Acute Unilateral Peripheral Vestibulopathy After COVID-19 Vaccination: Initial Experience in a Tertiary Neurotology Center

ObjectiveThe aim of the present study was to identify patients who developed acute unilateral peripheral vestibulopathy (AUPVP) after COVID-19 vaccination.MethodsFor this single-center, retrospective study, we screened the medical records of our tertiary interdisciplinary neurotology center for patients who had presented with AUPVP within 30 days after COVID-19 vaccination (study period: 1 June−31 December 2021). The initial diagnosis of AUPVP was based on a comprehensive bedside neurotological examination. Laboratory vestibular testing (video head impulse test, cervical and ocular vestibular evoked myogenic potentials, dynamic visual acuity, subjective visual vertical, video-oculography, caloric testing) was performed 1–5 months later.ResultsTwenty-six patients were diagnosed with AUPVP within the study period. Of those, n = 8 (31%) had developed acute vestibular symptoms within 30 days after COVID-19 vaccination (mean interval: 11.9 days, SD: 4.8, range: 6–20) and were thus included in the study. The mean age of the patients (two females, six males) was 46 years (SD: 11.7). Seven patients had received the Moderna mRNA vaccine and one the Pfizer/BioNTech mRNA vaccine. All patients displayed a horizontal(-torsional) spontaneous nystagmus toward the unaffected ear and a pathological clinical head impulse test toward the affected ear on initial clinical examination. Receptor-specific laboratory vestibular testing performed 1–5 months later revealed recovery of vestibular function in two patients, and heterogeneous lesion patterns of vestibular endorgans in the remaining six patients.Discussion and ConclusionsThe present study should raise clinicians' awareness for AUPVP after COVID-19 vaccination. The relatively high fraction of such cases among our AUPVP patients may be due to a certain selection bias at a tertiary neurotology center. Patients presenting with acute vestibular symptoms should be questioned about their vaccination status and the date of the last vaccination dose. Furthermore, cases of AUPVP occurring shortly after a COVID-19 vaccination should be reported to the health authorities to help determining a possible causal relationship

Postural stability and handicap of dizziness after preoperative vestibular ablation and vestibular prehabilitation in patients undergoing vestibular schwannoma resection

BACKGROUND: Surgical treatment of vestibular schwannoma (VS) leads to acute ipsilateral vestibular loss if there is residual vestibular function before surgery. To overcome the sequelae of acute ipsilateral vestibular loss and to decrease postoperative recovery time, the concept of preemptive vestibular ablation with gentamicin and vestibular prehabilitation before surgery has been developed (“vestibular prehab”). OBJECTIVE: Studying postural stability during walking and handicap of dizziness over a 1-year follow-up period in VS patients undergoing vestibular prehab before surgical treatment of VS. METHODS: A retrospective review of consecutive patients with a diagnosis of a VS undergoing surgical therapy from June 2012 to March 2018 was performed. All patients were included with documentation of the length of hospital duration and the Dizziness Handicap Inventory (DHI) and the Functional Gait Assessment (FGA) assessed preoperatively as well as 6 weeks and 1 year postoperatively. RESULTS: A total 68 VS patients were included, of which 29 patients received preoperative vestibular ablation by intratympanic injection of gentamicin. Mean VS diameter was 20.2 mm (SD 9.4 mm) and mean age at surgery was 49.6 years (SD 11.5 years). Vestibular prehab had no effect on DHI and FGA at any time point studied. CONCLUSIONS: We found no effect of vestibular prehab on postural stability during walking and on the handicap of dizziness. These findings add to the body of knowledge consisting of conflicting results of vestibular prehab. Therefore, vestibular prehab should be applied only in selected cases in an experimental setting

Internal auditory canal volume in normal and malformed inner ears

Purpose: A narrow bony internal auditory canal (IAC) may be associated with a hypoplastic cochlear nerve and poorer hearing performances after cochlear implantation. However, definitions for a narrow IAC vary widely and commonly, qualitative grading or two-dimensional measures are used to characterize a narrow IAC. We aimed to refine the definition of a narrow IAC by determining IAC volume in both control patients and patients with inner ear malformations (IEMs). Methods: In this multicentric study, we included high-resolution CT (HRCT) scans of 128 temporal bones (85 with IEMs: cochlear aplasia, n = 11; common cavity, n = 2; cochlear hypoplasia type, n = 19; incomplete partition type I/III, n = 8/8; Mondini malformation, n = 16; enlarged vestibular aqueduct syndrome, n = 19; 45 controls). The IAC diameter was measured in the axial plane and the IAC volume was measured by semi-automatic segmentation and three-dimensional reconstruction. Results: In controls, the mean IAC diameter was 5.5 mm (SD 1.1 mm) and the mean IAC volume was 175.3 mm3 (SD 52.6 mm3). Statistically significant differences in IAC volumes were found in cochlear aplasia (68.3 mm3, p < 0.0001), IPI (107.4 mm3, p = 0.04), and IPIII (277.5 mm3, p = 0.0004 mm3). Inter-rater reliability was higher in IAC volume than in IAC diameter (intraclass correlation coefficient 0.92 vs. 0.77). Conclusions: Volumetric measurement of IAC in cases of IEMs reduces measurement variability and may add to classifying IEMs. Since a hypoplastic IAC can be associated with a hypoplastic cochlear nerve and sensorineural hearing loss, radiologic assessment of the IAC is crucial in patients with severe sensorineural hearing loss undergoing cochlear implantation. Keywords: 3D segmentation; Cochlear malformation; Diagnosis; Inner ear malformation; Internal auditory canal; Volum

Endotype-Phenotype Patterns in Meniere's Disease Based on Gadolinium-Enhanced MRI of the Vestibular Aqueduct

Two histopathological subtypes of Meniere's disease (MD) were recently described in a human post-mortem pathology study. The first subtype demonstrated a degenerating distal endolymphatic sac (ES) in the affected inner ear (subtype MD-dg); the second subtype (MD-hp) demonstrated an ES that was developmentally hypoplastic. The two subtypes were associated with different clinical disease features (phenotypes), suggesting that distinct endotype-phenotype patterns exist among MD patients. Therefore, clinical endotyping based on ES pathology may reveal clinically meaningful MD patient subgroups. Here, we retrospectively determined the ES pathologies of clinical MD patients (n = 72) who underwent intravenous delayed gadolinium-enhanced inner ear magnetic resonance imaging using previously established indirect radiographic markers for both ES pathologies. Phenotypic subgroup differences were evidenced; for example, the MD-dg group presented a higher average of vertigo attacks (ratio of vertigo patterns daily/weekly/other vs. monthly, MD-dg: 6.87: 1; MD-hp: 1.43: 1; p = 0.048) and more severely reduced vestibular function upon caloric testing (average caloric asymmetry ratio, MD-dg: 30.2% ± 30.4%; MD-hp: 13.5% ± 15.2%; p = 0.009), while the MD-hp group presented a predominantly male sex ratio (MD-hp: 0.06:1 [f/m]; MD-dg: 1.2:1 [f/m]; p = 0.0004), higher frequencies of bilateral clinical affection (MD-hp: 29.4%; MD-dg: 5.5%; p = 0.015), a positive family history for hearing loss/vertigo/MD (MD-hp: 41.2%; MD-dg: 15.7%; p = 0.028), and radiographic signs of concomitant temporal bone abnormalities, i.e., semicircular canal dehiscence (MD-hp: 29.4%; MD-dg: 3.6%; p = 0.007). In conclusion, this new endotyping approach may potentially improve the diagnosis, prognosis and clinical decision-making for individual MD patients

Volumetry improves the assessment of the vestibular aqueduct size in inner ear malformation

Objectives: Enlarged vestibular aqueduct (EVA) is a common finding associated with inner ear malformations (IEM). However, uniform radiologic definitions for EVA are missing and various 2D-measurement methods to define EVA have been reported. This study evaluates VA volume in different types of IEM and compares 3D-reconstructed VA volume to 2D-measurements. Methods: A total of 98 high-resolution CT (HRCT) data sets from temporal bones were analyzed (56 with IEM; [cochlear hypoplasia (CH; n = 18), incomplete partition type I (IPI; n = 12) and type II (IPII; n = 11) and EVA (n = 15)]; 42 controls). VA diameter was measured in axial images. VA volume was analyzed by software-based, semi-automatic segmentation and 3D-reconstruction. Differences in VA volume between the groups and associations between VA volume and VA diameter were assessed. Inter-rater-reliability (IRR) was assessed using the intra-class-correlation-coefficient (ICC). Results: Larger VA volumes were found in IEM compared to controls. Significant differences in VA volume between patients with EVA and controls (p < 0.001) as well as between IPII and controls (p < 0.001) were found. VA diameter at the midpoint (VA midpoint) and at the operculum (VA operculum) correlated to VA volume in IPI (VA midpoint: r = 0.78, VA operculum: r = 0.91), in CH (VA midpoint: r = 0.59, VA operculum: r = 0.61), in EVA (VA midpoint: r = 0.55, VA operculum: r = 0.66) and in controls (VA midpoint: r = 0.36, VA operculum: r = 0.42). The highest IRR was found for VA volume (ICC = 0.90). Conclusions: The VA diameter may be an insufficient estimate of VA volume, since (1) measurement of VA diameter does not reliably correlate with VA volume and (2) VA diameter shows a lower IRR than VA volume. 3D-reconstruction and VA volumetry may add information in diagnosing EVA in cases with or without additional IEM. Keywords: 3D segmentation; Cochlear malformation; Diagnosis; Inner ear malformation; Volum

Der „Professional Ear User“ – Implikationen für die Prävention, Diagnostik und Therapie von Ohrerkrankungen

BACKGROUND Perfect hearing is crucial to the practice of various professions, such as instrument makers, musicians, sound engineers, and other professions not related to music, such as sonar technicians. For people of these occupational groups, we propose the term "professional ear user" (PEU) in analogy to "professional voice user". PEUs have special requirements for their hearing health, as they have well-known above-average auditory perceptual abilities on which they are professionally dependent. OBJECTIVE The purpose of this narrative review is to summarize selected aspects of the prevention, diagnosis, and treatment of ear disorders in PEUs. RESULTS AND CONCLUSION Prevention of hearing disorders and other ear diseases includes protection from excessive sound levels, avoidance of ototoxins and nicotine, and a safe manner of cleaning the outer auditory canal. Diagnosing hearing disorders in PEUs can be challenging, since subclinical but relevant changes in hearing cannot be reliably objectified by conventional audiometric methods. Moreover, the fact that a PEU is affected by an ear disease may influence treatment decisions. Further, physicians must be vigilant for non-organic ear diseases in PEUs. Lastly, measures to promote comprehensive ear health in PEUs as part of an educational program and to maintain ear health by means of a specialized otolaryngology service are discussed. In contrast to existing concepts, we lay the attention on the entirety of occupational groups that are specifically dependent on their ear health in a professional setting. In this context, we suggest avoiding a sole focus on hearing disorders and their prevention, but rather encourage the maintenance of a comprehensive ear health.Hintergrund Ein vollständig intaktes Hörvermögen ist zentral für die Ausübung verschiedener Berufe wie Instrumentenbaumeister, Musiker, Tonmeister sowie für weitere Berufsgruppen ohne Bezug zu Musik wie beispielsweise Sonar-Techniker. Für Personen all dieser Berufsgruppen schlagen wir in Analogie zum „Professional Voice User“ den Begriff „Professional Ear User“ (PEU) vor. PEU haben spezielle Anforderungen an ihre Ohrgesundheit, da sie über eine überdurchschnittliche auditive Wahrnehmungsfähigkeit verfügen, von der sie beruflich abhängig sind. Fragestellung Die vorliegende narrative Übersichtsarbeit hat zum Ziel, die sich daraus ergebenden speziellen Aspekte der Prävention, Diagnostik und Therapie von Ohrerkrankungen bei PEU zusammenzufassen. Ergebnisse und Schlussfolgerung Die Prävention von Hörstörungen und weiteren Ohrerkrankungen umfasst den Schutz vor zu hohen Schallpegeln, die Vermeidung von Ototoxinen oder Nikotin sowie die korrekte Durchführung einer Gehörgangsreinigung. Die Abklärung von Hörstörungen kann sich bei PEU herausfordernd gestalten, da subklinische, jedoch einschränkende Veränderungen des Hörvermögens mit konventionellen audiometrischen Methoden nicht zuverlässig objektiviert werden können. Schließlich kann das Vorliegen einer Ohrerkrankung bei einem PEU Therapieentscheidungen beeinflussen. Weiter muss bei PEU auch eine hohe Wachsamkeit bezüglich nichtorganischer Ohrerkrankungen bestehen. Abschließend werden Möglichkeiten diskutiert, um bei PEU eine umfassende Ohrgesundheit im Rahmen eines edukativen Programms zu fördern und mittels einer spezialisierten ohrenärztlichen Sprechstunde zu erhalten. Im Gegensatz zu bestehenden Konzepten ist der Fokus dabei auf die Gesamtheit der Berufsgruppen gerichtet, welche in professionellem Rahmen speziell von der Ohrgesundheit abhängig sind. Außerdem soll der Schwerpunkt hierbei nicht nur auf Hörstörungen und deren Prävention, sondern auch auf der Erhaltung einer ganzheitlichen Ohrgesundheit liegen

Der „Professional Ear User“ – Implikationen für die Prävention, Diagnostik und Therapie von Ohrerkrankungen

Zusammenfassung Hintergrund Ein vollständig intaktes Hörvermögen ist zentral für die Ausübung verschiedener Berufe wie Instrumentenbaumeister, Musiker, Tonmeister sowie für weitere Berufsgruppen ohne Bezug zu Musik wie beispielsweise Sonar-Techniker. Für Personen all dieser Berufsgruppen schlagen wir in Analogie zum „Professional Voice User“ den Begriff „Professional Ear User“ (PEU) vor. PEU haben spezielle Anforderungen an ihre Ohrgesundheit, da sie über eine überdurchschnittliche auditive Wahrnehmungsfähigkeit verfügen, von der sie beruflich abhängig sind. Fragestellung Die vorliegende narrative Übersichtsarbeit hat zum Ziel, die sich daraus ergebenden speziellen Aspekte der Prävention, Diagnostik und Therapie von Ohrerkrankungen bei PEU zusammenzufassen. Ergebnisse und Schlussfolgerung Die Prävention von Hörstörungen und weiteren Ohrerkrankungen umfasst den Schutz vor zu hohen Schallpegeln, die Vermeidung von Ototoxinen oder Nikotin sowie die korrekte Durchführung einer Gehörgangsreinigung. Die Abklärung von Hörstörungen kann sich bei PEU herausfordernd gestalten, da subklinische, jedoch einschränkende Veränderungen des Hörvermögens mit konventionellen audiometrischen Methoden nicht zuverlässig objektiviert werden können. Schließlich kann das Vorliegen einer Ohrerkrankung bei einem PEU Therapieentscheidungen beeinflussen. Weiter muss bei PEU auch eine hohe Wachsamkeit bezüglich nichtorganischer Ohrerkrankungen bestehen. Abschließend werden Möglichkeiten diskutiert, um bei PEU eine umfassende Ohrgesundheit im Rahmen eines edukativen Programms zu fördern und mittels einer spezialisierten ohrenärztlichen Sprechstunde zu erhalten. Im Gegensatz zu bestehenden Konzepten ist der Fokus dabei auf die Gesamtheit der Berufsgruppen gerichtet, welche in professionellem Rahmen speziell von der Ohrgesundheit abhängig sind. Außerdem soll der Schwerpunkt hierbei nicht nur auf Hörstörungen und deren Prävention, sondern auch auf der Erhaltung einer ganzheitlichen Ohrgesundheit liegen. Abstract Background Perfect hearing is crucial to the practice of various professions, such as instrument makers, musicians, sound engineers, and other professions not related to music, such as sonar technicians. For people of these occupational groups, we propose the term “professional ear user” (PEU) in analogy to “professional voice user”. PEUs have special requirements for their hearing health, as they have well-known above-average auditory perceptual abilities on which they are professionally dependent. Objective The purpose of this narrative review is to summarize selected aspects of the prevention, diagnosis, and treatment of ear disorders in PEUs. Results and conclusion Prevention of hearing disorders and other ear diseases includes protection from excessive sound levels, avoidance of ototoxins and nicotine, and a safe manner of cleaning the outer auditory canal. Diagnosing hearing disorders in PEUs can be challenging, since subclinical but relevant changes in hearing cannot be reliably objectified by conventional audiometric methods. Moreover, the fact that a PEU is affected by an ear disease may influence treatment decisions. Further, physicians must be vigilant for non-organic ear diseases in PEUs. Lastly, measures to promote comprehensive ear health in PEUs as part of an educational program and to maintain ear health by means of a specialized otolaryngology service are discussed. In contrast to existing concepts, we lay the attention on the entirety of occupational groups that are specifically dependent on their ear health in a professional setting. In this context, we suggest avoiding a sole focus on hearing disorders and their prevention, but rather encourage the maintenance of a comprehensive ear health

Radiological feature heterogeneity supports etiological diversity among patient groups in Meniere's disease

We aimed to determine the prevalence of radiological temporal bone features that in previous studies showed only a weak or an inconsistent association with the clinical diagnosis of Meniere's disease (MD), in two groups of MD patients (n = 71) with previously established distinct endolymphatic sac pathologies; i.e. the group MD-dg (ES degeneration) and the group MD-hp (ES hypoplasia). Delayed gadolinium-enhanced MRI and high-resolution CT data were used to determine and compare between and within (affected vs. non-affected side) groups geometric temporal bone features (lengths, widths, contours), air cell tract volume, height of the jugular bulb, sigmoid sinus width, and MRI signal intensity alterations of the ES. Temporal bone features with significant intergroup differences were the retrolabyrinthine bone thickness (1.04 ± 0.69 mm, MD-hp; 3.1 ± 1.9 mm, MD-dg; p < 0.0001); posterior contour tortuosity (mean arch-to-chord ratio 1.019 ± 0.013, MD-hp; 1.096 ± 0.038, MD-dg; p < 0.0001); and the pneumatized volume (1.37 [0.86] cm3, MD-hp; 5.25 [3.45] cm3, MD-dg; p = 0.03). Features with differences between the affected and non-affected sides within the MD-dg group were the sigmoid sinus width (6.5 ± 1.7 mm, affected; 7.6 ± 2.1 mm, non-affected; p = 0.04) and the MRI signal intensity of the endolymphatic sac (median signal intensity, affected vs. unaffected side, 0.59 [IQR 0.31-0.89]). Radiological temporal bone features known to be only weakly or inconsistently associated with the clinical diagnosis MD, are highly prevalent in either of two MD patient groups. These results support the existence of diverse-developmental and degenerative-disease etiologies manifesting with distinct radiological temporal bone abnormalities