Is it possible to diagnose Posterior Semicircular Canal BPPV from the sitting position? The role of the Head Pitch Test and the upright tests along the RALP and LARP planes.

PURPOSE: The diagnosis of benign paroxysmal positional vertigo (BPPV) involving the posterior semicircular canal (PSC) is traditionally entrusted to positioning tests where patients are rapidly brought in the supine position. This prospective study aims to define the role of a diagnostic protocol for PSC-BPPV including only upright tests. MATERIALS AND METHODS: 109 patients with PSC-BPPV were enrolled. The Head Pitch Test (HPT) was carried out first. If uneventful, the patient's head was turned 45° to each side and bent back-and-forth along the plane aligning either with the right anterior-left posterior (RALP) or left anterior-right posterior (LARP) canals, thus performing the upright RALP / upright LARP (uRALP/uLARP) test. Nystagmus observed was used to predict the diagnosis, which was therefore confirmed by Dix-Hallpike tests. RESULTS: PSC-BPPV could be correctly diagnosed in 75.2% of cases with the sole HPT and in 87.2% of cases by adding the uRALP/uLARP test (Upright Protocol). The time elapsed from symptoms onset was closely related to the protocol sensitivity, as it reached 100% (64/64) in acute patients while decreased to 68.9% (31/45) in cases evaluated after 7 days (p < 0.001). CONCLUSIONS: Upright maneuvers could correctly diagnose PSC-BPPV in most cases. uRALP/uLARP test demonstrated to improve the sensitivity of the HPT, mainly in recent-onset BPPV.

Pneumolabyrinth: a systematic review.

PURPOSE: The aim of this study is to provide a systematic review of the literature about the etiology, clinical and radiological presentation, surgical management, and outcomes of pneumolabyrinth (PNL). METHODS: A systematic review of the literature was performed including studies published up to September 2020 in electronic databases (PubMed/MEDLINE, EMBASE, Cochrane Library, and Scopus). The PRISMA standard was applied to identify English, Italian, or French-language studies mentioning PNL. Full texts lacking information on the etiology were excluded. Data concerning the cause, site of air bubbles/fistula, clinical presentation, treatment, and outcome were collected. A qualitative synthesis of the results was performed. RESULTS: Seventy-eight articles were eventually included; 132 patients were involved in the qualitative synthesis. The most common causes were: stapes surgery (24/132, 18.2%), temporal bone fracture (42/132, 31.8%), head trauma without temporal bone fracture (19/132, 14.4%), penetrating trauma (21/132, 15.9%), and barotrauma (15/132, 11.4%). The site most commonly involved was the vestibule (102/107, 95.3%), followed by cochlea (43/107, 40.2%) and semicircular canals (25/107, 23.4%). CONCLUSION: The etiopathogenesis of PNL can be summarized in traumatic, iatrogenic, or inflammatory/infective. Its management consists in exploratory tympanotomy and sealing the fistula, but also conservative treatments can be attempted. Vestibular symptoms disappear in the majority of cases. Instead, the prognosis of hearing function is widely variable, and complete recovery is less probable. The certainty of evidence is still too low to make it useful for clinical decision-making.

The effect of superior canal dehiscence size and location on audiometric measurements, vestibular-evoked myogenic potentials and video-head impulse testing.

PURPOSE: To correlate objective measures of audio-vestibular function with superior canal dehiscence (SCD) size and location in ears with SCD and compare results with literature. METHODS: We retrospectively evaluated 242 patients exhibiting SCD and/or extremely thinned bone overlying superior canals (SC) on CT scans and selected 73 SCD patients (95 ears with SCD). Data concerning audiometry, impedance audiometry, video-head impulse test (vHIT), cervical vestibular-evoked myogenic potentials (cVEMPs) and ocular VEMPs (oVEMPs) to air- (AC) and bone-conducted (BC) stimuli were collected for each pathologic ear and correlated with dehiscence size and location. RESULTS: AC pure-tone average (PTA) (p = 0.013), low-frequency air-bone gap (ABG) (p < 0.001), AC cVEMPs amplitude (p = 0.002), BC cVEMPs amplitude (p < 0.001) and both AC and BC oVEMPs amplitude (p < 0.001) positively correlated with increasing SCD size. An inverse relationship between dehiscence length and both AC cVEMPs and oVEMPs thresholds (p < 0.001) and SC vestibulo-ocular reflex (VOR) gain (p < 0.001) was observed. Dehiscences at the arcuate eminence (AE) exhibited lower SC VOR gains compared to SCD along the ampullary arm (p = 0.008) and less impaired BC thresholds than dehiscences at the superior petrosal sinus (p = 0.04). CONCLUSION: We confirmed that SCD size affects AC PTA, ABG and both amplitudes and thresholds of cVEMPs and oVEMPs. We also described a tendency for SC function to impair with increasing SCD size and when dehiscence is located at the AE. The latter data may be explained either by a spontaneous canal plugging exerted by middle fossa dura or by a dissipation through the dehiscence of mechanical energy conveyed to the endolymph during high-frequency impulses.

Safe tracheotomy for patients with COVID-19.

Patients affected by severe acute respiratory syndrome coronavirus 2 disease (COVID-19) with respiratory distress may need invasive mechanical ventilation for a long period of time. Head and neck surgeons are becoming increasingly involved in the care of COVID-19 patients because of the rapidly increasing number of tracheotomies required. This procedure, when performed without protection, may lead to the infection of the medical and nursing staff caring for the patient. The aim of this report is to share our protocol for performing a safe surgical tracheotomy in COVID-19 patients. Infection of the nursing/medical staff involved in the first 30 tracheotomies performed in patients affected by COVID-19 in the Intensive Care Unit of a tertiary referral center were evaluated. Mistakes that occurred during surgery were analyzed and discussed. None of the nursing/medical staff presented signs or symptoms of COVID-19 within 15 days after the procedure. Conclusion: The authors have prepared a protocol for performing a safe surgical tracheotomy in patients affected by COVID-19. Surgeons who might be involved in performing the tracheotomies should become familiar with these guidelines.

Isolated horizontal canal hypofunction differentiating a canalith jam from an acute peripheral vestibular loss.

OBJECTIVES: To describe a unique case of acute vertigo presenting with spontaneous horizontal nystagmus (SHN) and a clinical picture consistent with right acute peripheral vestibular loss (APVL) in which an isolated hypofunction of a horizontal semicircular canal (HSC) permitted to detect a spontaneous canalith jam and treat the patient accordingly. METHODS: Case report and literature review. RESULTS: A 74-year old woman presented with acute vertigo, left-beating SHN and a clinical picture consistent with right APVL. Nevertheless, vestibular evoked myogenic potentials were normal with symmetrical amplitudes and the video head impulse test (vHIT) revealed an isolated hypofunction of the right HSC. After repeated head shakings, the supine roll test evoked bilaterally a positioning paroxysmal geotropic horizontal nystagmus suggesting benign paroxysmal positional vertigo involving the non-ampullated arm of the right HSC. vHIT and caloric testing confirmed restitution of HSC function after repositioning maneuvers. CONCLUSIONS: In case of acute vertigo with SHN, a complete functional assessment of vestibular receptors and afferents should always be given in order to avoid misdiagnosis. Canalith jam should be considered in case of spontaneous nystagmus and isolated canal hypofunction.

Long-term outcomes of central neck dissection for cN0 papillary thyroid carcinoma.

OBJECTIVE: The risk-benefit ratio of central neck dissection (CND) in patients affected by papillary thyroid carcinoma (PTC) without clinical or ultrasonographic (US) evidence of neck lymph node metastasis (cN0) is currently debated. The aim of this study was to evaluate long-term outcome of CND on locoregional recurrence, distant metastasis, survival, and postoperative complications in a large series of patients with cN0-PTC. STUDY DESIGN: Observational retrospective controlled study. METHODS: Clinical records of patients (n=610) surgically treated for cN0-PTC at the Otolaryngology Unit of the Arcispedale Santa Maria Nuova-IRCCS, Reggio Emilia, Italy, from January 1984 to December 2008, were retrospectively reviewed. Study population was divided into three groups according to surgical treatment: Group A, total thyroidectomy (n=205); Group B, total thyroidectomy and elective ipsilateral CND (n=281); Group C, total thyroidectomy and bilateral CND (n=124). RESULTS: Of a total of 610 patients, 305 (50%) were classified as low-risk, 278 (45.57%) as intermediate-risk, and 27 (4.43%) as high-risk. Response to initial therapy was excellent in 567 patients (92.95%), acceptable in 21 (3.44%), and incomplete in 22 (3.61%), with no significant differences among groups. Locoregional recurrence was detected in 32 (5.2%) out of 610 patients. Distant metastasis was found in 15 patients (2.5%). Statistical analysis showed no significant differences in the rates of locoregional recurrence (p=0.890) or distant metastasis (p=0.538) among groups. Disease-specific mortality and overall survival did not significantly differ among groups (p=0.248 and 0.223, respectively). Rate of permanent hypoparathyroidism was significantly higher in Group C patients compared to those in Groups A and B. CONCLUSION: CND does not confer any clear advantage in the treatment of low-risk patients, regardless of surgical procedure. Instead, bilateral CND may be effective in limiting disease relapse and/or progression in patients at higher prognostic risk. Our data indicate that elective CND does not confer any clear advantage in terms of locoregional recurrence and long-term survival, as demonstrated by outcomes of the study Groups, regardless of their different prognostic risk. Elective CND allows a more accurate pathologic staging of central neck lymph nodes, despite its increasing the risk of permanent hypoparathyroidism. Intraoperative pathologic staging is a valuable tool to assess the risk of controlateral lymph node metastasis in the central neck compartment and to limit more aggressive surgery only to cases, otherwise understaged, with lymph node metastasis.

Audiovestibular Findings in Patients with Concurrent Superior Canal Dehiscence and Vestibular Schwannoma.

OBJECTIVE: To describe the clinical-instrumental findings in case of concurrent superior canal dehiscence (SCD) and ipsilateral vestibular schwannoma (VS), aiming to highlight the importance of an extensive instrumental assessment to achieve a correct diagnosis. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Five patients with concurrent SCD and VS. INTERVENTION: Clinical-instrumental assessment and imaging. MAIN OUTCOME MEASURE: Clinical presentation, audiovestibular findings, and imaging. RESULTS: The chief complaints were hearing loss (HL) and unsteadiness (80%). Other main symptoms included tinnitus (60%) and pressure-induced vertigo (40%). Mixed-HL was identified in three patients and pure sensorineural-HL in 1, including a roll-over curve in speech-audiometry in two cases. Vibration-induced nystagmus was elicited in all cases, whereas vestibular-evoked myogenic potentials showed reduced thresholds and enhanced amplitudes on the affected side in three patients. Ipsilesional weakness on caloric testing was detected in three patients and a bilateral hyporeflexia in one. A global canal impairment was detected by the video-head impulse test in one case, whereas the rest of the cohort exhibited a reduced function for the affected superior canal, together with ipsilateral posterior canal impairment in two cases. All patients performed both temporal bones HRCT scan and brain-MRI showing unilateral SCD and ipsilateral VS, respectively. All patients were submitted to a wait-and-scan approach, requiring VS removal only in one case. CONCLUSION: Simultaneous SCD and VS might result in subtle clinical presentation with puzzling lesion patterns. When unclear symptoms and signs occur, a complete audiovestibular assessment plays a key role to address imaging and diagnosis.

Rational approach to the prescription of anti-rheumatic drugs in rheumatoid arthritis: a product leaflet-based strategy in Italy.

The treatment of patients with rheumatoid arthritis (RA) has dramatically changed in the past 30 years. Currently, numerous conventional, biologic, and targeted synthetic DMARDs have been licensed and used following recommendations provided by international and national scientific societies. However, the availability of biosimilars and the increasing necessity of savings impacted on the local/national prescription of these drugs. The information provided by data sheet of every single drug is a decisive factor on the choice of a certain treatment merged with the patient's profile. Thus, our purpose was to construct a rational algorithm for the treatment strategy in RA according to costs and the product leaflet of the biologic and targeted-synthetic DMARDs currently licensed in Italy. We used the most recent available recommendations and then we performed a review of the literature considering all the factors that are known to influence drug safety/effectiveness. All these factors were considered in the context of the data sheets of currently available originators and biosimilars.

Skull Vibration-Induced Nystagmus in Superior Semicircular Canal Dehiscence: A New Insight into Vestibular Exploration-A Review.

The third window syndrome, often associated with the Tullio phenomenon, is currently most often observed in patients with a superior semicircular-canal dehiscence (SCD) but is not specific to this pathology. Clinical and vestibular tests suggestive of this pathology are not always concomitantly observed and have been recently complemented by the skull-vibration-induced nystagmus test, which constitutes a bone-conducted Tullio phenomenon (BCTP). The aim of this work was to collect from the literature the insights given by this bedside test performed with bone-conducted stimulations in SCD. The PRISMA guidelines were used, and 10 publications were included and analyzed. Skull vibration-induced nystagmus (SVIN), as observed in 55 to 100% of SCD patients, usually signals SCD with greater sensitivity than the air-conducted Tullio phenomenon (ACTP) or the Hennebert sign. The SVIN direction when the test is performed on the vertex location at 100 Hz is most often ipsilaterally beating in 82% of cases for the horizontal and torsional components and down-beating for the vertical component. Vertex stimulations are more efficient than mastoid stimulations at 100 Hz but are equivalent at higher frequencies. SVIN efficiency may depend on stimulus location, order, and duration. In SCD, SVIN frequency sensitivity is extended toward high frequencies, with around 400 Hz being optimal. SVIN direction may depend in 25% on stimulus frequency and in 50% on stimulus location. Mastoid stimulations show frequently diverging results following the side of stimulation. An after-nystagmus observed in 25% of cases can be interpreted in light of recent physiological data showing two modes of activation: (1) cycle-by-cycle phase-locked activation of action potentials in SCC afferents with irregular resting discharge; (2) cupula deflection by fluid streaming caused by the travelling waves of fluid displacement initiated by sound or vibration at the point of the dehiscence. The SVIN direction and intensity may result from these two mechanisms' competition. This instability explains the SVIN variability following stimulus location and frequency observed in some patients but also discrepancies between investigators. SVIN is a recent useful insight among other bedside examination tests for the diagnosis of SCD in clinical practice.

Consensus paper on the management of acute isolated vertigo in the emergency department.

Acute vertigo is defined as the perception of movement of oneself or the surroundings in the absence of actual motion and it is a frequent cause for emergency department admissions. The utilization of medical resources and the duration of hospital stay for this kind of symptom is high. Furthermore, the efficiency of brain imaging in the acute phase is low, considering the limited sensitivity of both CT and MRI for diagnosing diseases that are the causes of central type of vertigo. Relying on imaging tests can provide false reassurance in the event of negative results or prolong the in-hospital work-up improperly. On the other hand, clinical examinations, notably the assessment of nystagmus' features, have proven to be highly accurate and efficient when performed by experts. Literature data point out that emergency physicians often do not employ these skills or use them incorrectly. Several clinical algorithms have been introduced in recent years with the aim of enhancing the diagnostic accuracy of emergency physicians when evaluating this specific pathology. Both the 'HINTS and 'STANDING' algorithms have undergone external validation in emergency physician hands, showing good diagnostic accuracy. The objective of this consensus document is to provide scientific evidence supporting the clinical decisions made by physicians assessing adult patients with acute vertigo in the emergency department, particularly in cases without clear associated neurological signs. The document aims to offer a straightforward and multidisciplinary approach. At the same time, it tries to delineate benchmarks for the formulation of local diagnostic and therapeutic pathways, as well as provide a base for the development of training and research initiatives.

Vestibular Hypofunction in ARSACS Syndrome: A Possible Pitfall in the Differential Diagnosis of Recessive Cerebellar and Afferent Ataxias.

Objectives: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is an early-onset ataxia characterized by cerebellar dysfunction, spasticity, and sensory-motor polyneuropathy due to variations in the SACS gene (13q11). To date, no studies have instrumentally assessed vestibular function in this condition. Methods: We report a 36-year-old woman with diagnosis of ARSACS syndrome due to homozygous mutation (c.12232 C>T, p.Arg4078Ter) in the SACS gene. Neurologic examination showed spastic-ataxic gait, dysarthric speech, 4-limb ataxia, and spastic hypertonia with lower limb hyperreflexia. Results: A vestibular instrumental evaluation including bedside oculomotor testing found gaze-evoked and rebound nystagmus on horizontal and vertical gaze, saccadic movements within normality ranges, saccadic pursuit, and slightly impaired visually enhanced vestibulo-ocular reflex (VVOR). A near-normal VOR suppression (VORS) was recorded. Neither head shakings, skull vibrations, nor supine positionings could evoke nystagmus. Finally, the video-head impulse test detected a symmetrical VOR impairment for all the semicircular canals (SCs), mostly involving the horizontal SCs, with corrective saccades in all planes. Discussion: Vestibular hypofunction may be found in ARSACS syndrome and may represent a possible pitfall in the differential diagnosis of recessive cerebellar and afferent ataxias. In this setting, ARSACS syndrome should be considered in the differential diagnosis of CANVAS.

Posterior Semicircular Canal Dehiscence with Vestibulo-Ocular Reflex Reduction for the Affected Canal at the Video-Head Impulse Test: Considerations to Pathomechanisms.

Posterior semicircular canal dehiscence (PSCD) has been demonstrated to result in a third mobile window mechanism (TMWM) in the inner ear similar to superior semicircular canal dehiscence (SSCD). Typical clinical and instrumental features of TMWM, including low-frequency conductive hearing loss (CHL), autophony, pulsatile tinnitus, sound/pressure-induced vertigo and enhanced vestibular-evoked myogenic potentials, have been widely described in cases with PSCD. Nevertheless, video-head impulse test (vHIT) results have been poorly investigated. Here, we present six patients with PSCD presenting with a clinical scenario consistent with a TMWM and an impaired vestibulo-ocular reflex (VOR) for the affected canal on vHIT. In two cases, an additional dehiscence between the facial nerve and the horizontal semicircular canal (HSC) was detected, leading to a concurrent VOR impairment for the HSC. While in SSCD, a VOR gain reduction could be ascribed to a spontaneous "auto-plugging" process due to a dural prolapse into the canal, the same pathomechanism is difficult to conceive in PSCD due to a different anatomical position, making a dural herniation less likely. Alternative putative pathomechanisms are discussed, including an endolymphatic flow dissipation during head impulses as already hypothesized in SSCD. The association of symptoms/signs consistent with TMWM and a reduced VOR gain for the posterior canal might address the diagnosis toward PSCD.

Ocular vestibular evoked myogenic potentials in patients with acoustic neuroma.

To assess the usefulness of vestibular testing in patients with acoustic neuroma, considering two main aspects: to compare diagnostic sensitivity of the current vestibular tests, especially considering ocular vestibular evoked myogenic potentials (OVEMPs) and to identify pre-operative localization of the tumor (inferior vestibular nerve vs. superior vestibular nerve) only with the help of vestibular electrophysiological data. Twenty-six patients with unilateral acoustic neuroma (mainly intracanalicular type) were studied with a full audio-vestibular test battery (pure tone and speech audiometry, caloric bithermal test, vibration-induced nystagmus test (VIN), cervical and OVEMPs). 18 patients (69 %) showed abnormal caloric responses. 12 patients (46.2 %) showed a pattern of VIN test suggestive of vestibular asymmetry. 16 patients (61.5 %) showed abnormal OVEMPs (12 only to AC, 4 both to AC and BC). 10 patients (38.5 %) showed abnormal cervical vestibular evoked myogenic potentials (5 both to AC and BC, 5 only to AC). In one case, results of vestibular evoked potentials and caloric test were confirmed by intra-operative and post-operative findings. Results of electrophysiological tests in AN patients could be helpful for planning the proper surgical approach, considering that sensitivity of every exam is quite low in intracanalicular lesion; clinical data allow a better interpretation of vestibular evoked myogenic potentials.

Low-Frequency Air-Bone Gap and Pulsatile Tinnitus Due to a Dural Arteriovenous Fistula: Considerations upon Possible Pathomechanisms and Literature Review.

Low-frequency air-bone gap (ABG) associated with pulsatile tinnitus (PT) and normal impedance audiometry represents a common finding in patients with third window syndromes. Other inner disorders, including Meniere's disease (MD), perilymphatic fistula and intralabyrinthine schwannoma, might sometimes result in a similar scenario. On the other hand, PT is frequently associated with dural arteriovenous fistula (DAVF), while conductive hearing loss (CHL) is extremely rare in this clinical setting. A 47-year-old patient was referred to our center with progressive left-sided PT alongside ipsilateral fullness and hearing loss. She also experienced headache and dizziness. Otoscopy and video-oculographic examination were unremarkable. Conversely, a detailed instrumental audio-vestibular assessment revealed low-frequency CHL with normal impedance audiometry, slight left-sided caloric weakness, slightly impaired vestibular-evoked myogenic potentials on the left and normal results on the video-head impulse test, consistent with an MD-like instrumental profile. Gadolinium-enhanced brain MRI revealed an early enhancement of the left transverse sinus, consistent with a left DAVF between the left occipital artery and the transverse sinus, which was then confirmed by angiography. A trans-arterial embolization with Onyx glue was performed, resulting in a complete recession of the symptoms. Post-operatively, the low-frequency ABG disappeared, supporting the possible role of venous intracranial hypertension and abnormal pressure of inner ear fluids in the onset of symptoms and offering new insights into the pathomechanism of inner ear CHL.

A bone-conducted Tullio phenomenon-A bridge to understand skull vibration induced nystagmus in superior canal dehiscence.

Nystagmus produced in response to air-conducted sound (ACS) stimulation-the Tullio phenomenon-is well known in patients with a semicircular canal (SCC) dehiscence (SCD). Here we consider the evidence that bone-conducted vibration (BCV) is also an effective stimulus for generating the Tullio phenomenon. We relate the clinical evidence based on clinical data extracted from literature to the recent evidence about the physical mechanism by which BCV may cause this nystagmus and the neural evidence confirming the likely mechanism. The hypothetical physical mechanism by which BCV activates SCC afferent neurons in SCD patients is that traveling waves are generated in the endolymph, initiated at the site of the dehiscence. We contend that the nystagmus and symptoms observed after cranial BCV in SCD patients is a variant of Skull Vibration Induced Nystagmus (SVIN) used to identify unilateral vestibular loss (uVL) with the major difference being that in uVL the nystagmus beats away from the affected ear whereas in Tullio to BCV the nystagmus beats usually toward the affected ear with the SCD. We suggest that the cause of this difference is a cycle-by-cycle activation of SCC afferents from the remaining ear, which are not canceled centrally by simultaneous afferent input from the opposite ear, because of its reduced or absent function in uVL. In the Tullio phenomenon, this cycle-by-cycle neural activation is complemented by fluid streaming and thus cupula deflection caused by the repeated compression of each cycle of the stimuli. In this way, the Tullio phenomenon to BCV is a version of skull vibration-induced nystagmus.

Impaired Vestibulo-Ocular Reflex on Video Head Impulse Test in Superior Canal Dehiscence: "Spontaneous Plugging" or Endolymphatic Flow Dissipation?

Surgical plugging of the superior semicircular canal (SSC) represents an effective procedure to treat disabling symptoms in superior canal dehiscence (SCD), despite resulting in an impaired vestibulo-ocular reflex (VOR) gain for the SSC. On the other hand, SSC hypofunction on video head impulse test (vHIT) represents a common finding in patients with SCD exhibiting sound/pressure-induced vertigo, a low-frequency air-bone gap (ABG), and enhanced vestibular-evoked myogenic potentials (VEMPs). "Spontaneous canal plugging" has been assumed as the underlying process. Nevertheless, missing/mitigated symptoms and/or near-normal instrumental findings would be expected. An endolymphatic flow dissipation has been recently proposed as an alternative pathomechanism for SSC VOR gain reduction in SCD. We aimed to shed light on this debate by comparing instrumental findings from 46 ears of 44 patients with SCD exhibiting SSC hypofunction with post-operative data from 10 ears of 10 patients with SCD who underwent surgical plugging. While no difference in SSC VOR gain values was found between the two groups (p = 0.199), operated ears developed a posterior canal hypofunction (p = 0.002). Moreover, both ABG values (p = 0.012) and cervical/ocular VEMP amplitudes (p < 0.001) were significantly higher and VEMP thresholds were significantly lower (p < 0.001) in ears with SCD compared to operated ears. According to our data, canal VOR gain reduction in SCD should be considered as an additional sign of a third window mechanism, likely due to an endolymphatic flow dissipation.

Vestibular assessment in sudden sensorineural hearing loss: Role in the prediction of hearing outcome and in the early detection of vascular and hydropic pathomechanisms.

Introduction: Predicting hearing outcome in sudden sensorineural hearing loss (SSNHL) is challenging, as well as detecting the underlying pathomechanisms. SSNHL could be associated with vestibular damage since cochleo-vestibular structures share the same vascularization, along with being in close anatomical proximity. Whereas viral inflammations and autoimmune/vascular disorders most likely represent the involved aetiologies, early-stage Menière's disease (MD) can also present with SSNHL. Since an early treatment could beneficially influence hearing outcome, understanding the possible etiology plays a pivotal role in orienting the most appropriate treatment. We aimed to evaluate the extent of vestibular damage in patients presenting with SSNHL with or without vertigo, investigate the prognostic role of vestibular dysfunctions on hearing recovery and detect specific lesion patterns related to the underlying pathomechanisms. Methods: We prospectively evaluated 86 patients with SSNHL. Audio-vestibular investigation included pure-tone/speech/impedance audiometry, cervical/ocular-VEMPs, vHIT and video-Frenzel examination. White matter lesions (WML) were evaluated on brain-MRI. Patients were followed-up and divided into "SSNHL-no-vertigo," "SSNHL+vertigo" and "MD" subgroups. Results: Hearing was more impaired in "SSNHL+vertigo" patients who exhibited either down-sloping or flat-type audiograms, and was less impaired in "MD" where low frequencies were mostly impaired (p < 0.001). Otolith receptors were more frequently involved than semicircular canals (SCs). Although the "SSNHL-no-vertigo" subgroup exhibited the lowest vestibular impairment (p < 0.001), 52% of patients developed otolith dysfunctions and 72% developed nystagmus. Only "MD" subjects showed anterior SC impairment and upbeating spontaneous/positional nystagmus. They more frequently exhibited cervical-VEMPs frequency tuning (p = 0.036) and ipsilesional spontaneous nystagmus (p < 0.001). "SSNHL+vertigo" subjects presented with more frequently impaired cervical-VEMPs and posterior SC and with higher number of impaired receptors (p < 0.001). They mainly exhibited contralesional spontaneous and vibration-induced nystagmus (p < 0.05) and only they showed the highest WML score and "vascular" lesion patterns (p < 0.001). Concerning the outcomes, hearing was better in "MD" and worse in "SSNHL+vertigo" (p < 0.001). Hearing recovery was mostly affected by cervical-VEMPs impairment and the number of involved receptors (p < 0.05). Patients with "vascular" lesion patterns presented with the highest HL degree and WML score (p ≤ 0.001), while none of them exhibited a complete hearing recovery (p = 0.026). Conclusions: Our data suggest that vestibular evaluation in SSNHL can provide useful information on hearing recovery and underlying aetiologies.

Speech, Gait, and Vestibular Function in Cerebellar Ataxia with Neuropathy and Vestibular Areflexia Syndrome.

(1) Background: Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is characterized by late-onset cerebellar ataxia, bilateral vestibulopathy, and sensory neuronopathy mostly due to biallelic RFC1 expansion. (2) Objectives: The aim of this case series is to describe vestibular, gait, and speech alterations in CANVAS via a systematic approach. (3) Methods: All patients (n = 5) underwent a standardized clinical-instrumental examination, including the perceptual and acoustic analysis of speech, instrumental gait, and balance analysis (posturographic data were acquired using a force plate [Kistler, Winterthur, Switzerland] while 3D gait analysis, inclusive of surface electromyography, was acquired using a motion capture system [SMART DX, BTS Bioengineering, Milan, Italy], a wireless electromyograph [FreeEMG, BTS Bioengineering, Milan, Italy]), and vestibular assessment with video-oculography. (4) Results: Five patients were included in the analysis: three females (patients A, B, C) and two males (patients D and E) with a mean age at evaluation of 62 years (SD ± 15.16, range 36-74). The mean age of symptoms' onset was 55.6 years (SD ± 15.04, range 30-68), and patients were clinically and instrumentally evaluated with a mean disease duration of 6.4 years (SD ± 0.54, range 6-7). Video-Frenzel examination documented spontaneous downbeat nystagmus enhanced on bilateral gaze in all patients, except for one presenting with slight downbeat nystagmus in the supine position. All patients exhibited different degrees of symmetrically reduced VOR gain for allsix semicircular canals on the video-head impulse test and an unexpectedly normal ("false negative") VOR suppression, consistent with combined cerebellar dysfunction and bilateral vestibular loss. Posturographic indices were outside their age-matched normative ranges in all patients, while 3D gait analysis highlighted a reduction in ankle dorsiflexion (limited forward rotation of the tibia over the stance foot during the stance phase of gait and fatigue of the dorsiflexor muscles) and variable out-of-phase activity of plantar flexors during the swing phase. Finally, perceptual-acoustic evaluation of speech showed ataxic dysarthria in three patients. Dysdiadochokinesis, rhythm instability, and irregularity were observed in the oral diadochokinesis task. (5) Conclusions: CANVAS is a recently discovered syndrome that is gaining more and more relevance within late-onset ataxias. In this paper, we aimed to contribute to a detailed description of its phenotype.