Optimized Diagnostic Approach to Patients Suspected of Superior Semicircular Canal Dehiscence.

OBJECTIVES: Current methods of diagnosing superior semicircular canal dehiscence syndrome (SCDS) include a clinical exam, audiometric testing, temporal bone computer tomography (CT) imaging, and vestibular evoked myogenic potential (VEMP) testing. The main objective of this study was to develop an improved diagnostic approach to SCDS optimized for accuracy, efficiency, and safety that utilizes clinical presentation, audiometric testing, CT imaging, high-frequency cervical VEMP (cVEMP) testing, and patient treatment preference. A secondary aim was to investigate the cost associated with the current versus proposed diagnostic paradigms. DESIGN: All patients who underwent cVEMP testing since introduction of the 2 kHz cVEMP in our clinical protocol in July 2018 were screened. Patients suspected of SCDS based upon symptoms who also had available audiogram, CT scan, and 2 kHz cVEMP were included (58 ears). Patients were categorized as dehiscent, thin, or not dehiscent based on their CT scan. Symptom prevalence and cVEMP outcomes were analyzed and compared for all groups. The accuracy of the 2 kHz cVEMP was calculated using CT imaging as the standard. Using a combination of patient symptomatology, audiometric, CT and 2 kHz cVEMP data, as well as patient preference, a best clinical practice approach was developed. The cost associated with this approach was calculated and compared with cost of the current SCDS diagnostic workup using Medicare reimbursement rates. RESULTS: In the overall patient population suspected of SCDS based on clinical presentation, the sensitivity and specificity of 2 kHz cVEMP were 76% and 100%, respectively, while the positive and negative predictive values were 100% and 84.6%, assuming that the CT scan finding was correct. Autophony was the most common symptom in patients who had both superior semicircular canal dehiscence on CT imaging plus abnormal 2 kHz cVEMP (p < 0.001). Combining patient symptomatology, 2 kHz normalized peak to peak cVEMP amplitude, and patient treatment preference to determine, which patients should undergo CT scanning resulted in a potential cost reduction between 45% and 61%. CONCLUSION: In patients suspected of SCDS based on their clinical presentation, the combination of symptomatology, 2 kHz cVEMP data, and patient preference can be used to determine which patients should undergo CT scanning, resulting in a diagnostic cost reduction and reduced patient radiation exposure.

Inner ear pathologies impair sodium-regulated ion transport in Meniere's disease.

Meniere's disease (MD), a syndromal inner ear disease, is commonly associated with a pathological accumulation of endolymphatic fluid in the inner ear, termed "idiopathic" endolymphatic hydrops (iEH). Although numerous precipitating/exacerbating factors have been proposed for MD, its etiology remains elusive. Here, using immunohistochemistry and in situ protein-protein interaction detection assays, we demonstrate mineralocorticoid-controlled sodium transport mechanisms in the epithelium of the extraosseous portion of the endolymphatic sac (eES) in the murine and human inner ears. Histological analysis of the eES in an extensive series of human temporal bones consistently revealed pathological changes in the eES in cases with iEH and a clinical history of MD, but no such changes were found in cases with "secondary" EH due to other otological diseases or in healthy controls. Notably, two etiologically different pathologies-degeneration and developmental hypoplasia-that selectively affect the eES in MD were distinguished. Clinical records from MD cases with degenerative and hypoplastic eES pathology revealed distinct intergroup differences in clinical disease presentation. Overall, we have identified for the first time two inner ear pathologies that are consistently present in MD and can be directly linked to the pathogenesis of EH, and which potentially affect the phenotypical presentation of MD.

Normalizing cVEMPs: Which Method Is the Most Effective?

OBJECTIVES: To determine the most effective method for normalizing cervical vestibular evoked myogenic potentials (cVEMPs). DESIGN: cVEMP data from 20 subjects with normal hearing and vestibular function were normalized using 16 combinations of methods, each using one of the 4 modes of electromyogram (EMG) quantification described below. All methods used the peak to peak value of an averaged cVEMP waveform (VEMPpp) and obtained a normalized cVEMP by dividing VEMPpp by a measure of the EMG amplitude. EMG metrics were obtained from the EMG within short- and long-duration time windows. EMG amplitude was quantified by its root-mean-square (RMS) or average full-wave-rectified (RECT) value. The EMG amplitude was used by (a) dividing each individual trace by the EMG of this specific trace, (b) dividing VEMPpp by the average RMS or RECT of the individual trace EMG, (c) dividing the VEMPpp by an EMG metric obtained from the average cVEMP waveform, or (d) dividing the VEMPpp by an EMG metric obtained from an average cVEMP "noise" waveform. Normalization methods were compared by the normalized cVEMP coefficient of variation across subjects and by the area under the curve from a receiver-operating-characteristic analysis. A separate analysis of the effect of EMG-window duration was done. RESULTS: There were large disparities in the results from different normalization methods. The best methods used EMG metrics from individual-trace EMG measurements, not from part of the average cVEMP waveform. EMG quantification by RMS or RECT produced similar results. For most EMG quantifications, longer window durations were better in producing receiver-operating-characteristic with high areas under the curve. However, even short window durations worked well when the EMG metric was calculated from the average RMS or RECT of the individual-trace EMGs. Calculating the EMG from a long-duration window of a cVEMP "noise" average waveform was almost as good as the individual-trace-EMG methods. CONCLUSIONS: The best cVEMP normalizations use EMG quantification from individual-trace EMGs. To have the normalized cVEMPs accurately reflect the vestibular activation, a good normalization method needs to be used.

Toward Optimizing cVEMP: 2,000-Hz Tone Bursts Improve the Detection of Superior Canal Dehiscence.

BACKGROUND: The cervical vestibular evoked myogenic potential (cVEMP) test measures saccular and inferior vestibular nerve function. The cVEMP can be elicited with different frequency stimuli and interpreted using a variety of metrics. Patients with superior semicircular canal dehiscence (SCD) syndrome generally have lower cVEMP thresholds and larger amplitudes, although there is overlap with healthy subjects. The aim of this study was to evaluate which metric and frequency best differentiate healthy ears from SCD ears using cVEMP. METHODS: Twenty-one patients with SCD and 23 age-matched controls were prospectively included and underwent cVEMP testing at 500, 750, 1,000 and 2,000 Hz. Sound level functions were obtained at all frequencies to acquire threshold and to calculate normalized peak-to-peak amplitude (VEMPn) and VEMP inhibition depth (VEMPid). Third window indicator (TWI) metrics were calculated by subtracting the 250-Hz air-bone gap from the ipsilateral cVEMP threshold at each frequency. Ears of SCD patients were divided into three groups based on CT imaging: dehiscent, thin or unaffected. The ears of healthy age-matched control subjects constituted a fourth group. RESULTS: Comparing metrics at all frequencies revealed that 2,000-Hz stimuli were most effective in differentiating SCD from normal ears. ROC analysis indicated that for both 2,000-Hz cVEMP threshold and for 2,000-Hz TWI, 100% specificity could be achieved with a sensitivity of 92.0%. With 2,000-Hz VEMPn and VEMPid at the highest sound level, 100% specificity could be achieved with a sensitivity of 96.0%. CONCLUSION: The best diagnostic accuracy of cVEMP in SCD patients can be achieved with 2,000-Hz tone burst stimuli, regardless of which metric is used.

Toward Optimizing VEMP: Calculating VEMP Inhibition Depth With a Generic Template.

OBJECTIVES: Cervical vestibular evoked myogenic potentials (cVEMP) indirectly reveal the response of the saccule to acoustic stimuli through the inhibition of sternocleidomastoid muscle electromyographic response. VEMP inhibition depth (VEMPid) is a recently developed metric that estimates the percentage of saccular inhibition. VEMPid provides both normalization and better accuracy at low response levels than amplitude-normalized cVEMPs. Hopefully, VEMPid will aid in the clinical assessment of patients with vestibulopatholgy. To calculate VEMPid a template is needed. In the original method, a subject's own cVEMP was used as the template, but this method can be problematic in patients who do not have robust cVEMP responses. We hypothesize that a "generic" template, created by assembling cVEMPs from healthy subjects, can be used to compute VEMPid, which would facilitate the use of VEMPid in subjects with pathological conditions. DESIGN: A generic template was created by averaging cVEMP responses from 6 normal subjects. To compare VEMPid calculations using a generic versus a subject-specific template, cVEMPs were obtained in 40 healthy subjects using 500, 750, and 1000 Hz tonebursts at sound levels ranging from 98 to 123 dB peSPL. VEMPids were calculated both with the generic template and with the subject's own template. The ability of both templates to determine whether a cVEMP was present or not was compared with receiver operating characteristic curves. RESULTS: No significant differences were found between VEMPid calculations using a generic template versus using a subject-specific template for all frequencies and sound levels. Based on the receiver operating characteristic curves, the subject-specific and generic template did an equally good job at determining threshold. Within limits, the shape of the generic template did not affect these results. CONCLUSIONS: A generic template can be used instead of a subject-specific template to calculate VEMPid. Compared with cVEMP normalized by electromyographic amplitudes, VEMPid is advantageous because it averages zero when there is no sound stimulus and it allows the accumulating VEMPid value to be shown during data acquisition as a guide to deciding when enough data has been collected.

Toward Optimizing Vestibular Evoked Myogenic Potentials: Normalization Reduces the Need for Strong Neck Muscle Contraction.

BACKGROUND: The cervical vestibular evoked myogenic potential (cVEMP) represents an inhibitory reflex of the saccule measured in the ipsilateral sternocleidomastoid muscle (SCM) in response to acoustic or vibrational stimulation. Since the cVEMP is a modulation of SCM electromyographic (EMG) activity, cVEMP amplitude is proportional to muscle EMG amplitude. We sought to evaluate muscle contraction influences on cVEMP peak-to-peak amplitudes (VEMPpp), normalized cVEMP amplitudes (VEMPn), and inhibition depth (VEMPid). METHODS: cVEMPs at 500 Hz were measured in 25 healthy subjects for 3 SCM EMG contraction ranges: 45-65, 65-105, and 105-500 µV root mean square (r.m.s.). For each range, we measured cVEMP sound level functions (93-123 dB peSPL) and sound off, meaning that muscle contraction was measured without acoustic stimulation. The effect of muscle contraction amplitude on VEMPpp, VEMPn, and VEMPid and the ability to distinguish cVEMP presence/absence were evaluated. RESULTS: VEMPpp amplitudes were significantly greater at higher muscle contractions. In contrast, VEMPn and VEMPid showed no significant effect of muscle contraction. Cohen's d indicated that for all 3 cVEMP metrics contraction amplitude variations produced little change in the ability to distinguish cVEMP presence/absence. VEMPid more clearly indicated saccular output because when no acoustic stimulus was presented the saccular inhibition estimated by VEMPid was zero, unlike those by VEMPpp and VEMPn. CONCLUSION: Muscle contraction amplitude strongly affects VEMPpp amplitude, but contractions 45-300 µV r.m.s. produce stable VEMPn and VEMPid values. Clinically, there may be no need for subjects to exert high contraction effort. This is especially beneficial in patients for whom maintaining high SCM contraction amplitudes is challenging.

Evaluating Inhibition of Motoneuron Firing From Electromyogram Data to Assess Vestibular Output Using Vestibular Evoked Myogenic Potentials.

OBJECTIVES: Vestibular evoked myogenic potentials (VEMPs) are due to vestibular responses producing brief inhibitions of muscle contractions that are detectable in electromyographic (EMG) responses. VEMP amplitudes are traditionally measured by the peak to peak amplitude of the averaged EMG response (VEMPpp) or by a normalized VEMPpp (nVEMPpp). However, a brief EMG inhibition does not satisfy the statistical assumptions for the average to be the optimal processing strategy. Here, it is postulated that the inhibition depth of motoneuron firing is the desired metric for showing the influence of the vestibular system on the muscle system. The authors present a metric called "VEMPid" that estimates this inhibition depth from the EMG data obtained in a usual VEMP data acquisition. The goal of this article was to compare how well VEMPid, VEMPpp, and nVEMPpp track inhibition depth. DESIGN: To find a robust method to compare VEMPid, VEMPpp, and nVEMPpp, realistic physiological models for the inhibition of VEMP EMG signals were made using VEMP data from four measurement sessions on each of the five normal subjects. Each of the resulting 20 EMG-production models was adjusted to match the EMG autocorrelation of an individual subject and session. Simulated VEMP traces produced by these models were used to compare how well VEMPid, VEMPpp, and nVEMPpp tracked model inhibition depth. RESULTS: Applied to simulated and real VEMP data, VEMPid showed good test-retest consistency and greater sensitivity at low stimulus levels than VEMPpp or nVEMPpp. For large-amplitude responses, nVEMPpp and VEMPid were equivalent in their consistency across subjects and sessions, but for low-amplitude responses, VEMPid was superior. Unnormalized VEMPpp was always worse than nVEMPpp or VEMPid. CONCLUSIONS: VEMPid provides a more reliable measurement of vestibular function at low sound levels than the traditional nVEMPpp, without requiring a change in how VEMP tests are performed. The calculation method for VEMPid should be applicable whenever an ongoing muscle contraction is briefly inhibited by an external stimulus.

Current Demography and Treatment Strategy of Vestibular Migraine in Neurotologic Perspective.

OBJECTIVE: This study aims to describe the demographic features of vestibular migraine (VM) compared to other common peripheral vestibulopathies, and to assess the efficacy of trigger management as primary VM treatment. STUDY DESIGN: Retrospective study. SETTING: Tertiary referral medical center. METHODS: A retrospective chart review was performed of patients presenting with dizziness in a tertiary neurotology clinic. Among the newly diagnosed dizzy patients, those with a diagnosis of either VM, Ménière's disease, benign paroxysmal positional vertigo, or vestibular neuritis/labyrinthitis were selected for comparisons of the disease demographics. VM patients with multiple clinic visits in the study period were included for the treatment strategy analysis. RESULTS: A total of 1285 patients met the study inclusion criteria. In the newly diagnosed dizzy group comprising 814 patients, VM patients accounted for the largest (25%) and youngest (mean age 47 years) cohort, showing an obvious female preponderance of approximately 3.3:1. Furthermore, prevalence of VM declined significantly with advancing age, from 69%, to 34%, to 11%, in age groups of ≤30, 31 to 60, and >60 years, respectively. Trigger management, when employed as the primary VM first-year treatment, was effective for both sexes. Notably, it was more effective for women aged over than under 45 years. CONCLUSION: In our tertiary referral neurotologic center, VM was the most common diagnosis in newly diagnosed dizzy patients aged under 60 years, especially in women. For primary VM treatment, trigger management is an effective option deserving consideration.

Natural History of Untreated Idiopathic Sudden Sensorineural Hearing Loss.

OBJECTIVE: Although corticosteroids and other treatments for idiopathic sudden sensorineural hearing loss (ISSNHL) have been described, understanding its prognosis without intervention provides valuable information for patient management. The objective of this study is to provide a comprehensive, quantitative statistical analysis of the natural history of untreated idiopathic sudden sensorineural hearing loss (ISSNHL). STUDY TYPE AND DESIGN: A systematic review and meta-analyses. METHODS: Two independent searches of PubMed, Scopus, Web of Science, and Cochrane Library databases up to June 30, 2022, were performed. Adults with idiopathic sudden sensorineural hearing loss who received placebo or were untreated and had audiometric outcome measures in all study types were reviewed. These data indicative of the natural history of ISSNHL were analyzed, as were study characteristics related to risk of bias. Heterogeneity as assessed via I2 and random effects analyses were performed. RESULTS: Six studies meeting the inclusion criteria yielded 319 untreated patients whose natural history could be assessed. Heterogeneity among studies was moderate, with a variety of reported outcomes. A hearing improvement of at least 30 dB HL was observed in 36% (95% CI 0.28-0.44) of untreated patients, and of at least 10 dB HL was observed in 70% (95% CI 0.57-0.82) of untreated patients at 3 months. The mean hearing gain among untreated patients was 24.0 dB HL (95% CI 2.65-45.37) at 2-3 months. CONCLUSIONS: The observed natural history of ISSNHL suggests that patients can regain some hearing without active treatment. In the absence of future studies collecting prospective natural history data from untreated or placebo-treated ISSNHL patients, the data presented here provide the best available historical control data for reconsideration of results in past ISSNHL studies, as well as a roadmap for design and interpretation of future ISSNHL treatment clinical trials. Furthermore, knowing there is a statistically significant mean hearing gain of 24.0 dB HL in the untreated/placebo group provides an ethical basis for future placebo study of ISSNHL. The current status on ISSNHL management calls for a multi-institutional, randomized, double-blind placebo-controlled trial with validated outcome measures to provide science-based treatment guidance. Laryngoscope, 134:S1-S15, 2024.

Retrolabyrinthine Bone Thickness as a Radiologic Marker for the Hypoplastic Endotype in Menière Disease.

BACKGROUND AND PURPOSE: Menière disease (MD) manifests in 2 major endotypes: one with a hypoplastic, underdeveloped endolymphatic sac (MD-hp) and the other with a normally developed sac that degenerates over time (MD-dg). Determining the specific endotype in patients is important for predicting disease progression, tailoring patient counseling, and optimizing treatment strategies. Endotype diagnosis involves measuring an angular trajectory of the vestibular aqueduct (ATVA), with an ATVA ≥140° indicative of MD-hp and an ATVA ≤120° of MD-dg. However, assessing the ATVA can be challenging. This study aimed to explore the link between ATVA and the thickness of the retrolabyrinthine bone as an alternative diagnostic measure that could provide differentiation between MD endotypes using CT and MR imaging. MATERIALS AND METHODS: Retrospective review of CT temporal bone imaging from 32 adult patients with definite MD (60 ears) and 33 age-matched controls without MD or other inner ear symptoms (61 ears) was performed. The ATVA and retrolabyrinthine bone thickness were measured using uniform methodology on standardized axial CT images. Comparative analyses were performed to determine the correlation between ATVA and retrolabyrinthine bone thickness. Additionally, from a separate cohort of 11 patients (22 ears), CT and MR examinations of the temporal bone were retrospectively reviewed for retrolabyrinthine bone thickness measurements, to verify the correlation across the 2 modalities. RESULTS: The average retrolabyrinthine bone thickness was statistically significantly different between MD endotypes, being a mean of 0.8 (SD, 0.3) mm in patients with MD-hp (ATVA ≥140°) and 2.0 (SD, 0.9) mm in patients with MD-dg (ATVA ≤120°), with a consistent pattern of thin retrolabyrinthine bone in MD-hp and variable thickness in MD-dg. Receiver operating characteristic curve analysis within the MD cohort revealed that a retrolabyrinthine bone thickness ≥1.2 mm effectively rules out MD-hp. Excellent interrater reliability was noted for the retrolabyrinthine measurement, and there was near-perfect correlation between CT and MR measurements. CONCLUSIONS: Retrolabyrinthine bone thickness proved to be a useful and straightforward alternative marker for distinguishing MD endotypes, being particularly useful for excluding MD-hp. Including information on retrolabyrinthine bone thickness should be considered a routine part of reporting in the context of MD imaging.

Inner ear therapeutics and the war on hearing loss: systemic barriers to success.

Despite over 20 years of effort in academic research centers, start-up companies, and established pharmaceutical companies, there are no FDA-approved inner ear therapeutics for treatment of sensorineural hearing loss. There are a number of systemic barriers to creation of this new field of inner ear therapeutics. These include insufficient understanding of the particularity of different causes of hearing loss at the cellular and molecular level, lack of diagnostics of adequate sensitivity and specificity to discern these differences in vivo, a tendency for start-up biotech/pharma companies to prioritize competition over collaboration, and a drug development ecosystem that is really in the "pre-competitive" phase and a lack of infrastructure to develop, validate, gain regulatory approval, and successfully market an inner ear therapeutic. These issues will be discussed in this perspective article and a proposed remedy in the form of an inner ear therapeutics "moon shot" will be offered.

Improvement in word recognition score with level is associated with hearing aid ownership among patients with hearing loss.

Market surveys consistently show that only 22% of those with hearing loss own hearing aids. This is often ascribed to cosmetics, but is it possible that patients apply a different auditory criterion than do audiologists and manufacturers? We tabulated hearing aid ownership in a survey of 1000 consecutive patients. We separated hearing loss cases, with one cohort in which word recognition in quiet could improve with gain (vs. 40 dB HL) and another without such improvement but nonetheless with audiometric thresholds within the manufacturer's fitting ranges. Overall, we found that exactly 22% of hearing loss patients in this sample owned hearing aids; the same finding has been reported in many previous, well-accepted surveys. However, while all patients in the two cohorts experienced difficulty in noise, patients in the cohort without word recognition improvement were found to own hearing aids at a rate of 0.3%, while those patients whose word recognition could increase with level were found to own hearing aids at a rate of 50%. Results also coherently fit a logistic model where shift of the word recognition performance curve by level corresponded to the likelihood of ownership. In addition to the common attribution of low hearing aid usage to patient denial, cosmetic issues, price, or social stigma, these results provide one alternative explanation based on measurable improvement in word recognition performance.

Cochlear Implantation Hearing Outcome in Ménière's Disease.

OBJECTIVE: The aim of this study was to evaluate the hearing outcome of cochlear implantation in patients deafened by Ménière's disease. STUDY DESIGN: Retrospective single-institution study. SETTING: Tertiary medical center. METHODS: Our institutional database of 1400 patients with cochlear implants was reviewed to identify cases with deafness due to Ménière's disease. Twenty-nine patients were identified: 24 with unilateral and 5 with sequential bilateral cochlear implants. Pre- and postoperative speech recognition scores and medical data were extracted from the medical record and analyzed. RESULTS: Overall the mean difference between pre- and postoperative speech recognition after >1 year was 56% (95% CI, 47.08%-64.92%). The mean preoperative monosyllabic word score was 9.5%, and the mean postoperative scores at 1 month, 3 months, 6 months, 1 year, and >1 year were 37.1%, 46.1%, 54.1%, 59.1%, and 66.8%, respectively. Cochlear implantation resulted in improved word scores in all patients regardless of prior medical or surgical treatment (endolymphatic sac, labyrinthectomy). The mean postoperative hearing improvement in patients aged <70 and ≥70 years was 65.26% (95% CI, 54.79%-75.73%) and 40.00% (95% CI, 27.22%-52.77%). Postoperative word scores in patients with bilateral cochlear implants were not significantly different between the first and second implanted ears or between the monoaural and binaural testing conditions. CONCLUSION: Cochlear implant in patients deafened by Ménière's disease significantly improves word recognition scores regardless of whether medical or surgical treatment is used prior to implantation. The potential improvement in word recognition scores decreases after age 70 years.

A Systematic Review of Clinical Vestibular Symptom Triage, Tools, and Algorithms.

OBJECTIVE: The evaluation of peripheral vestibular disorders in clinical practice is an especially difficult endeavor, particularly for the inexperienced clinician. The goal of this systematic review is thus to evaluate the design, approaches, and outcomes for clinical vestibular symptom triage and decision support tools reported in contemporary published literature. DATA SOURCES: A comprehensive search of existing literature in August 2020 was conducted using MEDLINE, CINAHL, and EMBASE using terms of desired diagnostic tools such as algorithm, protocol, and questionnaire as well as an exhaustive set of terms to encompass vestibular disorders. REVIEW METHODS: Study characteristics, tool metrics, and performance were extracted using a standardized form. Quality assessment was conducted using a modified version of the Quality of Diagnostic Accuracy Studies 2 (QUADAS-2) assessment tool. RESULTS: A total of 18 articles each reporting a novel tool for the evaluation of vestibular disorders were identified. Tools were organized into 3 discrete categories, including self-administered questionnaires, health care professional administered tools, and decision support systems. Most tools could differentiate between specific vestibular pathologies, with outcome measures including sensitivity, specificity, and accuracy. CONCLUSION: A multitude of tools have been published to aid with the evaluation of vertiginous patients. Our systematic review identified several low-evidence reports of triage and decision support tools for the evaluation of vestibular disorders.

Assessment of Sudden Sensorineural Hearing Loss After COVID-19 Vaccination.

IMPORTANCE: Emerging reports of sudden sensorineural hearing loss (SSNHL) after COVID-19 vaccination within the otolaryngological community and the public have raised concern about a possible association between COVID-19 vaccination and the development of SSNHL. OBJECTIVE: To examine the potential association between COVID-19 vaccination and SSNHL. DESIGN, SETTING, AND PARTICIPANTS: This cross-sectional study and case series involved an up-to-date population-based analysis of 555 incident reports of probable SSNHL in the Centers for Disease Control and Prevention Vaccine Adverse Events Reporting System (VAERS) over the first 7 months of the US vaccination campaign (December 14, 2020, through July 16, 2021). In addition, data from a multi-institutional retrospective case series of 21 patients who developed SSNHL after COVID-19 vaccination were analyzed. The study included all adults experiencing SSNHL within 3 weeks of COVID-19 vaccination who submitted reports to VAERS and consecutive adult patients presenting to 2 tertiary care centers and 1 community practice in the US who were diagnosed with SSNHL within 3 weeks of COVID-19 vaccination. EXPOSURES: Receipt of a COVID-19 vaccine produced by any of the 3 vaccine manufacturers (Pfizer-BioNTech, Moderna, or Janssen/Johnson & Johnson) used in the US. MAIN OUTCOMES AND MEASURES: Incidence of reports of SSNHL after COVID-19 vaccination recorded in VAERS and clinical characteristics of adult patients presenting with SSNHL after COVID-19 vaccination. RESULTS: A total of 555 incident reports in VAERS (mean patient age, 54 years [range, 15-93 years]; 305 women [55.0%]; data on race and ethnicity not available in VAERS) met the definition of probable SSNHL (mean time to onset, 6 days [range, 0-21 days]) over the period investigated, representing an annualized incidence estimate of 0.6 to 28.0 cases of SSNHL per 100 000 people per year. The rate of incident reports of SSNHL was similar across all 3 vaccine manufacturers (0.16 cases per 100 000 doses for both Pfizer-BioNTech and Moderna vaccines, and 0.22 cases per 100 000 doses for Janssen/Johnson & Johnson vaccine). The case series included 21 patients (mean age, 61 years [range, 23-92 years]; 13 women [61.9%]) with SSNHL, with a mean time to onset of 6 days (range, 0-15 days). Patients were heterogeneous with respect to clinical and demographic characteristics. Preexisting autoimmune disease was present in 6 patients (28.6%). Of the 14 patients with posttreatment audiometric data, 8 (57.1%) experienced improvement after receiving treatment. One patient experienced SSNHL 14 days after receiving each dose of the Pfizer-BioNTech vaccine. CONCLUSIONS AND RELEVANCE: In this cross-sectional study, findings from an updated analysis of VAERS data and a case series of patients who experienced SSNHL after COVID-19 vaccination did not suggest an association between COVID-19 vaccination and an increased incidence of hearing loss compared with the expected incidence in the general population.

Oral vs intratympanic corticosteroid therapy for idiopathic sudden sensorineural hearing loss: a randomized trial.

CONTEXT: Idiopathic sudden sensorineural hearing loss has been treated with oral corticosteroids for more than 30 years. Recently, many patients' symptoms have been managed with intratympanic steroid therapy. No satisfactory comparative effectiveness study to support this practice exists. OBJECTIVE: To compare the effectiveness of oral vs intratympanic steroid to treat sudden sensorineural hearing loss. DESIGN, SETTING, AND PATIENTS: Prospective, randomized, noninferiority trial involving 250 patients with unilateral sensorineural hearing loss presenting within 14 days of onset of 50 dB or higher of pure tone average (PTA) hearing threshold. The study was conducted from December 2004 through October 2009 at 16 academic community-based otology practices. Participants were followed up for 6 months. INTERVENTION: One hundred twenty-one patients received either 60 mg/d of oral prednisone for 14 days with a 5-day taper and 129 patients received 4 doses over 14 days of 40 mg/mL of methylprednisolone injected into the middle ear. MAIN OUTCOME MEASURES: Primary end point was change in hearing at 2 months after treatment. Noninferiority was defined as less than a 10-dB difference in hearing outcome between treatments. RESULTS: In the oral prednisone group, PTA improved by 30.7 dB compared with a 28.7-dB improvement in the intratympanic treatment group. Mean pure tone average at 2 months was 56.0 for the oral steroid treatment group and 57.6 dB for the intratympanic treatment group. Recovery of hearing on oral treatment at 2 months by intention-to-treat analysis was 2.0 dB greater than intratympanic treatment (95.21% upper confidence interval, 6.6 dB). Per-protocol analysis confirmed the intention-to-treat result. Thus, the hypothesis of inferiority of intratympanic methylprednisolone to oral prednisone for primary treatment of sudden sensorineural hearing loss was rejected. CONCLUSION: Among patients with idiopathic sudden sensorineural hearing loss, hearing level 2 months after treatment showed that intratympanic treatment was not inferior to oral prednisone treatment. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00097448.

The Efficient Dizziness History and Exam.

Dizziness is a common complaint in otolaryngology clinics and can present a diagnostic challenge. A thorough history including onset, duration, and exacerbating and alleviating factors, along with physician persistence, can help differentiate between otologic and nonotologic forms of dizziness. An otologic and neurotologic physical examination, including vestibulo-ocular reflex and cranial nerve function evaluation and postural examination, can shed further light on symptom etiology. Otologic forms of dizziness often result in vertigo and may be associated with unilateral symptoms of hearing loss, aural fullness, or tinnitus. Primary causes of dizziness are more often constant and insidious in onset.

Efficacy and Durability of Intratympanic Gentamicin Treatment for Meniere's Disease.

Objective: To study the success of intratympanic gentamicin (ITG) treatment in reducing vertigo attacks in Meniere's disease (MD) and the value of the Halmagyi head thrust test (HTT) in predicting treatment durability. Study Design: Retrospective cohort study. Setting: Tertiary care vestibular clinic. Patients: Unilateral MD patients treated with ITG from 2006-2019 with ≥6 months follow-up. Main Outcome Measures: Demographics, audiometric data, subjective symptomatology, and HTT results were collected. Treatment success was defined as sufficient symptom relief. Treatment failure indicated vertigo control of less than 6 months duration. Treatment relapse indicated vertigo recurrence after 6 months. Results: Of 255 patients, treatment success, failure, and relapse occurred in 226 (88.6%), 29 (11.4%), and 121 (47.1%) patients, respectively. 48 (18.8%) patients who failed to respond or relapsed underwent labyrinthectomy. Mean follow-up time was 3.7 yrs (range 0.5-12.8). After ITG treatment, 25% patients reported worse hearing; mean pure tone average (PTA) increased by 18.6 ± 11.3 dB and mean word recognition score (WRS) decreased by 33 ± 21%. Of the 148 patients with negative pre-treatment HHT, 103 (69.6%) converted to positive after ITG treatment. Mean time-to-relapse in the converted and non-converted HTT cohorts was significantly different (49.7 vs. 27.0 months, p = 0.009) even after adjusting for gender, age, laterality, duration of symptoms, and number of ITG treatments. There were no significant differences between the two groups in hearing outcomes or subjective symptoms (e.g. lingering disequilibrium). Conclusions: ITG treatment effectively reduces the number of vertigo attacks in MD. HTT is valuable in predicting durability of treatment benefit.