Endoluminal Sigmoid Sinus Occlusion During Jugular Foramen Tumor Surgery: Novel Technique, Operative Nuances, and Clinical Experience With 33 Patients.

BACKGROUND AND IMPORTANCE: Surgery of jugular foramen tumors (JFTs) often requires vascular control by means of ligating the internal jugular vein and sigmoid sinus (SS) to allow intrabulbar access. Occlusion of the SS traditionally involves presigmoid and retrosigmoid durotomies allowing introduction of ligature devices, predisposing to cerebrospinal fluid (CSF) leakage and pseudomeningoceles. We describe a simple and novel endoluminal sigmoid sinus occlusion (ESSO) technique with Gelfoam that is entirely extradural. CLINICAL PRESENTATION: An extended anterolateral infralabyrinthine approach with ESSO was performed in 33 patients with JFTs. After ligating the internal jugular vein, the SS is opened and Gelfoam is placed endoluminally into the proximal SS. Care is taken to avoid occlusion of the venous outflow of the vein of Labbe to avoid temporal lobe venous infarction. Hemostatic gelatin matrix is injected distally to stop venous backflow from the inferior petrosal sinus. The jugular venous system is isolated, and the outer jugular wall can be opened to expose the JFT for resection. There were no complications of temporal lobe venous infarction or postoperative hematoma observed. Four patients with intradural tumor extension developed pseudomeningoceles. For patients with purely extradural JFTs, none developed postoperative incisional CSF leaks and one had pseudomeningocele. CONCLUSION: This ESSO technique is fast and effective, permitting occlusion of the SS during JFT surgery. It has the advantage of being entirely extradural, avoiding durotomy which can result in postoperative CSF leak. It is important to keep the Gelfoam distal to the transverse-sigmoid junction to avoid occlusion of the vein of Labbe inlet and temporal lobe venous infarction.

A review of bilateral sudden sensorineural hearing loss in pediatric patients.

OBJECTIVE: Bilateral, sudden sensorineural hearing loss (SSNHL) in the pediatric population is a rare phenomenon potentially detrimental to language acquisition and social development. This study comprehensively reviews and analyzes existing literature to determine any correlation or commonality in etiologies, presentations, and management of this condition. METHODS: PubMed, Cochrane, Scopus, and Web of Science databases were systematically searched for articles related to pediatric SSNHL from 1970 to 2021. Case series, case reports, and cohort studies were included. Data on patient demographics, etiology, diagnostic testing, management, and hearing recovery were collected. RESULTS: Excluding duplicates, 553 unique titles were identified by established search criteria, of which 342 titles were relevant to pediatric sudden hearing loss. Forty-six papers reported cases of bilateral SSNHL, totaling 145 individual cases. Not included in the analysis were 45 cases documented as non-organic hearing loss. The average age of the total 145 included patients was 8.5 years and 51 were male. Reported etiologies included cytomegalovirus (n = 3), meningitis (n = 13), mumps (n = 5), ototoxin exposure (n = 13), and enlarged vestibular aqueduct (n = 9). Tinnitus (n = 30) was the most reported concurrent symptom, followed by vertigo (n = 21). Systemic steroid therapy was the most common treatment and, when follow up was reported, most patients (51.2%) had complete or partial recovery of hearing. CONCLUSIONS: This is a comprehensive review of pediatric bilateral SSNHL. Though often idiopathic, etiologies also include infectious, structural, and autoimmune. Treatment largely consists of systemic steroid therapy, with variables rates of recovery. Further studies on intratympanic administration of steroids may guide future treatment.

Telemedicine and Otolaryngology in the COVID-19 Era.

OBJECTIVE: The global Coronavirus disease 2019 (COVID-19) pandemic has resulted in an expansion of telemedicine. The purpose of this study is to present our experience with outpatient telemedicine visits within a single institution's Department of Otolaryngology during the initial COVID-19 era. STUDY DESIGN: Retrospective chart review. METHODS: This was a single-institution study conducted within the Department of Otolaryngology at an urban tertiary care center. Data on outpatient visits was obtained from billing and scheduling records from January 6 to May 28, 2020. Visits were divided into "pre-shutdown" and "post-shutdown" based on our state's March 23, 2020 COVID-19 shutdown date. RESULTS: A total of 3447 of 4340 (79.4%) scheduled visits were completed in the pre-shutdown period as compared to 1451 of 1713 (84.7%) in the post-shutdown period. The proportion of telemedicine visits increased (0.7%-81.2%, P < .001). Overall visit completion rate increased following the shutdown (80.2%-84.7%, P < .001). Subspecialties with an increase in visit completion rate were general (76.9%-88.0%, P = .002), otology (77.4%-87.2%, P < .001), and rhinology (80.0%-86.2%, P = .003). Patients with Medicaid and Medicare had higher appointment completion rates following the transition to telemedicine visits (80.7%-85.7%, P = .002; 76.9%-84.7%, P = .001). Older age was associated with decreased appointment cancellation pre-shutdown (OR 0.994 [0.991-0.997], P < .001) but increased appointment cancellation post-shutdown (OR 1.008 [1.001-1.014], P = .015). Mean COVID-19 risk scores were unchanged (P = .654). CONCLUSIONS: COVID-19 has led to major changes in outpatient practice, with a significant shift from in-person to telemedicine visits following the mandatory shutdown. An associated increase in appointment completion rates was observed, reflecting a promising viable alternative to meet patient needs during this unprecedented time.

Association of Patient Frailty With Vestibular Schwannoma Resection Outcomes and Machine Learning Development of a Vestibular Schwannoma Risk Stratification Score.

BACKGROUND: Patient frailty is predictive of higher neurosurgical morbidity and mortality. However, existing frailty measures are hindered by lack of specificity to neurosurgery. OBJECTIVE: To analyze the association between 3 risk stratification scores and outcomes for nationwide vestibular schwannoma (VS) resection admissions and develop a custom VS risk stratification score. METHODS: We identified all VS resection admissions in the National Inpatient Sample (2002-2017). Three risk stratification scores were analyzed: modified Frailty Index-5, modified Frailty Index-11(mFI-11), and Charlson Comorbidity Index (CCI). Survey-weighted multivariate regression evaluated associations between frailty and inpatient outcomes, adjusting for patient demographics, hospital characteristics, and disease severity. Subsequently, we used k -fold cross validation and Akaike Information Criterion-based model selection to create a custom risk stratification score. RESULTS: We analyzed 32 465 VS resection admissions. High frailty, as identified by the mFI-11 (odds ratio [OR] = 1.27, P = .021) and CCI (OR = 1.72, P < .001), predicted higher odds of perioperative complications. All 3 scores were also associated with lower routine discharge rates and elevated length of stay (LOS) and costs (all P < .05). Our custom VS-5 score ( https://skullbaseresearch.shinyapps.io/vs-5_calculator/ ) featured 5 variables (age ≥60 years, hydrocephalus, preoperative cranial nerve palsies, diabetes mellitus, and hypertension) and was predictive of higher mortality (OR = 6.40, P = .001), decreased routine hospital discharge (OR = 0.28, P < .001), and elevated complications (OR = 1.59, P < .001), LOS (+48%, P < .001), and costs (+23%, P = .001). The VS-5 outperformed the modified Frailty Index-5, mFI-11, and CCI in predicting routine discharge (all P < .001), including in a pseudoprospective cohort (2018-2019) of 3885 admissions. CONCLUSION: Patient frailty predicted poorer inpatient outcomes after VS surgery. Our custom VS-5 score outperformed earlier risk stratification scores.

Characterization of Sensorineural Hearing Loss in Patients With MYH9-Related Disease: A Systematic Review.

OBJECTIVES: To review the characteristics and progression of hearing loss in MYH9-related disease (MYH9-RD) patients and present a unique case of bilateral non-simultaneous sudden sensorineural hearing loss (SNHL) in an MYH9-RD patient. MYH9-RD is a rare autosomal dominant platelet disorder. Patients with this disorder have a variable risk of developing SNHL. METHODS: A comprehensive literature search for scientific articles in PubMed, Scopus, and Web of Science that reported hearing loss outcomes in MYH9-RD patients. RESULTS: Initial search yielded 270 studies. Eight studies with a total of 23 patients met inclusion criteria and were used for data analysis. MYH9-RD patients typically present with progressive bilateral SNHL affecting predominantly the high frequencies. Mean age of hearing loss onset was 17.1 years, progressing to severe-profound SNHL over a mean period of 14.4 years. Seventeen of the 23 patients received cochlear implant (CI) at a mean age of 37.9 years. In comparison, the study patient presented initially with bilateral progressive SNHL as a teenager and developed bilateral non-simultaneous sudden SNHL, first in her right ear at the age of 31 and 7 months later in her left ear at the age of 32. She is now a successful bilateral CI user. CONCLUSIONS: This is the first systematic investigation of the relationship between MYH9-RD patients and SNHL. Hearing loss in MYH9-RD patients is generally characterized as progressive SNHL. However, the study patient presented with the unique feature of bilateral non-simultaneous sudden SNHL, potentially expanding the hearing loss sequela associated with this disorder.

Impact of Social Determinants of Health on Stereotactic Radiotherapy for Vestibular Schwannoma.

OBJECTIVES/HYPOTHESIS: Linear accelerator (LINAC) and Gamma Knife (GK) are common stereotactic radiation therapies for treating vestibular schwannoma (VS). There is currently limited literature examining specific demographic and socioeconomic factors, which influence the type of stereotactic radiation therapy a patient with VS receives. STUDY DESIGN: Retrospective database review. METHODS: The National Cancer Database was queried for cases of VS between 2004 and 2016. Patient demographic characteristics were compared using chi-squared and t-tests between GK and LINAC treated groups. Multivariate regression analysis was performed to assess predictors of stereotactic radiation therapy received. RESULTS: Of the 6,208 included patients, 5,306 (85.5%) received GK and 902 (14.5%) received LINAC. The mean age of GK patients was significantly lower than that of LINAC patients (58.0 vs. 59.7, P < .001). Individuals treated with GK had greater proportions of private insurance (P < .001) and incomes greater than $63,332 (P = .003). A greater proportion of GK patients were treated in academic centers (P < .001), in high-volume facilities (P < .001), in metropolitan areas (P < .001), and in the Northeastern United States (P < .001). On multivariate logistic regression analysis, region, metropolitan area, facility type, tumor size, and distance traveled by patients independently predict receipt of GK versus LINAC. CONCLUSION: Differences in patient demographics and other social determinants of health influence choice of GK versus LINAC therapy for VS patients. Future studies focused on addressing barriers to care, which may influence postprocedural quality of life and clinical outcomes associated with these two treatments are necessary to better understand the impact of these social differences. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:2232-2240, 2022.

Developments in Bio-Inspired Nanomaterials for Therapeutic Delivery to Treat Hearing Loss.

Sensorineural hearing loss affects millions of people worldwide and is a growing concern in the aging population. Treatment using aminoglycoside antibiotics for infection and exposure to loud sounds contribute to the degeneration of cochlear hair cells and spiral ganglion neurons. Cell loss impacts cochlear function and causes hearing loss in ∼ 15% of adult Americans (∼36 million). The number of individuals with hearing loss will likely grow with increasing lifespans. Current prosthesis such as hearing aids and cochlear implants can ameliorate hearing loss. However, hearing aids are ineffective if hair cells or spiral ganglion neurons are severely damaged, and cochlear implants are ineffective without properly functioning spiral ganglion neurons. As such, strategies that alleviate hearing loss by preventing degeneration or promoting cell replacement are urgently needed. Despite showing great promise from in vitro studies, the complexity and delicate nature of the inner ear poses a huge challenge for delivering therapeutics. To mitigate risks and complications associated with surgery, new technologies and methodologies have emerged for efficient delivery of therapeutics. We will focus on biomaterials that allow controlled and local drug delivery into the inner ear. The rapid development of microsurgical techniques in conjunction with novel bio- and nanomaterials for sustained drug delivery appears bright for hearing loss treatment.

Changing trends in management of vestibular schwannoma: A National Cancer Database study.

OBJECTIVE: To describe changes in management trends of vestibular schwannoma (VS) and the effects of median income, education level, insurance status, and tumor size on receipt of treatment. METHODS: Cross-sectional analysis using the National Cancer Database from 2004 to 2014. All patients with a diagnosis of VS were identified between 2004 and 2014. Trends in treatment modality over time were analyzed. Correlation between demographics, institution type, median income, education level, and insurance status were assessed using univariate and multivariate logistic regressions. RESULTS: Of the 22,290 VS patients identified, 16,011 (71.8%) received treatment, whereas 6,279 (28.2%) received observation. Management trend analysis over the 11-year period revealed patients with tumor size < 1 cm are more frequently observed (34.6% of cases in 2004 vs. 60.8% of cases in 2014) and less frequently received surgery (34.6% vs. 16.8%). Multivariate analysis showed observation was more frequently used in patients ≥ 65 years; black patients; and those on Medicaid, Medicare, and noninsured (P < 0.0001). All tumors > 2 cm were more likely to be treated with surgery, specifically subtotal resections (P < 0.0001). Patients treated at nonacademic centers were more likely to receive linear accelerator, or LINAC (Accuray Incorporated, Sunnyvale, CA) therapy than Gamma Knife (Elekta, Stockholm, Sweden) (P < 0.0001). CONCLUSION: Management of VS is shifting toward increased observation, most significantly in tumors size < 1 cm. Insurance provider plays a significant role on receipt of treatment and modality, whereas income and education do not. LEVEL OF EVIDENCE: NA Laryngoscope, 129:1197-1205, 2019.

Electronic Health Record Use Among American Neurotology Society Members.

OBJECTIVE: To describe the use of electronic health records (EHR) among members of the American Neurotology Society (ANS). STUDY DESIGN: Cross-sectional. SETTING: Active ANS members in November 2017. INTERVENTION: Internet-based survey. MAIN OUTCOME MEASURE: Survey that assessed the use of EHR in practice, types of EHR programs, different elements of EHR employed, and respondents' satisfaction and efficiency with EHR. RESULTS: One hundred twenty-seven ANS members responded to the survey with 67 (52.8%) respondents working in academic practice and 60 (47.2%) respondents working in private practice. Epic was the most commonly used EHR with 89 (70.1%) respondents using this system. Among all respondents, 84 (66.1%) respondents reported their efficiency was reduced by EHR use, and there was an even split between respondents who reported they were satisfied versus dissatisfied with their EHR (∼40% each). We found that younger members were more likely to feel EHR increased their efficiency compared with the older members (p = 0.04). In all other analyses, we found no significant difference in efficiency and satisfaction between age groups, practice settings, presence of residents or fellows, or specific EHR used. The main challenges reported by ANS members related to the EHR were increased time burden, poor user interface, lack of customizability, and the focus away from patients. CONCLUSIONS: The majority of ANS members felt their efficiency decreased as a result of EHR. These findings provide specific changes to the EHR that would improve efficiency and satisfaction among neurotologists.

The Role of Fast Imaging Employing Steady-State Acquisition (FIESTA) Magnetic Resonance Imaging for Assessment of Delayed Enhancement of Fat Graft Packing on Postoperative Imaging After Vestibular Schwannoma Surgery.

OBJECTIVE: To investigate the role and efficacy of fast imaging employing steady-state acquisition (FIESTA) imaging in distinguishing fat graft enhancement from residual or recurrent tumor after vestibular schwannoma (VS) surgery. METHODS: A retrospective study of 33 patients who underwent VS resection via the retrosigmoid or translabyrinthine approach with fat graft reconstruction was performed. Magnetic resonance imaging (MRI) was collected at different time points: preoperative, immediate postoperative (24-48 hours), delayed postoperative (3-6 months after surgery), and yearly postoperative. The image sets contained T1, T2, fat-suppressed T1-weighted with gadolinium, and FIESTA. The radiographs were analyzed for tumor recurrence by the primary neurosurgeon and an independent blinded neuroradiologist. If fat-suppressed T1-weighted images demonstrated postoperative enhancement in the resection bed, a comparison was made with FIESTA imaging. RESULTS: At 3-6 months postoperatively and at 1 year and beyond, 28 (84.8%) and 33 (100%) of patients, respectively, displayed delayed enhancement of the fat graft on postgadolinium fat-suppressed T1-weighted MRI. The enhancement seen on postgadolinium, fat-suppressed, T1-weighted MRI consistently correlated with the characteristic fat graft signal on FIESTA imaging and not tumor recurrence. FIESTA imaging was able to distinguish residual tumor from enhancing fat graft compared with postgadolinium, fat-suppressed, T1-weighted MRI (P < 0.0001) due to distinctive signaling patterns. CONCLUSIONS: FIESTA is an effective tool in discerning fat graft enhancement from residual or recurrent tumor on delayed postoperative imaging after VS resection. Fat graft used in reconstruction consistently enhances on delayed postoperative postgadolinium, fat-suppressed, T1-weighted imaging, which correlates with the fat graft signal seen on FIESTA images.

Traumatic Tympanic Membrane Perforations Diagnosed in Emergency Departments.

Importance: Tympanic membrane perforations (TMPs) are frequent events leading to evaluation in the primary care and otolaryngology offices or the emergency department (ED). Despite specific warning labels on packaging of cotton-tipped applicators regarding the risk of injury to the ear canal with personal use, these products are commonly used to remove ear cerumen. Objective: To analyze the mechanism of injury for traumatic TMPs among patients presenting to the ED. Design, Setting, and Participants: Cross-sectional analysis of cases from 100 emergency departments in the United States. The National Electronic Injury Surveillance System was searched on April 3, 2015, for ear-related injuries with analysis information regarding patient age, patient sex, time and date of injury, specific injury diagnoses, and specific injury mechanisms that occurred across 5 years, from January 1, 2010, through December 31, 2014. Main Outcomes and Measures: Diagnoses of traumatic TMP documented in the ED visit record as well as patient demographics, diagnoses, and other aspects of the injury, including mechanism of injury. Results: There were 949 case entries in the database for traumatic TMP, which extrapolates to 4852 ED visits nationally. Of 949 patients evaluated, 568 (59.8%) were men and 381 (40.2%) were women resulting in a male to female ratio of 1.49:1. Most injuries occurred in patients 18 years or younger (602 of 949 [63.4%]) with children younger than 6 years most at risk (331 of 949 [34.9%]). Ear canal instrumentation including foreign bodies was noted in 581 of 949 cases (61.2%), with cotton-tipped applicators noted in 261 (44.9%) of these cases. While foreign body instrumentation represented the leading cause of traumatic TMP in patients aged 0 to 5 years (284 of 331 cases [85.8%]), 6 to 12 years (108 of 158 [68.4%]), 19 to 36 years (85 of 223 [38.1%]), 37 to 54 years (48 of 91 [52.7%]), and 55 years or older (22 of 33 [66.7%]), water trauma was the leading cause of TMP in patients aged 13 to 18 years (43 of 113 cases [38.1%]). Conclusions and Relevance: Traumatic TMP represents a common reason for evaluation in the ED. Despite common warnings regarding risk of injury to the tympanic membrane with use of a cotton-tipped applicator, it is still a major cause of traumatic TMPs. Other injury mechanisms also play an important role in the teenage and young adult populations.

A Population Based Analysis of Melanoma of the External Ear.

OBJECTIVE: Malignant melanoma accounts for nearly 75% of all skin cancer deaths, and the incidence is on the rise in the United States. External ear melanoma (EEM) is rare, and there is little long-term data regarding the clinical behavior of this melanoma site. This study analyzes the demographic, clinicopathologic, and survival characteristics of EEM. METHODS: The SEER database was queried for EEM cases from 1973 to 2012 (8,982 cases). Data analyzed included patient demographics, incidence trends, and survival outcomes. RESULTS: External ear melanoma occurred most frequently in the sixth and seventh decades of life. Mean age at diagnosis was 65.5 (±16.8) years. However, the incidence of EEM in adolescents and young adults (ages 15-39 yr) has increased by 111.9% from 1973 to 2012. There was a strong male predilection with a male-to-female ratio of 6.40:1. The most common histologic subtype was malignant melanoma, NOS (46.8%), followed by superficial spreading melanoma (21.4%), and lentigo maligna melanoma (17.9%). The majority of cases were localized at the time of presentation (88.0%), with rare distant metastasis (1.9%). The most common treatment modality was surgery alone (97.6%), followed by surgery with radiotherapy (2.3%). Ten-year disease-specific survival was better among those treated with surgery alone (90.7%), than those treated with surgery with radiotherapy (37.1%) (p < 0.0001). Increasing Breslow's thickness and presence of an ulcerating lesion were both associated with poorer survival (p < 0.0001). CONCLUSION: This study represents the largest cohort of EEM. It has an excellent survival outcome with surgery being the treatment of choice.

Superior semicircular canal dehiscence: Diagnosis and management.

The authors provide an update on the clinical manifestations, diagnosis and various approaches to the treatment of superior semicircular canal dehiscence (SSCD). SSCD is a rare condition where the bone overlying the superior semicircular canal thins or dehisces causing characteristic clinical findings. Since this was first reported in 1998 by Minor and colleagues, there has been much advancement made in terms of diagnosis and treatment. Signs and symptoms include a wide variation of both vestibular and auditory manifestations. Diagnosis made solely on clinical signs is difficult due to how varied the presentations can be and the overlap with other otologic pathologies. High-resolution CT temporal scans have been the standard in confirming superior semicircular canal dehiscence, however, MRI FIESTA scans have recently been used to image SSCD. Additionally, audiometry and vestibular evoked myogenic potential (VEMP) testing are useful screening tools. Currently, the middle fossa approach is the most common and standard surgical approach to repair SSCD. The transmastoid, endoscopic and transcanal or endaural approaches have also been recently utilized. Presently, there is no consensus as to the best approach, material or technique for repair of SSCD. As we learn more, newer and less invasive approaches and techniques are being used to treat SSCD. We present a comprehensive review of SSCD, including clinical symptoms and presentation, histopathology, diagnosis, treatment strategies and outcomes of intervention.

Outcomes of Vestibular Schwannoma Surgery among the Elderly.

Objective To compare comorbidities and in-hospital complications between elderly and nonelderly patients undergoing vestibular schwannoma (VS) surgery. To examine average length of stay (LOS) and hospital charges among elderly patients. Study Design Population-based inpatient registry analysis. Setting Academic medical center. Subjects and Methods Retrospective analysis of the National Inpatient Sample for patients undergoing VS surgery from 2002 to 2010: 4137 patients met inclusion criteria, with 519 (12.5%) in the elderly cohort (≥65 years). Outcomes of elderly and nonelderly (<65 years) patient cohorts were compared. Results Compared with the nonelderly cohort, the elderly cohort had more comorbidities, including diabetes mellitus, hypertension, and pulmonary disease (all P < .001). Elderly patients had longer LOS (6.5 vs 5.4 days; P = .001) but did not incur significantly greater hospital charges. Rates of cerebrospinal fluid leak, meningitis, and facial nerve injury did not vary significantly between groups. The elderly cohort experienced higher rates of in-hospital complications, including acute cardiac events, iatrogenic cerebrovascular infarction/hemorrhage, postoperative bleeding (hemorrhage/hematoma), and in-hospital mortality (all P < .05). In binary logistic regression, correcting for patient demographics and presence of comorbidities, elderly status was associated with 1.848 (95% confidence interval, 1.167-2.927; P = .009) greater odds of medical complications and 13.188 (95% confidence interval, 1.829-95.113; P = .011) greater odds of in-hospital mortality. Conclusion Elderly patients undergoing VS surgery have more comorbidities, in-hospital complications, and longer LOS than nonelderly patients. The elderly cohort had a greater rate of in-hospital mortality, though rare. Interestingly, elderly patients did not have a higher rate of many known complications associated with VS surgery and did not incur more hospital charges.

Implementation of Lifestyle Modification Program Focusing on Physical Activity and Dietary Habits in a Large Group, Community-Based Setting.

BACKGROUND: Lifestyle modification programs improve several health-related behaviors, including physical activity (PA) and nutrition. However, few of these programs have been expanded to impact a large number of individuals in one setting at one time. Therefore, the purpose of this study was to determine whether a PA- and nutrition-based lifestyle modification program could be effectively conducted using a large group format in a community-based setting. METHOD: One hundred twenty-one participants enrolled in a 16-week, community-based lifestyle modification program and separated in small teams of 13 to 17 individuals. Height, weight, fruit and vegetable (FAV) consumption, physical fitness, and several psychosocial measures were assessed before and after the program. RESULTS: Significant improvements in 6-minute walk distance (+68.3 m; p < .001), chair stands (+6.7 repetitions; p < .001), FAV servings (+1.8 servings/day; p < .001), body weight (-3.2 lbs; p < .001), as well as PA social support and eating habits self-efficacy were observed. Our lifestyle modification program was also successful in shifting participants to higher levels of stages of change for nutrition and PA, increasing overall levels of self-efficacy for healthy eating, and improving levels of social support for becoming more active. CONCLUSIONS: A lifestyle modification program can be successfully implemented in a community setting using a large group format to improve PA and FAV attitudes and behaviors.

Malignant otitis externa hospitalizations: Analysis of patient characteristics.

OBJECTIVES/HYPOTHESIS: Malignant otitis externa (MOE) is a rare disorder that is not well studied in the inpatient setting. The Nationwide Inpatient Sample (NIS) database was utilized to analyze characteristics and predischarge outcomes of hospitalized MOE patients. METHODS: MOE hospitalizations were identified in the 2002 to 2013 NIS. Patient demographics, length of hospital stay, hospital charges, concomitant diagnoses, treatment-related procedures, complications, and in-hospital mortality rates were examined, with comparisons made among age cohorts and between diabetes mellitus (DM) and non-DM groups. RESULTS: A total of 8,300 cases of inpatient MOE were identified, with elderly DM patients compromising 22.7% of cases. Compared to adults, elderly patients had more inpatient procedures, longer hospitalizations (6.0 vs. 4.3 days), higher hospital charges ($26,712 vs. $19,047) (all P < 0.001), greater odds of in-hospital complications, and in-hospital mortality (odds ratio 14.435, 95% confidence interval 5.313-39.220). Adult/elderly patients with DM had more comorbidities, longer hospital stays (5.5 vs. 4.0 days), and higher hospital charges ($25,118 vs. $17,039) (all P < 0.001) than non-DM patients. However, DM was not associated with greater in-hospital mortality rates (0.6% vs. 0.5%; P = 0.640). Compared to the adult/elderly cohort, pediatric patients had higher rates of nonelective admissions (19.8% vs. 14.5%), shorter hospital stays (2.9 vs. 4.9 days), and lower hospital charges ($8,876 vs. $21,672) (all P < 0.05). CONCLUSION: Elderly diabetic patients made up a smaller fraction of hospitalized MOE cases than anticipated. Elderly patients had greater in-hospital complications and mortality. Diabetes mellitus in adult/elderly patients was not associated with increased mortality. Pediatric patients fared well with low complications rates and no instances of in-hospital mortality. LEVEL OF EVIDENCE: 2C. Laryngoscope, 127:2328-2336, 2017.

A case report comparing clinical, imaging and neuropsychological assessment findings in twins discordant for the VCP p.R155C mutation.

Inclusion body myopathy, Paget disease of bone and/or frontotemporal dementia is an autosomal dominant disease caused by mutations in the Valosin Containing Protein (VCP) gene. We compared clinical findings including MRI images and neuropsychological assessment data in affected and unaffected twin brothers aged 56 years from a family with the p.R155C VCP gene mutation. The affected twin presented with a 10 year history of progressive proximal muscle weakness, difficulty swallowing, gastroesophageal reflux, fecal incontinence, and peripheral neuropathy. Comprehensive neuropsychological testing revealed rapid cognitive decline in the absence of any behavioral changes in a span of 1 year. This case illustrates that frontotemporal dementia related cognitive impairment may precede behavioral changes in VCP disease as compared with predominance of behavioral impairment reported in previous studies. Our findings suggest that there is a need to establish VCP disease specific tools and normative rates of decline to detect pre-clinical cognitive impairment among affected individuals.

Subjective and objective findings in patients with true dehiscence versus thin bone over the superior semicircular canal.

OBJECTIVE: To compare subjective and objective findings between patients with true dehiscence versus thin bone over the superior semicircular canal (SSC). STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: All patients from our institution with true dehiscence or thin bone over the SSC on computed tomography temporal bone (oblique view) from 2007 to 2013. MAIN OUTCOME MEASURES: Subjective test: Dizziness Handicap Inventory (DHI). Objective tests: Infrared video eye recording with varying stimuli (Tulio, Fistula, and Vibration); vestibular evoked myogenic potential (VEMP); electrocochleography; videonystagmography; pure-tone audiometry (i.e., air-bone gap). RESULTS: Fifty-four patients (64 ears) were reviewed. Thirty-nine patients (47 ears) had true dehiscence of the SSC on temporal bone computed tomography. Fifteen patients (17 ears) had thin bone over the SSC. There was no statistical difference in DHI scores for patients with true dehiscence versus those with thin bone over the SSC. Only cervical VEMP and air-bone gap via pure-tone audiometry revealed a significant difference between the two groups. The remaining vestibular assessments did not demonstrate any difference. No significant correlations were revealed between DHI and objective test findings across and within the two groups. CONCLUSION: Among the objective tests, cervical VEMP and pure-tone audiometry are the only tools to distinguish between true dehiscence and thin bone over the SSC. DHI does not differentiate between these two groups. Furthermore, no correlation exists between DHI and any objective finding. Further investigation is necessary to develop a validated subjective symptom index of patients with SSC syndrome.

The relationship of age and radiographic incidence of superior semicircular canal dehiscence in pediatric patients.

OBJECTIVE: To determine if age affects radiographic incidence of superior semicircular canal dehiscence (SSCD) in pediatric patients. STUDY DESIGN: Retrospective case review. SETTING: Tertiary children's hospital. PATIENTS: Patients (0-18 yr) with high-resolution computed tomography (CT) temporal bone scans from April 2001 to February 2013. INTERVENTIONS: Diagnostic high-resolution CT temporal bone scans. MAIN OUTCOME MEASURES: Findings of dehiscent, thin, or normal SSC on CT scans (including reconstructed Poschl views). Interobserver radiographic interpretation rate between neuroradiologist and otologist. RESULTS: Seven-hundred CT scans (1,400 ears) were reviewed, and 1,188 ears were acceptable for analysis. Twenty-three ears (1.9%) had dehiscent SSC, 185 ears (15.6%) had thin SSC, and 980 ears (82.5%) had normal SSC. Median ages of dehiscent, thin, and normal canals were 5, 7, and 9 years, respectively ( p < 0.05). As age increased, the incidence of dehiscent and thin SCC cases decreased; for example, dehiscent or thin canal existed in 51.4% of children less than 12 months, 17.5% of children between 1 and 2 years, 18.5% of children between 3 and 10 years, and 10.9% of children between 11 and 18 years. The κ value of agreement between neuroradiologist and otologist was 0.814, demonstrating a high value of agreement (p < 0.05). CONCLUSION: Radiographic SSCD, although uncommon, appears to be more prevalent in younger children, especially infants younger than 12 months. This suggests that the SSC may develop more bony covering with age.

Cochlear trajectory in pediatric patients.

OBJECTIVES/HYPOTHESIS: As cochlear implantation increases, surgeons are noting possible anatomical differences in pediatric population. Outcome objectives were to study pediatric temporal bone anatomy using high-resolution temporal bone imaging, and analyze the anatomical differences in group 1 (<12 months) versus group 2 (1-4 years) versus group 3 (5-10 years) versus group 4 (10-18 years). STUDY DESIGN: Retrospective chart and radiologic review. METHODS: A retrospective chart and radiologic review of pediatric patients undergoing high-resolution computer tomography of the temporal bones from April 2001 to February 2013 was conducted. Scans were reviewed to record the transmastoid angle and transcanal angle. RESULTS: Seven hundred fifty patients were identified. A total of 1,426 ears were reviewed. The age range was 8 days to 21 years. Of the patients, 57.0% (n = 407) were male. The patients were divided into four groups: group 1 (<12 months), group 2 (1-4 years), group 3 (5-10 years), and group 4 (10-18 years). The transmastoid angle was observed to have variability. Significant differences were observed between groups 2 and 3 (P = .0028) and groups 2 and 4 (P = .0432). Analysis on the transcanal angle was performed. Significant differences existed between age groups 1 and 3 (P = .0150), groups 1 and 4 (P = .0038), and groups 2 and 4 (P = .0358). CONCLUSIONS: Considerable variation exists in pediatric temporal bones. The largest difference in the transmastoid angle was seen in children aged 1 to 4 years. The largest variability in the transcanal angle is between the infant (<12 months) and children >4 years of age. These differences are surgically relevant for round window identification and facial nerve safety during cochlear implant surgery in infants.