Faculty Bibliography

The novel Coronavirus (COVID-19) has created a deadly pandemic that is now significantly impacting the United States. Otolaryngologists are considered high risk for contracting disease, as the virus resides in the nasal cavity, nasopharynx, and oropharynx. While valuable work has been publicized regarding several topics in Rhinology, we discuss other aspects of our specialty in further detail. There are several issues regarding Rhinologic practice that need to be clarified both for the current epidemic as well as for future expected "waves." In addition, as the pandemic dies down, guidelines are needed to optimize safe practices as we start seeing more patients again. These include protocols pertinent to safety, in-office Rhinologic procedures, the substitution of imaging for endoscopy, and understanding the appropriate role of telemedicine. We discuss these aspects of Rhinology as well as practical concerns relating to telemedicine and billing, as these issues take on increasing importance for Rhinologists both in the present and the future.

BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing.RESULTSThirty-six USAID patients (55%) had elevated IRG-S. Neutrophilic panniculitis (40% vs. 0%), basal ganglia calcifications (46% vs. 0%), interstitial lung disease (47% vs. 5%), and myositis (60% vs. 10%) were more prevalent in patients with elevated IRG-S. Moderate IRG-S elevation and highly elevated serum IL-18 distinguished 8 patients with pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS). Among patients with panniculitis and progressive cytopenias, 2 patients were compound heterozygous for potentially novel LRBA mutations, 4 patients harbored potentially novel splice variants in IKBKG (which encodes NF-κB essential modulator [NEMO]), and 6 patients had de novo frameshift mutations in SAMD9L. Of additional 12 patients with elevated IRG-S and CANDLE-, SAVI- or Aicardi-Goutières syndrome-like (AGS-like) phenotypes, 5 patients carried mutations in either SAMHD1, TREX1, PSMB8, or PSMG2. Two patients had anti-MDA5 autoantibody-positive juvenile dermatomyositis, and 7 could not be classified. Patients with LRBA, IKBKG, and SAMD9L mutations showed a pattern of IRG elevation that suggests prominent NF-κB activation different from the canonical interferonopathies CANDLE, SAVI, and AGS.CONCLUSIONSIn patients with elevated IRG-S, we identified characteristic clinical features and 3 additional autoinflammatory diseases: IL-18-mediated PAP and recurrent MAS (IL-18PAP-MAS), NEMO deleted exon 5-autoinflammatory syndrome (NEMO-NDAS), and SAMD9L-associated autoinflammatory disease (SAMD9L-SAAD). The IRG-S expands the diagnostic armamentarium in evaluating USAIDs and points to different pathways regulating IRG expression.TRIAL REGISTRATIONClinicalTrials.gov NCT02974595.FUNDINGThe Intramural Research Program of the NIH, NIAID, NIAMS, and the Clinical Center.

BACKGROUND:To the authors' knowledge, the question of whether human papillomavirus (HPV) infection is associated with outcomes in patients with sinonasal squamous cell carcinoma (SNSCC) is not well studied at this time. In the current study, the authors investigated patterns of HPV testing and its association with survival in patients with SNSCC using the National Cancer Data Base. METHODS:The authors selected all SNSCC cases diagnosed between 2010 and 2016. HPV testing practices, clinicodemographic factors, treatments, and survival were analyzed. Multivariable Cox regression and propensity score-matched survival analyses were performed. RESULTS:A total of 6458 SNSCC cases were identified. Of these, only 1523 cases (23.6%) were tested for HPV and included in the current study. The median patient age was 64 years and the majority had advanced stage tumors (overall AJCC stage III-IV, 721 patients; 62.1%). HPV-positive SNSCC comprised 31.5% (447 of 1418 cases) of the final study cohort. Among 15 hospitals that routinely tested nonoropharyngeal SCCs for HPV, the percentage of HPV-positive SNSCCs was smaller (24.6%; P = .04). Patients with HPV-positive SNSCC were younger (aged 60 years vs 65 years; P < .001), with tumors that were more likely to be high grade (55.3% vs 41.7%; P < .001), and attributed to the nasal cavity (62.2% vs 44.0%; P < .001). HPV-positive SNSCC was associated with significantly improved overall survival in multivariable regression analysis (hazard ratio, 0.45; 95% CI, 0.28-0.72 [P = .001]) and propensity score-matched (hazard ratio, 0.61; 95% CI, 0.38-0.96 [P = .03]) analyses controlling for clinicodemographic and treatment factors. CONCLUSIONS:Currently, only a minority of patients with SNSCC are tested for HPV. However, a sizable percentage of SNSCC cases may be HPV related; furthermore, HPV-positive SNSCC is associated with improved overall survival. Routine HPV testing may be warranted in patients with SNSCC.

OBJECTIVE:We describe our experience using the extracorporeal video microscope, the "exoscope" for repair of a temporal bone encephalocele. METHOD/METHODS:The patient is a 69-year-old male with a right temporal lobe encephalocele herniating through a tegmen defect. He underwent definitive tegmen defect repair and bipolar cauterization of the encephalocele. The authors elected for a combined transmastoid and transtemporal approach in order to isolate the tegmen defect and provide watertight repair. The Synaptive robotic BrightMatter (Toronto, ON) drive video exoscope monitor system was used for the entirety of the case including both the transmastoid approach and transtemporal craniotomy. RESULTS:No intraoperative complications were encountered during either the transmastoid (mastoidectomy) or transtemporal craniotomy. The authors were able to complete the entire case without abandonment of the exoscope in favor of the traditional binocular microscope. Advantages of this technology in clinical practice includes high-resolution three-dimensional visualization, increased degrees of freedom for exoscope adjustment, and reduced surgeon fatigue in a fixed, unnatural posture. Limitations include decreased depth perception and increased operative time. CONCLUSION/CONCLUSIONS:The exoscope system is a safe and effective alternative or adjunct to the existing binocular operating microscope for lateral skull based procedures. The exoscope provides the surgeon with a comfortable, high-resolution visualization without compromising surgical exposure and patient safety.SDC video link: http://links.lww.com/MAO/A837.

OBJECTIVE:To identify risk factors and interventions affecting length of hospitalization (LOH) and clinical outcome in children with intracranial complications of rhinosinusitis. METHODS:Retrospective chart review of 12 children hospitalized at 2 academic medical centers for intracranial complications of rhinosinusitis over the past 5 years. RESULTS:12 patients were identified with an average age at presentation of 13 years old. 92% were male and 75% were African American. The most common presenting symptoms were fever and headache. Localizing neurological symptoms including hemiparesis and aphasia, in addition to seizures occurred in 33% of patients and increased LOH significantly (33 versus 15 days, p = 0.03). Epidural (EA) and subdural abscesses (SA) were the most common intracranial complications. 58% of patients were initially treated with a combination of open neurosurgical (ON) intervention and endoscopic sinus surgery (ESS) and LOH was significantly shorter for these patients compared to those treated otherwise (14 versus 31 days, p = 0.02). Streptococcus species were the most common group of bacteria identified in 75% of cases, with S. anginosus accounting for 42% of cases. The overall average LOH was 21 days with 92% of patients having complete resolution of symptoms by time of discharge. CONCLUSIONS:Treatment of intracranial complications of acute rhinosinusitis can have favorable outcomes after appropriate surgical management. Localizing neurologic symptoms and seizures portend longer hospital stay and recovery time. Shorter hospital stay was seen in those undergoing early combined ON and ESS interventions.

Background/UNASSIGNED:Upper Airway Stimulation (UAS) is a well-established therapy option for obstructive sleep apnea (OSA). Aims/UNASSIGNED:There are no data on whether respiratory sensing contributes to successful UAS therapy. Materials & Methods/UNASSIGNED:After initial measurements of 3 implanted patients (M1), the sensing signal was inverted (M2) without changing other parameters. Two weeks later, the signal was converted back again, and the sensitivity of breathing cycle detection was turned to a very low state (M3). Results/UNASSIGNED:At M2 and M3, the apnea-hypopnea index and oxygen desaturation index increased. Discussion/UNASSIGNED:Correct respiratory sensing is important for controlling OSA using UAS. Conclusions/UNASSIGNED:Therefore, implant centers should should optimize respiratory sensing placement and adjustment.

OBJECTIVES/HYPOTHESIS/OBJECTIVE:Description of a novel use of a submandibular gland (SMG) flap pedicled on the facial vessels to restore facial volume after parotidectomy, and comparison of clinical outcomes with alternative modes of reconstruction. STUDY DESIGN/METHODS:Retrospective chart review. METHODS:The surgical technique for a SMG flap is described. Retrospective chart review of cases of parotidectomy at a single tertiary medical center was conducted (n = 43). Cases were grouped in three cohorts of consecutive patients depending on reconstruction technique: SMG flap (n = 13), sternocleidomastoid muscle (SCM) flap (n = 15), and no flap (n = 15). Cohort characteristics and complication rates are reported. RESULTS:The SMG flap was more often used for a malignant pathology (92%) and in conjunction with a neck dissection (100%), compared to the SCM flap (47% and 15%) or no flap (0% and 0%), respectively. The mean House-Brackmann score in the immediate postoperative period in the SMG-flap group was slightly elevated compared to the other groups: 1.6 (standard deviation [SD] ± 0.5) versus 1.1 (SD ± 0.3) and 1.0 (SD ± 0.0). Otherwise, the complication rate was similar across groups. CONCLUSIONS:The SMG flap is a safe and effective option for volume restoration after parotidectomy. It provides durable volume that will not atrophy and is already exposed in the field of dissection for patients undergoing concurrent level I neck dissection. LEVEL OF EVIDENCE/METHODS:3.

OBJECTIVE:Cochlear implant (CI) technology and techniques have advanced over the years. There has not been the same degree of change in programming and there remains a lack of standardization techniques. The purpose of this study is to compare performance in cochlear implant subjects using experienced clinician (EC) standard programming methods versus an Artificial Intelligence, FOX based algorithm for programming. STUDY DESIGN/METHODS:Prospective, nonrandomized, multicenter study using within-subject experimental design SETTING:: Tertiary referral centers. PATIENTS/METHODS:Fifty-five adult patients with ≥ 3 months experience with a Nucleus 5, 6, Kanso, or 7 series sound processor. INTERVENTION/METHODS:Therapeutic Main Outcome Measures: CNC words and AzBio sentences in noise (+10 dB SNR) tests were administered in a soundproof booth followed by a direct connect psychoacoustic battery using the EC program. Tests were repeated 1 month later using the optimized FOX program. Subjective measures of patient satisfaction were also measured. RESULTS:Performance for the EC program was compared to the FOX program for both measures. Group mean results revealed equivalent performance (Kruskal-Wallis ANOVA p = 0.934) with both programming methods. While some patients had better performance with the FOX method and some performed more poorly, the majority had equivalent performance and preferred the FOX system. CONCLUSION/CONCLUSIONS:The study demonstrated that on average, FOX outcomes are equivalent to those using traditional programming techniques. In addition, the FOX programming method can effect standardization across centers and increase access for many individuals who could benefit.

The email address for Robert J. Ruben should be rruben@montefiore.org.