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Cochlear implantation in patients with neurofibromatosis type 2 and other retrocochlear pathology: A review of 32 cases over 25 years [Meeting Abstract]

Deep, N L; Patel, E; Shapiro, W H; Waltzman, S B; Jethanamest, D; McMenomey, S O; Roland, J T; Friedmann, D R
Objective: To describe cochlear implantation (CI) outcomes for rehabilitation of hearing loss due to retrocochlear pathology and/or its treatment.
Method(s): Retrospective review between 1995 and 2019 from a single tertiary care center of all patients with retrocochlear pathology who underwent CI. Demographics, clinical history, and audiometric data were reviewed. Study endpoints include (1) logged device use, (2) ability to achieve auditory perception, and (3) word recognition score (WRS) in the CI-only condition.
Result(s): Thirty-two patients (63% of females) with retrocochlear pathology were implanted at our center. The average age at implantation was 46.9 years (SD: 19, range: 13-80). Mean duration of deafness was 4.5 years (SD: 5.0, range: 0.4-19.0). Etiology of hearing loss included VS in 24 (75%), CNS malignancy treated with radiation in 4 (13%), intralabyrinthine schwannoma in 2 (6%), head and neck malignancy treated with radiation in 1 (3%), and superficial siderosis in 1 (3%). The mean preoperative PTA was 95.8 dBHL (SD 24.7) and WRS was 7.2% (SD 13.1). Of the 24 VSs, 21 were NF2-associated and 3 were sporadic. The mean tumor size was 1.64 cm (SD: 0.6, range: 0.5-2.6 cm). At the time of CI, 11 patients had prior microsurgery, 6 patients had prior radiation to the ipsilateral tumor, and 7 patients had stable tumors without prior surgery or radiation. Device use was classified as regular (>7 hours/day) in 15 (47%), limited (<7 hours/day) in 12 (38%), and nonuse is 5 (16%). The audiometric outcomes of 26 patients are reported, as the other 6 patients have been implanted too recently for review. Auditory perception was achieved in 24/26 patients. The two patients who failed to achieve auditory perception underwent prior surgery. Open-set speech recognition (WRS > 20%) was achieved in 18 patients. Meaningful sound perception but without significant open-set speech (WRS < 20%) was seen in six patients. Altogether, the mean WRS at most recent follow-up (mean: 3.4 years, SD: 1.8) for the observation, microsurgery, and radiation cohorts was 51% (SD: 15), 36% (SD: 28), and 39% (SD: 26), respectively. Over long-term follow-up, two patients experienced decline in CI performance associated with tumor regrowth and necessitated additional surgery; both underwent explantation of the CI and successful auditory brainstem implantation. The remaining patients have demonstrated durable benefit. A multivariate analysis is presented to evaluate the effects of the following variables: duration of deafness, time interval between treatment and CI, diagnosis of NF2, treatment cohort, pathology, and status of hearing in the contralateral ear.
Conclusion(s): In appropriately selected patients, cochlear implantation is feasible for the rehabilitation of hearing loss due to retrocochlear pathology and/or its treatment. Given the heterogeneity inherent to this population, outcomes are variable. In most cases, auditory percept was achieved and over half of the patients obtained open-set speech perception, irrespective of prior management and treatment
EMBASE:631114540
ISSN: 2193-6331
CID: 4387112

Osteotomies-When, Why, and How?

Locketz, Garrett D; Lozada, Kirkland N; Becker, Daniel G
An ideal nasal osteotomy should deliver precise, predictable, and reproducible cosmetic and functional results while minimizing soft-tissue trauma and postoperative complications. In addition to closing an open roof deformity after hump reduction, other common indications for osteotomies include the crooked nose and a wide bony vault. The literature has reported numerous and diverse osteotomy techniques as well as differences in timing of osteotomies. Each has its own merits and indications, and its proponents. In this article, we review the anatomy and nomenclature relating to osteotomies. We review the locations and paths of the osteotomies-lateral, intermediate, medial, and superior/transverse. We consider the percutaneous and endonasal approaches, as well as timing of osteotomies and other considerations. We also discuss technical considerations in the selection of instrumentation for osteotomies.
PMID: 32191960
ISSN: 1098-8793
CID: 4951722

Discontinuation of postoperative prophylactic antibiotics for endoscopic endonasal surgery [Meeting Abstract]

Benjamin, C G; Dastagirzada, Y; Bevilacqua, J; Gurewitz, J; Sen, C; Golfinos, J G; Placantonakis, D; Jafar, J J; Lebowtiz, R; Lieberman, S; Lewis, A; Pacione, D
Direct access through the sinuses and nasopharyngeal mucosa in the endoscopic endonasal approach (EEA) raises concern for a contaminated operative environment and subsequent infection. The reported rate of meningitis in endoscopic endonasal skull base surgery in the literature ranges from 0.7 to 3.0% [1, 2]. The only factor identified as being independently associated with meningitis in a statistically significant manner is cerebrospinal fluid (CSF) leak [1-5]. However, many centers performing high volume of EEAs use postoperative antibiotic coverage independent of the presence intraoperative or postoperative CSF leak. Furthermore, while meningitis remains a severe concern, most centers use postoperative gram-positive coverage to prevent toxic shock syndrome caused by Staphylococcus aureus infection in the setting of prolonged nasal packing. There are currently a multitude of approaches regarding perioperative antibiotic coverage in EEAs [1-4]. Given the lack of consensus in the literature and our experience regarding the benefit of discontinuation of prolonged prophylactic antibiotics throughout the breadth of neurosurgical procedures, we sought to analyze the need for postoperative antibiotics in EEAs further. As such, we performed a prospective analysis compared with a retrospective cohort to delineate whether discontinuation of postoperative antibiotics leads to a change in the rate of postoperative infections. The retrospective cohort consisted of patients who underwent an EEA from January 1, 2013 to May 31, 2019. These patients all received postoperative antibiotics while nasal packing was in place (median 7 days). Starting on April 1, 2019 until August 1, 2019, we discontinued postoperative antibiotic use. Patients from this group made up the prospective cohort. The retrospective cohort had 315 patients (66% pituitary macroadenomas vs. 7% microadenomas, 4% meningiomas, 4% craniopharyngiomas, 4% chordomas, and 15% others) while the prospective group had 23 patients (57% pituitary macroadenomas, 30% craniopharyngiomas, 8% meningiomas/chordomas, and 5% others). The primary endpoint was rate of postoperative infections and specifically, meningitis and multidrug resistant organism (MDRO) infections. There was no statistically significant difference in the use of nasal packing (p = 0.085), intraoperative CSF leak (p = 0.133), and postoperative CSF leak (p = 0.507) between the two groups. There was also no significant difference in the number of patients with positive preoperative MSSA and MRSA nasal swabs (p = 0.622). There was a significant decrease in the number of patients discharged with antibiotics (55.1% in the retrospective and 4.5% in the prospective group, p = 0.000). The number of patients with positive blood cultures (p = 0.701) and positive urine cultures (p = 0.691) did not differ significantly between the two groups. Finally, there was no statistically significant difference in postoperative CSF infections (p = 0.34) or MDRO infections (0.786) between the two groups. We describe promising preliminary results that demonstrate that discontinuation of postoperative antibiotics in EEAs do not lead to a statistically significant increase in the rate of postoperative CSF or MDRO infections. The previous algorithm for postoperative antibiotic coverage in our center, like many centers, called for gram-positive coverage, which may have contributed to the overall preponderance of gram-negative meningitis cases in this cohort
EMBASE:631114231
ISSN: 2193-6331
CID: 4387132

Alexis Retractor: Institutional Experience of Its Applications in Head and Neck Surgery and Review of the Literature

Lee, Jasmine; Yu, Jason W; Lee, Z-Hye; Levine, Jamie P; Jacobson, Adam S
BACKGROUND/UNASSIGNED:The Alexis retractor is a device that provides simultaneous radial retraction and wound protection during surgical procedures. Although typically used in abdominal and pelvic surgeries, there has been increased development of novel operative techniques utilizing the Alexis retractors in head and neck surgeries. METHODS/UNASSIGNED:We describe 2 cases of utilizing the Alexis retractor to attain transoral exposure in the setting of free flap reconstruction of intraoral defects. RESULTS/UNASSIGNED:In both cases, the Alexis retractor provided improved retraction, decreasing the number of instruments required for adequate exposure. Additionally, the polyurethane sheath component acted as a protective membrane over the lips and mucosa. CONCLUSIONS/UNASSIGNED:The Alexis retractor can be a powerful retraction tool for certain surgical procedures involving the head and neck regions.
PMID: 31960710
ISSN: 1545-1569
CID: 4273842

Patient-Specific 3D-Printed Rhinoplasty Operative Guides

Locketz, Garrett D; Silberthau, Kara; Lozada, Kirkland N; Becker, Daniel G
ORIGINAL:0015207
ISSN: 0748-8068
CID: 4951792

Immune Landscape of Viral- and Carcinogen-Driven Head and Neck Cancer

Cillo, Anthony R; Kürten, Cornelius H L; Tabib, Tracy; Qi, Zengbiao; Onkar, Sayali; Wang, Ting; Liu, Angen; Duvvuri, Umamaheswar; Kim, Seungwon; Soose, Ryan J; Oesterreich, Steffi; Chen, Wei; Lafyatis, Robert; Bruno, Tullia C; Ferris, Robert L; Vignali, Dario A A
Head and neck squamous cell carcinoma (HNSCC) arises through exposure to environmental carcinogens or malignant transformation by human papillomavirus (HPV). Here, we assessed the transcriptional profiles of 131,224 single cells from peripheral and intra-tumoral immune populations from patients with HPV- and HPV+ HNSCC and healthy donors. Immune cells within tumors of HPV- and HPV+ HNSCC displayed a spectrum of transcriptional signatures, with helper CD4+ T cells and B cells being relatively divergent and CD8+ T cells and CD4+ regulatory T cells being relatively similar. Transcriptional results were contextualized through multispectral immunofluorescence analyses and evaluating putative cell-cell communication based on spatial proximity. These analyses defined a gene expression signature associated with CD4+ T follicular helper cells that is associated with longer progression-free survival in HNSCC patients. The datasets and analytical approaches herein provide a resource for the further study of the impact of immune cells on viral- and carcinogen-induced cancers.
PMCID:7201194
PMID: 31924475
ISSN: 1097-4180
CID: 5482072

Oral submucous fibrosis: a contemporary narrative review with a proposed inter-professional approach for an early diagnosis and clinical management

Rao, Naman R; Villa, Alessandro; More, Chandramani B; Jayasinghe, Ruwan D; Kerr, Alexander Ross; Johnson, Newell W
Oral Submucous fibrosis (OSMF) has traditionally been described as "a chronic, insidious, scarring disease of the oral cavity, often with involvement of the pharynx and the upper esophagus". Millions of individuals are affected, especially in South and South East Asian countries. The main risk factor is areca nut chewing. Due to its high morbidity and high malignant transformation rate, constant efforts have been made to develop effective management. Despite this, there have been no significant improvements in prognosis for decades. This expert opinion paper updates the literature and provides a critique of diagnostic and therapeutic pitfalls common in developing countries and of deficiencies in management. An inter-professional model is proposed to avoid these pitfalls and to reduce these deficiencies.
PMCID:6951010
PMID: 31915073
ISSN: 1916-0216
CID: 4256992

Improving On-time Discharge in Otolaryngology Admissions

Gordon, Steven A; Garber, David; Taufique, Zahrah; Shao, Qianhui; Amin, Milan R; Roland, J Thomas; Givi, Babak
OBJECTIVE:We conducted a quality improvement project to increase the rate of discharges before noon (DBN) in the otolaryngology department at a tertiary care center. METHODS:Based on a Plan-Do-Study-Act framework, monthly discharge data and observed-to-expected (O:E) length of stay were collected and shared with the department members monthly. A target of 43% DBN was predetermined by the center (Plan). The following interventions were implemented (Do): discharge planning starting at the time of admission, focus on early attending-to-resident team communication, placement of discharge order prior to rounding, and weekly reminders to the entire department. RESULTS:Discharges were monitored for 3 years. For the year prior to this study, a minority of patients were discharged before noon (12 months: 75 of 190, 36%). During the first 6 months of monitoring (Study), no significant improvement was identified (34 of 95, 36%). After interventions, performance significantly improved (31 months: 250 of 548, 68%). The performance was consistently above the predetermined target of 43%. During the study time, O:E length of stay remained below the predetermined target (O:E ratio, 0.90; hospital target, 0.93). DISCUSSION/CONCLUSIONS:Comprehensive discharge planning beginning at the time of admission, weekly reminders, and improved communication (Act) can help to prioritize DBN and increase the percentage of discharges before noon. IMPLICATIONS FOR PRACTICE/CONCLUSIONS:By utilizing a quality improvement framework, significant improvements in timely discharge can be achieved and sustained with changes in workflow and departmental culture. These changes can be achieved without increases in resources or prolonging the length of stay.
PMID: 31906819
ISSN: 1097-6817
CID: 4257102

Efference Copies: Hair Cells Are the Link

Goldblatt, Dena S; Schoppik, David
Animals must distinguish external stimuli from self-generated sensory input to guide appropriate behaviors. A recent study elucidates a cellular mechanism by which zebrafish perform this distinction while maintaining sensitivity to external environmental signals.
PMID: 31910366
ISSN: 1879-0445
CID: 4257212

Zebrafish dscaml1 Deficiency Impairs Retinal Patterning and Oculomotor Function

Ma 马漫修, Manxiu; Ramirez, Alexandro D; Wang 王彤, Tong; Roberts, Rachel L; Harmon, Katherine E; Schoppik, David; Sharma, Avirale; Kuang, Christopher; Goei, Stephanie L; Gagnon, James A; Zimmerman, Steve; Tsai, Shengdar Q; Reyon, Deepak; Joung, J Keith; Aksay, Emre R F; Schier, Alexander F; Pan 潘於勤, Y Albert
Down Syndrome Cell Adhesion Molecules (dscam and dscaml1) are essential regulators of neural circuit assembly, but their roles in vertebrate neural circuit function are still mostly unexplored. We investigated the functional consequences of dscaml1 deficiency in the larval zebrafish (sexually undifferentiated) oculomotor system, where behavior, circuit function, and neuronal activity can be precisely quantified. Genetic perturbation of dscaml1 resulted in deficits in retinal patterning and light adaptation, consistent with its known roles in mammals. Oculomotor analyses revealed specific deficits related to the dscaml1 mutation, including severe fatigue during gaze stabilization, reduced saccade amplitude and velocity in the light, greater disconjugacy, and impaired fixation. Two-photon calcium imaging of abducens neurons in control and dscaml1 mutant animals confirmed deficits in saccade-command signals (indicative of an impairment in the saccadic premotor pathway), while abducens activation by the pretectum-vestibular pathway was not affected. Together, we show that loss of dscaml1 resulted in impairments in specific oculomotor circuits, providing a new animal model to investigate the development of oculomotor premotor pathways and their associated human ocular disorders.SIGNIFICANCE STATEMENTDscaml1 is a neural developmental gene with unknown behavioral significance. Using the zebrafish model, this study shows that dscaml1 mutants have a host of oculomotor (eye movement) deficits. Notably, the oculomotor phenotypes in dscaml1 mutants are reminiscent of human ocular motor apraxia, a neurodevelopmental disorder characterized by reduced saccade amplitude and gaze stabilization deficits. Population-level recording of neuronal activity further revealed potential subcircuit-specific requirements for dscaml1 during oculomotor behavior. These findings underscore the importance of dscaml1 in the development of visuomotor function and characterize a new model to investigate potential circuit deficits underlying human oculomotor disorders.
PMID: 31685652
ISSN: 1529-2401
CID: 4172342