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Objective: The objective of this study was to explore the useful of vestibular evoked myogenic potential (VEMP) testing for detecting endolymphatic hydrops, especially in the second ear of patients with unilateral Ménière disease (MD).Methods: This study was performed at a tertiary care academic medical center. Part I consisted of postmortem temporal bone specimens from the temporal bone collection of the Massachusetts Eye & Ear Infirmary; part II consisted of consecutive consenting adult patients (n = 82) with unilateral MD by American Academy of Otolaryngology-Head and Neck Surgery criteria case histories. Outcome measures consisted of VEMP thresholds in patients and histologic saccular endolymphatic hydrops in postmortem temporal bones.Results: Saccular hydrops was observed in the asymptomatic ear in six of 17 (35%) of temporal bones from donors with unilateral MD. Clinic patients with unilateral MD showed elevated mean VEMP thresholds and altered VEMP tuning in their symptomatic ears and, to a lesser degree, in their asymptomatic ears. Specific VEMP frequency and tuning criteria were used to define a "Ménière-like" response. This "Ménière-like" response was seen in 27% of asymptomatic ears of our patients with unilateral MD.Conclusions: Bilateral involvement is seen in approximately one third of MD cases. Saccular hydrops appears to precede symptoms in bilateral MD. Changes in VEMP threshold and tuning appear to be sensitive to these structural changes in the saccule. If so, then VEMP may be useful as a detector of asymptomatic saccular hydrops and as a predictor of evolving bilateral MD. [ABSTRACT FROM AUTHOR]

Objective: To test whether detection of serum antibody to a 68-kd inner ear protein distinguishes among different causes of sensorineural hearing loss, and identifies patients with active disease and those likely to respond to corticosteroid therapy.Design: Serum samples were tested by Western blot using bovine inner ear extract as antigen, and results were correlated with patient information obtained by chart review.Setting: Referral center.Subjects Of Study: Serum samples were obtained from patients with idiopathic, progressive, bilateral sensorineural hearing loss (IPBSNHL) (n = 72) otosclerosis (n = 11), Cogan's syndrome (n = 8), patients with positive tests for antinuclear antibodies (n = 10), and normal controls (n = 53).Main Outcome Measure: Detection of serum antibody to a 68-kd inner ear protein.Results: Serum from 42 to 72 patients with IPBSNHL reacted with a 68-kd protein constituent of inner ear extract. This reactivity was not detected in serum from 11 of 11 patients with otosclerosis, or in eight of eight with Cogan's syndrome. It was found in serum from one of 10 patients with a positive test for antinuclear antibody and in one of 53 normal controls. Antibody to the 68-kd protein was detected in serum from 89% of patients with actively progressing IPBSNHL and none of the 25 patients with inactive disease (P < .001). Patients who were antibody-positive responded to steroid treatment more frequently than did those who were antibody-negative (P < .001).Conclusions: These results indicate that the presence of circulating antibody to a 68-kd constituent of bovine inner ear extract serves as a marker for IPBSNHL and that its presence correlates with disease activity and responsiveness to corticosteroid treatment. [ABSTRACT FROM AUTHOR]

Seventy-four revision stapedectomies performed consecutively over 10 years (1986 to 1995) were reviewed retrospectively. The most common intraoperative findings were incus erosion, prosthesis displacement, and oval window closure. Incus erosion was more frequently associated with multiple revisions. The postoperative results were reported using the conventional method (postoperative air minus preoperative bone) as well as the guidelines recently published by the American Academy of Otolaryngology--Head and Neck Surgery (postoperative air minus postoperative bone), with success rates of postoperative air-bone gap closure to within 10 dB after revision surgery of 51.6% and 45.6%, respectively. Patients with persistent conductive hearing loss (large residual air-bone gaps) after primary stapedectomy had poorer postrevision hearing results. Sensorineural hearing loss (defined as a drop in bone pure-tone average of more than 10 dB) occurred in four cases (5.4%). The number of revision surgeries, variations in operative techniques using laser or drill, and the ossicle to which the prosthesis was attached did not statistically affect the postoperative air-bone gaps. These results were compared with previously published data. [ABSTRACT FROM AUTHOR]

The efficacy of surgery in controlling infection in 272 tympanomastoidectomy procedures for chronic otitis media (COM) was assessed by means of a four-point rating scale that incorporated both symptoms and signs, such as the presence or absence of otorrhea and granulation tissue. Of the 272 procedures, 170 were performed for COM with cholesteatoma and 102 were for active COM with granulation tissue but no cholesteatoma. Forty-seven percent were primary procedures, and 53% were revisions. Minimum follow-up was 12 months for all cases, with a mean of 30 months. Adequate control of infection occurred in 248 (91%) of the 272 cases. Of the 24 cases (9%) that developed persistent infection, 10 were controlled with a combination of oral and topical antibiotics and/or delayed skin grafting in the office. Thus overall satisfactory control of infection was achieved in 258 of 272 cases (95%). The outcome was influenced by the diagnostic category of COM: COM with cholesteatoma did significantly better than COM with granulation tissue (P = 0.02). The outcome was not influenced by the following variables: primary versus revision surgery, canal wall-up versus canal wall-down surgery, and extent of disease. The results suggest that active COM with granulation tissue may be more difficult to control than COM with cholesteatoma. [ABSTRACT FROM AUTHOR]

Topical administration of aminoglycoside antibiotics in the middle ear can achieve "chemical labyrinthectomy" in patients with intractable Meniere's disease. Herein we report our results of intratympanic gentamicin therapy in 21 patients using two different dosing protocols, twice weekly and twice daily (b.i.d.). Both hearing and vertigo outcome were evaluated. Complete control of episodic vertigo was achieved initially in 20 of 21 patients (95.2%). However, 6 of 20 responders (30%) developed relapsing symptoms within 12 months. Retreatment was successful in 75% of these patients. Overall, hearing was preserved or improved in 62% of cases, worse in 24%, and not yet tested in 14%. When the cumulative dose of gentamicin was < or = 4 injections in the first week, only 1 of 14 (7.1%) lost hearing. Intratympanic gentamicin offers better risk/benefit outcome than other invasive therapies for intractable Meniere's disease. [ABSTRACT FROM AUTHOR]

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., (© 2024 by American Journal of Neuroradiology.)

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., (© 2024 American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

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., Competing Interests: SA is the clinical advisory board member, Frequency Therapeutics, Inc., and the consultant, Spiral Therapeutics, Inc. The remaining author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Jiam and Rauch.)

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.

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.

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.

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Guan, Chari, Liu and Rauch.)

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., Competing Interests: Disclosure The authors have no disclosures to report., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: There are no approved pharmacologic therapies for chronic sensorineural hearing loss (SNHL). The combination of CHIR99021+valproic acid (CV, FX-322) has been shown to regenerate mammalian cochlear hair cells ex vivo. The objectives were to characterize the cochlear pharmacokinetic profile of CV in guinea pigs, then measure FX-322 in human perilymph samples, and finally assess safety and audiometric effects of FX-322 in humans with chronic SNHL., Study Designs: Middle ear residence, cochlear distribution, and elimination profiles of FX-322 were assessed in guinea pigs. Human perilymph sampling following intratympanic FX-322 dosing was performed in an open-label study in cochlear implant subjects. Unilateral intratympanic FX-322 was assessed in a Phase 1b prospective, randomized, double-blinded, placebo-controlled clinical trial., Setting: Three private otolaryngology practices in the US., Patients: Individuals diagnosed with mild to moderately severe chronic SNHL (≤70 dB standard pure-tone average) in one or both ears that was stable for ≥6 months, medical histories consistent with noise-induced or idiopathic sudden SNHL, and no significant vestibular symptoms., Interventions: Intratympanic FX-322., Main Outcome Measures: Pharmacokinetics of FX-322 in perilymph and safety and audiometric effects., Results: After intratympanic delivery in guinea pigs and humans, FX-322 levels in the cochlear extended high-frequency region were observed and projected to be pharmacologically active in humans. A single dose of FX-322 in SNHL subjects was well tolerated with mild, transient treatment-related adverse events (n = 15 FX-322 vs 8 placebo). Of the six patients treated with FX-322 who had baseline word recognition in quiet scores below 90%, four showed clinically meaningful improvements (absolute word recognition improved 18-42%, exceeding the 95% confidence interval determined by previously published criteria). No significant changes in placebo-injected ears were observed. At the group level, FX-322 subjects outperformed placebo group in word recognition in quiet when averaged across all time points, with a mean improvement from baseline of 18.9% (p = 0.029). For words in noise, the treated group showed a mean 1.3 dB signal-to-noise ratio improvement (p = 0.012) relative to their baseline scores while placebo-treated subjects did not (-0.21 dB, p = 0.71)., Conclusions: Delivery of FX-322 to the extended high-frequency region of the cochlea is well tolerated and enhances speech recognition performance in multiple subjects with stable chronic hearing loss., Competing Interests: No other authors have conflicts of interest., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc.)

Objective: Cognitive symptoms in patients with vestibular disorders are far from rare, but identification of patients at risk for cognitive impairment remains poor. The Dizziness Handicap Inventory (DHI) is a widely used patient-reported outcome questionnaire for dizzy patients with several questions that address cognitive function. However, the relationship between subjective cognitive symptomatology in patients with vestibular disorders and performance on DHI is poorly characterized., Study Design: Retrospective cohort study., Setting: Tertiary care vestibular clinic., Subjects: Individuals with diagnoses of vestibular migraine (VM), Menière's disease (MD), and concomitant vestibular migraine and Menière's disease (VMMD) presenting to clinic between January 2007 and December 2019., Results: Of 761 subjects, 365 had VM, 311 had MD, and 85 had VMMD. Symptoms of brain fog and chronic fatigue occurred more frequently in the VM and VMMD groups compared with the MD group (χ2 (df = 2, n = 761) = 67.8, 20.9, respectively, p < 0.0001). DHI scores were significantly higher in patients with VM and VMMD compared with those with MD (F[2,758] = 63.5, p < 0.001). A DHI score ≥ 13 suggested that the patient suffered from brain fog (sensitivity = 47.4%, specificity = 72.3%), whereas a score ≥ 15 indicated that the patient complained of chronic fatigue (sensitivity = 81.5%, specificity = 57.7%)., Conclusion: Our results indicate a high prevalence of interictal cognitive symptoms in patients with episodic vestibular disorders. The DHI cannot be reliably used to differentiate between vestibular disorders nor detect cognitive impairment in these patient populations. Alternative methods must be used to identify vestibulopathic patients with cognitive symptoms to initiate strategies for prevention and treatment., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2020, Otology & Neurotology, Inc.)

Objective: To characterize presurgical symptoms and treatment history and postoperative course in patients with medically recalcitrant Menière's disease undergoing transmastoid labyrinthectomy in the post-intratympanic gentamicin era., Study Design: Retrospective case series., Setting: Tertiary academic medical center., Patients: All patients who underwent transmastoid labyrinthectomy for medically recalcitrant Menière's disease in 2003 to 2019 by the senior author., Interventions: Review of patients' medical records for: preoperative history of drop attacks, gentamicin injections, endolymphatic sac decompression or vestibular neurectomy, preoperative audiograms, length of hospital stay, postoperative complications, and persistent symptoms or challenging recovery., Main Outcome Measures: Presurgical clinical history and proximal postoperative outcomes., Results: Seventy-two patients with a mean age of 56.7 (standard deviation [SD] 10.7) were included. All cases were unilateral. Forty-three patients (59.7%) suffered from drop attacks. Sixty-two (86.1%) had failed sufficient symptom control with gentamicin injections. The mean preoperative word recognition score was 36.4% (SD 23.7) versus 95.1% (SD 8.5) in the contralateral ear. The mean pure-tone average (PTA) of the ipsilateral ear before surgery was 65.5 dB (SD 18.0) versus 16.2 (SD 13.5) for the contralateral ear. Mean hospital stay was 2.0 days (SD 0.87 days, range of 1-5 d). Three patients (4.2%) had prolonged postoperative vertigo., Conclusions: Transmastoid labyrinthectomy at our center is performed for unilateral Menière's disease, generally when intratympanic gentamicin has failed. A majority of surgical patients suffer from drop attacks preoperatively. Hospital stay is typically brief.

As a result of the COVID-19 pandemic, telemedicine has been thrust to the forefront of health care. Despite its inherent limitations, telemedicine offers many advantages to both patient and physician as an alternative to in-person evaluation of select patients. In the near term, telemedicine allows nonpandemic care to proceed while observing appropriate public health concerns to minimize the spread of pandemic pathogens. Thus, it behooves practitioners to use telemedicine consultations for common otolaryngology complaints. Assessment of the dizzy patient is well-suited to an algorithmic approach that can be adapted to a telemedicine setting. As best practices for telemedicine have yet to be defined, we present herein a practical approach to the history and limited physical examination of the dizzy patient in the telemedicine setting for the general otolaryngologist. Indeed, once the acute crisis has abated, we suspect that this approach will continue to be an effective way to manage dizzy patients.

Superior semicircular canal dehiscence is a bony defect of the superior semicircular canal, which can lead to a variety of auditory and vestibular symptoms. The diagnosis of superior semicircular canal dehiscence (SCD) can be challenging, time consuming, and costly. The clinical presentation of SCD patients resembles that of other otologic disease, necessitating objective diagnostics. Although temporal bone CT imaging provides excellent sensitivity for SCD detection, it lacks specificity. Because the treatment of SCD is surgical, it is crucial to use a highly specific test to confirm the diagnosis and avoid false positives and subsequent unnecessary surgery. This review provides an update on recent improvements in vestibular evoked myogenic potential (VEMP) testing for SCD diagnosis. Combining audiometric and conventional cervical VEMP results improves SCD diagnostic accuracy. High frequency VEMP testing is superior to all other methods described to date. It is highly specific for the detection of SCD and may be used to guide decision-making regarding the need for subsequent CT imaging. This algorithmic sequential use of testing can substantially reduce radiation exposure as well as cost associated with SCD diagnosis., (Copyright © 2020 Noij and Rauch.)

Objectives/hypothesis: Few studies have specifically addressed auditory complaints in patients with nonblast mild traumatic brain injury (mTBI). Herein, we aimed to investigate auditory symptoms in patients following mTBI using patient-reported outcome measures., Study Design: Retrospective analysis of prospectively collected data in a tertiary-care hospital., Methods: The patients included those with mTBI (cases) and those without mTBI (controls). Individuals (≥18 years old) with and without mTBI were screened. Exclusion criteria included history of otologic disorders, blast injury, or occupational noise exposure. Primary outcomes included the Hearing Handicap Inventory for Adults (HHIA), Tinnitus Handicap Inventory (THI), and Hyperacusis Questionnaire (HQ). Secondary outcomes included subjective auditory complaints., Results: From September 2017 to September 2018, 52 patients with mTBI and 55 controls met inclusion and exclusion criteria. The mean time between mTBI and survey intake was 70.6 months. The mean age and gender were 51.5 years old and 73% female in the mTBI group, and 46.1 years old and 56.3% female in the control group (P = .112 and P = .105, respectively). Patients with mTBI reported hyperacusis (67.3% of all mTBI patients), hearing loss (61.5%), and tinnitus (61.5%), compared to 8.3%, 12.7%, and 16.4%, respectively, for control subjects (P < .0001). The mean HHIA score in the mTBI group was 38.3 versus 8.5 in controls (P = .002). The mean THI score was 27.4 in the mTBI group and 3.1 in controls (P < .0001). The mean HQ score was 26.5 in mTBI group and 7.3 in controls (P = .001)., Conclusions: Auditory symptoms and associated handicap were common in patients with nonblast mTBI compared to age-matched controls. Findings have implications for the pathophysiology and management of symptoms in this patient population., Level of Evidence: 3 Laryngoscope, 130:761-767, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: Vestibular symptoms are a common sequela of temporal bone fractures (TBFs). The mechanisms of injury to the peripheral vestibular system following TBF, however, are not well described. Herein, we aimed to investigate the histopathology of the peripheral vestibular system in patients who sustained TBFs., Study Design: Retrospective human specimen analysis., Methods: Specimens from the National Temporal Bone Pathology Registry with (cases) and without (controls) TBFs were evaluated. Specimens were analyzed by light microscopy for vestibular hair cell and/or dendritic degeneration, presence of endolymphatic hydrops, blockage of the endolymphatic duct, and number of Scarpa ganglion cells (ScGCs) in the superior and inferior vestibular nerves., Results: Seven temporal bones (TBs) from five individuals with TBFs, and seven TBs from six age-matched individuals without a history of head injury met inclusion and exclusion criteria. All fractures involved the otic capsule. Severe degeneration of the cristae was identified in the semicircular canals in all TBF cases. The utricular and saccular maculae showed mild to severe degeneration in the TBF cases. Vestibular hydrops (n = 2 TBs) and blockage of the endolymphatic duct (n = 3 TBs) were also present in the TBF cases. There was a decrease of 52.6% in the mean total ScGC count in the TBF cases (n = 3 TBs) compared to age-matched controls (n = 7 TBs, P = .015). There was a mean loss of 53% of the ScGCs in the superior vestibular nerve and a mean loss of 52.3% of the ScGCs in the inferior vestibular nerve compared to age-matched controls (P = .033 and P = .021, respectively)., Conclusions: In a cohort of patients with TBFs, there were distinct peripheral vestibular changes including reduction of ScGCs., Level of Evidence: NA Laryngoscope, 130:752-760, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To investigate if the cervical vestibular evoked myogenic potential (cVEMP) is predictive for developing bilateral Menière's disease (MD)., Study Design: Retrospective cohort study., Setting: Tertiary care center., Patients: Records of 71 patients previously diagnosed with unilateral MD at our institution who underwent cVEMP testing between 2002 and 2011 were screened., Intervention: Patients were contacted to answer a questionnaire to identify which patients had developed bilateral disease. Based on questionnaires and medical charts, 49 patients with a follow-up time of at least 5 years were included. The 49 originally asymptomatic ears are referred to as "study ears." Previously reported cVEMP criteria (original criteria) applied to study-ear cVEMPs separated them into Menière-like and normal-like groups., Main Outcome Measure: The main purpose was to determine if previously obtained cVEMP thresholds and tuning ratios of unilateral MD patients could predict who develops bilateral disease., Results: From the 49 included patients, 12 developed bilateral disease (24.5%). The study ears characterized by original cVEMP criteria as Menière-like were significantly more likely to develop bilateral disease compared with the normal-like study ears. The original criteria predicted development of bilateral disease with a positive predictive value (PPV) and negative predictive value (NPV) of 58.3% and 86.5% respectively. ROC curves were used to revise cVEMP criteria for predicting the progression to bilateral disease. A revised criterion combining three cVEMP metrics, reached a PPV and NPV of 85.7% and 93.7%., Conclusion: cVEMP threshold and tuning in unilateral MD patients are predictive of which patients will develop bilateral disease.

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.

Hypothesis: The vestibular aqueduct (VA) in Menière's disease (MD) exhibits different angular trajectories depending on the presenting endolymphatic sac (ES) pathology, i.e., 1) ES hypoplasia or 2) ES degeneration., Background: Hypoplasia or degeneration of the ES was consistently found in inner ears affected by MD. The two etiologically distinct ES pathologies presumably represent two disease "endotypes," which may be associated with different clinical traits ("phenotypes") of MD. Recognizing these endotypes in the clinical setting requires a diagnostic tool., Methods: 1) Defining the angular trajectory of the VA (ATVA) in the axial plane. 2) Measuring age-dependent normative data for the ATVA in postmortem temporal bone histology material from normal adults and fetuses. 3) Validating ATVA measurements from normative CT imaging data. 4) Correlating the ATVA with different ES pathologies in histological materials and CT imaging data from MD patients., Results: 1) The ATVA differed significantly between normal adults and MD cases with ES degeneration, as well as between fetuses and MD cases with ES hypoplasia; 2) a strong correlation between ATVA measurements in histological sections and CT imaging data was found; 3) a correlation between the ATVA, in particular its axial trajectory in the opercular region (angle αexit), with degenerative (αexit < 120°) and hypoplastic ES pathology (αexit > 140°) was demonstrated., Conclusion: We established the ATVA as a radiographic surrogate marker for ES pathologies. CT-imaging-based determination of the ATVA enables endotyping of MD patients according to ES pathology. Future studies will apply this method to investigate whether ES endotypes distinguish clinically meaningful subgroups of MD patients.

Objective: The cervical vestibular evoked myogenic potential (cVEMP) has been used to evaluate patients with Menière's disease (MD). Studied cVEMP metrics include: amplitude, threshold, frequency tuning, and interaural asymmetry ratio (IAR). However, few studies compared these metrics in the same set of MD patients, and methodological differences prevent such a comparison across studies. This study investigates the value of different cVEMP metrics in distinguishing one set of MD patients from age-matched controls., Study Design: Prospective study., Setting: Tertiary care center., Patients: Thirty patients with definite unilateral MD and 23 age-matched controls were prospectively included. All underwent cVEMP testing at 500, 750, 1000, and 2000 Hz on each side. Ears were separated into three groups: affected MD, unaffected MD, and control., Main Outcome Measures: Sound level functions were obtained at each frequency, and normalized peak-to-peak amplitude (VEMPn), VEMP inhibition depth (VEMPid), threshold, frequency-tuning ratio, and IAR were calculated. For all metrics, the differentiation between MD and control ears was compared using receiver operating characteristic (ROC) curves., Results: 500 Hz cVEMP threshold, VEMPn, and VEMPid were similarly good at distinguishing affected MD ears from healthy ears, with ROC area under the curves (AUCs) of more than 0.828 and optimal sensitivities and specificities of at least 80 and 70%. Combinations of these three metrics yielded slightly larger AUCs (>0.880). Tuning ratios and IAR were less effective in separating healthy from affected ears with AUCs ranging from 0.529 to 0.720., Conclusion: The cVEMP metrics most useful in distinguishing MD patients from healthy controls are threshold, VEMPn, and VEMPid, using 500 Hz stimuli.

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.

Objective: Vestibular symptoms such as dizziness and vertigo are common after head injury and may be due to trauma to the peripheral vestibular system. The pathophysiology of peripheral vestibular symptoms following head injury without temporal bone (TB) fracture, however, is not well understood. Herein, we investigate the histopathology of the peripheral vestibular system of patients who sustained head injury without a TB fracture., Study Design: Otopathology study., Setting: Otopathology laboratory., Subjects and Methods: TB of subjects with a history of head injury without TB fractures were included and evaluated by light microscopy. Specimens were assessed for qualitative and quantitative characteristics, such as number of Scarpa's ganglion cells in the superior and inferior vestibular nerves, vestibular hair cell and/or dendrite degeneration in vestibular end organs, presence of vestibular hydrops, and obstruction of the endolymphatic duct., Results: Five cases (n = 5 TBs) had evidence of vestibular pathology. There was a decrease of 48.6% (range, 40%-59%) in the mean count of Scarpa's ganglion cells as compared with that of normative historical age-matched controls. Moderate to severe degeneration of the vestibular membranous labyrinth was identified in the posterior, superior, and lateral canals in several cases (50%, n = 4 TBs). The maculae utriculi and sacculi showed mild to severe degeneration in 2 cases. Additional findings include vestibular hydrops (25%, n = 2 TBs) and blockage of the endolymphatic duct (n = 1 TB)., Conclusions: Otopathologic analysis of patients with a history of head injury without TB fracture demonstrated peripheral vestibular otopathology. Future studies are necessary to determine if otopathology findings are directly attributable to head injury.

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.

The development of therapies for the inner ear presents unique opportunities and challenges. On the one hand, the ear presents an opportunity for localized drug delivery to avoid systemic side effects. However, we do not understand the pathobiology of many common ear disorders clearly enough to develop rational therapeutic solutions. Further, identification of biomarkers beyond conventional audiometry and balance testing to track disease progress and recovery remain elusive. Because of the comparatively low incidence and prevalence of many inner ear disorders, as well as issues with respect to timing of drug delivery for certain diseases, multi-center, multi-investigator collaborative networks are required to promote effective clinical trial design., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objective: Evaluate the relationship between objective audiometric and vestibular tests and patient symptoms in superior canal dehiscence (SCD) syndrome., Study Design: Retrospective chart review., Setting: Tertiary care center., Patients: Ninety-eight patients with SCD, preoperative threshold audiograms, cervical vestibular evoked myogenic potential (cVEMP) thresholds, and computed tomography (CT) imaging were included. Clinical reports were reviewed for self-reported SCD symptoms. Twenty-five patients completed the Hearing Handicap Inventory (HHI), Dizziness Handicap Inventory (DHI), Autophony Index (AI), and the 36-item Short Form Survey (SF-36)., Main Outcome Measures: Correlations between preoperative low-frequency air-bone gap (ABG), cVEMP thresholds, and symptoms (including HHI, DHI, AI, and SF-36). Symptoms included hearing loss, aural fullness, autophony, hyperacusis, tinnitus, vertigo, imbalance and sound-, pressure and exercise provoked dizziness. Secondary outcome measure: Correlations between changes of objective and subjective measures before and after surgery., Results: Patients who reported hearing loss had larger ABGs at 250 Hz than patients without subjective hearing loss (p = 0.001). ABGs and cVEMP thresholds did not correlate with any other symptom. No significant correlation was found between ABG or cVEMP threshold and the HHI, DHI, AI or Health Utility Value (derived from the SF-36 quality of life score). Following SCD surgery, ABG decreased (p < 0.001), cVEMP thresholds increased (p < 0.001) and overall symptoms, handicap scores and quality-of-life improved; however, there was no significant relationship between these measures., Conclusion: While threshold audiometry and cVEMP are important tools to diagnose SCD and monitor surgical outcomes, these measures showed no significant correlation with vestibular and most auditory symptoms or their severity.

Objective: The standard evaluation of patients with suspected Menière's disease (MD) includes initial imaging to rule out tumors of the temporal bone. Few guidelines, however, advocate sequential imaging. We propose that sequential imaging may reveal other etiologies of auditory and vestibular symptoms as demonstrated in a patient with an endolymphatic sac tumor that was originally diagnosed Menière's after initial imaging., Patients: One patient with MD and initially unremarkable imaging. Repeat imaging several years after diagnosis after additional symptoms demonstrated interval development of an endolymphatic sac tumor (ELST)., Interventions: Resection of endolymphatic sac tumor., Main Outcome Measures: 1) Audiometry, 2) temporal bone imaging, and 3) otopathology RESULTS:: A 45-year-old man with diagnosis of asymmetric sensorineural hearing loss and intermittent vertigo underwent temporal bone magnetic resonance imaging that did not demonstrate any causative lesions. After an episode of sudden sensorineural hearing loss 4 years after initial presentation, repeat imaging was obtained. Magnetic resonance imaging and surgical resection confirmed diagnosis of ELST. The patient had no history of von Hippel-Lindau disease., Conclusions: A patient with a longstanding diagnosis of MD demonstrated interval development of an ELST. While ELSTs are rare, the study raises the question regarding whether interval imaging is indicated in patients with MD.

Objective: To develop a novel approach combining low-frequency air-bone gap (ABG) and cervical vestibular evoked myogenic potential (cVEMP) thresholds to improve screening for superior canal dehiscence (SCD) syndrome., Study Design: Retrospective study., Setting: Tertiary care center., Patients: One hundred forty patients with SCD and 21 healthy age-matched controls were included. Ears for each patient were divided into three groups based on computed tomography (CT) findings: 1) dehiscent, 2) thin, or 3) unaffected., Main Outcome Measures: cVEMP and audiometric thresholds were analyzed and differences among groups were evaluated., Results: We define the third window indicator (TWI) as the cVEMP thresholds at 500, 750, and 1000 Hz adjusted for the ABG at 250 Hz (i.e., subtracting ABG from cVEMP threshold). The TWI differentiates between dehiscent and nondehiscent control ears with a sensitivity of 82% and specificity of 100%, corresponding to a positive predictive value of 100%. ABGs and cVEMP thresholds were similar for healthy controls and patients with thin bone over the superior canal., Conclusion: This is the largest study to date examining the usefulness of cVEMPs in the diagnosis of SCD. Our "third window indicator" (TWI) combines cVEMP thresholds with the ABG at 250 Hz to improve the ability to screen patients with SCD symptoms.

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., (© 2019 The Author(s) Published by S. Karger AG, Basel.)

Objective: To compare the efficacy of intratympanic gentamicin injection (ITG) on vertigo control, drop attacks, and functional level in Menière's disease patients with and without a history of migraine., Patients: Menière's disease patients (patients with migraine and age- and sex-matched control patients without migraine) treated from 2002 to 2012 who failed medical management and received ITG, with a minimum 2-year follow up., Intervention: ITG., Main Outcome Measures: Vertigo control, drop attack prevalence, and change in functional level., Results: Twenty-eight Menière's disease patients were included in this study (14 with migraine and 14 matched patients without migraine). There were three men and 11 women in each groups, with a mean age of 53 ± 8.9 years. Baseline characteristics (Menière's stage, vertigo frequency, drop attack prevalence, and functional level) before ITG were not significantly different between the two groups. Two years after ITG, 71% of Menière's disease with migraine patients and 78% of Menière's disease without migraine patients had class A, B, or C vertigo control (p > 0.999). Change in prevalence of drop attacks (43-7% versus 50-0%, respectively) was also similar (p > 0.999). However, significantly fewer vertigo control class A, B, and C migraine patients achieved functional level 1 or 2 (40%) compared with non-migraine patients (91%) (p = 0.007)., Conclusions: Although ITG appears equally effective in treating vertigo and drop attacks in Menière's disease with and without migraine, patients with migraine derive significantly less benefit in terms of functional improvement.

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., (© 2018 S. Karger AG, Basel.)

Objective: To assess the cervical vestibular evoked myogenic potentials (cVEMPs) ability to track disease progression in Ménière's disease patients over time and identify the most sensitive outcome measurement., Study Design: Retrospective., Setting: Large specialty hospital, department of otolaryngology., Subjects: Twenty nine Ménière's patients and seven migraine associated vertigo (MAV) patients., Intervention: All patients underwent two cervical vestibular evoked myogenic potential tests at 250, 500, 750, and 1000 Hz with a minimum test interval of 3 months., Main Outcome Measures: Threshold, peak-to-peak (PP) amplitude, interaural asymmetry ratio, and effect size., Results: In affected Ménière's ears all outcome measures were worse during the second test, for threshold this difference was statistically significant at 750 and 1000 Hz compared with the first test. Compared with young healthy ears the threshold was significantly worse at all frequencies. PP amplitude was significantly decreased at the second test at 750 Hz compared with the first test. In MAV no significant difference between tests was found at any frequency in PP amplitude or threshold. In Ménière's ears, threshold showed a higher first-to-second effect size at 500, 750, and 1000 Hz compared with PP amplitude., Conclusion: cVEMP is able to track progression in Ménière's disease over time. Thresholds were the most effective outcome measure to both track progression and to distinguish between MAV and Ménière's patients.

Objective: Assessing the effect of a higher stimulation rate in cervical vestibular evoked myogenic potential (cVEMP) outcome measurements., Study Design: Prospective cohort study., Setting: Large specialty hospital, Department of otolaryngology., Subjects: Eleven healthy subjects were used in this study., Intervention: All subjects underwent a cervical vestibular evoked myogenic potential test at 500, 750, and 1000 Hz using 5 and 13 Hz stimulation rates., Main Outcome Measures: Threshold, peak-to-peak (PP) amplitude, and interaural asymmetry ratio (IAR)., Results: PP cVEMP amplitudes were larger at 5/s than at 13/s. The 5/s to 13/s differences were statistically significant at 500 and 750 Hz (p < 0.02). The coefficient of variation in PP amplitudes across subjects, was not significantly different at any frequency for 5/s versus 13/s stimuli. No significant difference was found in the IAR at any frequency. The cVEMP thresholds were similar between stimulation rates., Conclusion: In healthy young subjects no significant differences in threshold were found between 5/s and 13/s stimulation rates, though intrasubject PP amplitude was significantly lower at the faster stimulation rate. Increasing stimulation rate reduces test time and burden. Additional studies are needed to verify that these conclusions are also true for patients.

Objective: Test performance of a focused dizziness questionnaire's ability to discriminate between peripheral and nonperipheral causes of vertigo., Study Design: Prospective multicenter., Setting: Four academic centers with experienced balance specialists., Patients: New dizzy patients., Interventions: A 32-question survey was given to participants. Balance specialists were blinded and a diagnosis was established for all participating patients within 6 months., Main Outcomes: Multinomial logistic regression was used to evaluate questionnaire performance in predicting final diagnosis and differentiating between peripheral and nonperipheral vertigo. Univariate and multivariable stepwise logistic regression were used to identify questions as significant predictors of the ultimate diagnosis. C-index was used to evaluate performance and discriminative power of the multivariable models., Results: In total, 437 patients participated in the study. Eight participants without confirmed diagnoses were excluded and 429 were included in the analysis. Multinomial regression revealed that the model had good overall predictive accuracy of 78.5% for the final diagnosis and 75.5% for differentiating between peripheral and nonperipheral vertigo. Univariate logistic regression identified significant predictors of three main categories of vertigo: peripheral, central, and other. Predictors were entered into forward stepwise multivariable logistic regression. The discriminative power of the final models for peripheral, central, and other causes was considered good as measured by c-indices of 0.75, 0.7, and 0.78, respectively., Conclusion: This multicenter study demonstrates a focused dizziness questionnaire can accurately predict diagnosis for patients with chronic/relapsing dizziness referred to outpatient clinics. Additionally, this survey has significant capability to differentiate peripheral from nonperipheral causes of vertigo and may, in the future, serve as a screening tool for specialty referral. Clinical utility of this questionnaire to guide specialty referral is discussed.

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.

Clinical vignette: A 51-year-old man with right-sided sudden hearing loss presents to the otology clinic. He has a 4-year history of episodic vertigo of several hours' duration and fluctuating, progressive sensorineural hearing loss in his left ear. The vertigo attacks have not occurred for the last 18 months, and the left ear hearing is consistently poor. The patient's right ear hearing has dropped in the last 36 hours. MRI imaging of brain and temporal bone are normal. A 2-week "burst and taper" of oral prednisone is administered with no effect. Over the next 3 months, serial audiograms show rapidly progressive loss of threshold and word recognition scores on the right side. A trial of high-dose prednisone (60 mg/d for 30 days) results in full recovery of the right ear hearing and substantial improvement in the left ear. As the prednisone dose is slowly tapered over several months, the hearing drops again.