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OBJECTIVE The middle fossa approach is an effective option for the treatment of small (Koos grade I and II) vestibular schwannomas (VSs) when the goal is hearing preservation. The authors evaluated the rates of hearing preservation and examined the factors associated with improved hearing outcomes after the middle fossa approach for VSs. METHODS In this retrospective, single-center cohort study evaluating the clinical outcomes after resection of small VSs using the middle fossa approach, consecutive adult patients (> 18 years) who underwent surgery between January 2000 and December 2021 were included. Clinical and imaging characteristics were analyzed, including baseline hearing status, duration of surgery, anesthetic parameters, and imaging characteristics of the surgically treated tumors. RESULTS Among the 131 included patients, 102 had valid and discoverable pre- and postoperative audiology assessments. The mean follow-up was 26 months (range 1–180 months). There were 85 patients with serviceable hearing preoperatively, defined as American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS) class A or B, of whom 78% retained class A or B hearing at the last follow-up. Binary logistic regression demonstrated that preoperative hearing AAO-HNS class (OR 0.19, 95% CI 0.05–0.77; p = 0.02), overlap between fundus and cochlea (OR 0.32, 95% CI 0.11–0.96; p = 0.04), and duration of anesthesia (OR 0.98, 95% CI 0.97–0.99; p = 0.03) were independent predictors of hearing outcomes. Additionally, 75% of patients with high diffusion-weighted imaging signal in the tumor (p = 0.009) and 67% of patients with the tumor originating at the modiolus of the cochlea (p = 0.004) had poor hearing outcomes. CONCLUSIONS The hearing preservation rates after microsurgical resection of small VSs using the middle fossa approach are high, with 78% of patients maintaining AAO-HNS class A or B hearing. Poor hearing status at baseline, longer duration of anesthesia, and large overlap between the fundus of the internal auditory canal and the cochlea were independently associated with unfavorable hearing outcomes. Imaging characteristics can be used to stratify patients’ risk of hearing loss.

To evaluate the outcomes of cochlear implantation (CI) in adults with preoperatively diagnosed cognitive impairment.Retrospective cohort study.Tertiary-care academic center.Adults undergoing CI with preexisting cognitive impairment.Cochlear implantation.1) Hearing improvement after CI; 2) morbidity and mortality associated with surgery.Eight patients met inclusion criteria with mean age 77.8 years (SD, 9.6 y) at time of implantation; 7 were included in subsequent analysis as one did not have speech recognition scores. Average preoperative MoCA cognitive score of 22.6 (SD, 3.9, ≤25 demonstrates cognitive impairment). Average follow-up was 29.0 months (SD, 33.3 mo). Two patients passed away at an average 58.0 months (SD, 31.1 mo) after surgery. Median preoperative pure tone average was 86.3 dB HL ( interquartile range 31.3 dB HL) compared with 33.8 dB HL (IQR 5.0 dB HL) postoperatively ( p =0.001). Median preoperative speech testing score (AzBio/HINT) was 21% (IQR, 24%) compared with 44% (IQR, 21%) postoperatively ( p =0.001). There were no observed surgical complications during the follow-up period.This study demonstrates that patients with cognitive impairment before CI can experience improved hearing, no increased risk of complications, and good longevity after CI. Further prospective studies are needed to further define the utility of CI in patients with impaired cognition.

Unlike the autosomal dominant inheritance of neurofibromatosis 2, there are no known inherited risk factors for sporadic, unilateral vestibular schwannoma (VS), which comprise most VS cases. The authors tested a hypothesis positing a genetic contribution to predisposition to these lesions by analyzing familial clustering of cases.Familial clustering of individuals with unilateral VS was analyzed in two independent genealogical resources with linked diagnosis data: the Veterans Health Administration Genealogy Resource and the Utah Population Database. Tests for excess relatedness, estimation of relative risks (RRs) in close and distant relatives, and identification of pedigrees with a significant excess of unilateral VS among descendants were performed.The average pairwise relatedness of the Veterans Health Administration Genealogy Resource VS cases significantly exceeded the expected relatedness ( p = 0.016), even when close relationships were ignored ( p = 0.002). RR for third- and fifth-degree relatives developing VS were significantly elevated (RR, 60.83; p = 0.0005; 95% confidence interval [CI], 7.37-219.73) and (RR, 11.88; p = 0.013; 95% CI, 1.44-42.90), respectively. No VS-affected first-, second-, or fourth-degree relatives were observed. In the Utah Population Database population, no first- or second-degree relatives with VS were observed. RR for fifth-degree relatives developing VS was significantly elevated (RR, 2.23; p = 0.009; 95% CI, 1.15-3.90).These results provide strong evidence for an inherited predisposition to sporadic, unilateral VS. This study exhibits the value of genealogical resources with linked medical data for examining hypotheses regarding inherited predisposition. The high-risk unilateral VS pedigrees identified in two independent resources provide a powerful means of pursuing predisposition gene identification.

Objectives This study aims to assess the safety and feasibility of achieving His-Purkinje conduction system pacing (HPCSP) in consecutive patients with atrioventricular block (AVB) and to describe the site of conduction block in patients with infranodal AVB. Background HPCSP has evolved as the preferred form of physiologic pacing. Left bundle branch area pacing (LBBAP) has emerged as an effective alternative to His bundle pacing (HBP). Methods Consecutive patients with AVB referred for pacemaker implantation were included in the study. HBP or LBBAP was attempted in all patients. Site of conduction block was identified as nodal or infranodal (intra-Hisian or infra-Hisian) AVB. Results HPCSP was attempted in 333 consecutive patients with AVB and was successful in 322 (97%) patients. HBP was achieved in 140 patients, LBBAP in 179 patients, and both in 3 patients. Site of conduction block was nodal in 55% and infranodal in 45% (intra-Hisian 89%; infra-Hisian 4%; indeterminate 7%). QRS duration at baseline was 111±27 versus 129±31 (P Conclusions HPCSP pacing was successfully performed in 97% of unselected patients with AVB irrespective of the site of conduction block. True infra-Hisian block (distal His-Purkinje conduction disease) is rare. HBP and LBBAP were complementary in achieving stable and low capture thresholds.

This review examines the relationship between cochlear implantation and cognition and quality of life in older adults, as well as how frailty affects outcomes for older patients with cochlear implants. A growing body of evidence suggests that there is a strong association between hearing loss and cognitive impairment. Preliminary studies suggest that cochlear implantation in older adults may be protective against cognitive decline. While studies have observed a positive impact of cochlear implantation on quality of life, currently it is unclear what factors contribute the most to improved quality of life. Frailty, as a measurement of general health, likely plays a role in complication rates and quality-of-life outcomes after cochlear implantation, though larger prospective studies are required to further elucidate this relationship.

Describe a series of cases of idiopathic chronic auricular chondritis refractory to antibiotics and steroids treated successfully with surgery.Case series.Two tertiary academic medical centers.We analyzed four patients diagnosed with chronic auricular deformity, pain, and drainage for a period of 1 to 5 years who had failed prolonged treatment consisting of antibiotics, corticosteroids, and incision and drainage. All four patients were smokers, three were diabetic, and two had a history of bariatric surgery.Operative subcutaneous partial auriculectomy (removal of diseased cartilage and excess skin) was performed.Resolution of pain and drainage, need for additional procedures, and reduction in narcotics required for pain control were analyzed.Two of the four patients were given an immediate postoperative course of doxycycline and ciprofloxacin. With a minimum of 6 weeks' follow-up, all four patients had complete resolution of pain and recurrent drainage postoperatively. One patient requiring daily narcotic medication for pain and benzodiazepine for sleep preoperatively no longer required prescription medication. All specimens revealed chronic dermal and cartilage inflammation. Three of four cases had polymicrobial infection. One case had only skin contaminant growth on culture following multiple oral and parental antibiotic regimens.Surgical excision of diseased cartilage as a result of idiopathic chronic chondritis is an effective treatment in those cases refractory to antibiotics and incision and drainage, and should be considered in the treatment algorithm for similar patients, potentially offering definitive cure.

Thrombocytopenia is a common entity seen in ICU patients and is associated with increased morbidity such as bleeding and transfusions, and mortality in ICU patients. Various mechanisms such as decreased platelet production, sequestration, destruction, consumption, and sometimes a combination of these factors contribute to thrombocytopenia. An understanding of the mechanism is essential to diagnose the cause of thrombocytopenia and to help provide appropriate management. The management strategies are aimed at treating the underlying disorder, such as platelet transfusion to treat complications like bleeding. Several studies have aimed to provide the threshold for platelet transfusions in various clinical settings and recommend a conservative approach in the appropriate scenario. In this review, we discuss various pathophysiological mechanisms of thrombocytopenia and the diverse scenarios of thrombocytopenia encountered in the ICU setting to shed light on the varied thresholds for platelet transfusion, alternative agents to platelet transfusion, and future directions for the implementation of thromboelastography (TEG) in multiple clinical scenarios to assist in the administration of appropriate blood products to correct coagulopathy.

To examine the relationship among frailty index, hearing measures, and hearing-related quality of life (QOL) in older recipients of cochlear implants.Cross-sectional survey.Academic medical center.Adults aged ≥65 years at the time of receiving cochlear implants between July 13, 2000, and April 3, 2019, were asked to complete a questionnaire on hearing-related QOL. Chart review was performed to identify patients' characteristics. Correlations were calculated between frailty index and audiologic outcome measures as well as between speech recognition scores and QOL scores. Linear regression models were developed to examine the impact of clinical characteristics, frailty index, and hearing measures on hearing-related QOL.Data for 143 respondents were included. The mean age was 80.7 years (SD, 7.1), with a mean 27.8 years of hearing loss (SD, 17.4) before implantation. The mean frailty index was 11.1 (SD, 10.6), indicating that patients had 1 or 2 of the measured comorbidities on average. No correlation was found between lower frailty index (better health) and hearing scores, including pure tone averages (PTAs) and speech recognition scores. Lower frailty index and larger improvement in PTA after cochlear implantation predicted better QOL scores on univariate analysis (respectively,Frailty index does not correlate with hearing scores after cochlear implantation in older adults. Lower frailty index and more improvement in PTA predict better QOL scores after cochlear implantation in older adults.

OBJECTIVE To evaluate adverse events and feasibility of performing 1.5-T MRI in patients with cochlear implants (CI) and auditory brainstem implants (ABI). SETTING Single tertiary academic referral center. PATIENTS CI and ABI recipients undergoing 1.5-T MRI without internal magnet removal. INTERVENTION(S) MRI after tight headwrap application. MAIN OUTCOME MEASURES Adverse events, patient tolerance. RESULTS A total of 131 MR studies in 79 patients were performed, with a total of 157 study ears. Sixty-one patients (77%) had unilateral devices. Four patients (5%) underwent MRI with ABI magnets in place. Sixteen patients (20%) had MRI-compatible devices that did not require a head wrap. There were no instances of device stimulation, device malfunction, or excessive heating of the receiver-stimulator package. Magnet tilt requiring manual repositioning occurred during seven studies (4.5%) and magnet displacement requiring operative intervention occurred during seven studies (4.5%). Significant pain where imaging had to be discontinued occurred during three episodes (2%). No adverse events were noted among patients who underwent MRI with an MRI-compatible magnet. CONCLUSIONS MRI with CI or ABI magnets in place is associated with a low prevalence of adverse events when performed in a controlled setting. Many partial magnet displacements can be corrected with firm manual pressure. Devices with magnets that align with the field within their housing were not associated with any adverse events and do not require immobilization of the magnet during the scan. These may be valuable in patients with known or anticipated need for MRI.

Electrode impedances play a critical role in cochlear implant programming. It has been previously shown that impedances rise during periods of non-use, such as the post-operative recovery period. Then when the device is activated and use is initiated, impedances fall and are typically stable. In this study, we report a new pattern where electrode impedances increase with device use and decrease with device rest.Electrode impedances were measured three to four times every day over a span of 1-3 months for two cochlear implant patients.Two patients with a Nucleus cochlear implant participated in this study.Both subjects in this study show wide fluctuations in electrode impedances. By taking serial electrode impedance measurements throughout a day of use, we observe that electrode impedances consistently increase with device use and decrease with device rest.In this study, we report two cases of electrode impedances increasing as a function of device use. Numerous management strategies were employed to reduce this effect but none prevailed; a clear pathophysiologic mechanism remains elusive. Further study into the cause of this electrode impedance pattern is warranted to establish a management strategy for these cochlear implant users.

Objective Intraoperative far-field auditory brainstem response (ABR) and direct cochlear nerve action potential monitoring using neural response imaging (NRI) are techniques for monitoring the cochlear nerve during vestibular schwannoma (VS) surgery. A new paradigm has developed where the cochlear nerve is anatomically preserved during tumor removal to facilitate cochlear implantation in select circumstances. This report describes the use of an unmodified commercial cochlear implant (CI) to elicit electrically-evoked direct cochlear nerve and far-field ABR monitoring to evaluate the status of the cochlear nerve during tumor resection in real time. Patients Adult female with unilateral, sporadic VS. Intervention(s) Cochlear implantation followed by translabyrinthine resection of VS in single operation. Main outcome measures During tumor resection intra-cochlear electrodes were used to deliver electrical stimulation and measure NRI or the cochlear nerve action potential. Electrically-evoked ABR (eABR) was measured using surface electrodes and wave V was monitored (far-field ABR) during surgery. Results A 61-year-old female was evaluated for a unilateral, enlarging intracanalicular VS with asymmetric SNHL. The patient opted for microsurgery and due to her bilateral hearing loss was a candidate for CI. Cochlear implantation with an unmodified, commercially available lateral wall electrode was performed. The remainder of the approach and tumor resection was performed under continuous eABR and NRI monitoring. Gross total resection was achieved with intact eABR and NRI at the conclusion of the case. Changes in ABR and NRI consistently recovered after modifying dissection strategy. The patient reported auditory percepts with her cochlear implant postoperatively. Conclusions This report demonstrates the feasibility of using real-time NRI and eABR with a CI to facilitate preservation of the cochlear nerve during VS microsurgery. Using this method to mitigate cochlear nerve trauma during microsurgery may preserve the option of CI for hearing rehabilitation.

OBJECTIVE To evaluate factors affecting quality of life (QOL) in caregivers of older cochlear implant (CI) recipients. STUDY DESIGN Cross-sectional survey. SETTING Academic medical center. PATIENTS Adults over age 65 receiving CI between July 13, 2000 and April 3, 2019. INTERVENTIONS Cochlear implantation. MAIN OUTCOME MEASURES Linear regression models for caregiver QOL measured by Significant Other Scale for Hearing Disability (SOS-HEAR), with independent variables: caregiver role, patient gender, 11 factor modified frailty index (mFI), duration of hearing loss, hearing aid use, age at surgery, time since surgery, change in pure tone average (PTA), processor input type and Nijmegen Cochlear Implant Questionnaire (NCIQ). Correlations between SOS-HEAR and patient speech recognition scores. RESULTS Questionnaires were mailed to all 294 living CI recipients. Seventy-one caregivers completed the questionnaire. Only patient gender and mFi were significant predictors of caregiver QOL on both univariate (p ≤ 0.001, β= -20.26 [95% confidence interval -30.21, -10.3]; 0.005, -0.72 [-1.20, -0.23], respectively) and multivariate (p = 0.005, β = -20.09, -33.05 to -7.13; 0.003, -0.93 [-1.50, -0.37]) analysis, where caregivers of female patients with lower mFI (better health) had better QOL scores. Caregiver QOL was significantly associated with patient's change in PTA and self-reported QOL scores on univariate (p = 0.041, β = 0.52 [0.08, 0.96]; 0.024, -0.27 [-0.52, -0.02]) but not multivariate analysis. Time since CI was significant only on multivariate analysis (0.041, -0.17 [-0.33, -0.01]). Caregiver QOL did not correlate with patient speech recognition scores. CONCLUSIONS Higher QOL scores were found among caregivers of healthier, female CI recipients. Patient hearing measurements did not correspond with better caregiver QOL.

To describe the presentation, diagnosis, and management of chronic otitis media recidivism after subtotal petrosectomy and ear canal closure (STP).Patients with temporal bone pathology detected during follow-up after STP were identified in the electronic medical record. Pertinent clinical details regarding surveillance plan, presentation, imaging findings, and revision surgery were collected and analyzed.A total of 10 patients were identified with recurrent or persistent pathology after STP. The median time to detection was 46 months (IQR 24-84). Five patients (50 %) had non-specific symptomatology, 4 patients (40 %) were completely asymptomatic, and 1 patient (10 %) was asymptomatic outside of two instances of mastoiditis with cochlear implant device infection treated with incision and drainage. One patient (10 %) was noted to have proptosis on examination, but no other patients had objective signs of disease at detection of disease recidivism. Nine (90 %) patients had pathology identified on preoperative imaging. All patients underwent revision surgery, with identification of cholesteatoma in 8 cases (80 %) and cholesterol granuloma in 2 cases (20 %). Extensive disease was noted in 6 patients (60 %), all of whom followed-up greater than 3 years from surgery. All patients tolerated revision surgery without complication.Recidivistic disease often remains clinically silent for extended periods of time after STP. Planned follow-up - with imaging or second look surgery - to facilitate early detection should be considered. Undetected disease recurrence or development may result in morbidity in a proportion of patients if surveillance is not performed.

Objective To evaluate whether frailty or age increases the risk of postoperative complications following cochlear implant (CI) surgery. Study Design Retrospective cohort study. Setting Tertiary academic center. Methods An evaluation of all adult patients undergoing cochlear implantation between 2006 and 2020 was performed. The 5-item Modified Frailty Index (mFI-5, comprising preoperative history of pulmonary disease, heart failure, hypertension, diabetes, and partially/totally dependent functional status) was calculated for all patients included in analysis in addition to demographic characteristics. The primary outcome was postoperative complications following CI within a 3-month period. Major complications included myocardial infarction, bleeding, and cerebrospinal fluid leak, among others. Predictors of postoperative complications were examined using multivariable logistic regression reporting odds ratios (ORs) and 95% CIs. Results There were 520 patients included for review with a median age of 68 (range, 18-94) years and a slight male predominance (n = 283, 54.4%). There were 340 patients (65.4%) who were robust (nonfrail) with an mFI of 0, while 180 (34.6%) had an mFI of ≥1. There were 20 patients who experienced a postoperative complication (3.85%). There was no statistically significant association between postoperative complications as a result of preoperative frailty (OR, 1.56; 95% CI, 0.98-2.48, P = .06) or age as a continuous variable (OR, 0.99; 95% CI, 0.97-1.02, P = .51). Conclusions CI is safe for elderly and frail patients and carries no additional risk of complications when compared to younger, healthier patients. While medical comorbidities should always be considered perioperatively, this study supports the notion that implantation is low risk in older, frail patients.

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.

Cardiovascular disease (CAD) due to atherosclerosis is a major cause of death worldwide. The development of atherosclerosis involves intercellular communication facilitated by exosomes secreted from vascular endothelial cells (VECs), vascular smooth muscle cells (VSMCs), immune cells, and platelets. In this review, we summarize the current understanding of exosome biogenesis and uptake, and discuss atherogenic and atheroprotective functions of exosomes secreted from these cell types. In addition, we examine the potential of enhancing the therapeutic and targeting ability of exosomes exhibiting atheroprotective function by drug loading and surface modification with targeting ligands. We conclude with current challenges associated with exosome engineering for therapeutic use.

Objective To ascertain long-term hearing outcomes in patients with serviceable hearing following microsurgical resection of sporadic vestibular schwannoma (VS). Study design Retrospective cohort. Setting Tertiary academic referral center. Patients Forty-three adult subjects with unilateral sporadic VS who had serviceable hearing (American Academy of Otolaryngology-Head and Neck Surgery [AAO-HNS] class A or B) on initial postoperative audiogram following microsurgical resection between 2003 and 2016 with a minimum of two postoperative audiograms available for review. Intervention Surgical treatment with a retrosigmoid or middle cranial fossa approach. Main outcome measure Rate of maintaining serviceable hearing, as estimated using the Kaplan-Meier method, in accordance with the 1995 and 2012 AAO-HNS guidelines on reporting hearing outcomes. Results The median immediate postoperative pure-tone average (PTA) and word recognition score (WRS) were 31 dB and 95%, respectively. At last follow-up, the median PTA was 38 dB with a median change of 5 dB from initial postoperative audiogram, and the median WRS was 90% with a median change of 0% from initial postoperative audiogram. Eight patients developed non-serviceable hearing at a median of 4.1 years following microsurgical resection (interquartile range, 2.9-7.0). The median duration of hearing follow-up for the 35 patients who maintained serviceable hearing was 3.1 years (interquartile range, 2.2-7.5). Tumor control was achieved in 41 (95%) patients. The rate of maintaining serviceable hearing at 5 years was 81%. Conclusion Microsurgical resection provides excellent tumor control and durable long-term hearing in those with AAO-HNS class A or B hearing postoperatively. The paradigm of proactive microsurgical resection-when the tumor is small and hearing is good-hinges on the surgeon's ability to preserve residual hearing in a very high percentage of cases at or near preoperative hearing levels to maintain an advantage over conservative observation with regard to long-term hearing preservation.

Objective Previous research has shown that tumor growth during observation of small-to-medium sized sporadic vestibular schwannomas (VSs) occurs almost exclusively within 3 to 5 years following diagnosis. This has led some to consider ending surveillance after this interval. This study seeks to characterize a cohort of patients with tumors that exhibited late growth. Study design Retrospective cohort study. Setting Tertiary referral center. Patients Adults with sporadic VSs who initially elected observation with serial magnetic resonance imaging (MRI) surveillance. Intervention(s) None. Main outcome measure(s) Linear tumor growth was measured in accordance with AAO-HNS reporting guidelines. Delayed growth was defined as growth ≥2 mm in linear diameter that was first detected 5 years or more from the initial MRI. Results From a total of 361 patients, 172 experienced tumor growth during the interval of observation. Fourteen of these 172 patients (8.1%) experienced late growth occurring at 5 years or beyond. Among patients with delayed growth, the fastest growth rate after extended quiescence was 1.33 mm/yr, and the longest delay before tumor growth detection was 11.1 years. Additional treatment was recommended for six (42.9%) of the patients with delayed growth. Of 68 tumors that remained in the IAC, 11 (16.2%) demonstrated delayed growth. Of 66 tumors that presented in the CPA, 2 (3.0%) demonstrated delayed growth. Initial size was larger for tumors demonstrating early growth compared with those with delayed growth. For tumors within the IAC, those with early growth had a significantly higher median growth rate than those with delayed growth (1.40 vs. 0.45 mm/yr, p Conclusions Delayed growth encompassed 8.1% of growing VSs and 3.9% of all observed tumors. Patients with delayed growth exhibited slower growth rates compared with those who were diagnosed with growth early in their observation course. These findings support the need for lifelong surveillance of untreated VSs given the possibility of clinically significant delayed growth. Increasing the time interval between MRI studies after 5 years is a reasonable concession to balance practicalities of cost and convenience with risk of delayed of tumor growth.

OBJECTIVE To evaluate patients who become symptomatic from superior semicircular canal dehiscence (SSCD) following head trauma. STUDY DESIGN Case series assessing patients presenting with SSCD after a trauma. METHODS A case series was completed assessing patients presenting with SSCD after trauma. Data from three academic medical centers were evaluated, including the following: imaging, videonystagmography (VNG)/vestibular evoked myogenic potential (VEMP) testing, audiometric assessment, and surgical repair. Outcome measures included the following: 1) Description of audio-vestibular symptoms, 2) mean pre- and post-operative pure tone average (PTA), word recognition score (WRS), and air bone gap (ABG). RESULTS A total of 14 patients were included; 86% were male. Approximately 43% were found to have bilateral SSCD on imaging, with 57% of patients pursuing surgical management. The most common presenting symptoms included pulsatile tinnitus (93%), autophony (79%), and hearing loss (64%). Approximately 36% of patients underwent VNG/VEMP testing, with 83.3% of those demonstrating abnormal results. The mean audiometric findings on the symptomatic side included an air-conduction PTA of 38.0 dB, bone-conduction PTA of 24.3 dB, WRS of 81%, and ABG of 17.9 dB. Among patients who underwent surgery (57%), there was no significant change in the air-conduction PTA, bone-conduction PTA, or WRS (P > .05). However, there was an improvement in the ABG (preoperative = 22.8 dB versus postoperative = 9.7 dB; P = .005). CONCLUSION Head trauma may be a potentiating event for SSCD syndrome. This study advances the hypothesis that these patients likely have underlying radiographic SSCD prior to their trauma, and a traumatic event increases in intra-vestibular or intracranial pressures, unmasking SSCD syndrome. LEVEL OF EVIDENCE 4 Laryngoscope, 131:E2810-E2818, 2021.

Objective To test the hypothesis that severe to profound preoperative hearing loss predicts less acute postoperative vestibulopathy following microsurgical removal of vestibular schwannoma (VS) allowing for earlier postoperative mobilization and hospital discharge. Methods Patients with VS who underwent microsurgery and were found to have preoperative severe to profound hearing loss (pure tone average [PTA] > 70 dB HL) were matched 1:1 by age and tumor size to a group of randomly selected controls with preoperative serviceable hearing. Results A total of 57 patients met inclusion criteria and were matched to controls. Median age at the time of microsurgery was 56 years. The median PTA and WRS for cases were 91 dB HL (interquartile range [IQR] 78–120) and 0% (IQR 0–0), respectively. Median tumor size was 14.2 mm (IQR 10.9–20.9). A total of 35 (61%) patients exhibited nystagmus after surgery associated with acute vestibular deafferentation. Median time to ambulation in the hallway was 2 days. Controls exhibited similar tumor size (12.7 mm, p = 0.11) and age (57 years, p = 0.52). Preoperative hearing loss did not predict severity or duration of postoperative nystagmus or days to discharge; however, those with Class D hearing exhibited a shorter time to ambulation (p = 0.04). Conclusion Following microsurgical removal of VS, preoperative profound hearing loss was associated with a shorter time to postoperative mobilization; however, there were no observed associations with duration or severity of nystagmus and time to hospital discharge. Although not a predictor of nystagmus, preoperative profound hearing loss may portend quicker recovery from clinically significant postoperative vestibulopathy.

The degree to which various treatment modalities modify vestibular schwannoma (VS)-associated symptoms has received limited attention. The purpose of this study was to determine how different treatment modalities affect subjective symptoms in those presenting with VS.Prospective survey.Tertiary neurotology referral center.Patients with sporadic VS who received treatment at our institution were prospectively surveyed with a VS symptom questionnaire. Those who completed a baseline survey prior to treatment and at least 1 posttreatment survey were included. The prospective survey evaluated the severity of self-reported symptoms (Likert scale, 1-10), including tinnitus, dizziness or imbalance, headaches, and hearing loss.A total of 244 patients were included (mean age, 57 years). The mean duration of follow-up was 2.1 years, and the median number of surveys completed was 2 (interquartile range, 1-3). Seventy-eight (32%) cases were managed with observation, 118 (48%) with microsurgery, and 48 (20%) with radiosurgery. Multivariable analyses revealed no statistically significant difference in the change in tinnitus (Limited prospective data exist regarding the progression or resolution of subjective symptoms in those presenting with VS. This study suggests that tinnitus, dizziness or imbalance, and headaches are unlikely to be significantly modified by treatment modality and generally should not be used to direct treatment choice.

Introduction: Adolescents with heart disease report difficulty in communication about their health as a major inhibiting factor in their care. MHealth technologies collect health data in daily life and enable health data sharing between the provider and patient. The adolescent population has a high level of engagement with mobile devices and a willingness to use them for health-related activities. Hypothesis: We hypothesized that our novel gamified mHealth platform Heart Hero can engage adolescent patients in the collection of cardiac health data in their daily life. Methods: We designed the research app using ResearchKit to collect continuous physiological data from the Apple Watch and daily survey data on well-being, stress, medical adherence, and cardiac symptoms. Patients were provided the app, iPhone, and Apple Watch and enrolled for 27 days. A final in-app survey was provided to assess feedback. We enrolled 28 patients total who were scheduled for outpatient cardiopulmonary exercise testing. Results: Mean age was 14.3 years old (SD +/-3.08) with a 1:1 M:F ratio. 61% of patients were ≥ 15 years of age. 94% of patients completed the final survey. Subjects on average completed 64% (SEM +/-5) of the daily quizzes with an average daily adherence of over 50% wearing the watch. 100% reported they liked using the watch and app, and 89% would like to continue wearing the Apple Watch. 53% reported the study encouraged them to exercise more while 21% reported encouragement to walk more. Fig 1 demonstrates A) Apple Watch and b) daily survey data collected from a patient throughout the study. Conclusions: In conclusion, Heart Hero is a mHealth platform which can successfully be used to collect continuous health data from the adolescent population with high engagement characterized by adherence and positive patient feedback. Adherence to the app was notably superior to the initial 5 ResearchKit applications enrolling adult patients.

Introduction: Transcatheter pulmonary valve implantation (TPVi) is a widely available option to treat right ventricular outflow tract (RVOT) conduits, but coronary artery compression is an absolute contraindication. Cardiac magnetic resonance (CMR) may evaluate coronary anatomy, but its utility in predicting coronary compression is not well established. Hypothesis: CMR prior to TPVi can accurately predict coronary compression risk. Methods: We analyzed all patients with a recent CMR (≤ 12 months) and attempted TPVi in an RVOT conduit at 9 centers from 2007-2016. A core lab reviewed all CMRs for the shortest orthogonal distance from a coronary artery to the conduit, the shortest distance from a coronary artery to the most stenotic area of the conduit, and subjective assessment of coronary compression risk. Receiver operating characteristic curve was used to determine optimal predictive distances. Univariate and independent associations of the distances and qualitative assessment with coronary compression were examined using logistic regression. Results: Of 231 patients (62% male, median age 19.0 years), TPVi was successful in 198 (86%); in 24 (10%) balloon testing (documented coronary compression or high risk) precluded implantation. Distance to the RV to PA conduit ≤ 2.1 mm (area under the curve [AUC] 0.70) and distance to most stenotic area ≤ 13.1 mm (AUC 0.69) predicted coronary compression (Table). Subjective assessment had the highest AUC (0.78), with 96% negative predictive value. Both distances and qualitative assessment remained independently associated with coronary compression when controlling for abnormal coronary anatomy. Conclusions: CMR can predict the risk of coronary compression during TPVi in RVOT conduits but cannot completely exclude the risk of coronary compression. CMR may assist in patient selection and counselling families prior to TPVi, although balloon testing prior to TPVi remains essential.

BACKGROUND Previous cross-sectional studies analyzing quality of life (QOL) outcomes in patients with sporadic vestibular schwannoma (VS) have shown surprisingly little difference among treatment modalities. To date, there is limited prospective QOL outcome data available comparing baseline to posttreatment scores. STUDY DESIGN Prospective longitudinal study using the disease-specific Penn Acoustic Neuroma Quality of Life (PANQOL) scale. SETTING Large academic skull base center. PATIENTS Patients diagnosed with unilateral VS who completed a baseline survey before treatment and at least one posttreatment survey. MAIN OUTCOME MEASURES Change in PANQOL scores from baseline to most recent survey. RESULTS A total of 244 patients were studied, including 78 (32%) who elected observation, 118 (48%) microsurgery, and 48 (20%) stereotactic radiosurgery. Patients who underwent microsurgery were younger (p

Intracranial extension of rhinosinusitis is rare in children. We report a 9-year-old immunocompetent boy with central precocious puberty and obstructive sleep apnea-hypopnea syndrome who developed an intracranial epidural abscess secondary to rhinosinusitis while on continuous positive airway pressure (CPAP) treatment. A retrospective review of the medical record and imaging studies was performed. MEDLINE and Cochrane databases were searched for reports of epidural abscess developing in patients receiving CPAP treatment or in patients with precocious puberty. Intracranial extension of frontal rhinosinusitis is more common during puberty probably because of the active growth of the frontal sinuses and their rich blood supply. Controlled studies show no increase with rhinosinusitis in adults on CPAP; no published studies assess intracranial extension of rhinosinusitis in CPAP use. Patients with unexplained, severe headache and fever following CPAP use may require neuroimaging (magnetic resonance imaging [MRI] / contrast computed tomography) to rule out intracranial extension of sinusitis.

To ascertain the relationship among vestibular schwannoma (VS) tumor volume, growth, and hearing loss.Retrospective cohort study.Single tertiary center.Adults with observed VS and serviceable hearing at diagnosis were included. The primary outcome was the development of nonserviceable hearing as estimated using the Kaplan-Meier method. Associations of tumor volume with baseline hearing were assessed using Spearman rank correlation coefficients. Associations of volume and growth with the development of nonserviceable hearing over time were assessed using Cox proportional hazards models and summarized with hazard ratios (HRs).Of 230 patients with VS and serviceable hearing at diagnosis, 213 had serial volumetric tumor data for analysis. Larger tumor volume at diagnosis was associated with increased pure-tone average (PTA) (Larger initial VS tumor volume was associated with poorer hearing at baseline. Larger initial tumor volume was also associated with the development of nonserviceable hearing during observation in a univariable setting; however, this association was not statistically significant after adjusting for baseline hearing status.

Objectives To describe transnasal Eustachian tube (ET) occlusion with a liquid embolic solution for lateral skull base cerebrospinal fluid (CSF) leaks. Design A lateral skull base CSF fistula model was developed by the authors using fresh cadaveric heads. Using a transtympanic needle, regulated pressurized pigmented saline was continuously instilled into the middle ear space and visualized endoscopically in the nasopharynx. An angioembolization catheter was then placed through the cartilaginous ET orifice just medial to the bony ET. Under endoscopic and fluoroscopic guidance, a column of liquid embolic agent was deployed into the bony ET segment up to the middle ear space. Setting Tertiary care academic center. Participants Cadaveric specimens. Main Outcome Measures Cessation of CSF flow after occlusion at supraphysiologic pressures. Results In two cadavers, a CSF fistula model was developed and endoscopic visualization of irrigant flow into the nasopharynx was confirmed. Fluoroscopy provided adequate anatomic views of the ET and middle ear, in addition to dynamic views of embolization. Cessation of flow after occlusion was successfully achieved with pressures up to 25 mm Hg, which exceeds normal physiological intracranial pressure. Conclusion Eustachian tube occlusion with a liquid embolic solution is feasible in a novel cadaveric CSF leak model. In the future, this relatively short, straightforward procedure may become an outpatient alternative to manage intermittent or low-flow CSF fistulae following lateral skull base surgery.

Objective To describe audiometric outcomes following stereotactic radiosurgery (SRS) for jugular paraganglioma (JP). Study design Retrospective review. Setting Tertiary referral center. Patients Patients with pretreatment serviceable hearing (American Academy of Otolaryngology-Head and Neck Surgery [AAO-HNS] Class A or B) and serial post-SRS audiometric follow-up who underwent Gamma Knife SRS for JP between 1990 and 2017. Intervention(s) Gamma Knife SRS. Main outcome measures Progression to nonserviceable hearing; correlation between baseline hearing and treatment parameters with audiometric outcomes. Results Of 85 patients with JP who underwent SRS during the study period, 35 (66% female, median age 53) had pretreatment serviceable hearing and serial post-treatment audiometry available for review. Median tumor volume at the time of treatment was 7,080 mm, median cochlear point dose was 5.8 Gy (interquartile range [IQR] 4.1 to 7.3 Gy), and median marginal and maximum tumor doses were 16 and 32 Gy, respectively. After a median follow-up of 37 months (IQR 16 to 77 mo), the median change in pure-tone average and speech discrimination score in the treated ear was -1.2 dB HL/yr (IQR -4.5 to 0.3) and 0%/yr (IQR 0-3.5%), compared with 0.07 dB HL/yr (IQR -0.03 to 0.12) and 0 %/yr (IQR 0 to 0%) in the contralateral untreated ear. Seven patients developed nonserviceable hearing (AAO-HNS Class C or D) at a median of 13.2 months following SRS (IQR 4.8 to 24 mo). Among those who maintained serviceable hearing, median audiometric follow-up was 42 months (IQR 18 to 77 mo). The Kaplan-Meier estimated rates of serviceable hearing at 1, 3, and 5 years following SRS were 91%, 80%, and 80%, respectively. Sixty percent of patients with pulsatile tinnitus who underwent SRS experienced varying levels of symptomatic improvement following treatment. Conclusion The short- and intermediate-term risk of progression to nonserviceable hearing following SRS for JP is low. Data regarding the impact of cochlear dose from the vestibular schwannoma literature should not be freely applied to JP, since the impact of SRS parameters on hearing preservation seems to be less significant.

Objective Report an association between congenital stapes footplate fixation (CSFF) and radiological absence of the pyramidal eminence and stapedial tendon. Patients Children and adults with intraoperatively confirmed CSFF and an absent stapedial tendon. Interventions Computed tomography (CT); exploratory tympanotomy with stapedotomy. Main outcome measures Absence of a pyramidal eminence and stapedial tendon aperture identified on preoperative CT that was confirmed intraoperatively. Results Eight patients with intraoperative confirmation of CSFF and absent stapedial tendon were retrospectively identified. The average preoperative bone conduction and air conduction pure tone averages were 19.6 dB (SD 15.6 dB) and 55.9 dB (SD 23.6 dB), respectively. The average air-bone gap was 36.3 dB (SD 17.9 dB) preoperatively. In the seven patients who underwent preoperative CT, all were consistently identified to have an absent or hypoplastic pyramidal eminence and absent stapedial tendon aperture at the pyramidal eminence. In six cases, the stapedial footplate appeared normal, while in one case the footplate appeared abnormal which correlated with severe facial nerve prolapse observed intraoperatively. All eight cases underwent exploratory tympanotomy and demonstrated intraoperative stapes footplate fixation, absent stapedial tendon and either absent or hypoplastic pyramidal eminence, which correlated with preoperative CT findings. Conclusions This study identifies a clinically pragmatic association between an absent pyramidal eminence identified on high-resolution CT and the diagnosis of CSFF. In a condition that otherwise generally lacks distinctive radiological features, the absence of a pyramidal eminence on CT in a patient with nonprogressive, congenital conductive hearing loss may strengthen clinical suspicion for CSFF.

Objective To define relationships between the frontal sinus opening, ostia of other frontal recess cells, and endoscopic landmarks and to develop a clinically useful framework to guide frontal sinus surgery. Study Design Retrospective review. Setting Tertiary care academic referral center. Methods Adult patients with computed tomography (CT) without sinonasal pathology were included. Virtual endoscopy (using OsiriX) and corresponding CT reconstructions were used to identify all visible ostia in the frontal recess and characterize their positions in spaces between the uncinate/agger nasi (U), bulla ethmoidalis (EB), and middle turbinate (MT). Results Two hundred sides in 100 patients (median age 51 years, 62% female) were analyzed. The "center" of each map was defined as the intersection of spaces between U, EB, and MT. The frontal sinus opening was in the "center" in 53% of frontal recesses, lateral to this position in 29%, and anterior in 11%. When the frontal sinus opening was at the "center," anterior ostia drained frontal Kuhn T cells in 51% and intersinus septal cells in 23%. The skull base attachment of the apical strut of the uncinate process demarcated medial and lateral within the space between U and EB, with the opening to the frontal sinus medial in 68% and lateral in 31%. Left-right asymmetry in frontal sinus openings was noted in 46% of patients. Conclusion Combining preoperative imaging and knowledge of these anatomic relationships may facilitate more efficient frontal outflow tract identification and instrumentation. This represents the first and largest description of ostial configurations relative to endoscopic structural landmarks.4.

Objective Describe the first case of cochlear implantation (CI) for auditory rehabilitation of a patient with craniometaphyseal dysplasia (CMD) and progressive mixed hearing loss. Patients A 65-year-old woman with known autosomal dominant CMD presented with progressive mixed hearing loss and declining benefit from conventional hearing aids. Computed tomography and magnetic resonance imaging revealed hyperostosis of the entire craniofacial skeleton. Hearing evaluation demonstrated pure-tone thresholds in the profound range bilaterally by air conduction, and bone conduction thresholds that matched aided thresholds for her left ear, though testing was somewhat limited by inability to mask at high air-conduction thresholds. CI candidacy testing confirmed poor word and sentence scores in the right ear. Intervention Due to the inability to access the cochlea via a conventional mastoidectomy and facial recess approach, cochlear implantation via a postauricular subtotal petrosectomy approach with ear canal overclosure was performed. Main outcome measures Post-implantation word and sentence testing. Results Despite extensive internal auditory canal stenosis, the patient demonstrated excellent early speech understanding results 5 weeks after device activation. Postimplantation audiologic evaluation showed thresholds between 20 and 30 dB HL from 250 to 6000 Hz. Word and sentence testing scores were 76% Consonant-nucleus-consonant in quiet (up from 2% preoperatively) and 77% AzBio sentences in quiet (up from 10% preoperatively). Conclusion This report describes the first description of CI for CMD. Despite the extensive radiologic abnormalities, the patient has demonstrated excellent benefit from implantation. Further study of rare temporal bone dysplasias, such as CMD, is critical to better characterize the progression of otologic disease and determine optimal treatment.

OBJECTIVE The etiology of sensorineural hearing loss (SNHL) in patients with jugular paraganglioma (JP) whose tumors lack inner ear fistulae or vestibulocochlear nerve involvement is unknown. Recent literature has proposed that occlusion of the inferior cochlear vein may be causative. Herein, we assess the association between radiologic involvement of the cochlear aqueduct (CA) and the development of SNHL. STUDY DESIGN Blinded, retrospective review of imaging and audiometry. SETTING Tertiary center. PATIENTS Adults with JP. INTERVENTION(S) None. MAIN OUTCOME MEASURES Asymmetric SNHL was assessed continuously as the difference in bone conduction pure-tone average (BCPTA) between ears and as a categorical variable (≥15 dB difference at two consecutive frequencies, or a difference in speech discrimination score of ≥15%). Involvement of the CA was considered present if there was evidence of medial T2 fluid signal loss, contrast enhancement, or bony erosion/expansion. RESULTS Of 30 patients meeting inclusion criteria, 15 (50%) had asymmetric SNHL. CA involvement was observed in 87% of patients with asymmetric SNHL compared with 13% in those with symmetric hearing (p = 0.0001). Univariate analysis demonstrated that age, sex, and tumor volume were not associated with asymmetric SNHL. The median difference in BCPTA between ears in patients with CA involvement was 21.3 dB HL compared to 1.2 dB HL in those without CA involvement (p

The treatment paradigm for jugular paraganglioma (JP) has changed considerably over time with the wider adoption of stereotactic radiosurgery (SRS). To the best of the authors' knowledge, there are no published studies that use validated patient-reported outcome measures to ascertain quality of life (QoL) outcomes following SRS for JP when used in single or combined modality treatment regimens.Cross-sectional survey.Tertiary referral center.Adult patients with JP treated with primary SRS or SRS following primary surgery between 1990 and 2017.Surgery and/or Gamma Knife SRS.Global and treatment-related QoL and differences in QoL based on treatment approach.Sixty-nine surveys were distributed and a total of 26 completed surveys were received (38% response rate). Among respondents, the median age at SRS was 53 years and 16 of the 26 patients (62%) were female. Median follow-up was 97 months. Nineteen patients (73%) were treated with primary SRS or staged SRS following intentional subtotal resection (STR; hereafter referred to as "staged SRS"), while the remainder (n = 7, 27%) were treated with SRS for recurrent JP. Median physical and mental health QoL PROMIS-10 T-scores regardless of treatment strategy were 39.8 and 38.8, respectively, while median SF36 physical and mental component subscores were similar to national averages and non-tumor controls. When comparing general physical and mental health QoL scores, there was no significant difference between patients treated with primary or staged SRS and those treated with SRS for recurrent JP. However, age-adjusted swallowing function among patients treated with primary or staged SRS was better than in those patients treated with SRS for recurrent JP (p = 0.05).Patients treated with primary or staged SRS for JP tend to exhibit better swallowing outcomes than those treated with SRS for recurrent JP. However, the majority of overall and disease-specific quality of life measures were not different between groups. Based on the low incidence of new cranial neuropathy following SRS, it is likely that initial surgical morbidity is the primary contributor to this outcome. Disease-specific overall quality of life measures, akin to those already used for other benign skull base tumors, are necessary to better gauge physical and mental health outcomes following treatment for JP. Though limited by small sample size, this represents the first study to gauge QoL outcomes following treatment for JP.

INTRODUCTION AND OBJECTIVES:Opioid pain medications are overprescribed, but little data is available to appropriately tailor post-discharge opioid prescriptions after urologic surgery. The Opioid R...

Objectives To present a rare case of traumatic facial neuroma involving the geniculate ganglion and review relevant literature. Patient Thirty-year-old man. Intervention Microsurgical resection via combined mastoid-middle fossa approach with great auricular nerve interpositional graft. Main Outcome Measures Patient demographics and pre- and postoperative facial nerve function. Results A 30-year-old man with a reported history of prior Bell's palsy developed progressive complete (House–Brackmann VI) right facial paralysis following blunt trauma. Imaging was strongly suggestive of a geniculate ganglion hemangioma. As the patient had no spontaneous improvement in his poor facial function over the course of 9 months, he underwent resection of the facial nerve lesion with great auricular nerve graft interposition via a combined mastoid-middle fossa approach. Histopathology demonstrated disorganized fascicles, with axonal clustering reminiscent of sprouting/regeneration following trauma. No cellular proliferation or vascular malformation was present. Conclusion Traumatic facial nerve neuromas can occur following temporal bone trauma and can closely mimic primary facial nerve tumors. Akin to the management of geniculate ganglion hemangioma and schwannoma, preoperative facial function largely dictates if and when surgery should be pursued.

Trigeminal nerve schwannomas (TNSs) are rare lesions that typically present with symptoms of trigeminal neuropathy or other cranial nerve palsies. These lesions classically have a dumbbell shape, with an anterior component within Meckel's cave and posterior component extending into the posterior fossa through the porus trigeminus. Surgical resection of TNSs can often be achieved via an extradural subtemporal approach to Meckel's cave without an anterior petrousectomy, even for tumors with a significant posterior fossa component, as the tumor often erodes a portion of the petrous apex.1 We present the case of a 53-yr-old female presenting to our institution with complete trigeminal neuropathy secondary to a right-sided, previously resected and radiated TNS. Serial imaging demonstrated an interval growth of significant residual tumor despite multiple adjuvant therapies, and, thus, the patient was recommended to undergo additional surgical resection. The lesion was approached through a right-sided subtemporal approach to Meckel's cave,2 with a plan to utilize an anterior petrousectomy only if difficulty resecting the posterior fossa component of the tumor was encountered. Intraoperatively, the posterior fossa component was found to be densely adherent to the adjacent brainstem, likely secondary to prior surgery and radiation therapy, and, thus, an anterior petrousectomy was performed. Postoperatively, the patient had stable trigeminal neuropathy without any new neurological deficits and a magnetic resonance imaging (MRI) confirmed a gross total resection. In the accompanying video, we hope to demonstrate the steps and nuances of both the subtemporal approach to accessing Meckel's cave and anterior petrousectomy when employed for the resection of TNSs. The patient in question provided formal consent for the making of this video.