A diagnostic framework to identify vestibular involvement in multi-sensory neurological disease.

BACKGROUND AND PURPOSE: Identifying vestibular causes of dizziness and unsteadiness in multi-sensory neurological disease can be challenging, with problems typically attributed to central or peripheral nerve involvement. Acknowledging vestibular dysfunction as part of the presentation provides an opportunity to access targeted vestibular rehabilitation, for which extensive evidence exists. A diagnostic framework was developed and validated to detect vestibular dysfunction, benign paroxysmal positional vertigo or vestibular migraine. The specificity and sensitivity of the diagnostic framework was tested in patients with primary mitochondrial disease. METHODS: Adults with a confirmed diagnosis of primary mitochondrial disease were consented, between September 2020 and February 2022. Participants with and without dizziness or unsteadiness underwent remote physiotherapy assessment and had in-person detailed neuro-otological assessment. The six framework question responses were compared against objective neuro-otological assessment or medical notes. The output was binary, with sensitivity and specificity calculated. RESULTS: Seventy-four adults completed the study: age range 20-81 years (mean 48 years, ±SD 15.05 years); ratio 2:1 female to male. The framework identified a vestibular diagnosis in 35 participants, with seven having two diagnoses. The framework was able to identify vestibular diagnoses in adults with primary mitochondrial disease, with a moderate (40-59) to very high (90-100) sensitivity and positive predictive value, and moderate to high (60-74) to very high (90-100) specificity and negative predictive value. CONCLUSIONS: Overall, the clinical framework identified common vestibular diagnoses with a moderate to very high specificity and sensitivity. This presents an opportunity for patients to access effective treatment in a timely manner, to reduce falls and improve quality of life.

Cochlear implantation in patients with inner ear schwannomas: a systematic review and meta-analysis of audiological outcomes.

PURPOSE: In patients with inner ear schwannomas (IES), reports on hearing rehabilitation with cochlear implants (CI) have increased over the past decade, most of which are case reports or small case series. The aim of this study is to systematically review the reported hearing results with CI in patients with IES considering the different audiologic outcome measures used in different countries. METHODS: According to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline, a search of published literature was conducted. We included patients with IES (primary or with secondary extension from the internal auditory canal (IAC) to the inner ear, sporadic or NF2 related) undergoing cochlear implantation with or without tumour removal. The audiological results were divided into the categories "monosyllables", "disyllables", "multisyllabic words or numbers", and "sentences". RESULTS: Predefined audiological outcome measures were available from 110 patients and 111 ears in 27 reports. The mean recognition scores for monosyllabic words with CI were 55% (SD: 24), for bisyllabic words 61% (SD: 36), for multisyllabic words and numbers 87% (SD: 25), and 71% (SD: 30) for sentences. Results from for multisyllabic words and numbers in general showed a tendency towards a ceiling effect. Possible risk factors for performance below average were higher complexity tumours (inner ear plus IAC/CPA), NF2, CI without tumour removal ("CI through tumour"), and sequential cochlear implantation after tumour removal (staged surgery). CONCLUSION: Hearing loss in patients with inner ear schwannomas can be successfully rehabilitated with CI with above average speech performance in most cases. Cochlear implantation thus represents a valuable option for hearing rehabilitation also in patients with IES while at the same time maintaining the possibility of MRI follow-up. Further studies should investigate possible risk factors for poor performance. Audiological tests and outcome parameters should be reported in detail and ideally be harmonized to allow better comparison between languages.

[Increased report completeness and satisfaction with structured neurotological reporting in the interdisciplinary assessment of vertigo]. / Erhöhte Befundvollständigkeit und gesteigerte Zuweiserzufriedenheit bei strukturierter neurootologischer Befunderhebung in der interdisziplinären Schwindelabklärung.

BACKGROUND: Results of neurotological function diagnostics in the context of interdisciplinary vertigo assessment are usually formulated as free-text reports (FTR). These are often subject to high variability, which may lead to loss of information. The aim of the present study was to evaluate the completeness of structured reports (SR) and referrer satisfaction in the neurotological assessment of vertigo. MATERIALS AND METHODS: Neurotological function diagnostics performed as referrals (n = 88) were evaluated retrospectively. On the basis of the available raw data, SRs corresponding to FTRs from clinical routine were created by means of a specific SR template for neurotological function diagnostics. FTRs and SRs were evaluated for completeness and referring physician satisfaction (n = 8) using a visual analog scale (VAS) questionnaire. RESULTS: Compared to FTRs, SRs showed significantly increased overall completeness (73.7% vs. 51.7%, p < 0.001), especially in terms of patient history (92.5% vs. 66.7%, p < 0.001), description of previous findings (87.5% vs. 38%, p < 0.001), and neurotological (33.5% vs. 26.7%, p < 0.001) and audiometric function diagnostics (58% vs. 32.3%, p < 0.001). In addition, SR showed significantly increased referring physician satisfaction (VAS 8.8 vs. 4.9, p < 0.001). CONCLUSION: Neurotological SRs enable a significantly increased report completeness with higher referrer satisfaction in the context of interdisciplinary assessment of vertigo. Furthermore, SRs are particularly suitable for scientific data analysis, especially in the context of big data analyses.

COX inhibitor use during definitive radiotherapy is associated with worse hearing preservation in patients with vestibular schwannoma.

PURPOSE: Patients with vestibular schwannoma undergoing definitive radiotherapy commonly experience hearing loss due to tumor and treatment effects; however, there is limited data evaluating concurrent medication use and other clinicopathologic factors associated with hearing preservation during and after radiotherapy. We performed a retrospective cohort study reviewing consecutive patients from 2004 to 2019 treated with radiotherapy for vestibular schwannoma at our institution. METHODS: Ninety four patients with concurrent medications, baseline audiograms, and post-radiotherapy audiograms available were evaluable. We performed chi-squared analyses of the frequency of various clinicopathologic factors and t-tests evaluating the degree of hearing loss based on audiograms. RESULTS: At a median follow-up of 35.7 months (mean: 46.5 months), the baseline pure-tone average (PTA) of the ipsilateral ear worsened from 38.4 to 59.5 dB following completion of radiotherapy (difference: 21.1, 95% CI 17.8-24.4 dB, p < 0.001). 36 patients (38.3%) reported regular use of cyclooxygenase (COX) inhibitors (including acetaminophen and NSAIDs) during radiotherapy. The mean increase in PTA was significantly higher for patients taking COX inhibitors (25.8 dB vs 18.1 dB, p = 0.024) in the ipsilateral ear but not for the contralateral side. COX inhibitor use remained independently associated with worse PTA in the multivariate analysis. CONCLUSION: COX inhibitor use during definitive radiotherapy is associated with worse hearing loss in the affected ear but not for the contralateral side. This suggests the ototoxic effects of COX inhibitors may influence the effects of radiotherapy. These results could have clinical implications and warrant further investigation.

Natural history of facial paraganglioma with 2 decades of follow-up: A case report and literature review.

OBJECTIVE: To report 20 years of natural history data for a facial paraganglioma and provide a comprehensive review of the existing literature. PATIENT: 81-year-old female with a remote history of cardiac arrest while under anesthesia who elected to observe her facial paraganglioma for 20 years. INTERVENTIONS: Observation, clinical documentation, radiographic surveillance. MAIN OUTCOME MEASURES: Tumor progression, patient symptomatology, and review of management options. RESULTS: The initial presentation of the facial paraganglioma was facial spasm. Over the course of observation, symptoms progressed to include complete facial nerve paralysis, pulsatile tinnitus, and otalgia on the affected side. Radiologic surveillance demonstrated incremental growth and erosion of surrounding structures, including the posterior external auditory canal, stylomastoid foramen, and lateral semicircular canal with near-dehiscence. Twenty-four cases of facial paraganglioma were identified in the extended literature search and are summarized herein. CONCLUSIONS: This unique case contributes to the scarce literature surrounding facial paragangliomas by reporting the extended natural history of this disease.

Neurotological emergencies: a narrative review.

INTRODUCTION: Vestibular symptoms, including vertigo, dizziness, and gait unsteadiness, are a frequent reason of urgent medical assistance. Their causes are numerous and diverse, including neurological, otorhinolaryngological, and systemic diseases. Therefore, following a systematic approach is essential to differentiate striking but benign conditions from others that can compromise the patient's life. This study is intended to review vestibular disorders from a practical perspective and provide guidance to physicians involved in the emergency care of patients with vestibular symptoms. MATERIALS AND METHODS: A narrative review was performed, revisiting the main causes of vestibular disorders. RESULTS: Based on the speed of onset, duration, and history of similar episodes in the past, vestibular disorders can be categorized into three syndromic entities (acute, recurrent, and chronic vestibular syndromes). The most representative conditions pertaining to each group were reviewed (including their diagnosis and treatment) and a practical algorithm was proposed for their correct management in the acute care setting. CONCLUSIONS: Carrying out a correct categorization of the vestibular disorders is essential to avoid diagnostic pitfalls. This review provides useful tools for clinicians to approach their patients with vestibular symptoms at the emergency room.

Tinnitus Prevalence in the Adult Population-Results from the Gutenberg Health Study.

Background and Objectives: Tinnitus is a common symptom in medical practice, although data on its prevalence vary. As the underlying pathophysiological mechanism is still not fully understood, hearing loss is thought to be an important risk factor for the occurrence of tinnitus. The aim of this study was to assess tinnitus prevalence in a large German cohort and to determine its dependence on hearing impairment. Materials and Methods: The Gutenberg Health Study (GHS) is a population-based cohort study and representative for the population of Mainz and its district. Participants were asked whether they suffer from tinnitus and how much they are burdened by it. Extensive audiological examinations using bone- and air-conduction were also performed. Results: 4942 participants (mean age: 61.0, 2550 men and 2392 women) were included in the study. The overall prevalence of tinnitus was 26.1%. Men were affected significantly more often than women. The prevalence of tinnitus increased with age, peaking at ages 75 to 79 years. Considering only annoying tinnitus, the prevalence was 9.8%. Logistic regression showed that participants with severe to complete hearing loss (>65 dB) were more likely to have tinnitus. Conclusions: Tinnitus is a common symptom, and given demographic changes, its prevalence is expected to increase.

Otologic opioid usage and pain control in the postoperative period: An observational prospective study.

PURPOSE: Evaluate opioid prescribing patterns, opioid consumption, and patient pain patterns following otologic surgery. MATERIALS AND METHODS: Patients were included if they were ≥18 years old and received otologic surgery between November 2019 and August 2020. Patients were provided a survey which included a visual analog scale for recording their pain postoperatively and the amount of opioid they had remaining. Patients who did not complete all portions of the survey were excluded. RESULTS: Ninety-one patients completed the post-operative questionnaire. Collectively, patients were prescribed 5797 morphine milligram equivalents and used 3092: approximately 47% went unused. Of patients receiving a transcanal incision (n = 28/91, 31%), 70% went unused, whereas patients receiving a postauricular incision (n = 57/91, 63%), 38% went unutilized. The utilization difference between transcanal and postauricular cohorts was significant (p = 0.002). On multivariate analysis, patients who received a postauricular incision had 60% more opioid usage (p < 0.001), whereas those with a transcanal incision had an average reduction of 40% in opioid usage (p < 0.001). CONCLUSIONS: A significant amount of opioid medication went unused in this study. Patients with postauricular incisions had significantly increased opioid utilization as compared to those with transcanal incisions. Otologists may be able to successfully manage pain in the postoperative period with a reduced opioid prescription multimodal analgesia and increased patient education. Further study is needed to support this suggestion.

[The Dizziness Handicap Inventory for quality control in the treatment of vestibular dysfunction]. / Dizziness Handicap Inventory in der Qualitätssicherung der Therapie vestibulärer Funktionsstörung.

Treatment of vestibular dysfunction remains a challenge for many otolaryngologists. This is true not only for establishing the differential neurotologic diagnosis, but also for treatment and treatment monitoring in vestibular dysfunction patients. Particularly quality control of therapeutic measures is generally poorly documented. The validated German version of the Dizziness Handicap Inventory (DHI) represents a viable option for evaluation and monitoring of treatment outcomes. In this study, patients who were treated at the Otorhinolaryngology Department of the University Hospital Aachen because of unilateral peripheral vestibular disorders were asked to complete the DHI before and after treatment. Posttherapeutic DHI scores were collected by telephone. Evaluation of the DHI scores underlined the significant benefit of treatment in 92%. Furthermore, the DHI prove to be useful for documentation of patients' disorders and treatment and thus for quality assurance.

The utility of augmented reality in lateral skull base surgery: A preliminary report.

OBJECTIVE: To discuss the utility of augmented reality in lateral skull base surgery. PATIENTS: Those undergoing lateral skull base surgery at our institution. INTERVENTION(S): Cerebellopontine angle tumor resection using an augmented reality interface. MAIN OUTCOME MEASURE(S): Ease of use, utility of, and future directions of augmented reality in lateral skull base surgery. RESULTS: Anecdotally we have found an augmented reality interface helpful in simulating cerebellopontine angle tumor resection as well as assisting in planning the incision and craniotomy. CONCLUSIONS: Augmented reality has the potential to be a useful adjunct in lateral skull base surgery, but more study is needed with large series.

Normative video head impulse test data in subjects with and without vascular risk factors.

PURPOSE: There is a paucity of age- and vascular risk factor-stratified video head impulse test (vHIT) vestibulo-ocular reflex (VOR) data in the literature. The aim of this study was to investigate the vHIT VOR properties in healthy subjects of different ages and subjects with vascular risk factors. METHODS: This was a prospective observational single-center study at a tertiary referral university hospital in northern Sweden. Healthy participants and subjects with vascular risk factors were investigated with a floor standing external camera vHIT device. Age-stratified mean VOR gain among healthy adults and between group gain and gain asymmetry differences were calculated. RESULTS: We included eighty-eight healthy adults with a mean (range) age of 50 (22-85) years and n = 48 stroke ward patients with vascular risk factors (but without vestibular disease) with a mean (range) age of 74 (42-92) years. The mean VOR gain of horizontal canals decreased at higher ages in healthy subjects (r = - 0.32, p < 0.01, n = 167 canals). The age-stratified mean (SD) VOR gains were < 30 years: 0.98 (0.07), 30-39 years: 0.97 (0.07), 40-49 years: 0.98 (0.06), 50-59 years: 0.99 (0.06), 60-69 years: 0.93 (0.08), ≥ 70 years: 0.89 (0.15). No consistent differences between healthy subjects and subjects with vascular risk factors were seen except for a trend towards more pronounced gain asymmetries in the latter group. CONCLUSIONS: Age, but not vascular risk factors influence VOR gain. Age-adjusted vHIT-measurements may be useful in acute vertigo stroke risk differentiation.

Positron emission tomography visualized stimulation of the vestibular organ is localized in Heschl's gyrus.

The existence of a human primary vestibular cortex is still debated. Current knowledge mainly derives from functional magnetic resonance imaging (fMRI) and positron emission tomography (PET) acquisitions during artificial vestibular stimulation. This may be problematic as artificial vestibular stimulation entails coactivation of other sensory receptors. The use of fMRI is challenging as the strong magnetic field and loud noise during MRI may both stimulate the vestibular organ. This study aimed to characterize the cortical activity during natural stimulation of the human vestibular organ. Two fluorodeoxyglucose (FDG)-PET scans were obtained after natural vestibular stimulation in a self-propelled chair. Two types of stimuli were applied: (a) rotation (horizontal semicircular canal) and (b) linear sideways movement (utriculus). A comparable baseline FDG-PET scan was obtained after sitting motion-less in the chair. In both stimulation paradigms, significantly increased FDG uptake was measured bilaterally in the medial part of Heschl's gyrus, with some overlap into the posterior insula. This is the first neuroimaging study to visualize cortical processing of natural vestibular stimuli. FDG uptake was demonstrated in the medial-most part of Heschl's gyrus, normally associated with the primary auditory cortex. This anatomical localization seems plausible, considering that the labyrinth contains both the vestibular organ and the cochlea.

3D printed temporal bone as a tool for otologic surgery simulation.

PURPOSE: In this face validity study, we discuss the fabrication and utility of an affordable, computed tomography (CT)-based, anatomy-accurate, 3-dimensional (3D) printed temporal bone models for junior otolaryngology resident training. MATERIALS AND METHODS: After IRB exemption, patient CT scans were anonymized and downloaded as Digital Imaging and Communications in Medicine (DICOM) files to prepare for conversion. These files were converted to stereolithography format for 3D printing. Important soft tissue structures were identified and labeled to be printed in a separate color than bone. Models were printed using a desktop 3D printer (Ultimaker 3 Extended, Ultimaker BV, Netherlands) and polylactic acid (PLA) filament. 10 junior residents with no previous drilling experience participated in the study. Each resident was asked to drill a simple mastoidectomy on both a cadaveric and 3D printed temporal bone. Following their experience, they were asked to complete a Likert questionnaire. RESULTS: The final result was an anatomically accurate (XYZ accuracy = 12.5, 12.5, 5 µm) 3D model of a temporal bone that was deemed to be appropriate in tactile feedback using the surgical drill. The total cost of the material required to fabricate the model was approximately $1.50. Participants found the 3D models overall to be similar to cadaveric temporal bones, particularly in overall value and safety. CONCLUSIONS: 3D printed temporal bone models can be used as an affordable and inexhaustible alternative, or supplement, to traditional cadaveric surgical simulation.

The relationship of inter-implant time and hearing outcomes for bilateral cochlear implants.

PURPOSE: Determine the relationship between time elapsed between sequential bilateral cochlear implantation (BiCI) and speech intelligibility scores in post-lingually deafened adults. MATERIALS AND METHODS: Retrospective review of post-lingually deafened adults who received bilateral cochlear implants from January 1, 2011 to January 1, 2018 at an ambulatory tertiary referral center. RESULTS: 113 patients (226 cochlear implants) were initially reviewed, with 56 patients (112 implants) being included in the final analysis. Median inter-implant interval was 187.5 days (IQ range 54.25-346.5). Maximum interval was 1787 days. Mean age at first implant was 60.66 ± 13.37. Bilateral AzBio score in quiet and inter-implant interval showed no significant correlation (r = 0.034, p = 0.815). There was no significant difference in mean bilateral AzBio scores in quiet between the simultaneous and sequential implantation groups (p = 0.22). Similar non-significant results were seen when examining the correlation between AzBio Difference and inter-implant interval (r = -0.07, p = 0.66). No significant result between mean AzBio Difference of simultaneous and sequential implant recipients was found (p = 0.06). CONCLUSIONS: For the inter-implant intervals examined, there seems to be no significant decline in speech intelligibility scores for patients receiving sequential bilateral cochlear implants compared to simultaneously implanted patients. There was no significant correlation noted between increasing inter-implant intervals and speech intelligibility scores.

[Enlarged vestibular aqueduct syndrome-dehiscence syndromes-honeycomb mastoid : Pathophysiology and evidence for clinical differentiation]. / Der erweiterte vestibuläre Aquädukt ­ Dehiszenzsyndrome ­ "Honeycomb Mastoid" : Pathophysiologie und Evidenzlage für die klinische Differenzierung.

BACKGROUND: Differential diagnosis of dizziness with hearing loss requires standardized procedures for detection and classification of rare congenital and acquired malformations of the petrous part of the temporal bone. OBJECTIVE: The aim of this study was to present the physiology and pathophysiology of endolymphatic and perilymphatic pressure regulation, diagnostic guidelines, and aspects of prognosis and treatment. MATERIALS AND METHODS: Relevant publications and guidelines were evaluated and own cases are reported. RESULTS: Enlarged vestibular aqueduct (EVA) is the most frequently observed malformation of the inner ear, which leads to increased internal hydrostatic pressure and cochleovestibular dysfunction. Non-syndromic and syndromic forms, e.g., Pendred syndrome, are known. Other pressure-relevant malformations are semicircular canal dehiscence syndrome (SCDS) and enlargement of the cochlear aqueduct. There are currently no treatment options for EVAS and enlarged cochlear aqueduct. Pendred syndrome generally requires treatment with cochlea implants (CI) in early childhood. Dizziness and autophony in patients with SCDS syndrome can be effectively treated by semicircular canal occlusion and coverage. CONCLUSION: Complaints in non-syndromic EVA and SCD syndrome are mainly caused by exposure of the inner ear to provoked and spontaneous pressure increases. Deafness and vestibular dysfunction in syndromic EVA (i.e., Pendred syndrome, branchiootorenal syndrome) are caused by malformation of the cochlea, genetic maldevelopment of the hair cells, and pressure effects.

The clinical value of a thorough diagnostic evaluation for neurotologic complaints.

PURPOSE: Determine the clinical efficacy of comprehensive neurotologic testing in patients presenting with complaints of hearing loss, tinnitus and/or dizziness. METHODS: This is a retrospective analysis of 1170 consecutive charts of patients who presented between 1980 and 2013 with neurotologic complaints. Demographic data, chief complaint, diagnostic imaging, audiograms, and blood tests were evaluated. RESULTS: Retrospective analysis of 1170 patient charts was performed. 762/1170 (65%) patients presented with subjective hearing loss, 575/1170 (49%) with dizziness, and 657/1170 (56%) with tinnitus. Audiometric testing revealed hearing loss in 1059/1169 (91%) patients. 536/1120 (48%) patients had abnormalities on Magnetic Resonance Imaging, and 343/1087 (32%) on Computed Tomography imaging. Endocrine and immunologic testing revealed 108/1135 (9.5%) patients were hyperglycemic; 125/1124 (11%) patients had elevated TSH; 149/1141 (13%) patients had a positive ANA; and 82/1133 (7.2%) patients were positive for RF. 198/1083 (18%) of patients were positive for HLA-B35, 246/1083 (23%) for HLA-Cw4, 454/1083 (42%) for HLA-Cw7, and 747/1060 (70%) of patients had absent HLA-DR4. 112/1085 (10%) of patients were positive for anti-68kD antibodies and 154/936 (17%) for protein 0. Many patients were diagnosed with previously unrecognized medical conditions. CONCLUSION: Comprehensive neurotological workup results in diagnoses that would go unrecognized otherwise, allowing patients to receive prompt treatment for medically important conditions, some of which may be causally related to their neurotologic complaints. However, the value of each study for routine testing of patients with neurotologic complaints remains controversial; and the evidence presented herein should help practitioners determine what studies should be included in their patient assessments.

Acute evaluation of the acute vestibular syndrome: differentiating posterior circulation stroke from acute peripheral vestibulopathies.

This review article aims to provide an evidence-based approach to evaluating the patient who presents with acute prolonged, spontaneous vertigo in the context of the acute vestibular syndrome (AVS). Differentiation of posterior circulation stroke (PCS) presenting as an AVS has been regarded as an important diagnostic challenge for physicians involved in acute care. Current evidence suggests that a targeted approach to history taking and physical examination with emphasis on the oculomotor examination, more specifically the HINTS (Head Impulse/Nystagmus/Test-of-skew) examination battery, yields a higher sensitivity for the diagnosis of PCS than even standard magnetic resonance imaging with diffusion-weighted imaging. However, most studies have only validated the utility of the HINTS examination when performed by experts, who interpret the most powerful component of HINTS, namely the head impulse test (HIT), considerably different to the novice. Several investigations useful in the differentiation of the AVS are becoming more accessible and portable, such as videooculography with Frenzel goggles and video head impulse testing (vHIT), which allows for the quantitative assessment of the HIT. In clinical practice, vHIT has already become accepted as standard of care in the evaluation of AVS.