Faculty Bibliography

Management of head and neck squamous cell carcinoma necessitates a multimodal approach. The neck dissection has evolved over many years but is well established as the key surgical intervention for management of nodal disease in the neck. The open neck dissection has many varieties based on location and degree of disease but is the gold standard surgical technique. Robot-assisted neck dissections have emerged in recent years as an alternative. More research is required to establish long-term oncologic outcomes achieved with robot-assisted surgery and to assess whether cost and operative times decrease with experience.

Fanconi anemia (FA) is a clinically heterogenous and genetically diverse disease with 22 known complementation groups (FA-A to FA-W), resulting from the inability to repair DNA interstrand crosslinks. This rare disorder is characterized by congenital defects, bone marrow failure, and cancer predisposition. FANCA is the most commonly mutated gene in FA and a variety of mostly private mutations have been documented, including small and large indels, and point and splicing variants. Genotype-phenotype associations in FA are complex and a relationship between particular FANCA variants and the observed cellular phenotype or illness severity remains unclear. In this study, we describe two siblings with compound heterozygous FANCA variants (c.3788_3790delTCT and c.4199G>A) who both presented with esophageal squamous cell carcinoma at the age of 51. The proband came to attention when he developed pancytopenia after a single cycle of low-dose chemotherapy including platinum-based therapy. Other than a minor thumb abnormality, neither patient had prior findings to suggest FA, including normal blood counts and intact fertility. Patient fibroblasts from both siblings display increased chromosomal breakage and hypersensitivity to interstrand crosslinking agents as seen in typical FA. Based on our functional data demonstrating that the c.4199G>A/p.R1400H variant represents a hypomorphic FANCA allele, we conclude that the residual activity of the Fanconi anemia repair pathway accounts for lack of spontaneous bone marrow failure or infertility with the late presentation of malignancy as the initial disease manifestation. This and similar cases of adult-onset esophageal cancer stress the need for chromosome breakage testing in patients with early onset of aerodigestive tract squamous cell carcinomas before platinum-based therapy is initiated.

PURPOSE/OBJECTIVE:To report our experience of a sequence of events that resulted in an iatrogenic cholesteatoma originating from the external auditory canal (EAC) years after tympanoplasty that had included a tympanomeatal flap. METHODS:Data on the presentation and pathogenesis of iatrogenic cholesteatomas arising from misplaced tympanomeatal flaps during tympanoplasty without mastoidectomy were retrieved from the patients' medical records and analyzed. RESULTS:Five patients were identified with cholesteatomas involving the EAC. They all had recurrent ear infections and varying degrees of conductive hearing loss. Each patient's past surgical history included one or more tympanoplasties in which an ipsilateral tympanomeatal flap had been raised. None had undergone a mastoidectomy. Two patients presented with small cholesteatomas that had developed over an average of 6.5 years after surgery. Three patients had large cholesteatomas that had developed over an average of 33.7 years after surgery. Clinical presentations and imaging studies suggested a misplaced tympanomeatal flap as the most likely source of cholesteatoma. CONCLUSION/CONCLUSIONS:Tympanomeatal flap misplacement may cause iatrogenic cholesteatoma formation originating from the EAC during tympanoplasty even without mastoidectomy. These cholesteatomas can grow substantially before becoming symptomatic as they extend to and through the mastoid. They may not affect the sound conduction system until late in the course of the disease. Meticulous replacement of tympanomeatal flaps and exercising a high index of suspicion postoperatively can reduce the incidence of this complication.

PURPOSE/OBJECTIVE:Metastases should be considered in a patient with a cancer history and a sellar/suprasellar lesion, as this diagnosis can change the management strategy in such patients. Once the diagnosis is established, stereotactic radiosurgery (SRS) can be a safe and effective approach for these patients. METHODS:This case series describes five patients with pituitary metastases managed with GKRS at a single institution, taken from our prospective registry. All patients had SRS using the Gamma Knife Perfexion or Icon (Elekta), according to our standard institutional protocol. The optic nerves and chiasm were contoured, and the plan was adjusted to restrict dose to the optic apparatus as necessary. The tumor margin doses delivered were 11 Gy, 12 Gy, 14 Gy, 18 Gy (3 sessions of 6 Gy), and 12 Gy at the 50% isodose line. RESULTS:In this series, all sellar metastases were treated successfully with good radiographic and clinical response. The histology of the tumors included endometrial, gastrointestinal, and lung adenocarcinomas. Typically, histology is taken into consideration when choosing the treatment dose, along with size and location. In these patients, however, the dose used for the sellar metastases was chosen primarily for visual safety. This was typically lower than the dose for brain metastases in other locations. CONCLUSION/CONCLUSIONS:SRS provides an alternative treatment approach for sellar/suprasellar metastases with excellent local control, symptom improvement and maintenance of systemic therapy as desired. As such, CNS failure is rarely the proximate cause of demise in pituitary metastases provided that endocrinopathies are recognized and managed appropriately.

Chimeric antigen receptor T-cell therapy (CAR-T) is a novel immunotherapy used for the treatment of refractory B-cell leukemias and lymphoma. As clinical trials continue to expand, multiple treatment toxicities have been documented. Treatment-associated toxicities are typically systemic, however, focal manifestations have been described. We present a unique case of a 55-year-old female who developed oropharyngeal and laryngeal dystonia following CAR-T therapy. This case points to a possible association between CAR-T therapy and focal head and neck dystonia. Laryngoscope, 2020.

OBJECTIVE:To address variance in clinical care surrounding sporadic vestibular schwannoma, a modified Delphi study was performed to establish a general framework to approach vestibular schwannoma care. A multidisciplinary panel of experts was established with deliberate representation from key stakeholder societies. External validity of the final statements was assessed through an online survey of registered attendees of the 8th Quadrennial International Conference on Vestibular Schwannoma. STUDY DESIGN/METHODS:Modified Delphi method. METHODS:The panel consisted of 16 vestibular schwannoma experts (8 neurotology and 8 neurosurgery) and included delegates representing the AAOHNSF, AANS/CNS tumor section, ISRS, and NASBS. The modified Delphi method encompassed a four-step process, comprised of one prevoting round to establish a list of focus areas and three subsequent voting rounds to successively refine individual statements and establish levels of consensus. Thresholds for achieving moderate consensus, at ≥67% agreement, and strong consensus, at ≥80% agreement, were determined a priori. All voting was performed anonymously via the Qualtrics online survey tool and full participation from all panel members was required before procession to the next voting round. RESULTS:Through the Delphi process, 103 items were developed encompassing hearing preservation (N = 49), tumor control and imaging surveillance (N = 20), preferred treatment (N = 24), operative considerations (N = 4), and complications (N = 6). As a result of item refinement, moderate (4%) or strong (96%) consensus was achieved in all 103 final statements. Seventy-nine conference registrants participated in the online survey to assess external validity. Among these survey respondents, moderate (N = 21, 20%) or strong (N = 73, 71%) consensus was achieved in 94 of 103 (91%) statements, and no consensus was reached in 9 (9%). Of the four items with moderate consensus by the expert panel, one had moderate consensus by the conference participants and three had no consensus. CONCLUSION/CONCLUSIONS:This modified Delphi study on sporadic vestibular schwannoma codifies 100% consensus within a multidisciplinary expert panel and is further supported by 91% consensus among an external group of clinicians who regularly provide care for patients with vestibular schwannoma. These final 103 statements address clinically pragmatic items that have direct application to everyday patient care. This document is not intended to define standard of care or drive insurance reimbursement, but rather to provide a general framework to approach vestibular schwannoma care for providers and patients.

This erratum notifies the readers of the Dysphagia journal of an error in the original published version of this manuscript. In that manuscript, a previously available open source spreadsheet tool was used to calculate the position of the posterior laryngeal air column on lateral view videofluoroscopic images as a proxy for the bottom of the pharynx. We have subsequently been made aware of an error in the mathematical formula built into the spreadsheet, which resulted in a reversal of the results for the X and Y planes of measurement. This erratum provides corrections to the results and interpretations of the original manuscript.

Two notes sounded sequentially elicit melodic intervals and contours that form the basis of melody. Many previous studies have characterized pitch perception in cochlear implant (CI) users to be poor which may be due to the limited spectro-temporal resolution and/or spectral warping with electric hearing compared to acoustic hearing (AH). Poor pitch perception in CIs has been shown to distort melodic interval perception. To characterize this interval distortion, we recruited CI users with either normal (single sided deafness, SSD) or limited (bimodal) AH in the non-implanted ear. The contralateral AH allowed for a stable reference with which to compare melodic interval perception in the CI ear, within the same listener. Melodic interval perception was compared across acoustic and electric hearing in 9 CI listeners (4 bimodal and 5 SSD). Participants were asked to rank the size of a probe interval presented to the CI ear to a reference interval presented to the contralateral AH ear using a method of constant stimuli. Ipsilateral interval ranking was also measured within the AH ear to ensure that listeners understood the task and that interval ranking was stable and accurate within AH. Stimuli were delivered to the AH ear via headphones and to the CI ear via direct audio input (DAI) to participants' clinical processors. During testing, a reference and probe interval was presented and participants indicated which was larger. Ten comparisons for each reference-probe combination were presented. Psychometric functions were fit to the data to determine the probe interval size that matched the reference interval. Across all AH reference intervals, the mean matched CI interval was 1.74 times larger than the AH reference. However, there was great inter-subject variability. For some participants, CI interval distortion varied across different reference AH intervals; for others, CI interval distortion was constant. Within the AH ear, ipsilateral interval ranking was accurate, ensuring that participants understood the task. No significant differences in the patterns of results were observed between bimodal and SSD CI users. The present data show that much larger intervals were needed with the CI to match contralateral AH reference intervals. As such, input melodic patterns are likely to be perceived as frequency compressed and/or warped with electric hearing, with less variation among notes in the pattern. The high inter-subject variability in CI interval distortion suggests that CI signal processing should be optimized for individual CI users.