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.

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.

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.

Pericranial Flap-Based Multilayer Reconstruction of Endoscopic Transcribriform Craniectomy for Sinonasal Malignancies.

OBJECTIVE: Diffusion of endoscopic techniques for the resection of ethmoid bone malignancies through a transcribriform approach (TA) has raised new challenges regarding reconstruction options to reduce post-operative complications. Although there is consensus on the advantages of vascularized flaps over free grafts for large defects, no standard protocol exists on reconstruction procedures. In addition, although the pedicled nasoseptal flap has been extensively discussed, few studies have been published on extranasal pedicled flaps. The aim of this manuscript is to provide a detailed description of a reconstruction technique for large anterior skull base defects with the pericranial flap as part of a multilayered reconstruction. Moreover, patients treated with this approach were retrospectively assessed for post-operative complications. METHODS: A detailed description of the reconstruction procedure as performed in our departments is provided. Pictures depicting the main surgical steps are also included. In addition, preliminary functional results from a retrospective series of patients who underwent a TA and subsequent pericranial flap-based multilayer reconstruction for ethmoid roof malignancies between 2016 and 2022 at two institutional centers are reported. RESULTS: 16 patients were included in the study. Nine patients (56.3%) underwent adjuvant radiotherapy. Two patients had a biochemically-confirmed postoperative CSF leak. Only one of the two patients required surgical revision. During follow-up (mean 13 months), no other early nor delayed complications were observed. CONCLUSION: A standardized surgical technique with pericranial flap as part of a multilayered reconstruction for large anterior skull base defects following resection of sinonasal malignancies is proposed, which appears to be a safe choice when endonasal flaps are not available. LEVEL OF EVIDENCE: Level 4 Laryngoscope, 133:2942-2947, 2023.

Exclusive endoscopic ossiculoplasty with autologous material: step-by-step procedure and functional results.

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.