Cochlear Implantation in Patients With Osteolytic Labyrinthitis: A Case Series.

OBJECTIVE: To describe the rare process of osteolytic labyrinthitis, previously referred to as labyrinthine sequestrum, which involves progressive obliteration of the bony and membranous labyrinth with eventual supplantation with soft tissue and, in some cases, bony sequestrum. PATIENTS: Three patients with diverse presentations of osteolytic labyrinthitis from two tertiary care academic medical centers. INTERVENTIONS: Case series report analyzing the relevant clinical, radiologic, pathologic, and surgical data on our patients with osteolytic labyrinthitis and comparing these index cases to the existing literature. MAIN OUTCOME MEASURES: We describe the varying image findings seen in osteolytic labyrinthitis on computed tomography and magnetic resonance imaging. Also, we report successful surgical intervention and hearing rehabilitation with cochlear implantation in patients with osteolytic labyrinthitis. RESULTS: Our three patients presented with profound sudden sensorineural hearing loss and vertigo consistent with labyrinthitis. None of the three patients had a history of chronic otitis media. Imaging workup revealed varying degrees of erosion to the otic capsule bone demonstrating the spectrum of disease seen in osteolytic labyrinthitis. Although two cases showed osteolytic changes to the semicircular canals and vestibule, the first case revealed frank bony sequestrum within the obliterated labyrinth. The three cases were taken for surgical debridement and cochlear implantation. CONCLUSIONS: We propose the new term, osteolytic labyrinthitis-previously referred to as labyrinthine sequestrum-to describe the rare spectrum of disease characterized by destruction of the osseous and membranous labyrinth and potential supplantation with bony sequestrum. Cochlear implantation is a viable option in selected patients with osteolytic labyrinthitis.

A Proposed Mechanism for Visual Vertigo: Post-Concussion Patients Have Higher Gain From Visual Input Into Subcortical Gaze Stabilization.

Purpose: Post-concussion syndrome (PCS) is commonly associated with dizziness and visual motion sensitivity. This case-control study set out to explore altered motion processing in PCS by measuring gaze stabilization as a reflection of the capacity of the brain to integrate motion, and it aimed to uncover mechanisms of injury where invasive subcortical recordings are not feasible. Methods: A total of 554 eye movements were analyzed in 10 PCS patients and nine healthy controls across 171 trials. Optokinetic and vestibulo-ocular reflexes were recorded using a head-mounted eye tracker while participants were exposed to visual, vestibular, and visuo-vestibular motion stimulations in the roll plane. Torsional and vergence eye movements were analyzed in terms of slow-phase velocities, gain, nystagmus frequency, and sensory-specific contributions toward gaze stabilization. Results: Participants expressed eye-movement responses consistent with expected gaze stabilization; slow phases were fastest for visuo-vestibular trials and slowest for visual stimulations (P < 0.001) and increased with stimulus acceleration (P < 0.001). Concussed patients demonstrated increased gain from visual input to gaze stabilization (P = 0.005), faster slow phases (P = 0.013), earlier nystagmus beats (P = 0.003), and higher relative visual influence over the gaze-stabilizing response (P = 0.001), presenting robust effect sizes despite the limited population size. Conclusions: The enhanced neural responsiveness to visual motion in PCS, combined with semi-intact visuo-vestibular integration, presented a subcortical hierarchy for altered gaze stabilization. Drawing on comparable animal trials, findings suggest that concussed patients may suffer from diffuse injuries to inhibiting pathways for optokinetic information, likely early in the visuo-vestibular hierarchy of sensorimotor integration. These findings offer context for common but elusive symptoms, presenting a neurological explanation for motion sensitivity and visual vertigo in PCS.

Differentiation of peripheral and non-peripheral etiologies in children with vertigo/dizziness: The video-head impulse test and suppression head impulse paradigm.

OBJECTIVES: To identify the etiology of vertigo/dizziness and determine the effectiveness of the video-head impulse test (vHIT) and the suppression head impulse paradigm (SHIMP) tests in distinguishing between peripheral and non-peripheral etiologies in children who presented to the otolaryngology department with complaints of vertigo/dizziness. METHODS: The vHIT and SHIMP tests were applied to the children. The vestibulo-ocular reflex (VOR) gain and saccade parameters were compared. RESULTS: In 27 children presenting with vertigo/dizziness, the most common etiological factor was inner ear malformation (IEM) (n = 6/27, 22.2%), followed by cochlear implant surgery (11.1%) and migraine (11.1%). Vestibular hypofunction was indicated by the vHIT results at a rate of 60% (9/15 children) and SHIMP results at 73.3% (11/15 children) among the children with a peripheral etiology, while these rates were 8.3% (1/12 children) and 25% (3/12 children), respectively, in the non-peripheral etiology group. SHIMP-VOR and vHIT-VOR gain values had a moderate positive correlation (p = 0.01, r = 0.349). While there were overt/covert saccades in the vHIT, anti-compensatory saccade (ACSs) were not observed in the SHIMP test (p = 0.041). The rates of abnormal vHIT-VOR gain (p = 0.001), over/covert saccades (p = 0.019), abnormal vHIT response (p = 0.014), ACSs (p = 0.001), and abnormal SHIMP response (p = 0.035) were significantly higher in the peripheral etiology group. CONCLUSIONS: IEM was the most common etiological cause, and the rate of vestibular hypofunction was higher in these children with peripheral vertigo. vHIT and SHIMP are effective and useful vestibular tests for distinguishing peripheral etiology from non-peripheral etiology in the pediatric population with vertigo/dizziness. These tests can be used together or alone, but the first choice should be the SHIMP test, considering its short application time (approximately 4-5 min) and simplicity.

Vestibular migraine: an update.

PURPOSE OF REVIEW: We performed a narrative review of the recent findings in epidemiology, clinical presentation, mechanisms and treatment of vestibular migraine. RECENT FINDINGS: Vestibular migraine is an underdiagnosed condition that has a high prevalence among general, headache and neuro-otology clinics. Vestibular migraine has a bimodal presentation probably associated with a hormonal component in women. These patients could have a complex clinical phenotype including concomitant autonomic, inflammatory or connective tissue conditions that have a higher prevalence of psychological symptoms, which may mistakenly lead to a diagnosis of a functional neurological disorder. A high proportion of patients with postural perceptual persistent dizziness have a migraine phenotype. Independently of the clinical presentation and past medical history, patients with the vestibular migraine phenotype can respond to regular migraine preventive treatments, including those targeting the calcitonin gene-related peptide pathways. SUMMARY: Vestibular migraine is an underdiagnosed migraine phenotype that shares the pathophysiological mechanisms of migraine, with growing interest in recent years. A thorough anamnesis is essential to increase sensitivity in patients with unknown cause of dizziness and migraine treatment should be considered (see supplemental video-abstract).

Hearing loss in Takayasu's arteritis.

OBJECTIVES: Neurosensory hearing loss is well-documented in chronic autoimmune conditions such as systemic lupus erythematosus (SLE). However, the literature lacks data on the prevalence and characteristics of hearing impairment in Takayasu's arteritis (TAK). In this cross-sectional study, our principal objective was to systematically assess the auditory function of individuals diagnosed with TAK, against SLE patients and healthy controls (HC). METHODS: Age and gender matched TAK and SLE patients followed up in a tertiary centre along with healthy controls were included in a two-phase study. In the first phase, a questionnaire on ENT symptoms was administered to the patient (TAK: n=104 and SLE: n= 151) and HC (n=174) groups. In the second phase, patients (TAK: n=53 and SLE: n=33) and HC (n=45) underwent audiometric tests. RESULTS: The questionnaire survey revealed that both TAK and SLE patients reported hearing loss (27.9%, 25.8%, 7.4%, p<0.001), tinnitus (49%, 35.8%, 13.8%, p<0.001) and vertigo (46.2%, 33.8%, 16.7%, p<0.001) at significantly higher rates than HC. Audiometry results indicated that both TAK (30.2%) and SLE patients (18.2%) had increased hearing loss compared to HC (8.9%), however, only TAK patients were found to have significantly increased risk in age adjusted logistic regression analysis (OR= 3.915, 95%CI: 1.179-12.998, p=0.026). Hearing loss was mainly neurosensory in all groups. TAK patients were affected at both low (<6000 Hz) and high (>6000 Hz) frequencies, whereas SLE patients were affected only at high frequencies. Hearing loss was significantly associated only with older age. No association was observed with the anatomical location of vascular involvement or history of stroke. CONCLUSIONS: Our study reveals an increased prevalence of hearing loss in TAK. Further research is crucial to uncover the underlying causes.

Usefulness of thermography for differentiating Wallenberg's syndrome from noncentral vertigo in the acute phase.

OBJECTIVES: Wallenberg's syndrome (WS) is caused by a stroke in the lateral medulla and can present with various symptoms. One of the main symptoms is vertigo, which can be misdiagnosed as noncentral vertigo (NCV). Approximately 90% of the patients with acute WS have a lateral difference in body surface temperature (BST) due to autonomic pathway disturbances from infarction. Additionally, thermography can aid in WS diagnosis; however, whether BST differences occur in patients with acute NCV is unclear. METHODS: This study used thermography to measure the BST of patients with NCV and acute WS to determine the effectiveness of BST to differentiate between the conditions. Forty-eight consecutive patients diagnosed with NCV whose BST was measured using thermography during a hospital visit or admission were enrolled. The left and right BST of four sites (face, trunk, and upper and lower limbs) were measured and compared with obtained BST of nine patients with WS. RESULTS: Twenty-two patients had lateral differences in BST ≥ 0.5°C, three with ≥1.5°C, and none with ≥2.5°C. Only one patient with NCV had lateral differences in BST at two or more ipsilateral sites. When WS differentiated from NCV, a left-right difference ≥0.5°C in two or more ipsilateral sites had a sensitivity of 89% and specificity of 98%, and ≥1.0°C had a sensitivity of 78% and specificity of 98%. DISCUSSION: Acute WS can be differentiated from NCV through BST and the number of sites with lateral differences via thermography, even in rooms where conditions are unregulated.

Differences in semicircular canal function in the video head impulse test in patients in the chronic stage of sudden sensorineural hearing loss with vertigo and vestibular neuritis.

BACKGROUND: Sudden sensorineural hearing loss with vertigo (SHLV) and vestibular neuritis (VN) can result in prolonged dizziness. OBJECTIVES: This study aimed to compare the video head impulse test (vHIT) of patients with SHLV and VN. METHODS: Fifteen patients with SHLV and 21 patients with VN who visited the Vertigo/Dizziness Center of our hospital between December 2016 and February 2023 were included. vHIT was performed at the time of admission, and the VOR gain and catch up saccade (CUS) in the three types of semicircular canals (SCCs) were analyzed. RESULTS: Pathologic vHIT results were observed most frequently in the posterior SCC (73%), followed by lateral (53%) and anterior (13%) SCCs in the SHLV group. In contrast, pathologic vHIT results were observed most frequently in the lateral SCC (100%), followed by the anterior (43%) and posterior SCC (24%) SCCs in the VN group. Pathological vHIT results in the lateral and posterior SCC showed significant differences between the two groups, but for anterior SCC, no significant differences were found. CONCLUSIONS AND SIGNIFICANCE: Comparison of the two vHIT results revealed differences in the SCC dysfunction patterns. This may be due to the different pathophysiological mechanisms of the two vestibular disorders, which may result in prolonged vertigo.

Vestibular Decompensation Following COVID-19 Infection in a Person With Compensated Unilateral Vestibular Loss: A Rehabilitation Case Study.

BACKGROUND AND PURPOSE: Surgical removal of a vestibular schwannoma (vestibular schwannoma resection; VSR) results in a unilateral vestibular hypofunction with complaints of dizziness and imbalance. Although the anatomic lesion is permanent, recovery of balance and diminution of dizziness occurs through central neurophysiologic compensation. Compensation of the system is maintained through daily activity. Unfortunately, interruption of stimulus, such as decreased activities due to illness, can cause decompensation. Decompensation is described as the return of symptoms consistent with that experienced during the initial insult/injury (eg, dizziness, oscillopsia, balance difficulty). This case study describes a reoccurrence of vestibular dysfunction in a person with a history of VSR following hospitalization and protracted recovery from a COVID-19 infection. It further documents her recovery that may be a result of vestibular rehabilitation. CASE DESCRIPTION: A 49-year-old woman (M.W.) with a surgical history of VSR (10 years prior) and a medical history of significant COVID-19 infection, resulting in an intensive care unit stay and prolonged use of supplemental oxygen, presented to physical therapy with persistent dizziness and imbalance. The video head impulse test confirmed unilateral vestibular hypofunction. INTERVENTION: M.W. attended biweekly vestibular rehabilitation for 6 weeks and completed daily home exercises. OUTCOMES: At discharge, M.W. demonstrated improvements in patient-reported outcomes (Dizziness Handicap Inventory), functional testing (MiniBEST, 2-Minute Walk Test), and gaze stability measures (video head impulse testing, dynamic visual acuity). DISCUSSION: Vestibular decompensation preluded by a COVID-19 infection caused a significant decrease in functional mobility. Vestibular rehabilitation targeted at gaze and postural stability effectively reduced symptoms and facilitated recovery to M.W.'s pre-COVID-19 level of function. Video Abstract available for more insights from the authors (see the Video, Supplemental Digital Content 1 available at: http://links.lww.com/JNPT/A458 ).

Blood biomarkers for the differentiation between central and peripheral vertigo in the emergency department: a systematic review and meta-analysis.

BACKGROUND/INTRODUCTION: In patients with acute vestibular syndrome (AVS), differentiating between stroke and nonstroke causes is challenging in the emergency department (ED). Correct diagnosis of vertigo etiology is essential for early optimum treatment and disposition. OBJECTIVES: The aim of this systematic review and meta-analysis was to summarize the published evidence on the potential of blood biomarkers in the diagnosis and differentiation of peripheral from central causes of AVS. METHODS: A literature search was conducted for studies published until January 1, 2023, in PubMed, Ovid Medline, and EMBASE databases analyzing biomarkers for the differentiation between central and peripheral AVS. The Quality Assessment of Diagnostic Accuracy Studies questionnaire 2 was used for quality assessment. Pooled standardized mean difference and 95% confidence intervals were calculated if a biomarker was reported in two or more studies. Heterogeneity among included studies was investigated using the I2 metric. RESULTS: A total of 17 studies with 859 central and 4844 peripheral causes of acute dizziness or vertigo, and analysis of 61 biomarkers were included. The general laboratory markers creatinine, blood urea nitrogen, albumin, C-reactive protein, glucose, HbA1c, leukocyte counts, and neutrophil counts and the brain-derived biomarkers copeptin, S100 calcium-binding protein ß (S100ß), and neuron-specific enolase (NSE) significantly differentiated central from peripheral causes of AVS. CONCLUSIONS: This systematic review and meta-analysis highlights the potential of generalized inflammatory markers and brain-specific blood protein markers of NSE and S100ß as diagnostic biomarkers for central from peripheral differentiation in AVS. These results, as a complement to clinical characteristics, provide guidance for future large-scale diagnostic research, in this challenging ED patient population.

The Impact of Cigarette Smoking, Water-Pipe Use on Hearing Loss/Hearing Impairment: A Cross-Sectional Study.

OBJECTIVE: The aim of the present study was to determine the association between cigarette smoking, waterpipe smoking, and co-morbidity diseases on hearing loss. METHODS: A cross-sectional study was conducted among 1015 patients [386 males (38%) and 629 females (62%)] who were aged are between 25 and 65 years. The study used clinical, physical examinations and Pure-Tone Audiometry (PTA) to assess hearing. Univariate and multivariate stepwise logistic regression analyses were used for the statistical analysis. RESULTS: Out of 1015 patients assessed, 199 were cigarette smokers with hearing loss (21.6%) and 111 waterpipe smokers with hearing loss (12%). There were statistically significant differences between cigarette smokers with hearing loss regarding (p<0.001), gender (p<0.001), BMI (p<0.001), hypertension (p<0.001), tinnitus (p<0.001), vertigo and/or dizziness (p<0.001), and migraine/headaches (p<0.001). Also there were statistically significant differences between waterpipe smokers with hearing loss, none smokers concerning age groups (p<0.001), BMI (p<0.001), using MP3 players (p=0.004), family history of hypertension (p=0.026), ATP III metabolic syndrome (p=0.010), IDF metabolic syndrome (p=0.012), tinnitus (p<0.001), vertigo/dizziness (p<0.001), and migraine/headaches (p=0.025). Multivariate stepwise logistic regression analysis indicated that tinnitus (p<0.001), dizziness (p<0.001), nausea (p=0.001), headaches and migraine (p<=0.003), fatigue (p=0.004), and vertigo (p=0.022) were considered as risk predictors risk hearing loss related cigarette smokers. Also, analysis revealed that tinnitus (p<0.001), nausea (p=0.001), headaches and migraines (p<0.001), Type 2 diabetes mellitus (p<0.001), and vertigo (p=0.021), were considered as risk predictors for hearing loss related waterpipe smokers. CONCLUSION: The present study suggests cigarette smoking and waterpipe smoking, life-style factors are possible risk factors for hearing loss among smoker participants.

Etiologic distribution of dizziness/vertigo in a neurological outpatient clinic according to the criteria of the international classification of vestibular disorders: a single-center study.

OBJECTIVE: The study aimed to determine the etiological characteristics of patients with dizziness/vertigo attending a neurological clinic according to the criteria of the International Classification of Vestibular Disorders (ICVD), hoping to provide a valuable reference for clinicians to diagnose and treat dizziness/vertigo. METHOD: A total of 638 consecutive patients with a chief complaint of dizziness/vertigo who attended the vertigo clinic of our neurology department from January 2019 to January 2020 were included. Clinical data of patients, including baseline data, medical history, neurological, neuro-otological, and auxiliary examination results were collected. The etiologic distribution of dizziness/vertigo was determined by analyzing the diagnoses of patients. RESULTS: Of the 638 patients with dizziness/vertigo, 38.8% were males, 61.2% were females, with a male: female ratio of 1:1.58 and a mean age of 52.9 ± 16.9 years. Benign paroxysmal positional vertigo (BPPV) was the most common cause of dizziness/vertigo in both female (38.9%) and male patients (25.5%). Subgroup analysis based on sex showed that vestibular migraine (VM) and probable autoimmune inner ear disease (p-AIED) were more prevalent in female patients (10.7% and 3.8%, respectively), while vascular vertigo/dizziness was more common in male patients (10.1%). Subgroup analysis based on age showed that the most common diseases were VM in patients aged 0-30 years (27.4%), BPPV in patients aged 31-60 years (27.1%) and 61-100 years (46.0%). Episodic vestibular syndrome (EVS) was the most commonly observed, accounting for up to 60.6% (389/638) of all patients, and the most common diagnoses were BPPV (55.3%, 215/389), VM (15.2%, 59/389), primary unilateral peripheral vestibular dysfunction (p-UPVD) of unknown etiology (11.8%, 46/389), p-AIED (4.4%, 17/389), and vascular vertigo/dizziness (2.8%, 11/389) in these patients. Chronic vestibular syndrome (CVS) was found in 14.0% (90/638) of the patients, and the most common diagnoses were persistent postural-perceptual dizziness (PPPD, 35.6%, 32/90), psychogenic dizziness (18.9%, 17/90), p-UPVD of unknown etiology (15.6%, 14/90), vascular vertigo/dizziness (15.6%, 14/90), and bilateral vestibulopathy (7.8%, 7/90). Acute vestibular syndrome (AVS) was observed in 8.4% (54/638) of the patients, and the most common diagnoses were p-UPVD of unknown etiology (31.5%, 17/54), vestibular neuritis (24.1%, 13/54), probable labyrinthine apoplexy (16.7%, 9/54), stroke (13.0%, 7/54), and psychogenic dizziness (11.1%, 6/54). 16.4% (105/638) of the patients were found to have other disorders, including 15.2% (16/105) of patients with internal diseases, and 84.8% (89/105) of patients with unknown causes. In terms of localization diagnosis, 56.1%, 17.0%, 10.0%, and 16.4% of the patients were diagnosed with peripheral vestibular disorder, central vestibular disorder, psychiatric and functional vestibular disorders, and other disorders, respectively. CONCLUSION: (1) Dizziness/vertigo was more common in females, which was frequently caused by damage to the vestibular system. Non-vestibular or unknown etiologies were also seen in some patients; (2) VM was more prevalent in women than in men, vascular vertigo/dizziness was more commonly observed in men; (3) EVS was more common in patients with dizziness/vertigo. The most common causes of dizziness/vertigo were peripheral vestibular disorders in patients with AVS and EVS, PPPD and psychogenic dizziness in patients with CVS. The most common causes were BPPV and p-UPVD of unknown etiology in patients with a peripheral vestibular disorder, VM and vascular vertigo/dizziness in patients with central vestibular disorder, PPPD and psychogenic dizziness in patients with psychiatric and functional vestibular disorders.

Scuba Diving-Induced Inner-Ear Pathology: Imaging Findings of Superior Semicircular Canal and Tegmen Tympani Dehiscence.

BACKGROUND Superior semicircular canal dehiscence is an inner-ear pathology which presents with vertigo, disequilibrium, and hearing loss. Although the exact etiology of superior semicircular canal dehiscence is unknown, it is thought that an increase in middle-ear pressure disrupts a thin overlying temporal bone. Superior semicircular canal dehiscence is frequently seen in association with dehiscence of the tegmen tympani, which overlies the middle ear. Here, we present a case report of a 52-year-old Puerto Rican man with vertigo, dizziness, vomiting, and mild hearing loss associated with superior semicircular canal and tegmen tympani dehiscence after performing improper scuba diving techniques. CASE REPORT A 52-year-old Puerto Rican man presented to the emergency department with vertigo, dizziness, vomiting, and mild hearing loss in the right ear. The symptoms began shortly after scuba diving with inadequate decompression techniques on ascent. He was treated with recompression therapy with mild but incomplete improvement in symptoms. Bilateral temporal magnetic resonance imaging was suggestive of segmental dehiscence of the right superior semicircular canal and tegmen tympani. High-resolution computed tomography of the temporal bone confirmed right superior semicircular canal and tegmen tympani dehiscence with an intact left inner ear. CONCLUSIONS The increased inner-ear pressure that occurs during scuba diving can lead to dehiscence of the superior semicircular canal and tegmen tympani, causing vertigo and hearing loss. Performance of improper diving techniques can further increase the risk of dehiscence. Therefore, appropriate radiologic evaluation of the inner ear should be performed in such patients.

Vestibular function assessment in Idiopathic sudden sensorineural hearing loss: a prospective study.

PURPOSE: Idiopathic sudden sensorineural hearing loss (ISSHL) can cause acute damage not only to the auditory function, but also to the vestibular function in addition to damage to the hearing function. The aim of this study was to perform vestibular assessment using caloric test and video head impulse test in patients with idiopathic sudden sensorineural hearing loss. In addition, to evaluate the relationship of dizziness with vestibular tests and post-treatment responses of vestibular tests. METHODS: This is an observational, longitudinal and prospective study, including patients diagnosed with idiopathic sudden sensorineural hearing. Patients were divided into two groups according to the presence of vestibular complaints at presentation: Group 1: Patients with vestibular complaints, Group 2: Patients without vestibular complaints. All subjects underwent pure tone audiometry (PTA) testing, cold caloric test and video head impulse test (vHIT) during their admission and on the 10th day, 3rd month, and 1st year of their follow-up outpatient clinic controls. A unilateral weakness (UW) in the caloric test response was quantified according to the Jongkees formula. RESULTS: A positive and significant relationship was found between the degree of hearing loss according to the ASHA criteria pre-treatment and the level of improvement created according to Siegel criteria at the 10th day, 3rd month, 1st year after treatment (respectively p = 0.001, p = 0.001, p < 0.001). When both short-term and long-term results were evaluated after treatment, a positive improvement in the degree of hearing loss was observed. A statistically significant difference was observed between Groups 1 and 2 when the pre-treatment, at 10 days, 3 months and 1 year after treatment the caloric test UW value was compared (respectively p = 0.020, p = 0.004, p = 0.004, p = 0.004). A statistically significant difference was observed between Groups 1 and 2 when the pre-treatment, at 10 days, 3 months and 1 year after treatment vHIT lateral canal VOR value was compared (respectively p = 0.000, p = 0.001, p = 0.000, p = 0.004). When both short-term and long-term results were evaluated after treatment, a positive improvement was observed in both caloric test results and lateral vHIT VOR values. Pre-treatment, post-treatment 10th day, 3rd month, 1st year vHIT anterior and posterior canal VOR values were found to be VOR˃0.8 in all patients. No difference was observed in anterior and posterior canal VOR values. CONCLUSION: Vertigo in patients with ISSHL "as objectively confirmed through caloric testing and vHIT" can be considered a sign of severe cochlear damage. Our study demonstrated a significantly increased risk of vestibular affect in patients with ISSHL, especially in the presence of vertigo. Thus, we conclude that the focus in ISSHL should not only be on the cochlea but also on the vestibular system.

Arachnoid Cyst of the Cerebellopontine Angle: A Systematic Literature Review.

OBJECTIVE: The role of surgical management of arachnoid cyst (AC) of the cerebellopontine angle (CPA) is uncertain. This topic has remained controversial with varying contradictory recommendations in the literature, which is limited to mostly case reports. We aimed to provide a comprehensive summary and analysis of symptoms, operative techniques, outcomes, and recurrence of all available surgical cases of AC of the CPA to date. METHODS: A systematic literature search was performed in May 2022 querying several scientific databases. Inclusion criteria specified all studies and case reports of patients with AC located at the CPA for which any relevant surgical procedures were performed. RESULTS: A total of 55 patients from the literature and 5 treated at our institution were included. Mean patient age was 29 years (range, 0.08-79 years), with nearly twice (1.7×) as many female as male patients (37 female, 22 male). Headaches (35%), hearing loss (30%), vertigo (22%), and ataxia (22%) were the most common presentations. Following surgery, 95% experienced symptom improvement, with complete resolution in 64%. Of patients with hearing loss, 44% reported a return to normal. The rate of mortality was 1.69%, and 10% of tumors recurred (mean follow-up 2.3 years [range, 0-15 years]. CONCLUSIONS: Symptomatic AC of the CPA is rare. It exhibits a proclivity for females and commonly manifests with headache, hearing loss, vertigo, and ataxia. While careful selection for surgical candidacy is needed and intervention should be reserved for patients with severe symptoms, surgical decompression is an effective tool for symptom alleviation and recovery.

The acute vestibular syndrome: prevalence of new hearing loss and its diagnostic value.

OBJECTIVES: To assess the prevalence of new hearing losses in patients with acute vestibular syndrome (AVS) and to start to evaluate its diagnostic value for the differentiation between peripheral and central causes. DESIGN: We performed a cross-sectional prospective study in AVS patients presenting to our Emergency Department (ED) from February 2015 to November 2020. All patients received an MRI, Head-impulse test, Nystagmus test and Test of skew ('HINTS'), caloric testing and a pure-tone audiometry. RESULTS: We assessed 71 AVS patients, 17 of whom had a central and 54 a peripheral cause of dizziness. 12.7% had an objective hearing loss. 'HINTS' had an accuracy of 78.9% to diagnose stroke, whereas 'HINTS' plus audiometry 73.2%. 'HINTS' sensitivity was 82.4% and specificity 77.8% compared to 'HINTS' plus audiometry showing a sensitivity of 82.4% and specificity of 70.4%. The four patients with stroke and minor stroke had all central 'HINTS'. 55% of the patients did not perceive their new unilateral hearing loss. CONCLUSIONS: We found that almost one-eighth of the AVS patients had a new onset of hearing loss and only half had self-reported it. 'HINTS' plus audiometry proved to be less accurate to diagnose a central cause than 'HINTS' alone. Audiometry offered little diagnostic accuracy to detect strokes in the ED but might be useful to objectify a new hearing loss that was underestimated in the acute phase. Complete hearing loss should be considered a red flag, as three in four patients suffered from a central cause.

Long-Term Effects of Intervention on Vestibular Migraine: A Preliminary Study.

BACKGROUND: Vestibular migraine is a common cause of vertigo. Intervention often includes preventive and/or rescue medications. Lifestyle modifications are often used along with medications but can be used as the sole intervention. There is lack of clarity regarding the long-term benefits of these interventions. AIMS: The purpose of this study was to determine long-term effects of intervention types on dizziness in patients with vestibular migraine. METHODS: Twenty-three participants were grouped based on intervention into preventive medication plus lifestyle modifications, rescue medication plus lifestyle modifications, or lifestyle modifications only. Outcomes were determined at ~372 days post intervention by comparing pre- and post-Dizziness Handicap Inventory scores. A difference of ≥18 points was considered a change and we also evaluated change in severity scale on this measure. RESULTS: Using the group mean change score, only the rescue medication plus lifestyle modification group was significantly improved at 372 days of intervention. Considering all individual participants, 30% of the participants had improvement in dizziness at this point, regardless of intervention. Fifty percent of the rescue medication plus lifestyle modification group had significant reduction in dizziness, while the preventive medication plus lifestyle modification and the lifestyle modification only groups performed similarly using this criterion. Considering change in severity category, 43% of all participants improved by at least one category. The rescue medication plus lifestyle modifications and the lifestyle modifications only groups performed similarly with 50% of their respectively groups exhibiting improvement by at least one category. Notably, there was no worsening of dizziness for any participant in the lifestyle modification only group. CONCLUSION: Our findings suggest that improvement in dizziness is maintained at ~372days of intervention in patients with vestibular migraine. Intervention using rescue medications plus lifestyle modifications had the best outcomes, followed by lifestyle modifications only. There was no worsening in dizziness for the lifestyle modification only intervention. More work is needed to better understand intervention effects, but it is encouraging that effects are maintained at greater than one year.

Translation and validation of the PVSQ and DHI-PC questionnaires for pediatric dizziness.

AIMS: Validation of the PVSQ self-report questionnaire (diagnosis) and the DHI-PC caregiver report questionnaire (Dizziness Handicap Inventory) aims to improve the management of pediatric vertigo, which is often under-diagnosed. MATERIALS AND METHODS: The PVSQ and DHI-PC questionnaires were translated according to the Forward-Backward method and presented to a group of patients consulting for dizziness in a referral center and to a control group. A retest was performed at 2weeks for both questionnaires. Statistical validation consisted in calculating discriminatory capacity, ROC curve, reproducibility and internal consistency. The main study objective was the translation and validation of the PVSQ and DHI-PC questionnaires in French. The secondary objectives were to compare results in two subgroups according to the vestibular or non-vestibular etiology of dizziness and to assess the correlation between the two questionnaires. RESULTS: In total, 112 children, in two comparable groups (53 cases and 59 controls), were included. Mean PVSQ score was 14.62 for cases and 6.55 for controls (P<0.001). Reproducibility was moderate, and internal consistency and construct validity were satisfactory. A cut-off of 11 corresponded to maximum Younden index. Mean DHI-PC score was 41.6 (cases only). Reproducibility was moderate, and internal consistency and construct validity were satisfactory. CONCLUSION: The validation of the PVSQ and DHI-PC questionnaires offers two new tools in the management of dizziness, for both screening and follow-up.

Trans-mastoid plugging of superior semicircular canal dehiscence: long-term follow-up.

OBJECTIVE: To evaluate the long-term outcomes of trans-mastoid plugging of superior semicircular canal dehiscence (SSCD), focusing on complicated cases. METHODS: In this cohort study, we included all patients who underwent trans-mastoid plugging of SSCD between 2009 and 2019. We evaluated the symptoms (autophony, sound-/pressure-induced vertigo, disequilibrium, aural fullness and pulsatile tinnitus) before and 1 year after surgery in the medical records. We systematically assessed the current symptoms 6.2 ± 3 years postoperative (range 2.2-12.3 years) using questionnaires sent by post and validated by telephone interviews. We also documented any complications and the need for further procedures. We compared pure tone and speech audiometry before and 1 year after surgery. Finally, the degree of mastoid pneumatisation and mastoid tegmen anatomy were reviewed on preoperative CT scans. RESULTS: We included 24 ears in 23 patients. No complications were recorded, and none required a second procedure for SSCD. Following surgery, oscillopsia and Tullio phenomena resolved in all patients. Hyperacusis, autophony, and aural fullness were also settled in all patients except one. Balance impairment persisted to some degree in 35% of patients. No deterioration over the years was reported regarding the above symptoms. On average, bone conduction pure tone average pre- and 1 year postoperative were 13.7 ± 17 and 20.5 ± 18 dB, respectively (P = 0.002). Air bone gaps were reduced from 12.7 ± 8 to 5.9 ± 6 (P = 0.001). Two patients had a significant sclerotic mastoid, three had a prominent low-lying mastoid tegmen, and two had both. Anatomy had no effect on outcome. CONCLUSION: Trans-mastoid plugging of SSCD is a reliable and effective technique which achieves long-lasting symptom control, even in cases with sclerotic mastoid or low-lying mastoid tegmen.

Significance of endolymphatic sac surgery with and/or without simultaneous cochlea implant surgery in respect of vertigo control and speech perception in patients with Menière's disease.

PURPOSE: The focus on treating patients with Menière's Disease (MD) lies on the reduction of vertigo attacks and the preservation of sensory function. Endolympathic hydrops is considered as an epiphenomenon in MD, which can potentially be altered by endolymphatic sac surgery (ESS). Purpose of the study was to investigate the influences on vertigo control through manipulation of the perilymphatic system with or without ESS. METHODS: Retrospective data analysis of 86 consecutive patients with MD according to current diagnostic criteria after endolymphatic sac surgery alone (ESSalone; n = 45), cochlear implantation (CI) alone (CIalone; n = 12), and ESS with CI (ESS + CI; n = 29), treated at a tertiary referral center. MAIN OUTCOME MEASURES: vertigo control, speech perception pre- and postoperatively. RESULTS: Gender, side, and preoperative treatment were similar in all groups. Age was younger in the ESSalone-group with 56.2 ± 13.0 years (CIalone = 64.2 ± 11.4 years; ESS + CI = 63.1 ± 9.7 years). Definitive MD was present in all the CIalone, in 79.3% of the ESS + CI and in 59.6% of the ESSalone-patients. Likewise, vertigo control rate was 100% in the CIalone, 89.7% in the ESS + CI and 66.0% in the ESSalone-group. CONCLUSIONS: Vertigo control was improved in all three groups, however, superior in groups treated with CI, potentially contributed by the manipulation of both the endo- and perilymphatic systems. A more systematic characterization of the patients with larger case numbers and documentation of follow up data would be needed to evaluate a clinical effect more properly.

Superior canal dehiscence syndrome: A review.

Superior canal dehiscence syndrome (SCDS) is a vestibular disorder in which the presence of a pathological third window in the labyrinth causes several vestibular and cochlear symptoms. Herein, we review the diagnostic criteria and treatment of SCDS. The cause of SCDS is considered to be congenital or acquired; however, its etiology is not well known. Symptoms: Vertigo and/or oscillopsia induced by loud sounds (Tullio phenomenon) or stimuli that change the middle ear or intracranial pressure (fistula symptoms) with vestibular symptoms and hyperacusis and aural fullness with cochlear symptoms are characteristic clinical complaints of this syndrome. Neurological tests: Vertical-torsional eye movements can be observed when the Tullio phenomenon or fistula symptoms are induced. Conductive hearing loss with both a decrease in the bone conduction threshold at lower frequencies and an increase in the air conduction threshold at lower frequencies may be present on audiometry. Cervical and/or ocular vestibular evoked myogenic potentials are effective in strongly suspecting the presence of a pathologic third window in the labyrinth. Computed tomography (CT) imaging: High-resolution CT findings with multiplanar reconstruction in the plane of the superior semicircular canal consistent with dehiscence indicate SCDS. The Pöschl view along the plane of the superior semicircular canal and the Stenvers view perpendicular to it are recommended as CT imaging conditions. Findings from all three major diagnostic categories (symptoms, neurological tests, and/or CT imaging) are needed to diagnose SCDS. The surgical approaches for SCDS are as follows: the 1) middle cranial fossa approach, 2) transmastoid approach, and 3) round window and oval window reinforcement. Each technique has advantages and disadvantages.