Adverse events associated with Vibrant Soundbridge: A MAUDE study.

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 patient-related 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.

Four distinct ipsilateral vestibular schwannomas: A case of mosaic NF2-related schwannomatosis.

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.

Content Validity of a High-Fidelity Surgical Middle Ear Simulator: A Randomized Prospective International Multicenter Trial.

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 from 10 attempts, with trials 1, 4, 7, and 10 used for scoring. Three blinded experts graded videos of the simulated stapedectomy 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 from 24 to 90%. For LF and 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.

Impact of Cochlear Dose on Hearing Preservation Following Stereotactic Radiosurgery in Treatment of Vestibular Schwannomas: A Multi-Center Study.

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 parameters 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.

Malignant Pineal Parenchymal Tumors in Adults: A National Cancer Database Analysis.

BACKGROUND: Limited retrospective data exist on malignant pineal parenchymal tumors (PPTs) in adults, and there are no large previous studies that review clinical outcomes across the 3 treatment arms of surgery, radiotherapy, and chemotherapy. As a result, optimal disease management has yet to be defined. OBJECTIVE: To evaluate treatment trends and perform survival analysis in adult PPT. METHODS: The National Cancer Database was queried for histologically confirmed PPT diagnosed from 2007 to 2016. Univariate and multivariate Cox regressions were used to evaluate the prognostic impact of covariates. Kaplan-Meier survival curves were generated for comparative subanalyses. RESULTS: Of the 251 patients who met inclusion criteria, 172 had PPTs of intermediate differentiation (PPTID) and 79 had pineoblastoma. A plurality of patients with pineoblastoma were treated with trimodal therapy (39.1%), whereas patients with PPTID were commonly treated with either surgery alone or surgery and radiation (33.7% each). Factors independently associated with improved overall survival include younger patient age, female sex, lower comorbidity score, lower tumor grade, and treatment with surgery or radiation (each P < .05). Subanalyses confirm the effect of radiation on survival in patients with grade III PPTID with subtotal resection; however, no survival benefit of adjuvant radiation is demonstrated in patients with grade II PPTID with subtotal resection. CONCLUSION: Although radiotherapy and surgery were found to increase survival in all patients with PPT, there was no demonstrable survival benefit of adjuvant radiation in surgically treated patients with grade II PPTID. This suggests that adjuvant radiotherapy may not add significant survival benefit in many adult patients with grade II PPTID.

Idiopathic Intracranial Hypertension: A Comprehensive Overview.

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.

Adverse Events Associated With Ossicular Prostheses: Utility of a Federal Database.

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.

Impact of COVID-19 on Otolaryngology Literature.

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: 3 Laryngoscope, 132:1364-1373, 2022.

Vestibular Migraine and Its Comorbidities.

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.

Head and neck osteosarcomas: Analysis of treatment trends and survival outcomes in the United States (2004-2016).

BACKGROUND: Head and neck osteosarcoma (HNOS) is a rare primary bone tumor with limited data to guide treatment approaches. METHODS: The NCDB was used to identify patients diagnosed with HNOS. Kaplan-Meier and Cox multivariate regression were used to examine the impact of each treatment on overall survival (OS). RESULTS: We identified 821 patients diagnosed with HNOS. Utilization of neoadjuvant chemotherapy + surgery increased from zero cases in 2004 to 24% of cases in 2016. Among surgically treated patients, 5-year OS was 55.8% (CI: 51.7-60.1%). No difference was seen in OS between each treatment cohort on multivariate analysis. However, neoadjuvant chemotherapy + surgery + adjuvant chemotherapy had improved 18-month survival compared to surgery alone (95.8% vs. 78.5%, p = 0.031). CONCLUSIONS: Our study demonstrated no survival benefit in perioperative chemotherapy or radiation therapy; however, short-term survival improvement in patients receiving both neoadjuvant and adjuvant chemotherapy displays promise and requires further investigation.

High-Fidelity Surgical Middle Ear Simulator: A Pilot Study.

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.

Sensorineural Hearing Loss and Tinnitus Characteristics in Patients With Idiopathic Intracranial Hypertension.

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.

Biologics in Otolaryngology: Past, Present, and Future.

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.

Sensorineural Hearing Loss Caused by Labyrinthine Erosion Due to a Cerebrospinal Fluid Diverticulum.

OBJECTIVE: To present a case of progressive sensorineural hearing loss (SNHL) caused by labyrinthine erosion secondary to expanding geniculate ganglion cerebrospinal fluid (CSF) diverticulum extending along the path of the facial nerve. PATIENT: Thirteen-year-old man with no past medical history or risk factors presented with unilateral progressive SNHL and no other otologic or neurologic symptoms. INTERVENTIONS: Audiological data as well as imaging studies including a magnetic resonance imaging (MRI) of the brain and internal auditory canal and a temporal bone computed tomography (CT) are presented. Partial labyrinthectomy, CSF leak closure, and cochlear implantation were performed. A lumbar puncture was obtained 2 months postoperatively. MAIN OUTCOME MEASURE: Closure of the CSF leak. RESULTS: Mastoidectomy revealed a wide area of CSF leak through the geniculate ganglion and fallopian canal with partial destruction of the superior and lateral semicircular canals by an expanding diverticulum. CONCLUSIONS: This is an unusual presentation of progressive SNHL due to partial labyrinthine erosion due to a meningocele of the geniculate ganglion along the fallopian canal. It is likely that the patient had a congenital open pathway through the labyrinthine segment of the facial nerve and CSF pulsations created the meningocele over a few years. This case argues for early closure of such CSF diverticula to prevent possible SNHL due to labyrinthine erosion.

Prevalence of Sensorineural Hearing Loss in Pediatric Patients with Sickle Cell Disease: A Meta-analysis.

OBJECTIVES: To determine the prevalence of Sensorineural Hearing Loss (SNHL) attributable to Sickle Cell Disease (SCD) in the global pediatric population and to identify factors contributing to its severity. STUDY DESIGN: Meta-analysis. METHODS: We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations under 18 years of age with excluding studies analyzing patients on iron chelation therapy, adults, or those without objective audiological analysis. RESULTS: We identified 138 initial studies with 17 selected for analysis after applying the exclusion criteria. A total of 1,282 SCD patients and 553 controls were included in the meta-analysis. There was a statistically significant increase in the prevalence of SNHL in children with SCD compared to the general population with a cumulative risk ratio of 3.33. CONCLUSION: This is the first systematic investigation of the relationship between SCD and SNHL in pediatric patients across the globe. The increased prevalence of SNHL in the pediatric SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of SCD patients to reduce the social and developmental morbidity of hearing loss at a young age. PROSPERO Registration #: CRD42019132601. Laryngoscope, 131:1147-1156, 2021.

Characterization of Sensorineural Hearing Loss in Adult Patients With Sickle Cell Disease: A Systematic Review and Meta-analysis.

OBJECTIVES AND MAIN OUTCOME MEASURES: To determine the prevalence of sensorineural hearing loss (SNHL) attributable to sickle cell disease (SCD) in the global adult population and to identify factors contributing to its severity. STUDY DESIGN: Systematic Review and Meta-analysis. METHODS: We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations over 18 years of age with SCD. RESULTS: We identified 138 studies from the initial search, 12 of which met inclusion criteria and were utilized for data analysis. A total of 636 SCD patients and 360 controls were included in the Cochrane Review Manager 5.3 meta-analysis. There was a statistically significant increase in the prevalence of SNHL in adults with SCD compared with the general population with a cumulative risk ratio (RR) of 6.03. CONCLUSIONS: This is the first systematic investigation of the relationship between SCD and SNHL in adult patients across the globe. SNHL is more prevalent in patients with SCD, specifically those of the HbSS genotype, than the general population likely due to the pathophysiology of the disease and its effects on labyrinthine microvasculature. The increased prevalence of SNHL in the adult SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of adult SCD patients.

Pediatric head and neck rhabdomyosarcoma: An analysis of treatment and survival in the United States (1975-2016).

INTRODUCTION: Rhabdomyosarcoma (RMS) is the most common soft tissue head and neck sarcoma in children. Stringent analysis of survival data is imperative to optimize treatment. METHODS: The National Cancer Database (NCDB, 2004-2016) and the Surveillance, Epidemiology, and End Results Program (SEER, 1975-2016) were queried for patients ages 0-19 with RMS of the head and neck. Survival trends were analyzed using univariable logistic regression and Chi-square pairwise comparisons. Survival by treatment was analyzed using log-rank tests, Kaplan Meier, and multivariable Cox-proportional hazards regression. RESULTS: 1147 (63.3% age <10 years, 54.3% male) and 459 (71.4% age <10 years, 53.6% male) patients were identified in NCDB and SEER, respectively. In NCDB, embryonal (n = 625, 54.5%) and alveolar (n = 300, 26.2%) were the most common histology and nonparameningeal/non-orbital (n = 634, 55.3%), followed by parameningeal (n = 303, 26.4%) and orbital (n = 210, 18.3%) the most common location. Five-year overall survival (OS) was 70.3% with lower mortality risk for embryonal histology (adjusted HR [aHR] = 0.69, p = 0.0038). Orbital tumors had greatest survival (5-year OS = 92.4%) of all sites, and their mortality risk was higher with chemotherapy and radiation compared to surgery and radiation (aHR = 6.27, p = 0.0302). SEER analysis showed average increased survival by 4% per year (p < 0.0001), but no significant 5-year OS difference when comparing 1976-1980 and 2006-2010 (p = 0.0843). CONCLUSIONS: Major prognostic factors for survival in childhood RMS of the head and neck were embryonal histology, orbital site, extent of disease, and use of SRT for orbital tumors. Larger population studies are required to demonstrate survival differences between treatment modalities for other sites.

A Case of Incus Necrosis Following Oral and Intratympanic Steroid Administration for Sensorineural Hearing Loss.

OBJECTIVE: Intratympanic (IT) steroid injections are often used for the treatment of inner ear pathologies, including sudden sensorineural hearing loss. IT steroids are also commonly used in conjunction with or in case of systemic steroid therapy failure. They are viable options for patients who cannot endure side effects or are at high risk of complications from systemic therapy. The duration of treatment, strength, and type of steroid, and number of IT injections are important factors that impact possible treatment complications. Reported complications, though uncommon, include tympanic membrane perforation, otalgia, vertigo, otitis media, and acne. In this report, we explore a novel case of incus necrosis associated with oral and/or IT steroid administration. STUDY DESIGN: Clinical Capsule Report. SETTING: Tertiary Academic Center. PATIENT: We present a case of a 70-year-old male with sudden sensorineural hearing loss who developed incus necrosis and conductive hearing loss following treatment with oral and intratympanic steroids. INTERVENTION: The patient underwent a left tympanoplasty with ossicular chain reconstruction to treat the conductive component of his hearing loss. RESULTS: The left tympanoplasty almost fully-resolved the conductive component of his hearing loss. However, intraoperative and CT assessment of the temporal bone revealed resorption of the long process of the incus, with otherwise normal-appearing ossicles. CONCLUSION: We report the first case of possible steroid-induced necrosis of the long process of the incus. Owing to its tenuous blood supply, the incus may be predisposed to steroid-induced osteonecrosis similar to that of the femoral head.