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In the senescence-accelerated mouse prone 8 (SAMP8) mouse model, oxidative stress leads to premature senescence and age-related hearing impairment (ARHI). CMS121 inhibits oxytosis/ferroptosis by targeting fatty acid synthase. The aim of our study was to determine whether CMS121 is protective against ARHI in SAMP8 mice. Auditory brainstem responses (ABRs) were used to assess baseline hearing in sixteen 4-week-old female SAMP8 mice, which were divided into two cohorts. The control group was fed a vehicle diet, while the experimental group was fed a diet containing CMS121. ABRs were measured until 13 weeks of age. Cochlear immunohistochemistry was performed to analyze the number of paired ribbon-receptor synapses per inner hair cell (IHC). Descriptive statistics are provided with mean ± SEM. Two-sample t-tests were performed to compare hearing thresholds and paired synapse count across the two groups, with alpha = 0.05. Baseline hearing thresholds in the control group were statistically similar to those of the CMS121 group. At 13 weeks of age, the control group had significantly worse hearing thresholds at 12 kHz (56.5 vs. 39.8, p = 0.044) and 16 kHz (64.8 vs. 43.8, p = 0.040) compared to the CMS121 group. Immunohistochemistry showed a significantly lower synapse count per IHC in the control group (15.7) compared to the CMS121 group (18.4), p = 0.014. Our study shows a significant reduction in ABR threshold shifts and increased preservation of IHC ribbon synapses in the mid-range frequencies among mice treated with CMS121 compared to untreated mice.

Predict hearing preservation after middle cranial fossa approach for vestibular schwannomas.Application of machine learning algorithms, including classification and regression trees and random forest models to observational data.Single-tertiary referral center.Patients (n = 144) with a previously untreated sporadic vestibular schwannoma who underwent microsurgical resection by middle cranial fossa approach between November 2017 and November 2021.Middle cranial fossa approach.Hearing preservation, defined by postoperative word recognition score of 50% or greater and pure tone average below 50 dB HL or less than 10% reduction in word recognition score. Model performance was evaluated with classification accuracy in an independent validation sample. Variable importance for the random forest model is reported according to entropy, a measure of mean decrease in model accuracy incurred by excluding each variable from the model.Hearing preservation was achieved in 60% of patients (86 of 144) overall. The classification and regression tree model identified preoperative pure tone average with a cut point of 30 dB HL, and more posterior tumor position to be the most important prognostic features for hearing preservation. Model accuracy was 0.68. The random forest model demonstrated perfect accuracy (1). Baseline pure tone average, word recognition score, and anteroposterior tumor position were among the most influential features for hearing preservation prediction.Machine learning algorithms have the potential for accurate prediction of hearing preservation rates after middle fossa approach for vestibular schwannomas at a single institution. These models have the capacity for continued refinement with ongoing addition of data.

Purpose: High-volume hospitals are associated with improved surgical outcomes for acoustic neuromas (ANs). Due to the benign and slow-growing nature of ANs, many patients travel to geographically distant cities, states, or countries for their treatment. However, the impact of travel burden to high-volume centers, as well as its relative benefit are poorly understood. We compared post-operative outcomes between AN patients that underwent treatment at local, low-volume hospitals with those that traveled long distances to high-volume hospitals. Methods: The National Cancer Database was used to analyze AN patients that underwent surgery (2004–2015). Patients in the lowest quartile of travel distance and volume (Short-travel/Low-Volume: STLV) were compared to patients in the highest quartile of travel distance and volume (Long-travel/High-Volume: LTHV). Only STLV and LTHV cases were included for analysis. Results: Of 13,370 cases, 2,408 met inclusion criteria. STLV patients (n = 1,305) traveled a median of 6 miles (Interquartile range [IQR] 3-9) to low-volume centers (median 2, IQR 1-3 annual cases) and LTHV patients (n = 1,103) traveled a median of 143 miles [IQR 103-230, maximum 4,797] to high-volume centers (median 34, IQR 28–42 annual cases). LTHV patients had lower Charlson/Deyo scores (p=0.001), mostly received care at academic centers (81.7% vs 39.4%, pConclusion: Although traveling farther to high-volume centers increased the time between diagnosis and treatment for AN patients, they experienced superior postoperative outcomes compared to patients who received treatment locally at low-volume centers. Enabling access and travel to high-volume centers may improve AN patient outcomes.

Objective Vestibular schwannoma (VS) are benign, often slow growing neoplasms. Some institutions opt for radiosurgery in symptomatic patients of advanced age versus surgical resection. The aim of the study is to analyze surgical outcomes of VS in patients over the age of 65 who were either not candidates for or refused radiosurgery. Methods This includes retrospective analysis of VS patients between 1988 and 2020. Demographics, tumor characteristics, surgical records, and clinical outcomes were recorded. Patient preference for surgery over radiosurgery was recorded in the event that patients were offered both. Facial nerve outcomes were quantified using House-Brackmann (HB) scores. Tumor growth was defined by increase in size of >2 mm. Results In total, 64 patients were included of average age 72.4 years (65–84 years). Average maximum tumor diameter was 29 mm (13–55 mm). Forty-five patients were offered surgery or GKRS, and chose surgery commonly due to radiation aversion (48.4%). Gross total resection was achieved in 39.1% (n = 25), near total 32.8% (n = 21), and subtotal 28.1% (n = 18). Average hospitalization was 5 days [2–17] with 75% (n = 48) discharged home. Postoperative HB scores were good (HB1–2) in 43.8%, moderate (HB3–4) in 32.8%, and poor (HB5–6) in 23.4%. HB scores improved to good in 51.6%, moderate in 31.3%, and remained poor in 17.1%, marking a rate of facial nerve improvement of 10.9%. Tumor control was achieved in 95.3% of cases at an average follow-up time of 37.8 months. Conclusion VS resection can be safely performed in patients over the age of 65. Advanced age should not preclude a symptomatic VS patient from being considered for surgical resection.

OBJECTIVE Analyze delayed facial nerve palsy (DFNP) following resection of vestibular schwannoma (VS) to describe distinct characteristics and facial nerve (FN) functional course. STUDY DESIGN Prospective cohort with retrospective review. SETTING Academic medical center. PATIENTS Consecutive patients undergoing VS resection 11/2017 to 08/2020. Exclusion criteria: preoperative House-Brackmann (HB) ≥ III, postoperative HB ≥ III without delayed palsy

Genome-wide association studies (GWAS) provide an unbiased first look at genetic loci involved in aging and noise-induced sensorineural hearing loss and tinnitus. The hearing phenotype, whether audiogram-based or self-report, is regressed against genotyped information at representative single nucleotide polymorphisms (SNPs) across the genome. Findings include the fact that both hearing loss and tinnitus are polygenic disorders, with up to thousands of genes, each of effect size of < 0.02. Smaller human GWAS’ were able to use objective measures and identified a few loci, however, hundreds of thousands of participants have been required for the statistical power to identify significant variants, and GWAS is unable to assess rare variants with mean allele frequency < 1%. Animal studies are required as well because of inability to access the human cochlea. Mouse GWAS builds on linkage techniques and the known phenotypic differences in auditory function between inbred strains. With the advantage that the laboratory environment can be controlled for noise and aging, the Hybrid Mouse Diversity Panel (HDMP) combines 100 strains sequenced at high resolution. Lift-over regions between mice and humans have identified over 17,000 homologous genes. Since most significant SNPs are either intergenic or in introns, and binding sites between species are poorly preserved between species, expression quantitative trait locus information is required to bring humans and mice into agreement. Transcriptome-wide analysis studies (TWAS) can prioritize putative causal genes and tissues. Diverse species, each making a distinct contribution, carry a synergistic advantage in the quest for treatment and ultimate cure of sensorineural hearing difficulties.

Objective Examine the impact of preoperative cochlear Fast Imaging Employing Steady-state Acquisition (FIESTA) and Constructive Interference in Steady State (CISS) signal intensity on hearing outcomes after middle cranial fossa (MCF) resection of acoustic neuroma (AN). Methods Adult patients (≥18 years) who underwent MCF AN resection for hearing preservation (HP) between November 2017 and September 2019 were retrospectively reviewed. All patients had preoperative word recognition score (WRS) ≥50%. HP was defined as postoperative WRS ≥50%. A neuroradiologist blinded to patients' clinical and audiometric outcomes reviewed patients' preoperative magnetic resonance images. Ipsilateral-to-contralateral cochlear FIESTA/CISS signal intensity ratios were determined using hand-drawn regions of interest focused on the cochlear fluid. Preoperative and postoperative pure tone average (PTA) and WRS were reviewed. Results Fifty-one patients were reviewed (60.8% female). Mean age was 47 years and mean tumor size 9.2 mm (±3.8). Hearing was preserved in 56.9% (n = 29). FIESTA/CISS signal ratios did not significantly differ between patients with and without HP. Lower FIESTA/CISS signal ratios correlated with greater declines in hearing (r = 0.322, p = 0.011 for PTA; and r = 0.384, p = 0.004 for WRS). On multivariate analysis accounting for tumor size and preoperative PTA/WRS, decreases in FIESTA/CISS signal ratios independently predicted greater declines in hearing by PTA (b = -100.9, p = 0.012) and WRS (b = 76.208), although the latter result was not statistically significant (p = 0.078). Conclusions Cochlear FIESTA/CISS signal intensity may be a predictor of postoperative hearing loss after MCF AN resection. In this cohort, degraded preoperative cochlear FIESTA/CISS signal strongly predicted postoperative hearing loss.Level of Evidence: IV.

Age-related hearing loss (ARHL), or presbycusis, is one of the most prevalent conditions affecting the global population. A substantial fraction of patients with ARHL have no identifiable mutation despite over a hundred having been discovered, suggesting unidentified monogenic or polygenic causes. In this study, we investigated the hearing function of the aging outbred CFW mice through auditory brainstem response (ABR) thresholds. Through the characterization of 1,132 ABRs, we observed significant variation in both absolute thresholds and the effect of aging. We identify eight distinct patterns of hearing loss and were able to categorize nearly all data within these eight categories. Proportions within each category varied immensely between aging timepoints. We observe a small but consistent hearing deficit in female CFW mice. The resulting phenotypic data are a necessity for ARHL association mapping at a higher resolution than has previously been achieved and provides a new resource for studying ARHL.

Vestibular schwannomas (VS) are benign neoplasms that arise from the Schwann cells enveloping the vestibular division of the eighth cranial nerve. These tumors typically exhibit indolent growth and commonly cause audiovestibular dysfunction. If allowed to grow large enough, VS can cause brainstem compression and become fatal. Treatment options include observation with serial imaging, radiotherapy, and microsurgical resection. This review will summarize the literature regarding treatment outcomes for patients with small VS. Advances in diagnostic imaging have allowed for earlier and more frequent detection of small VS. Management of small VS remains controversial and is influenced by physician preference. Recent studies have summarized outcomes in small VS in conservative, radiosurgery, and microsurgery cohorts. Additional data on quality of life, volumetry, and morphometry allow for more informed decision-making in the treatment of small VS. In this article, we review contemporary literature describing outcomes of each treatment modality, with emphasis on tumor control, facial nerve function, hearing preservation, and quality of life.

ImportanceHigh surgical vestibular schwannoma case volume in a medical institution may decrease the risk of adverse outcomes among patients undergoing vestibular schwannoma surgery.ObjectiveTo study the association between surgical vestibular schwannoma case volume and excess time in the hospital after vestibular schwannoma surgery.Design, Setting, and ParticipantsThis cohort study evaluated data from the National Cancer Database from January 1, 2004, through December 31, 2019, on Commission on Cancer–accredited facilities in the US. The hospital-based sample comprised adult patients aged 18 years or older with a vestibular schwannoma treated with surgery.ExposuresFacility case volume, defined as the mean number of surgical vestibular schwannoma cases per year in the 2 years preceding the index case.Main Outcomes and MeasuresThe primary outcome was a composite of prolonged hospital stay (>90th percentile) or 30-day readmission. Risk-adjusted restricted cubic splines were used to model the probability of the outcome according to facility volume. The inflection point (in cases per year) when the declining risk of excess time in the hospital began to plateau was selected as the threshold to define high- and low-volume facilities. Outcomes were compared among patients treated at high- and low-volume facilities, with mixed-effects logistic regression models adjusting for patient sociodemographic characteristics, comorbidities, tumor size, and clustering within facilities. Collected data were analyzed between June 24 and August 31, 2022.ResultsAmong 11 524 eligible patients (mean [SD] age, 50.2 [12.8] years; 53.5% female; 46.5% male) who underwent surgical resection of vestibular schwannoma at 66 reporting facilities, the median length of stay was 4 (IQR, 3-5) days, and 655 patients (5.7%) were readmitted within 30 days. The median case volume was 16 (IQR, 9-26) cases per year. An adjusted restricted cubic spline model identified a downtrending probability of excess time in the hospital with increasing volume. The declining risk of excess time in the hospital began to plateau at a facility volume of 25 cases per year. Surgery at a facility with an annual case volume at or above this threshold was independently associated with a 42% reduction in the odds of excess time in the hospital compared with surgery at a low-volume center (odds ratio, 0.58; 95% CI, 0.44-0.77).Conclusions and RelevanceThis cohort study found that among adults undergoing vestibular schwannoma surgery, a higher facility case volume was associated with a reduced risk of prolonged hospital stay or 30-day readmission. A facility case volume of 25 cases per year may represent a risk-defining threshold.

Objectives To analyze the relationship between preoperative and intraoperative auditory brain stem response (ABR) characteristics and hearing outcomes in patients with vestibular schwannomas (VS) undergoing hearing preservation (HP) surgery via a middle cranial fossa (MCF) approach. Design Prospective study. Setting Academic tertiary skull base referral center. Methods Pre- and postoperative pure-tone average (PTA) and word recognition score (WRS) were examined. Intraoperative ABR wave III latency, wave V latency, and amplitude were recorded. HP was defined as postoperative WRS ≥50%. Participants Adult patients with VS and WRS ≥50% who underwent MCF tumor resection between November 2017 and September 2019. Main Outcome Measures Postoperative hearing outcomes. Results Sixty patients were included. Mean tumor size was 9.2 mm (range, 3–17). HP rates were 56.7% for the cohort and 69.7% for tumors Conclusion Of the examined preoperative and intraoperative ABR characteristics, a stable wave V intraoperatively was the strongest predictor of HP after MCF resection of VS. Level of Evidence Level III.

Pediatric cerebellopontine angle (CPA) meningiomas are extremely rare and are usually treated with a retrosigmoid surgical approach or radiation. The authors present the use of a middle fossa approach for the treatment of a symptomatic CPA meningioma in a 22-month-old female. The patient initially presented at 17 months with isolated progressive, long-standing right-sided facial weakness. MRI demonstrated a 5.0 × 5.0–mm right CPA lesion just superior to the cisternal segment of cranial nerve (CN) VII, which demonstrated growth on interval imaging. At 22 months of age she underwent a successful middle fossa craniotomy, including wide exposure of the porus acusticus, allowing for a gross-total resection with preservation of CNs VII and VIII. Pathological analysis revealed a WHO grade I meningioma. The patient remained neurologically stable on follow-up. The middle fossa approach can be used to safely access the CPA in properly selected pediatric patients.

To examine whether simple mobility assessments can predict functional limitations and length of hospitalization after acoustic neuroma (AN) resection.Prospective case series.A prospective clinical study of adult patients undergoing AN resection by either the translabyrinthine, retrosigmoid, or middle fossa approach was conducted at a tertiary center. Preoperative mobility assessments included the functional gait assessment (FGA) and the 10-m walk (10 MW). Postoperatively, the Activity Measure for Post-Acute Care (AMPAC, at 48 hours), FGA, and 10 MW (at 1 week) were obtained. Demographic and medical data were collected.One hundred and thirty-eight patients were analyzed (mean age: 48.3 years, 68.8% female). Mean length of stay (LOS) was 3.1 days. The translabyrinthine approach was most commonly performed (48.6%). On regression analyses, preoperative FGA (P = 0.03) and 48-hour postoperative AM-PAC (P 0.001) independently predicted LOS, even after accounting for age, gender, body mass index, and tumor size. On receiver operating characteristic analysis, a preoperative FGA cut score of 25.5 predicted a protracted hospital stay (4 days) with a sensitivity of 77% and specificity of 50% (area under curve: 68.5).This study demonstrated that preoperative mobility assessments can predict functional limitations and LOS after AN resection. These objective tools can be used by clinicians to manage expectations and guide preoperative counseling in patients considering surgery.3 Laryngoscope, 131:644-648, 2021.

This is the first genome-wide association study with the Hybrid Mouse Diversity Panel (HDMP) to define the genetic landscape of the variation in the suprathreshold wave 1 amplitude of the auditory brainstem response (ABR) both pre- and post-noise exposure. This measure is correlated with the density of the auditory neurons (AN) and/or the compliment of synaptic ribbons within the inner hair cells of the mouse cochlea. We analyzed suprathreshold ABR for 635 mice from 102 HMDP strains pre- and post-noise exposure (108 dB 10 kHz octave band noise exposure for 2 h) using auditory brainstem response (ABR) wave 1 suprathreshold amplitudes as part of a large survey (Myint et al., Hear Res 332:113–120, 2016). Genome-wide significance levels for pre- and post-exposure wave 1 amplitude across the HMDP were performed using FaST-LMM. Synaptic ribbon counts (Ctbp2 and mGluR2) were analyzed for the extreme strains within the HMDP. ABR wave 1 amplitude varied across all strains of the HMDP with differences ranging between 2.42 and 3.82-fold pre-exposure and between 2.43 and 7.5-fold post-exposure with several tone burst stimuli (4 kHz, 8 kHz, 12 kHz, 16 kHz, 24 kHz, and 32 kHz). Immunolabeling of paired synaptic ribbons and glutamate receptors of strains with the highest and lowest wave 1 values pre- and post-exposure revealed significant differences in functional synaptic ribbon counts. Genome-wide association analysis identified genome-wide significant threshold associations on chromosome 3 (24 kHz; JAX00105429; p

Objective A small subset of children with congenital hearing loss have abnormal cochleovestibular nerves (i.e., absent, aplastic, or deficient cochlear nerves), with largely unknown etiology. Our objective was to investigate the underlying pathways and identify novel genetic variants responsible for cochleovestibular malformations and nerve abnormalities. It is our hypothesis that several cochleovestibular nerve abnormalities might share common causative pathways. Design We used a family-based exome sequencing approach to study 12 children with known rare inner ear and/or cochleovestibular nerve malformations. Results Our results highlight a diverse molecular etiology and suggest that genes important in the developing otic vesicle and cranial neural crest, e.g., MASP1, GREB1L, SIX1, TAF1, are likely to underlie inner ear and/or cochleovestibular nerve malformations. Conclusions We show that several cochleovestibular nerve malformations are neurocristopathies, which is consistent with the fact that cochleovestibular nerve development is based on otic placode-derived neurons in close association with neural crest-derived glia cells. In addition, we suggest potential genetic markers for more severely affected phenotypes, which may help prognosticate individual cochlear implantation outcomes. Developing better strategies for identifying which children with abnormal nerves will benefit from a cochlear implantation is crucial, as outcomes are usually far less robust and extremely variable in this population, and current neuroimaging and electrophysiologic parameters cannot accurately predict outcomes. Identification of a suitable treatment early will reduce the use of multiple interventions during the time-sensitive period for language development.

Numerous shown consequences of age-related hearing loss have been unveiled; however, the relationship of the cortical and subcortical structures of the auditory pathway with aging is not well known. Investigations into neural structure analysis remain sparse due to difficulties of doing so in animal models; however, recent technological advances have been able to achieve a resolution adequate to perform such studies even in the small mouse. We utilize 12 members of the BXD family of recombinant inbred mice and aged separate cohorts. Utilizing novel magnetic resonance histology imaging techniques, we imaged these mice and generated high spatial resolution three dimensional images which were then comprehensively labeled. We completed volumetric analysis of 12 separate regions of interest specific to the auditory pathway brainstem nuclei and cortical areas with focus on the effect of aging upon said structures. Our results showed significant interstrain variation in the age-related effect on structure volume supporting a genetic influence in this interaction. Through multivariable modeling, we observed heterogenous effects of aging between different structures. Six of the 12 regions of interests demonstrated a significant age-related effect. The auditory cortex and ventral cochlear nucleus were found to decrease in volume with age, while the medial division of the medial geniculate nucleus, lateral lemniscus and its nucleus, and the inferior colliculus increased in size with age. Additionally, no sex-based differences were noted, and we observed a negative relationship between auditory cortex volume and mouse weight. This study is one of the first to perform comprehensive magnetic resonance imaging and quantitative analysis in the mouse brain auditory pathway cytoarchitecture, offering both novel insights into the neuroanatomical basis of age-related changes in hearing as well as evidence toward a genetic influence in this interaction. High resonance magnetic resonance imaging provides a promising efficacious avenue in future mouse model hearing loss investigations.

Objectives Dispersion of bone dust in the posterior fossa during retrosigmoid craniectomy for vestibular schwannoma (VS) resection could be a source of meningeal irritation and lead to development of persistent postoperative headaches (POH). We aim to determine risk factors, including whether the presence of bone spicules that influence POH after retrosigmoid VS resection. Design Present study is a retrospective case series. Setting The study was conducted at a tertiary skull-base referral center. Participants Adult patients undergoing VS resection via a retrosigmoid approach between November 2017 and February 2020 were included for this study. Main Outcome Measures Development of POH lasting ≥ 3 months is the primary outcome of this study. Results Of 64 patients undergoing surgery, 49 had complete data (mean age, 49 years; 53% female). Mean follow-up time was 2.4 years. At latest follow up, 16 (33%) had no headaches, 14 (29%) experienced headaches lasting Conclusion The presence of bone spicules in the posterior fossa on postoperative CT did not contribute to headaches following retrosigmoid craniectomy approach for VS resection.

Excessive opioid prescription is a source of prescription diversion and could contribute to chronic opioid abuse. This study describes the opioid prescribing patterns and risk factors for additional opioid prescription after surgical resection of vestibular schwannoma (VS).Retrospective chart review.Single tertiary referral center.Adult VS patients undergoing surgical resection between May 2019 and March 2020.Opioid use postoperatively and up to 60 days following surgery were characterized from medical records and by querying the state-wide Controlled Substance Utilization Review and Evaluation System.The presence of additional opioid prescriptions within 60 days of surgery.A total of 109 patients (mean age 50 yrs, 65.5% female) were prescribed an average of 138.2 ± 117.8 mg of morphine equivalents (MME). Twenty-two (20.9%) required additional prescriptions of 163.2 ± 103.2 MME. Age, gender, tumor size, or surgical approach (translabyrinthine, retrosigmoid, versus middle fossa) were not associated with additional prescriptions. Patients with additional prescriptions had higher body mass index (BMI 28.8 vs. 25.8 kg/m2, p = 0.015) and required more opioid medications during hospitalization (51.8 vs. 29.1 MME, p = 0.002). On multivariate logistic regression, higher BMI (odds ratio [OR] 1.32; p = 0.001), history of headaches (OR 11.9, p = 0.011), and history of opioid use (OR 29.3, p = 0.008) were associated with additional prescription.Additional opioid prescriptions may be necessary in a portion of VS patients undergoing surgery. The choice of surgical approach is not associated with excess opioid requirements. Patients with higher BMI, history of headaches, or preoperative opioid use may require additional prescriptions.

ABR wave I amplitude represents the synapse of auditory nerve fibers with the inner hair cell and is highly correlated with synapse counts. Cochlear synaptopathy, the loss of synaptic connections between inner hair cells and auditory nerve fibers, has been well-demonstrated in animal models of noise-induced hearing loss. The peak-to-peak wave I amplitude was determined at baseline and 2 weeks after noise exposure. We determined the ABR wave I amplitude at 80 dB SPL at the frequencies of 8, 12, 16, 24, and 32 kHz. A total of 69 strains (1–8 mice/strain) were analyzed. A statistically significant post-noise reduction in wave I amplitude was observed in all the tested frequencies (p

The pathophysiology of age-related hearing loss (ARHL), or presbycusis, involves a complex interplay between environmental and genetic factors. The fundamental biomolecular mechanisms of ARHL have been well described, including the roles of membrane transport, reactive oxygen species, cochlear synaptopathy, vascular insults, hormones, and microRNA, to name a few. The genetic basis underlying these mechanisms remains under-investigated and poorly understood. The emergence of genome-wide association studies has allowed for the identification of specific groups of genes involved in ARHL. This review highlights recent advances in understanding of the pathogenesis of ARHL, the genetic basis underlying these processes and suggests future directions for research and potential therapeutic avenues.

Modern imaging techniques allow early detection of small vestibular schwannomas (VSs) with minimal or no hearing impairment. While controversy surrounds the management of these tumors, given their benign nature and unpredictable natural history, microsurgical excision is the only modality that offers the opportunity to cure the tumor and preserve hearing. Hearing preservation in VS surgery may be accomplished via the middle fossa or retrosigmoid approaches. Appropriate patient selection and surgical approach is critical in achieving the best hearing outcomes. This article highlights the preoperative assessment, patient selection and prognostic factors, intraoperative monitoring of hearing, and surgical approaches to optimize hearing preservation during VS removal.

Tophaceous gout of the middle ear is a rare occurrence that presents as a granular white-colored mass. It is frequently misdiagnosed as cholesteatoma or tympanosclerosis in patients who otherwise may not manifest any clinical or biochemical signs of gout. While uncommon, it can lead to clinically significant disease such as conductive hearing loss. The present report describes 2 cases of middle ear gouty tophi initially mistaken for another entity. Both patients underwent surgery, and the diagnosis of gout was revealed after final histopathological analysis. A review of the literature is also presented.

To investigate the factors associated with unilateral hearing loss (UHL) and its impact on communication in US adults.Cross-sectional study.Nationally representative sample of US adults.We analyzed data from the 2011-2012 and 2015-2016 National Health and Nutritional Examination Survey, in which participants aged 20 to 69 years completed an audiometric evaluation (n = 8138). UHL was defined as a speech frequency pure-tone average ≥25 dB in the worse hearing ear and25 dB in the better hearing ear. Logistic regression was used to examine the association between UHL and relevant factors.The prevalence of UHL was 8.1% (95% CI, 7.3%-9.0%) in US adults. Factors associated with UHL included older age, male sex, white race, lower level of education, diabetes, cardiovascular disease, and off-work noise exposure. Among adults with UHL, 40% (95% CI, 32%-48%) reported subjective trouble with hearing, a rate higher than the 12% (95% CI, 11%-14%) among normal-hearing adults. After adjusting for relevant factors, adults with UHL were more likely to report difficulties with following conversations with noise (odds ratio [OR], 1.7; 95% CI, 1.2-2.5) and frustration when talking to family and friends (OR, 3.0; 95% CI, 1.9-4.6). Higher levels of communication difficulties were observed with worsening level of UHL.Adults with UHL report significant communication difficulties in comparison to normal-hearing adults. Further research is needed to understand the psychosocial impact of UHL on adults and ways to improve communication support for adults with UHL.

OBJECTIVE To examine the relationship between the Functional Gait Assessment (FGA) and quality of life (QOL) measurements relating to balance before and after vestibular schwannoma (VS) resection and to assess the role of preoperative FGA in predicting postoperative QOL. STUDY DESIGN A prospective clinical study of adult patients undergoing VS resection between September 2018 and December 2019. FGA was administered 1 week before and after surgery. Dizziness Handicap Inventory (DHI) and Penn Acoustic Neuroma Quality of Life (PANQOL) were administered preoperatively and at 3 months postoperatively. SETTING Single tertiary center. PATIENTS Patients (age ≥ 18 years old) with VS undergoing microsurgical resection. Excluded were patient with previous surgery or radiation. INTERVENTION VS resection. MAIN OUTCOMES AND MEASURES Primary outcome: correlation between FGA and QOL surveys. Secondary outcome: correlation between preoperative measurements of balance and postoperative PANQOL. RESULTS One hundred thirty-eight patients were analyzed (mean age: 48 years old, 65.9% female). The translabyrinthine approach was most commonly performed. Under multivariate analysis, preoperative FGA significantly correlated with preoperative PANQOL balance score (p

Background: During the COVID-19 pandemic, measures were taken to decrease viral spread by transitioning health care appointments to virtual mediums. This study evaluates the use of telemedicine within the Divisions of Colon and Rectal Surgery and Otolaryngology-Neurotology at a single academic institution during the COVID-19 pandemic. Study Design: An online survey modeled after the TeleENT Satisfaction Questionnaire and the Medical Communication Competence Scale (MCCS) was administered to gauge patient’s experience with Electronic Medical Record-based telemedicine visits. Results: Participants noticed several advantages of telemedicine visits over traditional in-office visits: shorter visits, saving money, and avoiding potential exposure during the pandemic. A total of 36% at least somewhat agreed that the quality of care was hindered by the lack of a physical examination;61.7% participants at least somewhat agreed that they prefer a face-to-face visit rather than telemedicine consultation. Most patients were satisfied with the care they received via telemedicine (Likert 6.19/7) and 95.8% would use telemedicine again. Participants self-reported an average saving of $108.70 when using telemedicine. When comparing the telemedicine cohort with the in-office control, the telemedicine patients noticed an improved ability to communicate with their physician in five out of eight domains of the MCCS. Conclusion: Surgical preoperative consultation, postoperative follow-up, and routine visits were conducted via telemedicine during the COVID-19 pandemic, representing a new direction for surgical appointments and consultations. This study shows that telehealth can provide satisfactory care and increases access to surgical care for patients when external factors prevent the traditional physician–patient interaction. With better-perceived communication via telemedicine appointments, patients may subjectively feel that they can express their symptoms and gather information from the doctor regarding their diagnosis more efficiently.

Background: Although several candidate-gene association studies have been conducted to investigate noise-induced hearing loss (NIHL) in humans, most are underpowered, unreplicated, and account for only a fraction of the genetic risk. Mouse genome-wide association studies (GWASs) have revolutionized the field of genetics and have led to the discovery of hundreds of genes involved in complex traits. The hybrid mouse diversity panel (HMDP) is a collection of classic inbred and recombinant inbred strains whose genomes have been either genotyped at high resolution or sequenced. To further investigate the genetics of NIHL, we report the first GWAS based on distortion product otoacoustic emission (DPOAE) measurements and the HMDP. Methods: A total of 102 strains (n = 635) from the HMDP were evaluated based on DPOAE suprathreshold amplitudes before and after noise exposure. DPOAE amplitude variation was set at 60 and 70 dB SPL of the primary tones for each frequency separately (8, 11.3, 16, 22.6, and 32 kHz). These values provided an indirect assessment of outer hair cell integrity. Six-week-old mice were exposed for 2 h to 10 kHz octave-band noise at 108 dB SPL. To perform local expression quantitative trait locus (eQTL) analysis, gene expression microarray profiles were generated using cochlear RNA from 64 hybrid mouse strains (n = 3 arrays per strain). Results: Several new loci were identified and positional candidate-genes associated with NIHL were prioritized, especially after noise exposure (1 locus at baseline and 5 loci after exposure). A total of 35 candidate genes in these 6 loci were identified with at least 1 probe whose expression was regulated by a significant cis-eQTL in the cochlea. After careful analysis of the candidate genes based on cochlear gene expression, 2 candidate genes were prioritized: Eya1 (baseline) and Efr3a (post-exposure). Discussion and Conclusion: For the first time, an association analysis with correction for population structure was used to map several loci for hearing traits in inbred strains of mice based on DPOAE suprathreshold amplitudes before and after noise exposure. Our results identified a number of novel loci and candidate genes for susceptibility to NIHL, especially the Eya1 and Efr3a genes. Our findings validate the power of the HMDP for detecting NIHL susceptibility genes.

To identify clinical predictors of facial nerve (FN) outcomes after microsurgical resection of vestibular schwannoma (VS).Prospective cohort study.Academic medical center.Consecutive patients undergoing VS resection from November 2017 to October 2019 were included. FN function was evaluated with the House-Brackmann (HB) scale and stratified into good (HB I-II) and poor (HB III-VI) function. Analyses included descriptive statistics, correlation, and logistic regression.Of 256 patients who met criteria (mean age, 47.7 years; 62.5% female), 227 (88.7%) achieved good FN function postoperatively and 238 (93.0%) at latest follow-up (mean, 154.8 days). Operative approaches consisted of translabyrinthine (50.8%), retrosigmoid (25.0%), and middle fossa craniotomies (24.2%). Extent of resection was decided intraoperatively, and gross or near total resection was accomplished in 237 (92.6%) cases. Postoperative HB grade correlated with latest HB grade (0.615,Microsurgical resection of VS can be accomplished with excellent FN outcomes. Gross total resection and ≥100-µV intraoperative FN electromyographic response predicted excellent FN outcomes. Immediate postoperative FN function is a prognosticator of long-term FN function.

OBJECTIVE The middle fossa transpetrosal approach to the petroclival and posterior cavernous sinus regions includes removal of the anterior petrous apex (APA), an area well studied in adults but not in children. To this end, the authors performed a morphometric analysis of the APA region during pediatric maturation. METHODS Measurements of the distance from the clivus to the internal auditory canal (IAC; C-IAC), the distance of the petrous segment of the internal carotid artery (petrous carotid; PC) to the mesial petrous bone (MPB; PC-MPB), the distance of the PC to the mesial petrous apex (MPA; PC-MPA), and the IAC depth from the middle fossa floor (IAC-D) were made on thin-cut CT scans from 60 patients (distributed across ages 0–3, 4–7, 8–11, 12–15, 16–18, and > 18 years). The APA volume was calculated as a cylinder using C-IAC (length) and PC-MPB (diameter). APA pneumatization was noted. Data were analyzed by laterality, sex, and age. RESULTS APA parameters did not differ by laterality or sex. APA pneumatization was seen on 20 of 60 scans (33.3%) in patients ≥ 4 years. The majority of the APA region growth occurred by ages 8–11 years, with PC-MPA and PC-MPB increasing 15.9% (from 9.4 to 10.9 mm, p = 0.08) and 23.5% (from 8.9 to 11.0 mm, p < 0.01) between ages 0–3 and 8–11 years, and C-IAC increasing 20.7% (from 13.0 to 15.7 mm, p < 0.01) between ages 0–3 and 4–7 years. APA volume increased 79.6% from ages 0–3 to 8–11 years (from 834.3 to 1499.2 mm3, p < 0.01). None of these parameters displayed further significant growth. Finally, IAC-D increased 51.1% (from 4.3 to 6.5 mm, p < 0.01) between ages 0–3 and adult, without significant differences between successive age groups. CONCLUSIONS APA development is largely complete by the ages of 8–11 years. Knowledge of APA growth patterns may aid approach selection and APA removal in pediatric patients.

Objective Comprehensive molecular profiling of radioresistant and cystic vestibular schwannoma (VS) subtypes. Study design Our study utilized whole-exome sequencing (WES), RNA-sequencing (RNAseq), and correlated clinical data from 12 samples (2 samples of solid sporadic subtype, 8 with cystic changes, and 2 previously irradiated). Setting Academic medical center. Patients Patients diagnosed with VS who required surgical treatment. Inclusion: Cystic and radioresistant tumors matched to age and tumor volume, with solid sporadic VS samples as control; Exclusion: NF-2 patients. Intervention(s) WES using custom probes for copy number analysis. A modified version of the Agilent Human Whole Exome sequencing hybrid capture system was used to process samples. Recurrent variants were identified and compared between groups. Leukocyte-derived DNA was utilized as internal control to reduce false-positives. Main outcome measure(s) Analysis of genetic landscape of VS subtypes (naive solid VS, cystic VS, and previously irradiated VS) by performing deep next-generation sequencing. Results WES data achieved a mean coverage of 202X and RNAseq generated an average of 74 million total reads. As a group, 25% of samples had 22q loss. Somatic analysis identified previously reported genes and multiple novel mutations across samples. Differential expression analysis of RNAseq data found significantly mutated genes such as COL6A3, CLMP, ART4, Lumican that were shared by both cystic VS and irradiated VS, but not seen in sporadic VS. Conclusions Using WES we were able to demonstrate that cystic and irradiated samples are subtypes of VS with an increased mutation burden and a unique genetic fingerprint. We identified differences between the genomic and molecular profile of cystic VS and radioresistant VS. Our results help advance the understanding of the pathophysiology of these tumor subtypes and suggest possible molecular targets for novel treatment strategies.

The pathophysiology underlying spiral ganglion cell defect–induced deafness remains elusive. Using the whole exome sequencing approach, in combination with functional assays and a mouse disease model, we identified the potentially novel deafness-causative MAP1B gene encoding a highly conserved microtubule-associated protein. Three novel heterozygous MAP1B mutations (c.4198A>G, p.1400S>G; c.2768T>C, p.923I>T; c.5512T>C, p.1838F>L) were cosegregated with autosomal dominant inheritance of nonsyndromic sensorineural hearing loss in 3 unrelated Chinese families. Here, we show that MAP1B is highly expressed in the spiral ganglion neurons in the mouse cochlea. Using otic sensory neuron–like cells, generated by pluripotent stem cells from patients carrying the MAP1B mutation and control subject, we demonstrated that the p.1400S>G mutation caused the reduced levels and deficient phosphorylation of MAP1B, which are involved in the microtubule stability and dynamics. Strikingly, otic sensory neuron–like cells exhibited disturbed dynamics of microtubules, axonal elongation, and defects in electrophysiological properties. Dysfunctions of these derived otic sensory neuron–like cells were rescued by genetically correcting MAP1B mutation using CRISPR/Cas9 technology. Involvement of MAP1B in hearing was confirmed by audiometric evaluation of Map1b heterozygous KO mice. These mutant mice displayed late-onset progressive sensorineural hearing loss that was more pronounced in the high frequencies. The spiral ganglion neurons isolated from Map1b mutant mice exhibited the deficient phosphorylation and disturbed dynamics of microtubules. Map1b deficiency yielded defects in the morphology and electrophysiology of spiral ganglion neurons, but it did not affect the morphologies of cochlea in mice. Therefore, our data demonstrate that dysfunctions of spiral ganglion neurons induced by MAP1B deficiency caused hearing loss., Dysfunctions of spiral ganglion neurons caused by Map1b deficiency leads to sensorineural hearing loss.

OBJECTIVE To determine and itemize surgical costs associated with the translabyrinthine (TL), retrosigmoid (RS), and middle cranial fossa (MCF) approaches for microsurgical excision of vestibular schwannoma (VS). STUDY DESIGN Retrospective cost analysis study. SETTING Tertiary referral center. PATIENTS Thirty consecutive adult patients underwent microsurgical excision of VS by either TL, RS, or MCF approach (10 per approach). INTERVENTIONS Microsurgical excision of VS by one of the three major approaches. Medical and financial data were collected. MAIN OUTCOME MEASURES Total operating room time (minutes), skin-to-skin time (minutes), operating room cost ($US), and surgical supplies cost ($US). RESULTS The MCF approach was associated with the shortest skin-to-skin time (230.3 min, p

BACKGROUND Hospital readmissions are commonly linked to elevated health care costs, with significant financial incentive introduced by the Affordable Care Act to reduce readmissions. OBJECTIVE To study the association between patient, hospital, and payer factors with national rate of readmission in acoustic neuroma surgery. METHODS All adult inpatients undergoing surgery for acoustic neuroma in the newly introduced Nationwide Readmissions Database from 2013 to 2014 were included. We identified readmissions for any cause with a primary diagnosis of neurological, surgical, or systemic complication within 30- and 90-d after undergoing acoustic neuroma surgery. Multivariable models were employed to identify patient, hospital, and administrative factors associated with readmission. Hospital volume was measured as the number of cases per year. RESULTS We included patients representing a weighted estimate of 4890 admissions for acoustic neuroma surgery in 2013 and 2014, with 355 30-d (7.7%) and 341 90-d (9.1%) readmissions. After controlling for patient, hospital, and payer factors, procedural volume was significantly associated with 30-d readmission rate (OR [odds ratio] 0.992, p = 0.03), and 90-d readmission rate (OR 0.994, p = 0.047). The most common diagnoses during readmission in both 30- and 90-d cohorts included general central nervous system complications/deficits, hydrocephalus, infection, and leakage of cerebrospinal fluid (rhinorrhea/otorrhea). CONCLUSION After controlling for patient, hospital, and payer factors, increased procedural volume is associated with decreased 30- and 90-d readmission rate for acoustic neuroma surgery. Future studies seeking to improve outcomes and reduce cost in acoustic neuroma surgery may seek to further evaluate the role of hospital procedural volume and experience.

Author(s): Tu, Nathan C; Friedman, Rick A | Abstract: Age-related hearing loss (ARHL) is the most common cause of hearing loss in the world. The development of ARHL in each individual is multifactorial, involving both intrinsic and extrinsic factors. This review highlights several of the key findings in the ARHL literature and discusses future directions. Level of Evidence:NA.