The Diagnosis of Granulomatosis With Polyangiitis When Serology and Biopsies are Negative.

Objective: Granulomatosis with polyangiitis (GPA) is a potentially fatal condition which often manifests in the head and neck. Currently, diagnosis relies on antineutrophil cytoplasmic autoantibody (c-ANCA) serology and mucosal or renal biopsy. However, a significant proportion of patients with GPA limited to the head and neck are seronegative and biopsy negative. This study evaluates the role of clinical diagnosis of GPA in the absence of positive laboratory findings. Study Design: Case series with chart review. Setting: Academic Tertiary Medical Center. Methods: This was a retrospective review of 143 patients treated in an outpatient otolaryngology clinic at a tertiary care hospital for known or suspected GPA from 1998 to 2021. Presenting symptoms, C-ANCA status at initial presentation, biopsy results, long-term serology results, and time to initiation of treatment were analyzed. Results: Twenty-six of 143 (18.2%) patients were seronegative; only 3 of these patients (12%) had positive biopsies. Seventeen (73.9%) of these patients presented with nasal and sinus disease and 12 (52.2%) presented with airway involvement. Only 4 (17.4%) patients had renal involvement. Delay in treatment of patients with negative laboratory workup ranged from 0 months to 11 years. All patients who were seronegative and/or biopsy negative at presentation responded clinically to immunosuppressive therapy. Conclusion: GPA cases are often limited to the upper respiratory tract, making diagnosis difficult, particularly in seronegative patients. These results suggest that, when GPA is suspected, despite negative serology, the diagnosis of GPA should be made on clinical grounds, and empiric therapy encouraged to prevent delay in treatment.

Four-Dimensional Computed Tomography for Parathyroid Adenoma Localization: A Pre-Operative Imaging Protocol.

OBJECTIVE: Primary hyperparathyroidism (PHPT) affects approximately 0.86% of the population, with surgical resection as the treatment of choice. A 4D computed tomography (CT) is a highly effective tool in localizing parathyroid adenomas; however, there is currently no defined role for 4D CT when stratified against ultrasonography (USG) and nuclear medicine Technetium Sestamibi SPECT/CT (SES) imaging. STUDY DESIGN: Retrospective Study. SETTING: University Hospital. METHODS: All patients who underwent parathyroidectomy for PHPT between 2014 and 2019 at a single institution were reviewed. Patients who had a 4D CT were included. We compared outcomes of 4D CT as a second line imaging modality to those of USG and SES as first line modalities. An imaging algorithm was proposed based on these findings. RESULTS: There were 84 patients identified who had a 4D CT after unsuccessful first line imaging. A 4D CT localized parathyroid adenoma to the correct quadrant in 64% of cases, and to the correct laterality in 75% of cases. Obese patients had significantly lower rates of adenoma localization with USG (33.4%), compared to non-obese patients (67.5%; P = .006). In determining multigland disease the sensitivity of 4D CT was 86%, while the specificity was 87%. CONCLUSIONS: A 4D CT has impressive rates of accurate localization of parathyroid adenomas; however due to the radiation exposure involved, it should remain a second line imaging modality. PHPT patients should first be evaluated with USG, with 4D CT used if this is unsuccessful and patients are greater than 40 years old, have a high BMI, or are having revision surgery.

Optimization of Subglottic View During Flexible Laryngoscopy With Patient Positioning.

OBJECTIVE: Determine the ideal head position to optimize visualization of the subglottis using flexible laryngoscopy. STUDY DESIGN: Prospective cohort study. SETTING: Outpatient multidisciplinary airway clinic at a tertiary care center. METHODS: Patients presenting to a multidisciplinary airway clinic undergoing nasoendoscopic airway examination were enrolled. Three head positions were utilized to examine the subglottis during laryngoscopy: "sniffing," chin tuck, and stooping positions. In-office reviewers and blinded clinician participants evaluated views of the airway based on Cormack-Lehane (CL) scale, airway grade (AG), and visual analog scale (VAS). Demographic data were obtained. Statistical analysis compared head positions and demographic data using Student's t test, analysis of variance, and Tukey's post hoc analysis. RESULTS: One hundred patients participated. No statistical differences existed among in-clinic or blinded reviewers for the CL score in any head position (p = .35, .5, respectively). For both AG and VAS, flexed and stooping positions were rated higher than the sniffing positions by both in-clinic and blinded reviewers (p < .01 for all analyses), but there was no statistical difference between these two positions (p = .28, .18, respectively). There was an inverse correlation between age and scores for AG and VAS in the flexed position for both sets of reviewers (p = .02, <.01 respectively), and a higher body mass index was significantly associated with the need to perform tracheoscopy for full airway evaluation (p < .01). CONCLUSION: Both flexion and stoop postures can be implemented by an experienced endoscopist in awake, transnasal flexible laryngoscopy to enhance visualization of the subglottic airway.

Customizable interdental splinting for repair of pediatric mandibular fractures in children with mixed dentition: A novel technique.

OBJECTIVE: Repair of pediatric mandibular fractures (PMFs) can be challenging due to the lack of permanent dentition for immobilization, and the presence of unerupted teeth and growth plates in the mandible limiting the space for fixation. Interdental splinting (IDS) has been advocated to provide temporary fixation without the need for mandibular plating; however, there is sparse description of the surgical methodology, and data on long term outcomes are even more limited. The aim of this study is to present our technique and outcomes using a novel technique for IDS repair of pediatric mandible fractures. STUDY DESIGN: Observational retrospective chart review. SETTING: Tertiary care pediatric hospital. SUBJECTS AND METHODS: Pediatric patients requiring operative repair for mandibular fracture at our tertiary care institution between 2004 and 2021 were included. Patients over 18 years of age, those who died due to associated injuries, or those who underwent non-IDS repairs were excluded. Subjects with at least 3 months of follow-up were assessed for efficacy of surgical repair and short-term adverse outcomes, and at least 1 year for long-term adverse events. Descriptive statistics were obtained. RESULTS: Twenty-three children were included in the study with an average age of 7.4 years (range 2-17 years). Fifty-two percent (52.2%) were female. The most common fracture site was the condyle, occurring in 16 children (70%). The indication for operative repair in all cases was malocclusion. The average duration of maxillomandibular fixation (MMF) with the novel IDS was 21 days (range 12-42 days). The average length of follow up was 1.6 years (range 3 months-11 years). All children had restored, functional occlusion at follow up with none requiring further orthodontic or dental intervention. Three children of the total cohort (13.0%) had prolonged hospitalization beyond 48 h for poor oral intake. Five children (21.7%) experienced minor long-term complications including persistent temporomandibular joint pain (n = 1, 4.3%), infection (n = 2, 8.7%), hypertrophic scar (n = 1, 4.3%) and exposure of hardware (n = 1, 4.3%). CONCLUSION: PMFs resulting in malocclusion are safely and effectively managed with operative repair utilizing a customizable IDS, with few observed short- and long-term complications.

Ototoxicity and Teprotumumab.

OBJECTIVES: Teprotumumab, a novel monoclonal antibody, targets the insulin-like growth factor 1 (IGF-1) receptor. IGF-1 receptors, found in muscle and fat adjacent to the eye and implicated in Graves Ophthalmopathy, are also in the cochlea. In clinical trials, 5 participants reported self-limited audiologic symptoms but there are no objective data in the literature. The aim of this report is to describe one of the first known cases of teprotumumab-induced irreversible sensorineural hearing loss. METHODS: Case report at a tertiary referral center. RESULTS: A 61 year old female with Graves ophthalmopathy presented with bilateral hearing loss, sound distortion, and tinnitus following treatment with teprotumumab. Audiogram showed mild sloping to moderately-severe sensorineural hearing loss. Repeat audiometry obtained 4 months after cessation of teprotumumab and treatment with oral corticosteroids was unchanged. CONCLUSIONS: This is one of the first descriptive cases of ototoxicity resulting in irreversible sensorineural hearing loss in the setting of treatment with teprotumumab. Periodic audiologic evaluations should be recommended to patients on teprotumumab.

Non-invasive Fungal Sinusitis as a Complication of a Steroid-Eluting Stent Following Endoscopic Sinus Surgery: A Case Report.

OBJECTIVE: Steroid eluting stents have proven to be a highly useful adjunctive therapy for chronic rhinosinusitis (CRS) and play an important role in the treatment of many inflammatory diseases of the sinuses. Few reports of adverse events were reported in clinical trials and are described in the literature. However, we describe the first known case of an immunocompetent patient developing non-invasive fungal tissue infection as a sequelae of stent-related tissue necrosis requiring surgical debridement. METHODS: A 69-year-old immunocompetent male with CRS had Propel™ stents placed in the bilateral frontal sinus outflow tracts during revision endoscopic sinus surgery. He presented 2 weeks post-operatively with severe facial pain without vision changes, fevers, mental status changes, or evidence of cranial neuropathies. On rigid nasal endoscopy, necrotic tissue and gross fungal elements were visualized in the left frontal sinus outflow tract at the area of previous steroid stent position. RESULTS: The patient was taken for urgent endoscopic sinus surgery and debridement given significant symptoms and concern for invasive fungal infection. A revision left maxillectomy, ethmoidectomy, and draf 2b frontal sinus drillout were performed, with healthy bleeding tissue encountered beneath necrotic tissue. Pathology revealed tissue necrosis, exudative lumenal debris, and extensive fungal elements with no evidence of tissue invasion, and cultures yielded growth of aspergillus niger. The patient's symptoms improved significantly on post-operative day 1, he had normal post-operative changes at 2 weeks following debridement, and had no recurrence of fungal infection with complete healing at 4 months. CONCLUSION: While likely rare, steroid-eluting stents may pose a risk of saprophytic tissue infection as a result of tissue necrosis and local immunosuppression. Caution should be taken in using these devices in immunocompromised patients.

Trust in physicians among patients with head and neck cancer before and after treatment.

BACKGROUND: Little is known about physician trust in patients with head and neck cancer. This study's aim is to evaluate trust pre- and post-treatment. METHODS: A study was conducted of 50 patients with head and neck cancer at a tertiary referral center. Surveys administered before and after treatment included several validated survey tools including the Trust in Oncologist Scale Short Form. RESULTS: There was an increase in overall trust scores (p < 0.001). Female patients (p = 0.034) and those who received chemotherapy (p = 0.001) were less trusting post-treatment. Patients with more comorbidities (p = 0.045) and progression of disease (p = 0.029) had higher final trust than those without. Patients with high initial distress scores showed a decrease in trust (p = 0.023). CONCLUSIONS: Patients with head and neck cancer trust their surgeon more after completion of treatment, with specific characteristics having a variable impact on trust scores.

Transmastoid Hydroxyapatite Resurfacing for Sigmoid Sinus Wall Anomalies Causing Pulsatile Tinnitus.

OBJECTIVE: Comprising 4% of tinnitus, pulsatile tinnitus (PT) can be particularly difficult for affected patients as well as surgeons looking to address their symptoms. Often the cause is not identified but can be secondary to turbulent flow in or near the sigmoid sinus, particularly if there is an identifiable sigmoid sinus dehiscence (SSDe) and/or diverticulum (SSDi). These sigmoid sinus wall anomalies (SSWA) may be treated with transmastoid sigmoid sinus resurfacing; however, this intervention remains relatively novel and its technique, materials used, resolution success, and complications need to be continuously reviewed. METHODS: A retrospective case series of patients with PT due to SSWA at a tertiary referral center was reviewed. A total of 6 patients (7 ears) treated by transmastoid resurfacing using hydroxyapatite (HA) were retrospectively assessed. Pre-operative demographics and symptoms, pre- and post-operative hearing results, and post-operative outcomes were reviewed. RESULTS: All patients were female with an average BMI of 32.9 (±5.4) and a mean age of 45.5 years (±15.3). Mean follow-up was 648 days. Objective tinnitus was noted in all ears with SSDi (100%); however, no objective tinnitus was noted with purely SSDe. In 100% of ears, PT was diminished with ipsilateral jugular compression and was amplified with contralateral head turn. Pre-operative symptoms of PT resolved in all patients, but delayed recurrence (>1 year) occurred in 1 ear (14%). No patient had pre- or post-operative hearing loss. No major complications were encountered. CONCLUSIONS: Transmastoid resurfacing for SSWA with HA bone cement is a safe, reliable intervention in properly identified PT patients.

Graduating otolaryngology resident preparedness for fellowship as assessed by fellowship faculty.

OBJECTIVES/HYPOTHESIS: The purpose of this study was to evaluate fellowship program directors' perceptions of incoming clinical fellows' preparedness for subspecialty training and to thereby identify strengths and shortcomings in otolaryngology training programs' ability to prepare residents for fellowship. STUDY DESIGN: Validated e-mail survey. METHODS: Two hundred eleven otolaryngology subspecialty fellowship program directors and faculty directly involved with training fellows were contacted. A validated survey by the American College of Surgeons was modified and distributed to otolaryngology fellowship faculty in six otolaryngology subspecialties. The 59-item survey employed a five-response Likert scale tailored to each subspecialty. Responses were collected between November 2016 and January 2017. RESULTS: One hundred ten otolaryngology faculty responded to the survey (52%). Respondents had worked with fellows for a mean of 12 years (standard deviation = 8). Respondents felt fellows were competent in the areas of professionalism, clinical evaluation, and management. Pediatric faculty were more likely to disagree about fellows' independence in the operating room (P = .004) and during call (P = .002) compared to other specialties. Laryngology and facial plastic and reconstructive surgery faculty felt more neutral about anatomy recognition (P = .008), tissue manipulation (P = .002), and use of energy sources (e.g., cautery, lasers) (P < .001). Fellows in all subspecialties were felt to be least prepared in research and academic interest. CONCLUSIONS: Faculty involved in fellowship training feel that fellows are well-prepared overall upon entering fellowship. Residency programs may benefit from providing more experience with facial plastic reconstructive surgery, laryngology, and pediatrics. Regardless of specialty, residents could benefit from increased training in research design, data analysis, and basic statistics. LEVEL OF EVIDENCE: NA Laryngoscope, E280-E286, 2018.

CRTC2 activation in the suprachiasmatic nucleus, but not paraventricular nucleus, varies in a diurnal fashion and increases with nighttime light exposure.

Entrainment of the intrinsic suprachiasmatic nucleus (SCN) molecular clock to the light-dark cycle depends on photic-driven intracellular signal transduction responses of SCN neurons that converge on cAMP response element-binding protein (CREB)-mediated regulation of gene transcription. Characterization of the CREB coactivator proteins CREB-regulated transcriptional coactivators (CRTCs) has revealed a greater degree of differential activity-dependent modulation of CREB transactivational function than previously appreciated. In confirmation of recent reports, we found an enrichment of crtc2 mRNA and prominent CRTC2 protein expression within the SCN of adult male rats. With use of a hypothalamic organotypic culture preparation for initial CRTC2-reactive antibody characterization, we found that CRTC2 immunoreactivity in hypothalamic neurons shifted from a predominantly cytoplasmic profile under basal culture conditions to a primarily nuclear localization (CRTC2 activation) 30 min after adenylate cyclase stimulation. In adult rat SCN, we found a diurnal variation in CRTC2 activation (peak at zeitgeber time of 4 h and trough at zeitgeber time of 16-20 h) but no variation in the total number of CRTC2-immunoreactive cells. There was no diurnal variation of CRTC2 activation in the hypothalamic paraventricular nucleus, another site of enriched CRTC2 expression. Exposure of rats to light (50 lux) for 30 min during the second half of their dark (nighttime) phase produced CRTC2 activation. We observed in the SCN a parallel change in the expression of a CREB-regulated gene (FOS). In contrast, nighttime light exposure had no effect on CRTC2 activation or FOS expression in the paraventricular nucleus, nor did it affect corticosterone hormone levels. These results suggest that CRTC2 participates in CREB-dependent photic entrainment of SCN function.