Bone-Anchored Hearing Aid Effects on Vestibular Function: A Preliminary Report.

OBJECTIVE: Cochlear receptors are sensitive to vibratory stimuli. Based on this sensibility, bone-anchored hearing aids have been introduced to correct unilateral or bilateral conductive or mixed hearing loss and unilateral deafness. The vestibular system is also sensitive to the vibratory stimulus and this type of response is used in clinics to test its functionality. Being aware of this double separated sensibility, we wondered whether bone vibration, which activates the acoustic receptors of patients with bone conduction aids, can also influence the functionality of the vestibular system. METHODS: To this end, we recruited 12 patients with a bone-anchored hearing aid and evaluated their vestibular function with and without an activated vibratory acoustic device. RESULTS: Our results show that the vibratory stimulus delivered by the bone conduction aid also reaches and stimulates the vestibular receptors; this stimulation is evidenced by the appearance or modification of some nystagmus findings during bedside vestibular testing. Despite this, none of these patients complained of dizziness or vertigo during prosthesis use. Nystagmus that appeared or changed during acoustic vibratory stimulation through the prosthesis was almost all predominantly horizontal, unidirectional with respect to gaze or body position, inhibited by fixation, and most often consistent with vestibular function tests indicating peripheral vestibular damage. CONCLUSIONS: The findings of sound-evoked nystagmus seem to indicate peripheral rather than central vestibular activation. The occurrence of some predominantly horizontal and high-frequency induced nystagmus seems to attribute the response mainly to the utricle and lateral semicircular canal.

Phenotypes and clinical subgroups in vestibular migraine: a cross-sectional study with cluster analysis.

OBJECTIVE: The aim of this multicentric cross-sectional study was to collect phenotypes and clinical variability on a large sample of 244 patients enrolled in different university centers in Italy, trying to differentiate subtypes of VM. BACKGROUND: VM is one of the most frequent episodic vertigo characterized by a great clinical variability for duration of attacks and accompanying symptoms. Diagnosis is based only on clinical history of episodic vertigo in 50% of cases associated with migrainous headache or photo/phonophobia. METHODS: We enrolled in different university centers 244 patients affected by definite VM according to the criteria of the Barany Society between January 2022 and December 2022. An audiometric examination and a CNS MRI were performed before inclusion. Patients with low-frequency sensorineural hearing loss were not included, as well as patients with an MRI positive otherwise that for microischemic lesions. Patients were asked to characterize vestibular symptoms choosing among (multiple answers were allowed): internal vertigo, dizziness, visuo-vestibular symptoms/external vertigo; onset of vertigo and duration, neurovegetative, and cochlear accompanying symptoms (hearing loss, tinnitus, and fullness during attacks) were collected as well as migrainous headache and/or photo/phonophobia during vertigo; autoimmune disorders were also analyzed. A bedside examination was performed including study of spontaneous-positional nystagmus with infrared video goggles, post head shaking ny, skull vibration test, and video head impulse test. RESULTS: We included 244 subjects, 181 were females (74.2%). The age of onset of the first vertigo was 36.6 ± 14.5 while of the first headache was 23.2 ± 10.1. A positive correlation has been found between the first headache and the first vertigo. The mean duration of vertigo attacks was 11 ± 16 h. We carried on a cluster analysis to identify subgroups of patients with common clinical features. Four variables allowed to aggregate clusters: age of onset of vertigo, duration of vertigo attacks, presence of migrainous headache during vertigo, and presence of cochlear symptoms during vertigo. We identified 5 clusters: cluster 1/group 1 (23 subjects, 9.4%) characterized by longer duration of vertigo attacks; cluster 2/group 2 (52 subjects, 21.3%) characterized by absence of migrainous headache and cochlear symptoms during vertigo; cluster 3/group 3 (44 subjects, 18%) characterized by presence of cochlear symptoms during vertigo but not headache; cluster 4/group 4 (57 subjects, 23.4%) by the presence of both cochlear symptoms and migrainous headache during vertigo; cluster 5/group 5 (68 subjects, 27.9%) characterized by migrainous headache but no cochlear symptoms during vertigo. CONCLUSION: VM is with any evidence a heterogeneous disorder and clinical presentations exhibit a great variability. In VM, both symptoms orienting toward a peripheral mechanism (cochlear symptoms) and central ones (long lasting positional non-paroxysmal vertigo) may coexist. Our study is the first published trying to characterize subgroups of VM subjects, thus orienting toward different pathophysiological mechanisms.

External auditory canal ectopic atypical meningioma: A case report and brief literature review.

Meningiomas are tumours typically derived from the meningothelial cells of the arachnoid mater. They most often arise in intracranial, intraspinal, or orbital locations. Ectopic meningiomas, described as primary meningiomas with no intracranial involvement, are definitely unconventional. In fact, most of the extracranial meningiomas described in the literature, particularly in the outer ear, are effectively spreads of disease with primary intracranial localization. We describe a case of a primary external auditory canal meningioma with demonstrated absence of intracranial involvement, and we provide a full radiological, histological, immunohistochemical and molecular characterization of the lesion.

Treating benign paroxysmal positional vertigo of the lateral semicircular canal with a shortened forced position.

Benign paroxysmal positional vertigo (BPPV) is the peripheral vestibular disorder that is most frequently encountered in routine neuro-otological practice. Among the three semicircular canals, the lateral semicircular canal (LSC) is the second most frequently interested in the pathological process. In most cases, LSC BPPV is attributable to a canalithiasis or cupulolithiasis mechanism. The clinical picture of LSC BPPV is that of positional nystagmus and vertigo evoked by turning the head from the supine to the side lateral position. With such a movement, a horizontal positional (and often also paroxysmal) direction-changing nystagmus is generated. Depending on whether the pathogenetic mechanism is that of canalithiasis or cupulolithiasis and depending on where the dense particles are located, LSC BPPV direction-changing positional nystagmus is geotropic or apogeotropic on both lateral sides. Due to its mechanical nature, BPPV is effectively treated by means of physical therapy. In the case of a LSC BPPV, one of the most effective therapies is the forced prolonged position (FPP), in which the patient is invited to lie for 12 h on the lateral side on which vertigo and nystagmus are less intense, to move the canaliths out from the canal (or to shift them inside of the canal from one tract to another) exploiting the force of gravity. Despite its efficacy, FPP is not always well tolerated by every patient, and it cannot be done during the diagnostic session because of its duration. The present study aimed to verify the efficacy of a different forced position, shortened forced position (SFP), with respect to the original FPP. SFP treatment would allow patients to more easily bear the forced position and physicians to control the outcome almost immediately, possibly enabling them to dismiss patients without vertigo. After 1 h of lying on the side where vertigo and nystagmus are the less intense, 38 out of 53 (71.7%) patients treated with SFP were either healed or improved. Although the outcomes are not as satisfying as those of the original FPP, SFP should be considered as a therapeutic prospect, especially by those physicians who work in collaboration with emergency departments or otherwise encounter acute patients to cure them of vertigo as soon as possible.

Otoendoscopy in the era of narrow-band imaging: a pictorial review.

PURPOSE: Otoendoscopy represents the initial non-invasive diagnostic cornerstone for external and middle ear disorders. Recently, new techniques of enhanced imaging such as narrow-band imaging (NBI) have been introduced but their role as a potential aid in otological practice remains unproven. In this pictorial review, we want to present the potential application of this endoscopic method, highlight its limitations, and give some hints regarding its future implementation. METHODS: Representative cases of external and/or middle ear pathologies were selected to illustrate the role of NBI in this regard. RESULTS: NBI may represent a useful aid in the otological work-up, in the differential diagnosis of ear tumor-like masses, and, possibly, in the prognosis of tympanic perforations. For other ear disorders, instead, this technique does not seem to add anything to the standard clinical practice. CONCLUSIONS: NBI might prove useful in the assessment of selected external and middle ear disorders but its role must be prospectively validated.

Hearing outcomes and patient satisfaction after stapes surgery: local versus general anaesthesia.

Objective: Otosclerosis is a frequent ear disorder causing a stapedo-ovalar ankylosis and conductive hearing loss. Stapedoplasty, performed under both general (GA) and local anaesthesia (LA), is the most advisable surgical solution. Auditory recovery relies on the patient's conditions and on the intervention itself. The aim of our work was to compare hearing outcomes with stapedoplasty performed under GA or LA and to investigate patients' compliance to both methods. Methods: Fifty-five otosclerotic patients underwent stapedoplasty both under GA (32/55) and LA (23/55). Pre- and post-operative air and bone tone audiometry threshold values as well as the air-bone gap and its closure score, were analysed. All patients filled in a satisfaction questionnaire regarding their concern and level of appreciation of the type of anaesthesia. Results and conclusions: Our data show that the auditory results with stapedoplasty are good and do not differ between LA and GA. Even considering the advantages and limits of the two methods, one cannot favour one or the other type of anaesthesia. Finally, the patient's satisfaction cannot be considered a criterion of choice, since this was found to be high in both cases.

Vitamin D Insufficiency/Deficiency in Patients with Recurrent Benign Paroxysmal Positional Vertigo.

BACKGROUND: The aim of this study is to verify if (1) there is a link between hypovitaminosis D and benign paroxysmal positional vertigo, (2) the number of benign paroxysmal positional vertigo relapses decreases after vitamin D supplementation; and (3) benign paroxysmal positional vertigo response to physical therapy improves after hypovitaminosis D correction. METHODS: We enrolled 26 patients with benign paroxysmal positional vertigo and 24 subjects, who never suffered from vertigo, as a control group. All benign paroxysmal positional vertigo patients underwent physical therapy, once a week, until benign paroxysmal positional vertigo resolution. All participants were subjected to a dosage of serum 25(OH) vitamin D. In patients with hypovitaminosis D, we prescribed cholecalciferol. After 3 months of therapy, all patients were asked to undergo a second dosage of serum 25(OH) vitamin D. For each patient, we counted the number of maneuvers required to resolve each episode of benign paroxysmal positional vertigo before and after vitamin D supplementation. RESULTS: Our results suggest that (1) there is a relationship between vitamin D deficiency and the onset of BPPV and (2) hypovitaminosis correction is able to reduce both the number of patients relapsing and the number of relapses per patient. CONCLUSIONS: We have not found a significant effect of vitamin D supplementation as regards the responsivity of benign paroxysmal positional vertigo to physical therapy.

Syncope-presyncope and dizziness, differential diagnosis.

To date, there are no doubts about the evaluation of patients with syncope, while differential diagnoses between presyncope and nonspecific symptoms, generically referred to as dizziness, are complex and not clearly standardized. This paper aims to highlight the most frequent vestibular diseases, which can mimic a presyncopal episode in adult and older patients. It should be noted that nonspecific symptoms may be caused by multiple conditions, and they can coexist in the same person, making diagnosis even more difficult. Due to nonspecific symptoms and diagnostic complexity, evaluation of patients with presyncope or dizziness should be multidisciplinary, involving the syncope expert and the audiologist, since the first evaluation of the patient in the Emergency Department.

Papillary-cystic neoplasms of the middle ear are distinct from endolymphatic sac tumours.

AIMS: Papillary neoplasms of the middle and inner ear are rare and poorly characterised. The current World Health Organization classification divides them into two major subtypes: aggressive papillary tumours (APTs) and endolymphatic sac tumours (ELSTs). The aim of this article is to present two papillary neoplasms of the middle ear that do not fit into either the classic APT category or the classic ELST category, and compare them with three ELSTs. METHODS AND RESULTS: The patients were a 48-year-old female and a 59-year-old male without a history of other neoplasms. Histology showed papillary-cystic growth of predominantly oncocytic (Case 1) or mucinous (Case 2) cells surrounded by a p63-positive basal layer. The overall histology was reminiscent of oncocytic sinonasal papilloma (Case 1) and pancreatobiliary or salivary intraductal papillary mucinous neoplasms (Case 2). Ovarian-type stroma, invasion and malignant features were absent. Immunohistochemistry revealed expression of cytokeratin (CK) 7, but not carbonic anhydrase IX (CAIX) or paired box gene 8 (PAX8) (except for very focal PAX8 expression in Case 1). The TST15 gene panel and HRAS sequencing revealed no pathogenic mutations in BRAF, KRAS, EGFR, AKT1, or HRAS. The TruSight RNA fusion panel revealed an MKRN1-BRAF fusion in Case 1. No fusion was detected in Case 2. The three ELSTs showed classic features of the entity, expressed CK7, epithelial membrane antigen, PAX8, and CAIX, and lacked a basal cell layer. CONCLUSION: These novel cases suggest that papillary tumours of the ear represent a heterogeneous spectrum of distinct neoplasms unified by a prominent papillary-cystic pattern rather than a single entity. Future studies should clarify whether the MKRN1-BRAF fusion is a defining recurrent driver event, especially in those cases reported as sinonasal-type middle ear papillomas.

Double-Blind Randomized Trial on the Efficacy of the Forced Prolonged Position for Treatment of Lateral Canal Benign Paroxysmal Positional Vertigo.

OBJECTIVES/HYPOTHESIS: The need for class I and II studies on the efficacy of liberatory maneuvers in the treatment of lateral canal benign paroxysmal positional vertigo (LC-BPPV) motivated the present double-blind randomized trial on the short-term efficacy of the forced prolonged position (FPP). STUDY DESIGN: Double-blind, randomized controlled trial. METHODS: Two hundred twenty-one patients with unilateral LC-BPPV met the inclusion criteria for a multicentric study. Patients were randomly assigned to treatment by FPP (116 subjects) or sham treatment (105 subjects). Subjects were followed up at 24 hours with the supine roll test by blinded examiners. RESULTS: Among the sample, 67.4% and 32.6% of the patients showed respectively geotropic and apogeotropic variant of LC-BPPV. At the 24-hour follow-up, the effectiveness of FFP compared to the sham maneuver was, respectively, 57.8% versus 12.4% (P < .0001) in the total sample, 76.9% versus 11.3% (P < .0001) in the geotropic variant group, and 60.5% versus 17.6% (P = .0003) in the apogeotropic variant group, including resolution or transformation to geotropic variant. CONCLUSIONS: FPP proved highly effective compared to the sham maneuver. The present class 2 study of the efficacy of the FPP changes the level of recommendation of the method for treating LC-BPPV into a strong one. LEVEL OF EVIDENCE: 2 Laryngoscope, 131:E1296-E1300, 2021.

Upright BPPV Protocol: Feasibility of a New Diagnostic Paradigm for Lateral Semicircular Canal Benign Paroxysmal Positional Vertigo Compared to Standard Diagnostic Maneuvers.

Background: The diagnosis of benign paroxysmal positional vertigo (BPPV) involving the lateral semicircular canal (LSC) is traditionally entrusted to the supine head roll test, also known as supine head yaw test (SHYT), which usually allows identification of the pathologic side and BPPV form (geotropic vs. apogeotropic). Nevertheless, SHYT may not always allow easy detection of the affected canal, resulting in similar responses on both sides and intense autonomic symptoms in patients with recent onset of vertigo. The newly introduced upright head roll test (UHRT) represents a diagnostic maneuver for LSC-BPPV, supplementing the already-known head pitch test (HPT) in the sitting position. The combination of these two tests should enable clinicians to determine the precise location of debris within LSC, avoiding disturbing symptoms related to supine positionings. Therefore, we proposed the upright BPPV protocol (UBP), a test battery exclusively performed in the upright position, including the evaluation of pseudo-spontaneous nystagmus (PSN), HPT and UHRT. The purpose of this multicenter study is to determine the feasibility of UBP in the diagnosis of LSC-BPPV. Methods: We retrospectively reviewed the clinical data of 134 consecutive patients diagnosed with LSC-BPPV. All of them received both UBP and the complete diagnostic protocol (CDP), including the evaluation of PSN and data resulting from HPT, UHRT, seated-supine positioning test (SSPT), and SHYT. Results: A correct diagnosis for LSC-BPPV was achieved in 95.5% of cases using exclusively the UBP, with a highly significant concordance with the CDP (p < 0.000, Cohen's kappa = 0.94), regardless of the time elapsed from symptom onset to diagnosis. The concordance between UBP and CDP was not impaired even when cases in which HPT and/or UHRT provided incomplete results were included (p < 0.000). Correct diagnosis using the supine diagnostic protocol (SDP, including SSPT + SHYT) or the sole SHYT was achieved in 85.1% of cases, with similar statistical concordance (p < 0.000) and weaker strength of relationship (Cohen's kappa = 0.80). Conclusion: UBP allows correct diagnosis in LSC-BPPV from the sitting position in most cases, sparing the patient supine positionings and related symptoms. UBP could also allow clinicians to proceed directly with repositioning maneuvers from the upright position.

Congruous Torsional Down Beating Nystagmus in the Third Position of the Semont's Maneuver in Patients Treated for Canalithiasis of Posterior Semicircular Canal Benign Paroxysmal Positional Vertigo: Its Significance and Prognostic Value.

Due to its mechanical pathogenesis, benign paroxysmal positional vertigo treatment is mainly physical: when posterior semicircular canal is involved, Semont's maneuver is reported as one of the most effective liberating procedures. In the case of a canalolithiasis, the efficacy of the maneuver is corroborated by the appearance of some nystagmus findings during its performance. Liberating nystagmus, that can occur in the second position of Semont's maneuver and whose direction is congruous with the excitation of the affected posterior semicircular canal has proven to be a favorable prognostic sign. On the other hand, in clinical experience, we've frequently verified the appearance of another nystagmus during the execution of the maneuver: upon reaching the third position, when replacing the patient seated, a torsional down beating nystagmus, with the torsional component "congruous" with the stimulation of the vertical semicircular canals of the affected side, can often be appreciated. Such a sign can occur with or without having had the previous liberating nystagmus in the second position and is almost always associated with an intense vertigo and/or body pulsion. In this study, we describe the incidence and characteristics of the congruous torsional down beating nystagmus that can arise by assuming the third position of Semont's maneuver in a cohort of patients treated for posterior semicircular canal benign paroxysmal positional vertigo due to canalolithiasis. In the best of our knowledge, such a sign has never been described and explained before. On the basis of the pathophysiology and of the possible canal receptors stimulation during the different phases of Semont's maneuver, we formulated different hypothesis on how such a nystagmus can be generated. We observed that such a sign, when elicited, has a very good prognostic meaning for healing purposes, even better than that of liberating nystagmus. Therefore, congruous torsional down beating nystagmus should always be checked when performing Semont's maneuver because it could help in predicting success of physical treatment and in managing patients.

Corrigendum: Clinical Features of Headache in Patients With Diagnosis of Definite Vestibular Migraine: The VM-Phenotypes Projects.

[This corrects the article DOI: 10.3389/fneur.2018.00395.].

Clinical Features of Headache in Patients With Diagnosis of Definite Vestibular Migraine: The VM-Phenotypes Projects.

Migraine is a common neurological disorder characterized by episodic headaches with specific features, presenting familial aggregation. Migraine is associated with episodic vertigo, named Vestibular Migraine (VM) whose diagnosis mainly rely on clinical history showing a temporary association of symptoms. Some patient refers symptoms occurring in pediatric age, defined "episodic symptoms which may be associated with migraine." The aim of this cross sectional observational study was to assess migraine-related clinical features in VM subjects. For the purpose, 279 patients were recruited in different centers in Europe; data were collected by a senior neurologist or ENT specialist through a structured questionnaire. The age of onset of migraine was 21.8 ± 9. The duration of headaches was lower than 24 h in 79.1% of cases. Symptoms accompanying migrainous headaches were, in order of frequency, nausea (79.9%), phonophobia (54.5%), photophobia (53.8%), vomiting (29%), lightheadedness (21.1%). Visual or other auras were reported by 25.4% of subjects. A familial aggregation was referred by 67.4%, while migraine precursors were reported by 52.3% of subjects. Patients reporting nausea and vomiting during headaches more frequently experienced the same symptoms during vertigo. Comparing our results in VM subjects with previously published papers in migraine sufferers, our patients presented a lower duration of headaches and a higher rate of familial aggregation; moreover some common characters were observed in headache and vertigo attacks for accompanying symptoms like nausea and vomiting and clustering of attacks.

Retrospective analysis of patients with dizziness evaluated in Syncope Unit: a real life experience.

PURPOSE: To evaluate the characteristics of patients referred for dizziness to a Syncope Unit. METHODS: This is a retrospective study. Of 491 patients referred to the Syncope Unit of Careggi Hospital in 2015, 198 (40.3%) who experienced dizziness alone or associated with a history of transient loss of consciousness were enrolled. All the patients underwent an initial evaluation according to the European Society of Cardiology guidelines on syncope. We compared the clinical characteristics and final diagnosis of patients referred for dizziness alone (n = 64) to those of patients with dizziness and history of transient loss of consciousness (n = 134). RESULTS: The study population had a mean age of 62 ± 20 years (range 16-96 years) and 101 (51%) were female. A final diagnosis of pre-syncope was made in about the 80% of the patients without a previous history of transient loss of consciousness. In this group, other diagnoses were benign paroxysmal positional vertigo (6.3%), transient ischemic attack (4.7%) or psychogenic dizziness (7.8%). Syncope was diagnosed in the 82.7% of the patients with dizziness and history of transient loss of consciousness. CONCLUSION: Dizziness was the main reason for referral to the Syncope Unit in almost one-third of the patients, in whom pre-syncope was the most frequent final diagnosis. Otological, neurological and psychiatric disorders should be also considered as differential diagnosis, highlighting the importance of a multidisciplinary approach.

Duplex Sonography of Vertebral Arteries for Evaluation of Patients with Acute Vertigo.

We evaluated the role of vertebral artery extracranial color-coded duplex sonography (VAECCS) in predicting vertebrobasilar stroke in consecutive patients presenting to the emergency department with vertigo of suspected ischemic origin. The final diagnosis was established by a panel of experts consisting of an emergency physician, a neurologist, and an otoneurologist. Vertebrobasilar stroke was diagnosed when an acute brain ischemic lesion congruent with symptoms was detected by neuroimaging during the index visit or a stroke was diagnosed within a 3-mo period after emergency department presentation. Among 126 patients, 28 (22%) were diagnosed with vertebrobasilar stroke. Fifteen (75%) of 20 patients with abnormal VAECCS results and 13 (12%) of 106 with normal VAECCS results had a final diagnosis of vertebrobasilar stroke. The sensitivity and specificity of VAECCS were 53.6% and 94.9%, respectively. Detecting an abnormal flow pattern at VAECCS significantly increased the risk of vertebrobasilar stroke (odds ratio = 21.5). The flow patterns most frequently related to vertebrobasilar stroke were absence of flow and high resistance pattern velocity (odds ratio = 9.3 and 22.7, respectively). VAECCS predicts vertebrobasilar stroke and could be a useful bedside screening tool in patients with vertigo.

Differential Diagnosis of Vertigo in the Emergency Department: A Prospective Validation Study of the STANDING Algorithm.

OBJECTIVE: We investigated the reliability and accuracy of a bedside diagnostic algorithm for patients presenting with vertigo/unsteadiness to the emergency department. METHODS: We enrolled consecutive adult patients presenting with vertigo/unsteadiness at a tertiary hospital. STANDING, the acronym for the four-step algorithm we have previously described, based on nystagmus observation and well-known diagnostic maneuvers includes (1) the discrimination between SponTAneous and positional nystagmus, (2) the evaluation of the Nystagmus Direction, (3) the head Impulse test, and (4) the evaluation of equilibrium (staNdinG). Reliability of each step was analyzed by Fleiss' K calculation. The reference standard (central vertigo) was a composite of brain disease including stroke, demyelinating disease, neoplasm, or other brain disease diagnosed by initial imaging or during 3-month follow-up. RESULTS: Three hundred and fifty-two patients were included. The incidence of central vertigo was 11.4% [95% confidence interval (CI) 8.2-15.2%]. The leading cause was ischemic stroke (70%). The STANDING showed a good reliability (overall Fleiss K 0.83), the second step showing the highest (0.95), and the third step the lowest (0.74) agreement. The overall accuracy of the algorithm was 88% (95% CI 85-88%), showing high sensitivity (95%, 95% CI 83-99%) and specificity (87%, 95% CI 85-87%), very high-negative predictive value (99%, 95% CI 97-100%), and a positive predictive value of 48% (95% CI 41-50%) for central vertigo. CONCLUSION: Using the STANDING algorithm, non-sub-specialists achieved good reliability and high accuracy in excluding stroke and other threatening causes of vertigo/unsteadiness.