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Vestibular schwannoma (VS) may be associated with endolymphatic hydrops (EH). EH may account for symptomatology in a subset of patients with VS.Presenting symptoms of VS and EH overlap, and MRI evaluation of the membranous labyrinth in some patients with VS demonstrates EH. The aim of the current study is to evaluate whether EH is present in temporal bones of patients with VS.The NIDCD and House Temporal Bone Laboratory at UCLA Eccles database was queried for the diagnosis of "acoustic neuroma." Exclusion criteria included concomitant ear disease and surgery. Temporal bones were analyzed for EH of the basal, middle, and apical turns and vestibule. Premortem audiometric and clinical data were gathered.Of 43 human temporal bones with VS, 6 met inclusion criteria. All temporal bones demonstrated VS that was undisturbed by surgery. Three of six demonstrated EH of at least one cochlear turn as well as vestibular hydrops. Three patients had severe to profound hearing loss. One patient carried a diagnosis of Menière's disease.EH is demonstrated in the setting of VS in human temporal bones. EH may be one mechanism of hearing loss and dizziness in patients with VS.The underlying mechanisms of symptoms of VS may be multifactorial. The association of EH in some patients with VS would modify our clinical approach to management.To discover if EH may be associated with VS.To broaden understanding of pathophysiologic mechanisms in patients with VS.Level IVIRB Approved: UCLA IRB No. 10-001449.

OBJECTIVE To evaluate the safety of 3 Tesla (T) magnetic resonance imaging (MRI) in patients with auditory brainstem implants (ABI) with the magnet removed at implantation and report incidence of complications. STUDY DESIGN Retrospective chart review. SETTING Tertiary neurotology ambulatory practice. PATIENTS Patients with diagnosis of Neurofibromatosis, type 2 (NF2) with functional ABIs. INTERVENTIONS Observational recordings. MAIN OUTCOME MEASURES Of the 89 patients meeting inclusion criteria, 7 patients underwent 3T MRI, with a total of 39 scans done. Three patients had 1 scan each, one patient had 4 scans, one patient had 5 scans, one patient had 6 scans, and one patient had 21 scans. The mean time between ABI placement and first 3 T scan was 118 ± 73 months. The most common indication for imaging was surveillance of NF2 lesions. The most frequent scans were MRI brain (25.6%), followed by MRI of cervical (15%), thoracic (15%) and lumbar (15%) spine, and MRI IAC (8%). There were no reported complications for any of the scans. No scans were interrupted due to patient discomfort. There were no device malfunctions. CONCLUSIONS 3 T MRIs are safe in patients with ABIs as long as the magnet is removed. It is recommended that the magnet be removed at the time of implantation in all NF2 patients, who require frequent surveillance.

Hypothesis MicroRNA (miRNA) expression profiles from human perilymph correlate to post cochlear implantation (CI) hearing outcomes. Background The high inter-individual variability in speech perception among cochlear implant recipients is still poorly understood. MiRNA expression in perilymph can be used to characterize the molecular processes underlying inner ear disease and to predict performance with a cochlear implant. Methods Perilymph collected during CI from 17 patients was analyzed using microarrays. MiRNAs were identified and multivariable analysis using consonant-nucleus-consonant testing at 6 and 18 months post implant activation was performed. Variables analyzed included age, gender, preoperative pure tone average (PTA), and preoperative speech discrimination (word recognition [WR]). Gene ontology analysis was performed to identify potential functional implications of changes in the identified miRNAs. Results Distinct miRNA profiles correlated to preoperative PTA and WR. Patients classified as poor performers showed downregulation of six miRNAs that potentially regulate pathways related to neuronal function and cell survival. Conclusion Individual miRNA profiles can be identified in microvolumes of perilymph. Distinct non-coding RNA expression profiles correlate to preoperative hearing and postoperative cochlear implant outcomes.

Diagnosis and treatment of Ménière's disease remains a significant challenge because of our inability to understand what is occurring on a molecular level. MicroRNA (miRNA) perilymph profiling is a safe methodology and may serve as a "liquid biopsy" equivalent. We used machine learning (ML) to evaluate miRNA expression profiles of various inner ear pathologies to predict diagnosis of Ménière's disease.Prospective cohort study.Tertiary academic hospital.Perilymph was collected during labyrinthectomy (Ménière's disease, n = 5), stapedotomy (otosclerosis, n = 5), and cochlear implantation (sensorineural hearing loss [SNHL], n = 9). miRNA was isolated and analyzed with the Affymetrix miRNA 4.0 array. Various ML classification models were evaluated with an 80/20 train/test split and cross-validation. Permutation feature importance was performed to understand miRNAs that were critical to the classification models.In terms of miRNA profiles for conductive hearing loss versus Ménière's, 4 models were able to differentiate and identify the 2 disease classes with 100% accuracy. The top-performing models used the same miRNAs in their decision classification model but with different weighted values. All candidate models for SNHL versus Ménière's performed significantly worse, with the best models achieving 66% accuracy. Ménière's models showed unique features distinct from SNHL.We can use ML to build Ménière's-specific prediction models using miRNA profile alone. However, ML models were less accurate in predicting SNHL from Ménière's, likely from overlap of miRNA biomarkers. The power of this technique is that it identifies biomarkers without knowledge of the pathophysiology, potentially leading to identification of novel biomarkers and diagnostic tests.

Introduction: Although sensorineural hearing loss (SNHL) is the most common neurodegenerative disease in humans, no approved pharmaceutical interventions are currently available. The progre...

OBJECTIVE: With the increasing numbers of COVID-19 vaccinations available there are some reports of new onset of otologic symptoms. We present our experience in recently vaccinated patients over a 30-day time frame. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary otology ambulatory practice. PATIENTS: All patients with available diagnostic codes, COVID-19 questionnaires and clinical notes. INTERVENTIONS: Observational recordings. MAIN OUTCOME MEASURES: Within the same 30-day time period in 2019, 2020, and 2021, 1.6, 2.4, and 3.8% respectively, of all office visits were for patients with the diagnosis of new onset idiopathic sensorineural hearing loss (SNHL) without other underlying otologic diagnoses. In this time frame in 2021, 30 patients out of the 1,325 clinical visits had new or significantly exacerbated otologic symptoms that began shortly after COVID-19 vaccination. Specifically, 18 patients received Moderna and 12 patients received Pfizer vaccine. Their mean age was 60.9±13.8 years old; 11 were women and 19 men. The mean onset of symptoms was 10.18 ±â€Š9 days post-vaccination. Symptoms included 25 patients (83.3%) with hearing loss, 15 (50%) with tinnitus, eight (26.7%) with dizziness, and five (16.7%) with vertigo. Eleven patients had previous otologic diagnoses, including six patients with Meniere's disease, two with autoimmune inner ear disease (AIED), and three having both. CONCLUSIONS: There are no definite correlations to the COVID-19 pandemic or vaccination and new or worsened otologic symptoms. Vaccinated patients with new or exacerbated otologic symptoms should be promptly referred for evaluation. Suspected cases of post-vaccination otologic symptoms should be reported to the Center for Disease Control (CDC) vaccine adverse event reporting system (VAERS).

Objectives: The cochlear aqueduct is a bony duct connecting the scala tympani with the subarachnoid space. Given the pathophysiology of otosclerosis, including bone resorption and new bone deposition, we hypothesize that the cochlear aqueduct in otosclerotic ears is narrowed. Methods: A retrospective review of patients with otosclerosis who have undergone high-resolution computed tomography (HRCT) of the temporal bone was completed. The control cohort included 20 patients with the diagnosis of noise-induced hearing loss, without the diagnosis of otosclerosis. Uniform measurements of cochlear aqueduct dimensions were performed using the axial plane. Results: The otosclerosis cohort included 25 males and 52 females with mean age of 52.2 ± 17.6 years. The control group included 10 males and 10 females with mean age of 64.0 ± 18.5 years. The mean cochlear aqueduct length, width mid canal, aperture base, aperture widest diameter, and funnel diameter in millimeters were 12.19 ± 1.66, 0.68 ± 0.28, 4.21 ± 1.67, 3.23 ± 1.47, and 2.70 ± 1.05 in the ears with otosclerotic foci and 11.57 ± 1.66, 0.69 ± 0.29, 2.56 ± 1.59, 2.77 ± 1.67, and 2.58 ± 1.03 in control group, respectively. Statistical difference was seen in length of cochlear aqueduct, aperture base, and aperture widest diameters ( P = .017, Conclusions: The length of the cochlear aqueduct and the funnel width are statistically longer in the otosclerotic population compared to control. The width of the cochlear aqueduct is not statistically different.

Hearing loss (HL) is the most common neurodegenerative disease worldwide. Despite its prevalence, clinical testing does not yield a cell or molecular based identification of the underlying etiology of hearing loss making development of pharmacological or molecular treatments challenging. A key to improving the diagnosis of inner ear disorders is the development of reliable biomarkers for different inner ear diseases. Analysis of microRNAs (miRNA) in tissue and body fluid samples has gained significant momentum as a diagnostic tool for a wide variety of diseases. In previous work, we have shown that miRNA profiling in inner ear perilymph is feasible and may demonstrate distinctive miRNA expression profiles unique to different diseases. A first step in developing miRNAs as biomarkers for inner ear disease is linking patterns of miRNA expression in perilymph to clinically available metrics. Using machine learning (ML), we demonstrate we can build disease specific algorithms that predict the presence of sensorineural hearing loss using only miRNA expression profiles. This methodology not only affords the opportunity to understand what is occurring on a molecular level, but may offer an approach to diagnosing patients with active inner ear disease.

Hypothesis: Menière's disease microRNA (miRNA) profiles are unique and are reflected in the perilymph and serum of patients.Background: Development of effective biomarkers for Menière's disease are needed. miRNAs are small RNA sequences that downregulate mRNA translation and play a significant role in a variety of disease states, ultimately making them a promising biomarker. miRNAs can be readily isolated from human inner ear perilymph and serum, and may exhibit disease-specific profiles.Methods: Perilymph sampling was performed in 10 patients undergoing surgery; 5 patients with Meniere's disease and 5 patients with otosclerosis serving as controls. miRNAs were isolated from the serum of 5 patients with bilateral Menière's disease and compared to 5 healthy age-matched controls. For evaluation of miRNAs an Agilent miRNA gene chip was used. Analysis of miRNA expression was carried out using Qlucore and Ingenuitey Pathway Analysis software. Promising miRNAs biomarkers were validated using qPCR.Results: In the perilymph of patients with Menière's disease, we identified 16 differentially expressed miRNAs that are predicted to regulate over 220 different cochlear genes. Six miRNAs are postulated to regulate aquaporin expression and twelve miRNAs are postulated to regulate a variety of inflammatory and autoimmune pathways. When comparing perilymph with serum samples, miRNA-1299 and−1270 were differentially expressed in both the perilymph and serum of Ménière's patients compared to controls. Further analysis using qPCR confirmed miRNA-1299 is downregulated over 3-fold in Meniere's disease serum samples compared to controls.Conclusions: Patients with Ménière's disease exhibit distinct miRNA expression profiles within both the perilymph and serum. The altered perilymph miRNAs identified can be linked to postulated Ménière's disease pathways and may serve as biomarkers. miRNA-1299 was validated to be downregulated in both the serum and perilymph of Menière's patients.

Hypothesis MicroRNAs predicted to regulate neurotrophin signaling can be found in human perilymph. Background Animal and human temporal bone studies suggest that spiral ganglion health can affect cochlear implant (CI) outcomes. Neurotrophins have been identified as a key factor in the maintenance of spiral ganglion health. Changes in miRNAs may regulate neurotrophin signaling and may reflect neurotrophin expression levels. Methods Perilymph sampling was carried out in 18 patients undergoing cochlear implantation or stapedotomy. Expression of miRNAs in perilymph was evaluated using an Agilent miRNA gene chip. Using ingenuity pathway analysis (IPA) software, miRNAs targeting neurotrophin signaling pathway genes present in a cochlear cDNA library were annotated. Expression levels of miRNAs in perilymph were correlated to the patients' preoperative pure-tone average. Results Expression of mRNAs coding for neurotrophins and their receptors were identified in tissue obtained from normal human cochlea during skull base surgery. We identified miRNAs predicted to regulate these signaling cascades, including miR-1207-5p, miR-4651, miR-103-3p, miR-100-5p, miR-221-3p, miR-200-3p. There was a correlation between poor preoperative hearing and lower expression of miR-1207 (predicted to regulate NTR3) and miR-4651 (predicted to regulate NTR2). Additionally, miR-3960, miR-4481, and miR-675 showed significant differences in expression level when comparing mild and profound hearing loss patients. Conclusions Expression of some miRNAs that are predicted to regulate neurotrophin signaling in the perilymph of cochlear implant patients vary with the patient's level of residual hearing. These miRNAs may serve as biomarkers for changes in neurotrophin signaling.

Objective Our objective is to evaluate the safety in patients with cochlear implants (CIs) and auditory brainstem implants (ABI) undergoing 1.5 Tesla (T) magnetic resonance imaging (MRI). Secondly, we want to raise awareness on CI and MRI safety, and advocate for continued improvement and advancement to minimize morbidity for our CI patients. Methods Retrospective case series from 2006 to 2018 at a single tertiary academic center. Data was collected on patients with CI or auditory brainstem implants undergoing MRI. Outcomes collected include demographic data, age at time of MRI, MRI characteristics, complications, CI manufacturer, and image quality. Results Eighteen patients with CI or ABI collectively underwent a total of 62 MRI scans. Five of 15 (33%) CI patients with magnet had complications: five total of 24 MRI scans (21%). Two patients had magnet removal prior to 29 MRI scans without complications. Four of five MRI-related complications were equipped with a U.S. Food and Drug Administration-approved head wrap. Three of five required a trip to the operating room to explore and reposition the CI magnet; two could not complete MRI secondary to pain. Of the complications, two were Cochlear (Sydney, Australia), two Advanced Bionics (Valencia, CA), and one MED-EL (Innsbruck, Austria). Synchrony model (MED-EL) had 0 of seven complications, with a total of 19 MRI scans, which features a freely rotating and self-aligning magnet. Conclusion Our series offers a diverse number of CI manufacturers and is in accordance with other literature that CI MRI-related adverse events are occurring at an unacceptable frequency. We can promote CI MRI safety through our institutions' MRI CI patient protocols, raise awareness that diagnostic MRI benefits must outweigh CI-related complications, and advocate for continued industry technological innovation. Level of evidence 4 Laryngoscope, 129:482-489, 2019.

Purpose Vagal nerve stimulation in conjunction with sound therapy has been proposed as a treatment for subjective tinnitus. The purpose of this study is to retrospectively review the effect of VNS on perception of tinnitus in epilepsy patients. We explore the incidence of tinnitus and its perceived reduction in patients requiring implantation of VNS for medically refractory seizures. Materials and methods A phone survey was conducted in adult patients with prior VNS implantation. A questionnaire including the visual analog scale (VAS) of tinnitus loudness was used to determine the presence and severity of tinnitus. Results Out of the 56 patients who had completed the phone survey, 20 (35%) reported the presence of pre-operative tinnitus. The tinnitus positive group was significantly older (p = 0.019). Of the 20 pre-operative tinnitus positive patients, all patients continued to have tinnitus post-operatively. Four (20%) noted no changes in VAS of tinnitus loudness while 16 (80%) had at least a one-point decrease. The mean difference between pre- and post-operative VAS of loudness was 2.05, with a standard deviation of 1.84 and this was statistically significant (p Conclusions In this study, we evaluate the potential of vagal nerve stimulation to alter the perception of tinnitus in patients with refractory epilepsy. Eighty percent of patients noted some level of subjective tinnitus improvement after VNS implantation. Given this finding, there may be a potential additional benefit to the use of VNS in patients with epilepsy.

Objectives: The topic of prescription opioid overuse remains a growing concern in the United States. Our objective is to provide insight into pain perception and opioid use based on a patient cohort undergoing common otologic and neurotologic surgeries. Study Design: Prospective observational study with patient questionnaire. Setting: Single academic medical center. Subjects and Methods: Adult patients undergoing otologic and neurotologic procedures by two fellowship trained neurotologists between June and November of 2018 were included in this study. During first postoperative follow-up, participants completed a questionnaire assessing perceived postoperative pain and its impact on quality of life, pain management techniques, and extent of prescription opioid use. Results: A total of 47 patients met inclusion and exclusion criteria. The median pain score was 3 out of 10 (Interquartile Range [IQR] = 2-6) with no significant gender differences ( P = .92). Patients were prescribed a median of 15.0 (IQR = 10.0-15.0) tablets of opioid pain medication postoperatively, but only used a median of 4.0 (IQR = 1.0-11.5) tablets at the time of first follow-up. Measured quality of life areas included sleep, physical activity, work, and mood. Sleep was most commonly affected, with 69.4% of patients noting disturbances. Conclusions: This study suggests that practitioners may over-estimate the need for opioid pain medication following otologic and neurotologic surgery. It also demonstrates the need for ongoing patient education regarding opioid risks, alternatives, and measures to prevent diversion.

HYPOTHESIS Profiling of microRNA (miRNA) within perilymph samples collected at the time of stapedectomy can be used to identify active gene expression pathways in otosclerosis as compared with controls. BACKGROUND miRNAs are small non-coding RNAs that effect gene expression by post-transcription regulation and silencing. Perilymph sampling allows for a novel way to collect material actively involved in the disease process. METHODS Perilymph was collected at time of stapedectomy, underwent a microarray analysis, and significantly expressed miRNAs were correlated to known bone morphology pathways using a cochlear transcriptome library. To determine miRNA related specifically to otosclerosis, cochlear implant controls were used for statistical analysis. RESULTS A total of 321 significantly expressed miRNAs were identified within the four otosclerosis perilymph samples. miRNAs associated with 23 genes involved in bone morphology pathways were significantly expressed. A significant difference in the otosclerotic samples as compared with control was noted in miRNA expression regulating HMGA2, ITGB3, SMO, CCND1, TP53, TP63, and RBL2 gene pathways. No significant difference was noted in miRNAs expression associated with ACE, RELN, COL1A1, and COL1A2 genes which were previously correlated with otosclerosis. CONCLUSIONS Perilymph miRNA profiling obtained at the time of stapedectomy consistently identifies differentially expressed genes compared with controls. Perilymph miRNA sampling with cochlear transcriptome library cross-referencing can be successfully used to identify active gene expression pathways in otosclerosis.

The aim of this report is to present a case of mesial temporal lobe sclerosis (MTS) causing medically refractory seizures, which was initially disguised as temporal lobe encephalocele secondary to prior otologic surgery. Temporal lobe encephaloceles are characterized by a defect within the middle cranial fossa that results in the abnormal communication of the meninges into the pneumatized skull base. After the temporal lobe encephalocele repair, the patient continued to have seizures and was subsequently diagnosed with mesial temporal lobe sclerosis. Imaging revealed the serial progression of hippocampal atrophy and loss of internal architecture. Differentiation between mesial temporal sclerosis and encephalocele as the underlying epileptic etiology is critical. While repairing encephaloceles is necessary to address other potential sequelae, patients with mesial temporal lobe sclerosis will require additional interventions.

Post-operative rhabdomyolysis is a rare but life-threatening condition. Less than ten cases have been described in the otolaryngic literature and, to our knowledge, no reports exist in the setting of oral reconstructive free tissue transfer. Case report presentation. We discuss the clinical course that lead to the diagnosis of rhabdomyolysis with special consideration to simultaneous microvascular anastomosis. Serial lab values were closely followed to prevent kidney injury while preventing flap congestion. Excessive fluid resuscitation in free flap reconstruction has been associated with increased post-operative complications and flap failure. We present a cautionary case to highlight the need for early diagnosis of rhabdomyolysis, a condition that may become more prevalent in the head and neck population as obesity, the main risk factor, continues to increase worldwide.

Our objective is to evaluate the safety in patients with cochlear implants (CIs) and auditory brainstem implants (ABI) undergoing 1.5 Tesla (T) magnetic resonance imaging (MRI). Secondly, we want to raise awareness on CI and MRI safety, and advocate for continued improvement and advancement to minimize morbidity for our CI patients.Retrospective case series from 2006 to 2018 at a single tertiary academic center. Data was collected on patients with CI or auditory brainstem implants undergoing MRI. Outcomes collected include demographic data, age at time of MRI, MRI characteristics, complications, CI manufacturer, and image quality.Eighteen patients with CI or ABI collectively underwent a total of 62 MRI scans. Five of 15 (33%) CI patients with magnet had complications: five total of 24 MRI scans (21%). Two patients had magnet removal prior to 29 MRI scans without complications. Four of five MRI-related complications were equipped with a U.S. Food and Drug Administration-approved head wrap. Three of five required a trip to the operating room to explore and reposition the CI magnet; two could not complete MRI secondary to pain. Of the complications, two were Cochlear (Sydney, Australia), two Advanced Bionics (Valencia, CA), and one MED-EL (Innsbruck, Austria). Synchrony model (MED-EL) had 0 of seven complications, with a total of 19 MRI scans, which features a freely rotating and self-aligning magnet.Our series offers a diverse number of CI manufacturers and is in accordance with other literature that CI MRI-related adverse events are occurring at an unacceptable frequency. We can promote CI MRI safety through our institutions' MRI CI patient protocols, raise awareness that diagnostic MRI benefits must outweigh CI-related complications, and advocate for continued industry technological innovation.4 Laryngoscope, 129:482-489, 2019.