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Vestibular atelectasis (VA) is a rare clinical entity characterized by a collapse of the endolymphatic space resulting in vestibular loss with the possible onset of positional and/or sound/pressure-induced vertigo. It could be idiopathic or secondary to other inner-ear diseases including Meniere’s disease (MD). A collapse of the membranous labyrinth involving the semicircular canals (SCs) and the utricle represents its distinctive histopathological feature. While specific radiological patterns consistent with VA have been described on contrast-enhanced MRI with delayed acquisitions, an impairment of the blood–labyrinthine barrier (BLB) could be detected in several disorders leading to vestibular loss. We conducted a narrative review of the literature on VA focusing on the putative pathomechanisms accounting for positional and sound/pressure-induced nystagmus despite unilateral vestibular loss (UVL) in this condition, providing two novel cases of VA. Both patients presented with a clinical picture consistent with unilateral MD that rapidly turned into progressive UVL and positional and/or sound/pressure-induced vertigo. In both cases, the posterior SC was initially impaired at the video-head impulse test (vHIT) and both cervical and ocular VEMPs were initially reduced. Progressively, they developed unsteadiness with paretic spontaneous nystagmus, an impairment also for the lateral and anterior SCs, caloric hypo/areflexia and VEMPs areflexia. They both exhibited ipsilesional nystagmus to sound/pressure stimuli and in one case a persistent geotropic direction-changing positional nystagmus consistent with a “light cupula” mechanism involving the lateral SC of the affected side. A collapse of the membranous labyrinthine walls resulting in contact between the vestibular sensors and the stapes footplate could explain the onset of nystagmus to loud sounds and/or pressure changes despite no responses to high- and low-frequency inputs as detected by caloric irrigations, vHIT and VEMPs. On the other hand, the onset of positional nystagmus despite UVL could be explained with the theory of the “floating labyrinth”. Both patients received contrast-enhanced brain MRI with delayed acquisition exhibiting increased contrast uptake in the pars superior of the labyrinth, suggesting an impairment of the BLB likely resulting in secondary VA. A small intralabyrinthine schwannoma was detected in one case. VA should always be considered in case of positional and/or sound/pressure-induced vertigo despite UVL.

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.

(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.

Purpose To describe the surgical procedure of exclusive endoscopic ossiculoplasty (EEO) with autologous grafts and evaluate audiological results, focusing on the advantages or drawbacks compared to the corresponding microscopic technique. Methods A retrospective review of consecutive adult and pediatric patients affected by chronic otitis media (COM) with or without cholesteatoma who underwent EEO was conducted. Only autologous ossiculoplasty was included in the study. The procedure was performed by experienced surgeons of our institution between November 2014 and September 2019. Hearing outcomes were evaluated using postoperative air–bone gap (ABG) and success rates in different subgroups of patients and different types of ossiculoplasty (OPL) were analyzed. Our results were finally compared with the existing literature regarding both microscopic and endoscopic ossicular chain reconstruction. Results In total, 74 endoscopic ossicular chain repair procedures performed within the study period met the inclusion criteria. Of these, 21 were pediatric patients (28%) and 53 were adults (72%). Surgical reconstruction procedures included 43 partial ossicular reconstructions (POR) and 31 total ossicular reconstructions (TOR). The postoperative ABG improved significantly compared to preoperative measurements, and the mean ABG closure was 7.85 dB HL ( p  = 0.00064). No statistically significant differences in audiological outcomes between TOR/POR techniques and pediatric/adult groups were found in our study cohort, with p values of 0.10 and 0.88, respectively. Conclusions At present, EEO can be considered a valid surgical option for re-establishing a functioning ossicular chain with acceptable hearing restoration in children and adults. Further reports in wider case series are required to confirm these results.

Aims The association between gestational diabetes mellitus (GDM) and common carotid artery (CCA) intima-media thickness (IMT) is still controversial. This systematic review and meta-analysis was performed to assess the correlation between GDM and CCA-IMT in and after pregnancy. Methods PubMed and EMBASE databases were systematically reviewed on April 2023. Studies measuring CCA-IMT in both pregnant women with GDM and women with previous history of GDM (pGDM) vs. healthy controls were included. The subtotal and overall standardized mean differences (SMDs) of CCA-IMT were calculated using the random-effect model. Results Nineteen studies with a total of 302 GDM and 861 pGDM women were analyzed. The average value of CCA-IMT measured in GDM/pGDM (0.59 ± 0.12 mm) was slightly increased in comparison to the accepted reference limits of IMT according to age classes. Substantial heterogeneity was detected for the studies involving both GDM and pGDM women, with an overall statistic I 2 of 86.0% ( p  < 0.001). Large SMDs were obtained for the studies conducted on both GDM and pGDM women, with an overall SMD of 0.89 (95%CI 0.63–1.15, p  < 0.001). Egger's test for a regression intercept gave a p -value of 0.37, indicating no publication bias. On meta-regression analysis, all potential confounders (number of patients, age at pregnancy, body mass index, measuring time, follow-up duration and GDM criteria) were not significantly associated with effect modification. Conclusions GDM in and after pregnancy is independently associated with subclinical atherosclerosis. The association between GDM and carotid remodeling is potentially mediated by the longstanding underlying risk.