hit" NF2 variants, including mosaic loss of chromosome 22 in the IAC tumor seen only with OGM, consistent with mosaic NF2-related schwannomatosis. CONCLUSIONS:Multimodality sequencing, including NGS, MLPA, and OGM, was required to ensure appropriate diagnosis of mosaic NF2-related schwannomatosis in this patient. A similar approach can be used for other patients with multiple ipsilateral tumors and suspected tumor predisposition.
Objective: To summarize adverse events and their root causes reported to the United States Food and Drug Administration (FDA) on Vibrant Soundbridge (VSB) hearing device (Med-El, Innsbruck, Austria), an active middle ear implant for patients with moderate to severe hearing loss. Materials and methods: The FDA 's Manufacturer and User Facility Device Experience (MAUDE) database was queried for reports of VSB adverse events from January 1, 2012, to July 27, 2022. Results: Six hundred sixty-three total medical device reports were identified, from which 913 adverse events were extracted. Of these, 498 (54.5 %) were adverse events to patients (AEPs), while 415 (45.5 %) were device malfunctions (DMs). The most common AEPs were hearing performance issues 428 (85.9 %). The most common DMs were compromised conductive link 125 (30.1 %). Root causes identified for DMs were iatrogenic 85 (58.6 %), patient -related 28 (19.3 %), and trauma and external causes 32 (22.1 %). The most common iatrogenic root cause 12 (14.1 %) involved damage to the conductive link during revision surgery. The most common patientrelated causes of DMs were excessive middle ear tissue growth 16 (57 %), and abrupt body movements 5 (28.6 %). The most common external cause of DM was cleaning of the ear canal or mastoid cavity 20 (62.5 %). Conclusions: Despite its well-known limitations, the MAUDE database provides valuable information on possible complications of VSB as it relates to device malfunction or adverse events for patients. Implementation of standardized reports with relevant and well-defined categories could certainly allow for a more meaningful analysis.
OBJECTIVE: Stereotactic radiosurgery (SRS) is a well-established treatment for vestibular schwannomas (VS). Hearing loss remains a main morbidity of VS and its treatments, including SRS. The effects of radiation param-eters of SRS on hearing remain unknown. The goal of this study is to determine the effect of tumor volume, patient demographics, pretreatment hearing status, cochlear radiation dose, total tumor radiation dose, fractionation, and other radiotherapy parameters on hearing deterioration.METHODS: Multicenter retrospective analysis of 611 patients who underwent SRS for VS from 1990-2020 and had pre-and post-treatment audiograms.RESULTS: Pure tone averages (PTAs) increased and word recognition scores (WRSs) decreased in treated ears at 12- 60 months while remaining stable in untreated ears. Higher baseline PTA, higher tumor radiation dose, higher maximum cochlear dose, and usage of single fraction resulted in higher post radiation PTA; WRS was only predicted by baseline WRS and age. Higher baseline PTA, single fraction treatment, higher tumor radiation dose, and higher maximum cochlear dose resulted in a faster deterioration in PTA. Below a maximum cochlear dose of 3 Gy, there were no statistically significant changes in PTA or WRS.CONCLUSIONS: Decline of hearing at one year in VS patients after SRS is directly related to maximum cochlear dose, single versus 3-fraction treatment, total tumor radiation dose, and baseline hearing level. The maximum safe cochlear dose for hearingtbrowd preservation at one year is 3 Gy, and the use of 3 fractions instead of one fraction was better at preserving hearing.
Objective: After demonstration of face validity of a surgical middle ear simulator (SMS) previously, we assessed the content validity of the simulator with otolaryngology residents. Study Design: Multicenter randomized prospective international study. Setting: Four academic institutions. Methods: Novice participants were randomized into control, low-fidelity (LF), and high-fidelity (HF) groups. Control and LF produced 2 recordings from 2 attempts, and HF produced 4 recordings from10 attempts, with trials 1, 4, 7, and 10 used for scoring. Three blinded experts graded videos of the simulated stape-dectomy operation using an objective skills assessment test format consisting of global and stapedotomy-specific scales. Results: A total of 152 recordings from 61 participants were included. Baseline characteristics did not differ significantly between groups. Depending on the step of the operation, inter-rater reliability ranged from24 to 90%. For LFand HF, years of training was significantly associated with improved scores in certain objective skills assessment test subparts. HF outperformed the control group on stapes and global scores (p < 0.05). The HF group demonstrated improvement in global score over trials, but plateaued after four trials. Scores varied greatly for participants from different institutions in certain operative steps, such as transecting incudostapedial joints, likely due to differences in instrumentation and time elapsed since manufacture. Conclusion: Practice with SMS led to better performance in both global and stapes-specific scores. Further studies are needed to examine construct validity and to create otology-appropriate grading systems. Variables like instrumentation and decline in flexibility of the simulator after 12 months greatly affect performance on the simulator.
Objectives/Hypothesis To understand the effect of the COVID-19 pandemic on the volume, quality, and impact of otolaryngology publications. Study Design Retrospective analysis. Methods Fifteen of the top peer-reviewed otolaryngology journals were queried on PubMed for COVID and non-COVID-related articles from April 1, 2020 to March 31, 2021 (pandemic period) and pre-COVID articles from the year prior. Information on total number of submissions and rate of acceptance were collected from seven top-ranked journals. Results Our PubMed query returned 759 COVID articles, 4,885 non-COVID articles, and 4,200 pre-COVID articles, corresponding to a 34% increase in otolaryngology publications during the pandemic period. Meta-analysis/reviews and miscellaneous publication types made up a larger portion of COVID publications than that of non-COVID and pre-COVID publications. Compared to pre-COVID articles, citations per article 120 days after publication and Altmetric Attention Score were higher in both COVID articles (citations/article: 2.75 +/- 0.45, P < .001; Altmetric Attention Score: 2.05 +/- 0.60, P = .001) and non-COVID articles (citations/article: 0.03 +/- 0.01, P = .002; Altmetric Attention Score: 0.67 +/- 0.28, P = .016). COVID manuscripts were associated with a 1.65 times higher acceptance rate compared to non-COVID articles (P < .001). Conclusions COVID-19 was associated with an increase in volume, citations, and attention for both COVID and non-COVID articles compared to pre-COVID articles. However, COVID articles were associated with lower evidence levels than non-COVID and pre-COVID articles. Level of Evidence Level 3 Laryngoscope, 2021
Idiopathic intracranial hypertension (IIH), also called pseudotumor cerebri syndrome, is a disorder defined as elevated intracranial pressure (ICP) of unknown cause. It is a diagnosis of exclusion in most cases, and all other forms of elevated ICP must be ruled out. With its increasing prevalence, it is much more likely for physicians, otolaryngologists included, to encounter this condition. It is important to have a clear understanding of the typical and atypical presentation of this disease, along with its evaluative workup and management options. This article reviews IIH with a focus on those factors that are specifically relevant to otolaryngologic care.
Objectives: Ossicular prostheses have demonstrated utility in ossicular chain reconstruction, but their use also is associated with complications. This study aims to explore the utility of the U.S. Food and Drug Administration's Manufacturer and User Facility Device Experience (MAUDE) database for the systematic analysis of complications associated with ossicular prostheses. It also summarizes adverse events to patients (AEPs) and device malfunctions (DMs) reported in the MAUDE database, and attempts to examine the consequences brought about by these events and to identify likely root causes. Study Design: Retrospective analysis of the MAUDE database. Methods: The MAUDE database was queried for reports from January 2010 to May 2020. Data were extracted from reports involving the use of ossicular prostheses. Results: Seventy-three medical device reports, reports submitted to the Food and Drug Administration detailing suspected device-associated malfunctions, injuries, and deaths, involved ossicular prostheses and were included for analysis, from which 126 adverse events were extracted. Of these, 50 (39.7%) were AEPs, while 76 (60.3%) were DMs. The most common AEPs were foreign body and hearing loss, while the DMs reported most commonly were broken prosthesis and displaced prosthesis. Of the 76 DMs, 19 (25%) were attributed to mishandling by the operator, while 6 (7.9%) were due to a defective device. Broken prosthesis was the DM most commonly attributed to mishandling by the operator in 16 (32.7%) cases. Conclusion: Ossicular prostheses are used extensively for ossicular chain reconstruction, but also are associated with adverse events. The MAUDE database in its current form does not appear sufficient for the systematic review of adverse events associated with ossicular prostheses. A more standardized reporting format with clearly defined categories and inclusion of relevant variables might improve the quality of information provided by the MAUDE database.
Idiopathic intracranial hypertension (IIH), also called pseudotumor cerebri syndrome, is a disorder defined as elevated intracranial pressure (ICP) of unknown cause. It is a diagnosis of exclusion in most cases, and all other forms of elevated ICP must be ruled out. With its increasing prevalence, it is much more likely for physicians, otolaryngologists included, to encounter this condition. It is important to have a clear understanding of the typical and atypical presentation of this disease, along with its evaluative workup and management options. This article reviews IIH with a focus on those factors that are specifically relevant to otolaryngologic care.
Objectives: To characterize patterns of sensorineural hearing loss (SNHL) and tinnitus in patients with idiopathic intracranial hypertension (IIH). Study Design: Retrospective chart review. Setting: Tertiary referral center. Patients: Adult patients diagnosed with IIH via lumbar puncture (LP) between 2010 and 2020 who had available audiograms. The study included a total of 40 patients; 33 women, and 7 men with a median age of 43. Interventions: Diagnostic LP and audiogram. Main Outcome Measures: Otologic symptoms, ophthalmologic signs, hearing thresholds, cerebrospinal fluid opening pressures. Results: The most commonly reported symptoms were tinnitus in 28 (70%, 23 pulsatile and 5 tonal), aural fullness in 11 (28%), and vertigo in 10 (25%). Twenty-nine patients had ophthalmologic examinations and 18 had evidence of papilledema. Twenty-five (63%) patients had hearing loss in at least one ear at one frequency range. Patients presented with both unilateral and bilateral hearing loss across low, middle, and high frequency ranges. No significant association was observed between hearing loss threshold and LP opening pressure except for 250 Hz in the left ear. After stratification by tinnitus group (pulsatile, tonal, and no tinnitus), no significant difference was found between mean hearing loss threshold at different frequencies. In addition, no significant mean age difference was identified in patients within each tinnitus group. Conclusions: There was no classic pattern or presentation for hearing loss in our IIH patients. They developed sudden, unilateral, or bilateral SNHL in low, middle, or high frequency range. The degree of hearing loss did not correlate with CSF opening pressure.
Vestibular migraine (VM) is one of the most common neurologic causes of vertigo. Symptoms and International Classification of Headache Disorders criteria are used to diagnose VM because no objective tests, imaging or audiologic, have been shown to reliably diagnose this condition. Central auditory, peripheral, and central vestibular pathway involvement has been associated with VM. Although the interaction between migraine and other vestibular disorders can be a challenging scenario for diagnosis and treatment, there are data to show that vestibular rehabilitation and a variety of pharmacologic agents improve reported symptoms and vertigo frequency.
OBJECTIVES:To assess a middle ear simulator as a surgical training tool among a cohort of novice trainees.STUDY DESIGN:Prospective pilot study.SETTING:The George Washington University Hospital (tertiary care academic hospital).PARTICIPANTS:Twenty one medical students and physician assistant students completed the protocol. Each student produced four recordings over 10 attempts. The two raters were attending surgeons from the George Washington University Hospital.INTERVENTIONS:Stapedotomies performed on a high-fidelity middle ear simulator. Participants were assessed at baseline with a Purdue pegboard test and trained with video and a low fidelity middle ear simulator.MAIN OUTCOME MEASURES:Two experts rated recordings on scales based upon a validated objective skills assessment test (OSAT) format.RESULTS:Inter-rater reliability was strong across all stapedotomy skills scores and global rating scores. Participants demonstrated statistically significant improvement up to the third recording (seventh attempt), but the scores on the fourth recording (tenth attempt) were not significantly different from the third. One participant failed to improve in score over 10 attempts. Pegboard test performance was not correlated with score improvement. Low-fidelity trial time was significantly correlated to stapedotomy and global rating scores.CONCLUSIONS:This pilot study serves as the first investigation of this middle ear simulator amongst a cohort of trainees. A high-fidelity middle ear simulator may serve as a measurement tool to select future surgical trainees, customize training pathways, and assess surgical capacity before graduation.
Data sharing is not applicable to this article as no data sets were generated or analyzed during the current study.
Biologics have been widely adopted in multiple subspecialties of otolaryngology. This article provides an overview of past, present, and future uses of biologics in otolaryngology with emphasis on allergic rhinitis, chronic rhinosinusitis with polyposis, head and neck squamous cell carcinoma, salivary and skull base tumors, hearing loss, and other otologic disorders.