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.

A Bilateral Vestibular Schwannoma is Not Always Related to Neurofibromatosis Type 2.

Bilateral vestibular schwannomas are commonly diagnosed in patients affected by neurofibromatosis type 2, a genetic disease caused by a heterozygous mutation in the gene region encoding neurofibromin-2. Sporadic bilateral vestibular schwannomas are very rare entities affecting almost exclusively elderly people. We present the case of a senior woman who was followed up with the "wait-and-scan" strategy for a unilateral vestibular schwannoma that later developed as a contralateral tumor, compatible with vestibular schwannoma, raising questions about its nature and risk of having been transmitted in offspring. Genetic testing excluded mutations of the neurofibromatosis type 2 gene. The presence of bilateral vestibular schwannomas is often considered pathognomonic of neurofibromatosis type 2, but the estimated probability of sporadic bilateral tumors in the absence of other neurofibromatosis type 2 features is 50% over 70 years of age. Therefore, the NF2 gene assessment is in any case recommended in these patients not only for an evaluation of the risk of being transmitted. The treatment strategy should be carefully personalized for each patient, considering the size of the tumors, symptoms, and hearing function together with the patient's age.

Evidence-based surveillance protocol for vestibular schwannomas: a long-term analysis of tumor growth using conditional probability.

OBJECTIVE: The growth characteristics of vestibular schwannomas (VSs) under surveillance can be studied using a Bayesian method of growth risk stratification by time after surveillance onset, allowing dynamic evaluations of growth risks. There is no consensus on the optimum surveillance strategy in terms of frequency and duration, particularly for long-term growth risks. In this study, the long-term conditional probability of new VS growth was reported for patients after 5 years of demonstrated nongrowth. This allowed modeling of long-term VS growth risks, the creation of an evidence-based surveillance protocol, and the proposal of a cost-benefit analysis decision aid. METHODS: The authors performed an international multicenter retrospective analysis of prospectively collected databases from five tertiary care referral skull base units. Patients diagnosed with sporadic unilateral VS between 1990 and 2010 who had a minimum of 10 years of surveillance MRI showing VS nongrowth in the first 5 years of follow-up were included in the analysis. Conditional probabilities of growth were calculated according to Bayes' theorem, and nonlinear regression analyses allowed modeling of growth. A cost-benefit analysis was also performed. RESULTS: A total of 354 patients were included in the study. Across the surveillance period from 6 to 10 years postdiagnosis, a total of 12 tumors were seen to grow (3.4%). There was no significant difference in long-term growth risk for intracanalicular versus extracanalicular VSs (p = 0.41). At 6 years, the residual conditional probability of growth from this point onward was seen to be 2.28% (95% CI 0.70%-5.44%); at 7 years, 1.35% (95% CI 0.25%-4.10%); at 8 years, 0.80% (95% CI 0.07%-3.25%); at 9 years, 0.47% (95% CI 0.01%-2.71%); and at 10 years, 0.28% (95% CI 0.00%-2.37%). Modeling determined that the remaining lifetime risk of growth would be less than 1% at 7 years 7 months, less than 0.5% at 8 years 11 months, and less than 0.25% at 10 years 4 months. CONCLUSIONS: This multicenter study evaluates the conditional probability of VS growth in patients with long-term VS surveillance (6-10 years). On the basis of these growth risks, the authors posited a surveillance protocol with imaging at 6 months (t = 0.5), annually for 3 years (t = 1.5, 2.5, 3.5), twice at 2-year intervals (t = 5.5, 7.5), and a final scan after 3 years (t = 10.5). This can be used to better inform patients of their risk of growth at particular points along their surveillance timeline, balancing the risk of missing late growth with the costs of repeated imaging. A cost-benefit analysis decision aid was also proposed to allow units to make their own decisions regarding the cessation of surveillance.

Which Inner Ear Disorders Lie Behind a Selective Posterior Semicircular Canal Hypofunction on Video Head Impulse Test?

OBJECTIVE: To assess all different patterns of associated abnormalities on audiometry, bithermal caloric test (BCT) and cervical/ocular vestibular-evoked myogenic potentials (VEMPs) to air/bone-conduction in patients with selective posterior semicircular canal (PSC) hypofunction and to correlate them with underlying disorders. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: 51 patients (23 men, 28 women, mean age 57.5 yr) with isolated PSC deficit (one bilateral). INTERVENTIONS: Correlation with instrumental data and underlying diagnoses. MAIN OUTCOME MEASURES: Video-oculographic findings, objective measurements on audiometry, BCT, VEMPs and video-head impulse test (vHIT). RESULTS: Ongoing or previous acute vestibular loss (AVL) was diagnosed in 13 patients (25.5%, 3 inferior vestibular neuritis, 10 AVL with sudden sensorineural hearing loss [SSNHL]), Meniere's disease (MD) in 12 (23.5%), cerebellopontine angle (CPA) lesion in 9 (17.6%), various causes in 7 (13.7%), benign paroxysmal positional vertigo (BPPV) involving the non-ampullary arm of PSC in 5 cases (9.8%) whereas unknown pathology in 5 (9.8%). Involvement of at least one additional receptor besides PSC was seen in 89.8% of cases. Cochlear involvement was diagnosed in 74.5% with pure-tone average significantly greater in patients with AVL+SSNHL (p < 0.05). Overall involvement of labyrinthine receptors or afferents was highest in patients with AVL+SSNHL (p < 0.01), MD and CPA lesions (p < 0.05). CONCLUSIONS: Isolated loss of PSC function on vHIT is mostly accompanied by additional labyrinthine deficits that could only be identified through an accurate instrumental evaluation. Assessment of all receptors and afferents should be always pursued to identify the lesion site and better understand the underlying pathophysiological mechanisms.

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.

Early experience in tracheostomy and tracheostomy tube management in Covid-19 patients.

In Italy, we have experienced Europe's first and largest coronavirus outbreak. Based on our preliminary experience, we discuss the challenges in performing tracheotomy and tracheostoma care in the setting of a new pathogen.

Two Different Extranodal Lymphomas in an HIV+ Patient: A Case Report and Review of the Literature.

Human immune deficiency virus- (HIV-) infected individuals present a higher risk of developing malignancies. Herein, we are presenting an unusual case of an untreated HIV+ patient, who developed two distinct lymphoproliferative disorders in a period of 4 years: a primary cutaneous T-cell lymphoma (PCTCL) and a diffuse large B-cell lymphoma (DLBCL) not otherwise specified (NOS), the latter developed while commencing combined antiretroviral therapy (cART). The two lymphomas also showed peculiar features: PCTCL are rarely described in HIV+ setting and particularly at such a low clinical stage, and the DLBCL showed uncommon cytology, non-GCB phenotype, EBER negativity, and absence of c-MYC translocation, all atypical features in this clinical context. This report not only confirms the increased risk of lymphoma for HIV+ patients and HIV infection being one of the major risk factors for lymphoid disorders but draws the attention on the possible occurrence of unusual features, suggesting that HIV serology should always be investigated in the clinical suspicion of lymphoma.

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.

Superior canal dehiscence with tegmen defect revealed by otoscopy: Video clip demonstration of pulsatile tympanic membrane.

Superior canal dehiscence is a pathologic condition of the otic capsule acting as aberrant window of the inner ear. It results in reduction of inner ear impedance and in abnormal exposure of the labyrinthine neuroepithelium to the action of the surrounding structures. The sum of these phenomena leads to the onset of typical cochleo-vestibular symptoms and signs. Among them, pulsatile tinnitus has been attributed to a direct transmission of intracranial vascular activities to labyrinthine fluids. We present the first video-otoscopic documentation of spontaneous pulse-synchronous movements of the tympanic membrane in two patients with superior canal dehiscence. Pulsating eardrum may represent an additional sign of third-mobile window lesion.

IFI16 Expression Is Related to Selected Transcription Factors during B-Cell Differentiation.

The interferon-inducible DNA sensor IFI16 is involved in the modulation of cellular survival, proliferation, and differentiation. In the hematopoietic system, IFI16 is consistently expressed in the CD34+ stem cells and in peripheral blood lymphocytes; however, little is known regarding its regulation during maturation of B- and T-cells. We explored the role of IFI16 in normal B-cell subsets by analysing its expression and relationship with the major transcription factors involved in germinal center (GC) development and plasma-cell (PC) maturation. IFI16 mRNA was differentially expressed in B-cell subsets with significant decrease in IFI16 mRNA in GC and PCs with respect to naïve and memory subsets. IFI16 mRNA expression is inversely correlated with a few master regulators of B-cell differentiation such as BCL6, XBP1, POU2AF1, and BLIMP1. In contrast, IFI16 expression positively correlated with STAT3, REL, SPIB, RELA, RELB, IRF4, STAT5B, and STAT5A. ARACNE algorithm indicated a direct regulation of IFI16 by BCL6, STAT5B, and RELB, whereas the relationship between IFI16 and the other factors is modulated by intermediate factors. In addition, analysis of the CD40 signaling pathway showed that IFI16 gene expression directly correlated with NF-κB activation, indicating that IFI16 could be considered an upstream modulator of NF-κB in human B-cells.

Management of growing vestibular schwannomas.

Conservative management of small vestibular schwannomas is frequently proposed as most tumours do not grow. Anyway, tumour growth is reported in 30-40 % of the cases, so that surgery is consequently generally proposed. We primarily observed 161 patients affected by unilateral vestibular schwannomas. All patients were examined by means of gadolinium-enhanced magnetic resonance imaging scans. Tumour growth was recorded in 58 cases (35.8 %) and these subjects set up the group of study. Twenty-two (37.9 %) patients were surgically treated; tumour was always completely removed, all patients had normal facial function after surgery and only one patient suffered from a major complication (cerebellar haematoma). Fourteen patients (24.1 %) were submitted to radiotherapy, while one patient was lost at follow-up and another one died because of other medical reasons. Finally, 20 (34.5 %) subjects continued to be observed for different reasons. The mean follow-up period after identification of growth was 6.1 years. Nine tumours continued to grow, nine tumours stopped growing, one tumour grew and then regressed in size and one tumour decreased. Sixty percent of patients with useful hearing at diagnosis preserved it during the entire observation period. In conclusion, most of VS do not grow; in case of tumour growth, a surgical procedure may be suggested and the outcomes are not negatively influenced by the delay of the procedure. But in some cases, patients can still follow the "wait and scan" policy. In fact, only less than half of the growing tumours continued to grow. Moreover, most of the patients continued to retain a useful hearing.

Diuretics in Meniere disease: a therapy or a potential cause of harm?

Despite the lack of clear evidence for their effectiveness in treating vertigo, tinnitus, hearing loss and aural fullness, diuretics, represent a common treatment for Meniere disease (MD), as they are supposed to decrease volume and pressure in the endolymphatic partition of the labyrinth. Our group have outlined the possibility of an adverse effect on inner ear function derived from an abrupt lowering of blood pressure: a subsequent exaggerated vasomotor response inducing local ischemia could be responsible for more or less permanent damage. The inner ear, owing both to its terminal vascular supply and to the necessity of a steady metabolism, seems a reliable target for any hemodynamic imbalance that acutely affect its perfusion. In our opinion, the complexity of the inner ear anatomy and function argues against the usefulness of diuretics to reduce endolymphatic volume, in analogy to their effect on the volemia: too many active mechanisms and "buffer" systems are involved in the labyrinth. Even considering that the finding of mean low pressure values is relatively common in subjects with MD, an attempt should be to maintain a stable blood perfusion to the labyrinth; in fact, an abrupt decrease in systemic blood pressure can trigger an adverse sympathetic reaction and transmit misleading information to the cochlear vasopressin receptors.