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.

A NSQIP study comparing surgical outcomes between primary and non-primary TEPs after total laryngectomy.

OBJECTIVE: Tracheoesophageal puncture with voice prosthesis (TEP) is considered the gold standard for voice rehabilitation after total laryngectomy; however, there is debate as to whether it should be inserted concurrently with removal of the larynx (primary TEP), or as a separate, additional procedure at a later date (secondary TEP). We utilized the National Surgical Quality Improvement Program Database (NSQIP) to compare postoperative complications, readmission rates, and reoperation rates among individuals who underwent total laryngectomy with or without concurrent TEP placement. METHODS: We conducted a retrospective study using the American College of Surgeons National Surgical Quality Improvement Program database (ACS-NSQIP) from 2012 to 2019. Patients were categorized into primary and non-primary TEP groups using a variation of CPT codes for total laryngectomy, tracheoesophageal prosthesis, and type of reconstruction. Univariate analyses were performed and significance was determined at p < 0.05. RESULTS: A total of 1974 patients who underwent total laryngectomy were identified from the database: 1505 (77.3 %) in the non-primary TEP group and 442 (22.7 %) in the primary TEP group. Patients in the non-primary TEP group were more likely to have an ASA class greater than or equal to three (91.2 % primary vs. 84.6 % non-primary, p < 0.001). Patients in the non-primary TEP group were also more likely to require intraoperative or postoperative blood transfusions within the first 72 h of surgery (20.5 % non-primary vs. 15.3 % primary, p = 0.016). Both groups had similar rates of wound breakdown and dehiscence. There remained no significant difference based on type of reconstruction. CONCLUSIONS: This study suggests that patients receiving primary TEPs are not at a greater risk of developing wound complications such as pharyngocutaneous fistulas in the 30-day postoperative period. This remained true when patients were stratified by type of flap reconstruction. Patients in the non-primary TEP group were more likely to have an ASA category of 3 or greater, which may explain why they experienced higher rates of complications such as blood transfusions intra-operatively or post-operatively.

Cost-analysis of in-office versus operating room sialendoscopy: Comparison of cost burden and outcomes.

PURPOSE: Office-based procedures in otolaryngology are increasingly utilized to increase efficiency, reduce cost, and eliminate risks associated with surgery. Gland-preserving surgical management of sialadenitis and sialolithiasis are often performed in the operating room, although many surgeons are moving this practice to clinic. We aim to determine the difference in patient charges and perioperative outcomes for salivary gland procedures performed in the clinic versus the OR. METHODS: Retrospective series of patients presenting with sialolithiasis, acute or chronic sialadenitis, and stricture between 2010 and 2019. Demographics, perioperative variables, setting, and charge data were collected. RESULTS: 528 patients underwent operative intervention (n = 427 office, n = 101 OR). Cohort demographics were comparable. Sialolithiasis was the most common presenting diagnosis in both cohorts. Both cohorts had similar rates of complete (p = 0.09) and partial (p = 0.97) response to treatment. A higher percentage of patients in the OR group reported no improvement (21.4 vs 12.2%, p = 0.034). Overall complications were similar (p = 0.582). Mean charges were statistically greater in the OR ($5560.35 OR vs $1298.33 office, p < 0.001). Operative time was significantly reduced in the office group (21.8 min vs 60.85 min, p < 0.001). CONCLUSIONS: Appropriately selected patients can be successfully treated in outpatient clinic without compromising patient safety or quality while significantly reducing the financial burden to patients and the healthcare system.

Intraoperative stretching microbreaks reduce surgery-related musculoskeletal pain in otolaryngologists.

OBJECTIVE: The purpose of this study was to examine the role intraoperative otolaryngology stretching microbreak (OSMB) has on musculoskeletal (MSK) pain and discomfort in otolaryngologists. BACKGROUND: Otolaryngology procedures subject surgeons to significant amounts of pain and strain over their years of training. MSK pain is a serious concern for otolaryngologists' career longevity as well as their general wellbeing. METHODS: Participants from two different hospitals and one private practice were recruited to participate in this study. An initial ergonomic survey was obtained to assess baseline MSK pain, and its subjective impact on operative performance. The participants then completed three control days without OSMB exercises, followed by three intervention days with OSMB exercises which were completed at 20-40 minute intervals. Preoperative and postoperative pain rating surveys were completed before and after each procedure and at the end of the day to determine changes in pain and/or discomfort. RESULTS: Ten otolaryngologists (50 % men, 50 % women; mean age 35.6 years) participated in this study. Half of the participants indicated that they were concerned their pain would limit their ability to operate in the future. 70 % of participants indicated that they have not attempted to treat this pain and 60 % did not try any stretching exercises outside the operating room (OR) to mitigate their symptoms. Participants reported neck, upper back, and lower back to be the primary MSK discomfort. OSMB improved participants' pain scores in neck, shoulders, hands, and lower back pain (p < 0.05). CONCLUSION: MSK pain has shown to be a serious concern for the ability of otolaryngologists to continue performing surgery in the future. OSMB may be an effective strategy that can be implemented by otolaryngologists intraoperatively to improve MSK pain and overall well being.

Detection of internal exon deletion with exon Del.

BACKGROUND: Exome sequencing allows researchers to study the human genome in unprecedented detail. Among the many types of variants detectable through exome sequencing, one of the most over looked types of mutation is internal deletion of exons. Internal exon deletions are the absence of consecutive exons in a gene. Such deletions have potentially significant biological meaning, and they are often too short to be considered copy number variation. Therefore, to the need for efficient detection of such deletions using exome sequencing data exists. RESULTS: We present ExonDel, a tool specially designed to detect homozygous exon deletions efficiently. We tested ExonDel on exome sequencing data generated from 16 breast cancer cell lines and identified both novel and known IEDs. Subsequently, we verified our findings using RNAseq and PCR technologies. Further comparisons with multiple sequencing-based CNV tools showed that ExonDel is capable of detecting unique IEDs not found by other CNV tools. CONCLUSIONS: ExonDel is an efficient way to screen for novel and known IEDs using exome sequencing data. ExonDel and its source code can be downloaded freely at https://github.com/slzhao/ExonDel.

In-office Transoral Hilar Sialolithotomy: A Cost-Effective and Patient-Centered Procedure.

Transoral sialolithotomy performed in-office under local anesthesia is routinely performed for distal submandibular stones. We demonstrate the senior author's novel practice of in-office transoral sialolithotomy for hilar and intraglandular stones. A review of cases performed by the senior author revealed similar rates of complication and stone recurrence as those reported in the literature from removal under general anesthesia. Laryngoscope, 2024.

IgG4-related systemic disease: an uncommon presentation for an uncommon disease.

IgG4-related systemic disease is an emerging disease process that manifests with a constellation of features, most commonly but not exclusive to swelling and tuberous growth in the lacrimal and salivary glands, potentially involving many other organ systems. This condition often prompts investigations into malignancy or needless radical surgical procedures. A 58-year-old male was presented to a rheumatologist after several biopsies were done that were suspicious for neoplasia, involving the lacrimal gland and lung. The diagnosis was confirmed when tissue from the lacrimal gland biopsy was reviewed with special stains for IgG4, performed at the Mayo Clinic. This patient is interesting because his disease included bilateral lacrimal glands--at different intervals, the submandibular glands, the lung, and the thyroid gland. His disease responded to immunosuppression. Literature has shown resolution of the tumors upon starting glucocorticoids or rituximab. Our patient was given a course of prednisone and methotrexate with normal follow-up CT chest and physical exam.

Torus mandibularis and its implication as a risk factor for the formation of sialolithiasis.

Sialolithiasis is the formation of a stone within the salivary gland, and approximately 80% of them occur within the submandibular gland. Sialolithiasis can lead to swelling and pain of the gland in the immediate phase and if left untreated, can lead to sialadenitis and even atrophy of the gland. Certain factors have already been established in the formation of sialolithiasis such as dehydration, tobacco smoke, autoimmune disorders and certain medications. One factor we theorise contributes to the formation of sialolithiasis is bony outgrowths on the tongue surface of the jaw, called mandibular tori. These outgrowths can lead to external compression of the submandibular duct, which leads to impeded salivary flow and the potential for sialolith formation. We present two cases in which individuals had submandibular sialolithiasis in the setting of extensive mandibular tori.

Placement of a tracheoesophageal prosthesis using intraoperative ultrasound: A novel technique.

Introduction: In patients who undergo a total laryngectomy, one of the most common techniques for voice rehabilitation is a tracheoesophageal prosthesis. Typically, this is accomplished using an endoscope down the oesophagus to visualise puncturing of the posterior tracheal wall; however, we present a case in which the use of an endoscope was not feasible, and a novel technique had to be utilised. Case report: A mid-70s male who had a total laryngectomy secondary to laryngeal carcinoma was undergoing tracheoesophageal puncture for prosthesis placement, when it was noted that he had significant stenosis of his proximal oesophagus, precluding the use of an endoscope. The senior author decided to use intraoperative ultrasound to visualise the oesophageal lumen for proper placement of the puncture. Discussion: This case demonstrates the ability of intraoperative ultrasound to visualise the oesophageal lumen and accurately place the tracheoesophageal puncture in cases where an endoscope cannot be used. Conclusion: We present a novel technique in which ultrasound was used to visualise the oesophageal lumen and identify the needle puncturing the posterior tracheal wall for placement of a tracheoesophageal prosthesis.

Minor Salivary Gland Carcinosarcoma of the Floor of the Mouth.

Carcinosarcoma is an uncommon tumor consisting of malignant epithelial and mesenchymal elements. Salivary gland carcinosarcoma is aggressive in nature, and given its biphasic histologic appearance, it has the potential to be mistaken for a less concerning entity. Intraoral minor salivary gland carcinosarcoma is exceedingly rare with the palate being the site most frequently involved. Only two cases of carcinosarcoma arising from the floor of the mouth (FOM) have been reported. We present a case of a non-healing FOM ulcer that was identified as a minor salivary gland carcinosarcoma on surgical pathology along with the steps and importance of accurate diagnosis.

Complications Associated with Microdebriders in Otolaryngology Procedures from 2011 to 2021: A MAUDE Study.

Objective: The microdebrider is a powered surgical instrument that is widely used in the field of otolaryngology. We aim to identify the type and frequency of device malfunctions, patient complications, and subsequent interventions related to the use of microdebriders. Study Design: Cross-sectional analysis. Setting: The US Food and Drug Administration (FDA) 2011 to 2021 Manufacturer and User Facility Device Experience (MAUDE) database. Methods: The MAUDE database was queried for reports of "microdebrider," with adverse events selected that pertained to usage in head and neck surgeries from January 1, 2011 to December 31, 2021. Results: There were 282 adverse events in 267 individual medical device reports (MDR). Although the majority of the reports did not specify the specific operation, endoscopic sinus surgery was the most common reported procedure (89, 33.3%). The most common cause of device malfunction was due to a broken piece (120, 48.2%) followed by overheating of the microdebrider motor (78, 31.3%). Of the reports which specified patient injury, the most commonly reported was "unintentional tissue damage," (10, 32.3%). Conclusion: The microdebrider has demonstrated utility within the field of otolaryngology, but is not without risk of malfunction that can cause patient injury. By understanding possible risks of microdebrider usage, including tissue damage, burns, and bleeds caused by device malfunction or operator error, physicians can be better prepared to address complications and educate patients.

Radiographic Scapholunate Interval in the Pediatric Population Decreases in Size as Age Increases.

BACKGROUND: Widening of the scapholunate (SL) interval greater than 2 mm is diagnostic of an injury in adults; however, this absolute number cannot be used for skeletally immature individuals due to the ossification of the carpal bones. The purpose of this study was to determine age-appropriate normative values for the radiographic SL interval in the skeletally immature population. METHODS: Normal pediatric wrist radiographs were identified. The radiographic SL interval was measured as the distance between the scaphoid and the lunate at the mid-joint space between the scaphoid and the lunate, and the SL and capitolunate angles were measured on lateral views. An initial inter-rater reliability assessment was completed with strong inter-rater reliability. RESULTS: In all, 529 radiographic series of children (276 male), aged 4 to 17 years, were reviewed. A negative linear correlation between chronological age and distance at the mid-joint space was observed. The average values for the radiographic SL interval ranged from 9.07 to 1.57 mm. The average SL and capitolunate angles were 52.0° and 10.6°, respectively. No linear relationship was found between chronological age and SL or capitolunate angle (R = 0.07 and 0.03, respectively). CONCLUSIONS: The visible radiographic distance between the scaphoid and the lunate decreases with increasing age as the carpal bones ossify. The normative values defined in this study can be used to determine whether a true widening of the SL interval is present in the pediatric population. No linear relationship exists between chronological age and SL or capitolunate angle.

Neoadjuvant chemotherapy enhances tumor-specific T cell immunity in patients with HPV-associated oropharyngeal cancer.

BACKGROUND: Treatment of patients with newly diagnosed HPV-associated oropharyngeal squamous cell carcinoma (OPSCC) with neoadjuvant chemotherapy (NAC) results in a high rate of 5-year recurrence free survival with few patients requiring adjuvant treatment. We hypothesized that NAC enhances primary tumor HPV-specific T cell responses. METHODS: HPV-specific responses in tumor infiltrating lymphocytes (TILs) before and after NAC were determined using autologous co-culture assays. RESULTS: Greater HPV16-specific TIL responses, sometimes polyclonal, were observed after NAC compared to before in 8 of 10 patients (80%) with PCR-verified HPV16-positive tumors. A significant association was observed between net-negative change in HPV-specific TIL response and disease relapse (p = 0.04, Mann-Whitney test), whereas pathologic complete response at time of surgery did not correlate with recurrence. CONCLUSIONS: NAC induces HPV-specific tumor T cell responses in patients with newly diagnosed HPV-associated OPSCC; whereas lack of an increase following NAC may associate with risk of relapse.

Remission of rheumatoid arthritis in clinical practice: application of the American College of Rheumatology/European League Against Rheumatism 2011 remission criteria.

OBJECTIVE: To describe use of the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) rheumatoid arthritis (RA) remission criteria in clinical practice. METHODS: Remission was examined using data on 1,341 patients with RA (91% men) from the US Department of Veterans Affairs RA (VARA) registry (total of 9,700 visits) and 1,153 patients with RA (25.8% men) in a community rheumatology practice (Arthritis and Rheumatology Clinics of Kansas [ARCK]) (total of 6,362 visits). Cross-sectional and cumulative probabilities were studied, and agreement between the various remission criteria was assessed. Aspects of reliability of the criteria were determined using Boolean-based definitions, as well as the Clinical Disease Activity Index (CDAI) and Simplified Disease Activity Index (SDAI) scoring methods proposed by the ACR/EULAR joint committee. RESULTS: When the 3-variable ACR/EULAR definition of remission recommended for use in community practice (swollen and tender joint counts ≤1, and visual analog scale score for patient's global assessment of disease activity ≤1) was applied, cross-sectional remission was 7.5% (95% confidence interval [95% CI] 6.4, 8.7%) for ARCK and 8.9% (95% CI 7.9, 9.9%) for VARA, and cumulative remission (remission at any observation) was 18.0% (for ARCK) and 24.4% (for VARA), over a mean followup of ∼2.2 years. Addition of the erythrocyte sedimentation rate or C-reactive protein level to the criteria set reduced remission to 5.0-6.2%, and use of the CDAI/SDAI increased the proportions to 6.9-10.1%. Moreover, 1.8-4.6% of the patients met remission criteria at ≥2 visits. Agreement between criteria definitions was good, as assessed by kappa statistics and Jaccard coefficients. Among patients in remission, the probability of a remission lasting 2 years was 6.0-14.1%. Among all patients, the probability of a remission lasting 2 years was <3%. Remission status and examination results for each patient varied substantially among physicians, as determined by multilevel analyses. CONCLUSION: Cross-sectional remission occurred in 5.0-10.1% of the patients in these cohorts, with cumulative remission being 2-3 times greater; however, long-term remission was rare. Problems with reliability and agreement limit the usefulness of these criteria in the individual patient. However, the criteria can be an effective method for measuring clinical status and treatment effect in groups of patients in the community.

Sjogren Syndrome-Associated Autonomic Neuropathy.

Sjogren syndrome (SS) is one of the common autoimmune diseases characterized by dryness of the eyes and mouth described as "xerophthalmia" and "xerostomia," respectively. Affected patients may also experience complex neurological symptoms characterized by extremity paresthesia and pain as well as dizziness and presyncope. In fact, patients may present initially with pure neurological symptoms. Thus, the diagnosis of SS remains a clinical challenge. We report a case here of a patient with primary Sjogren syndrome (pSS) experiencing severe autonomic neuropathy symptoms. A 53-year-old female patient diagnosed with pSS at the age of 30 years presented with a four-month history of palpitations and dizziness. A tilt table test showed positive findings with significant symptomatic orthostatic hypotension. The patient was started on midodrine therapy followed by a beta-blocker with improvement in her symptoms. There was also augmentation of her SS disease management by introducing IV IgG therapy.  The diagnosis of neuropathy in SS is complex and requires a combination of clinical signs and symptoms. This case report focuses on the neurological manifestations of SS, especially the ones related to autonomic neuropathy. We aim to share awareness of the neurological manifestations of patients with pSS and alert physicians that it could be the initial presentation of this systemic disease.

The Use of Ethanol Sclerotherapy To Treat a Large Cervical Lymphocele.

Cervical lymphoceles are atypical lymphatic accumulations that develop within the subcutaneous tissue of the neck. While these accumulations have traditionally been removed via surgical excision, sclerotherapy has recently emerged as a reasonable option to prevent injury to surrounding vascular, neurologic, and pulmonary structures. The purpose of this case report is to describe the efficacy of ethanol sclerotherapy for a cervical lymphocele refractory to surgical embolization. We present the case of a 70-year-old male with a large cervical lymphocele that was initially treated with surgical embolization. The mass rapidly reaccumulated within two weeks and the patient subsequently underwent ethanol sclerotherapy with no evidence of re-accumulation after 18 months. This case highlights the utility of ethanol due to its better side-effect profile, widespread availability, and cheaper cost when compared to better-described agents.

The lupus family registry and repository.

The Lupus Family Registry and Repository (LFRR) was established with the goal of assembling and distributing materials and data from families with one or more living members diagnosed with SLE, in order to address SLE genetics. In the present article, we describe the problems and solutions of the registry design and biometric data gathering; the protocols implemented to guarantee data quality and protection of participant privacy and consent; and the establishment of a local and international network of collaborators. At the same time, we illustrate how the LFRR has enabled progress in lupus genetics research, answering old scientific questions while laying out new challenges in the elucidation of the biologic mechanisms that underlie disease pathogenesis. Trained staff ascertain SLE cases, unaffected family members and population-based controls, proceeding in compliance with the relevant laws and standards; participant consent and privacy are central to the LFRR's effort. Data, DNA, serum, plasma, peripheral blood and transformed B-cell lines are collected and stored, and subject to strict quality control and safety measures. Coded data and materials derived from the registry are available for approved scientific users. The LFRR has contributed to the discovery of most of the 37 genetic associations now known to contribute to lupus through 104 publications. The LFRR contains 2618 lupus cases from 1954 pedigrees that are being studied by 76 approved users and their collaborators. The registry includes difficult to obtain populations, such as multiplex pedigrees, minority patients and affected males, and constitutes the largest collection of lupus pedigrees in the world. The LFRR is a useful resource for the discovery and characterization of genetic associations in SLE.

Malignancy-associated multicentric reticulohistiocytosis.

Multicentric reticulohistiocytosis (MR) is a rare systemic disorder of unknown etiology. Up to 25% of patients with MR have associated malignancies. We are reporting a case of a patient with MR who initially had an excellent clinical response to oral methotrexate and prednisone. Subsequent attempts of prednisone taper failed because of disease flare-up. Workup for associated malignancy revealed localized squamous cell carcinoma of the lung. The tumor was resected; methotrexate and prednisone were tapered and discontinued with complete remission of MR. A review of literature and the new advances and treatment modalities for MR is provided.

Predictors of Tracheostomy Decannulation in Adult Laryngotracheal Stenosis.

OBJECTIVE: Predictors of tracheostomy decannulation in patients with laryngotracheal stenosis are not fully known, making prognosis difficult. The aim was to identify predictors of tracheostomy decannulation in adult patients with acquired stenosis of the larynx and/or trachea who were tracheostomy dependent. STUDY DESIGN: Case series. SETTING: Academic teaching hospital. METHODS: A total of 103 consecutive adult patients with laryngotracheal stenosis who were tracheostomy dependent and seen by the otolaryngology clinic from January 1, 2013, to August 2, 2018, were included. Exclusion criteria included age <18 years, history of laryngeal cancer or head and neck radiation, or history of laryngeal fracture. The primary outcome was the presence of tracheostomy at last follow-up. The patients' etiology of stenosis, comorbid conditions, and characteristics of the stenosis were analyzed to determine if there was a statistically significant relationship with decannulation. RESULTS: A total of 103 patients were included: 67% of patients were women and the average age was 53.5 years. Sixty-four patients (62%) were successfully decannulated. In multivariate analysis, patients who were successfully decannulated presented to the otolaryngology clinic earlier after tracheostomy was performed, were more likely to have been intubated due to trauma, and were less likely to have gastroesophageal reflux disease. In patients with subglottic or tracheal stenosis, those with granulation tissue without firm scar were more likely to be decannulated, and those who underwent rigid dilation were less likely to be decannulated. CONCLUSION: Early evaluation by an otolaryngologist may increase the likelihood of tracheostomy decannulation in patients with laryngotracheal stenosis. Patient comorbidities may assist in predicting which patients will be successfully decannulated.

Acquisition of aneuploidy drives mutant p53-associated gain-of-function phenotypes.

p53 is mutated in over half of human cancers. In addition to losing wild-type (WT) tumor-suppressive function, mutant p53 proteins are proposed to acquire gain-of-function (GOF) activity, leading to novel oncogenic phenotypes. To study mutant p53 GOF mechanisms and phenotypes, we genetically engineered non-transformed and tumor-derived WT p53 cell line models to express endogenous missense mutant p53 (R175H and R273H) or to be deficient for p53 protein (null). Characterization of the models, which initially differed only by TP53 genotype, revealed that aneuploidy frequently occurred in mutant p53-expressing cells. GOF phenotypes occurred clonally in vitro and in vivo, were independent of p53 alteration and correlated with increased aneuploidy. Further, analysis of outcome data revealed that individuals with aneuploid-high tumors displayed unfavorable prognoses, regardless of the TP53 genotype. Our results indicate that genetic variation resulting from aneuploidy accounts for the diversity of previously reported mutant p53 GOF phenotypes.