Unilateral enlarged vestibular aqueduct syndrome and bilateral endolymphatic hydrops

Enlarged vestibular aqueduct (EVA) syndrome is a common congenital inner ear malformation characterized by a vestibular aqueduct with a diameter larger than 1.5 mm, mixed or sensorineural hearing loss that ranges from mild to profound, and vestibular disorders that may be present with a range from mild imbalance to episodic objective vertigo. In our study, we present the case of a patient with unilateral enlarged vestibular aqueduct and bilateral endolymphatic hydrops (EH). EH was confirmed through anamnestic history and audiological exams; EVA was diagnosed using high-resolution CT scans and MRI images. Therapy included intratympanic infusion of corticosteroids with a significant hearing improvement, more evident in the ear contralateral to EVA. Although most probably unrelated, EVA and EH may present with similar symptoms and therefore the diagnostic workup should always include the proper steps to perform a correct diagnosis. Association between progression of hearing loss and head trauma in patients with a diagnosis of EVA syndrome is still uncertain; however, these individuals should be advised to avoid activities that increase intracranial pressure to prevent further hearing deterioration. Intratympanic treatment with steroids is a safe and well-tolerated procedure that has demonstrated its efficacy in hearing, tinnitus, and vertigo control in EH

Day-case management of chronic suppurative otitis media with cholesteatoma with canal wall down technique surgery: long-term follow-up

The overall number of day-case otologic surgery cases is increasing; however, there is limited experience about performing canal wall down tympanoplasty in patients with chronic suppurative otitis media with cholesteatoma in this setting. The objective of this study was to assess the success of this technique as day-case surgery in terms of results and complications over an 8-year follow up period. We included in this study 42 patients undergoing canal wall down technique tympanoplasty surgery for chronic suppurative otitis media with cholesteatoma performed as day cases during a 2-year period. 30 cases (71.4%) were discharged on the day of surgery, whereas 12 cases (28.6%) were hospitalized and discharged the day after. The principal reasons for failure of discharge on the day of surgery were asthenia (6 cases), vertigo and asthenia (4 cases), undetermined (2 cases). Based on our experience, with a proper preoperative selection, assessment and screening of the patients, mastoidectomy with timpanoplasty for chronic suppurative otitis media with cholesteatoma can be carried out in a day surgery setting with no significant effects on effectiveness of surgery, post-operative symptoms and relapse of disease even in the long term

Quality of life measurements for patients with chronic suppurative otitis media: Italian adaptation of "Chronic Ear Survey". La misura della qualità della vita in pazienti con otite media suppurativa cronica: adattamento in italiano del “Chronic Ear Survey”

Il Chronic Ear Survey (CES) è una misura specifica della Qualità della Vita (QoL) nei pazienti affetti da Otite Media Suppurativa Cronica (CSOM). È un questionario composto da 13 domande che indagano frequenza, durata e severità dei sintomi associati a questa malattia. Il CES genera tre sottoscale con rispettivo punteggio che riguardano limitazioni nelle attività fisiche e sociali, sintomi e trattamento medico. Attraverso le risposte ottenute dai pazienti è possibile ricavare un punteggio che va da 0 a 100; il punteggio più alto indica una QoL migliore, mentre quello più basso indica una QoL peggiore. Il questionario è stato creato in lingua inglese. Lo scopo del lavoro è di validare in lingua italiana il CES. La traduzione è stata condotta seguendo le linee guida internazionali. La versione italiana del CES (CES-I) è stata proposta a 54 pazienti con CSOM. Nello stesso tempo, è stato somministrato a tutti i pazienti anche il questionario SF-36. Un modello trasversale è stato usato per esaminare la consistenza interna (Cronbach alpha) e la validità esterna (coefficiente di Pearson). Per confermare la validità esterna del CES-I è stato poi analizzato il test di correlazione di Pearson considerando il punteggio totale, le singole sottoscale del CES e le 8 scale dello Short Form Health Survey (SF-36). Il coefficiente di Cronbach è stato pari a 0.737. Il coefficiente di correlazione interno ha dato un risultato pari a 0.737 (95% CI: 0.600-0.835, p < 0.001) di media e 0.412 (95% CI: 0.237-0.559, p < 0.001) per le singole misure. Sulla base dei nostri risultati il questionario CES-I è risultato essere concorde con l’originale in lingua inglese e può essere considerato uno strumento adeguato per valutare la Qualità della Vita nei pazienti con CSOM di lingua italianaThe chronic ear survey (CES) is a sensitive and disease specific quality of life (QoL) measurement tool in patients with chronic suppurative otitis media (CSOM). It is a 13-item survey that evaluates the frequency, duration and severity of problems associated with this disease. It is composed of three subscales that describe activity restrictions, symptoms and medical resource utilisation. Based on patient's answers, it is possible to obtain a score resulting in a scale ranging from 0 to 100; the highest indicates the best health, while the lowest denotes poor health. The questionnaire was originally created in English. The aim of this study is to validate the CES questionnaire in Italian (CES-I). Translation was made following international guidelines. The application follows the stages of translation from English to Italian and linguistic adaptation, and grammatical and idiomatic equivalence review. The CES-I and the Short Form Health Survey 36 (SF-36) questionnaires were administered to 54 patients with CSOM. A cross-sectional design was used to examine the internal consistency (Cronbach's alpha) and concurrent validity (Pearson's product moment correlation). To confirm the external validity of CES-I, Pearson correlation coefficient, considering the total score and single subscales of CES and the 8 scales of the SF-36, was calculated. Cronbach's alpha coefficient for internal consistency was 0.737. The intraclass correlation coefficient, measured through mixed effects, was 0.737 (95% CI: 0.600-0.835, p < 0.001) for average measures and 0.412 (95% CI: 0.273-0.559, p < 0.001) for individual measures. According to our results, CES-I is a reliable tool for evaluation of QoL in patients with CSOM among the Italian-speaking population

Ocular vestibular evoked myogenic potentials and intravestibular intralabyrinthine schwannomas.

Intravestibular intralabyrinthine schwannomas (ILSs) are uncommon benign tumors that arise from the sac-cular, utricular, and lateral and superior ampullary nerves. According to the literature, there is an average delay of 8 years between the onset of symptoms and diagnosis. Te diagnosis is based on an audiovestibular examination and magnetic resonance imaging (MRI). We describe a case of intravestibular ILS in which we included the ocular vestibular evoked myogenic potentials (oVEMPs) test in the diagnostic workup. The oVE-MPs test is a relatively new neurophysiologic diagnostic modality that evaluates the superior vestibular pathway and the ascending contralateral pathway through the vestibulo-ocular reflex. In our case, a 65-year-old man presented with progressive right-sided sensorineu-ral hearing loss, dizziness, and tinnitus and fullness in his right ear. Audiovestibular examination and MRI detected an intravestibular ILS on the right. We found that oVEMPs were absent on the contralateral side, which contributed to the diagnostic process. Te detection of oVEMPs can provide detailed information on the functionality of the macula of the utricle and the lateral and superior ampullary nerves, with a precise identification of the affected area. Based on our findings, we discuss the role of oVEMPs in the diagnosis of an intravestibular ILS

Idiopathic benign paroxysmal vertigo in children, a migraine precursor.

OBJECTIVES: We describe the case of a young girl in whom transient deafness occurred when her core body temperature rose. METHODS: The patient was referred for a series of audiological and neurologic evaluations performed over time in both afebrile and febrile states, as well as after a stress test (with a treadmill) in which the body temperature rise simulated the febrile state. RESULTS: The patient was found to have a temporary bilateral hearing loss, but had normal distortion product otoacoustic emissions. Moreover, auditory brain stem responses revealed the absence of neural synchrony when her core body temperature increased. CONCLUSIONS: These results are consistent with a temperature-dependent auditory neuropathy, a rare condition in which patients show normal outer hair cell function and abnormal neural function of the eighth cranial nerve. The symptom is reminiscent of Uhthoff's phenomenon, which is described as transient visual loss and is usually observed in multiple sclerosis. This case of temperature-dependent auditory neuropathy is noteworthy because it sheds light on a disorder of which there have been few reports in the literature. We discuss its similarity to Uhthoff's phenomenon

Septal Nasal Extramedullary Plasmacytoma: A Rare Tumor in an Unusual Area

We present an extreme rare case of extramedullary nasal plasmacitoma that arise from nasal septum. The mass surgically removed was analyzed by a pathologist who diagnosed an extramedullary nasal plasmacytoma. The patient did not present systemic involvement. A short cycle of radiotherapy was performed after the surgery. At 9-month follow-up, the patient is recurrence free

Fetal alcohol spectrum disorders in pediatrics. FASD and the pediatrician

Fetal alcohol syndrome (FAS) is a complex and malformative condition due to the teratogenic effect of alcohol consumed during pregnancy. Several epidemiological studies have shown that maternal alcohol use during pregnancy is the most common preventable cause of mental retardation in childhood. The effects of alcohol on the fetus range from abortion to a spectrum of clinical manifestations called Fetal Alcohol Spectrum Disorders (FASD) that includes partial FAS (PFAS), neonatal Alcohol Related Birth Defects (ARBD) and Alcohol Related Neurodevelopmental Disorders (ARND) up to the most severe disease which is the so-called FAS

The cochleo-vestibular secretory senescence

The vestibular and cochlear senescence is a complex degenerative disease and one of the most prevalent chronic conditions of elderly, affecting tens of millions of people worldwide. In this review, we will try to summarize the most important sites and involved mechanism trying to figure out strategies to prevent its clinical consequences

RIPK3 activation leads to cytokine synthesis that continues after loss of cell membrane integrity

Necroptosis is a form of programmed cell death that is defined by activation of the kinase RIPK3 and subsequent cell membrane permeabilization by the effector MLKL. RIPK3 activation can also promote immune responses via production of cytokines and chemokines. How active cytokine production is coordinated with the terminal process of necroptosis is unclear. Here, we report that cytokine production continues within necroptotic cells even after they have lost cell membrane integrity and irreversibly committed to death. This continued cytokine production is dependent on mRNA translation and requires maintenance of endoplasmic reticulum integrity that remains after plasma membrane integrity is lost. The continued translation of cytokines by cellular corpses contributes to necroptotic cell uptake by innate immune cells and priming of adaptive immune responses to antigens associated with necroptotic corpses. These findings imply that cell death and production of inflammatory mediators are coordinated to optimize the immunogenicity of necroptotic cells

Comparison and combination of a hemodynamics/biomarkers-based model with simplified PESI score for prognostic stratification of acute pulmonary embolism: findings from a real world study

Background: Prognostic stratification is of utmost importance for management of acute Pulmonary Embolism (PE) in clinical practice. Many prognostic models have been proposed, but which is the best prognosticator in real life remains unclear. The aim of our study was to compare and combine the predictive values of the hemodynamics/biomarkers based prognostic model proposed by European Society of Cardiology (ESC) in 2008 and simplified PESI score (sPESI).Methods: Data records of 452 patients discharged for acute PE from Internal Medicine wards of Tuscany (Italy) were analysed. The ESC model and sPESI were retrospectively calculated and compared by using Areas under Receiver Operating Characteristics (ROC) Curves (AUCs) and finally the combination of the two models was tested in hemodinamically stable patients. All cause and PE-related in-hospital mortality and fatal or major bleedings were the analyzed endpointsResults: All cause in-hospital mortality was 25% (16.6% PE related) in high risk, 8.7% (4.7%) in intermediate risk and 3.8% (1.2%) in low risk patients according to ESC model. All cause in-hospital mortality was 10.95% (5.75% PE related) in patients with sPESI score ≥1 and 0% (0%) in sPESI score 0. Predictive performance of sPESI was not significantly different compared with 2008 ESC model both for all cause (AUC sPESI 0.711, 95% CI: 0.661-0.758 versus ESC 0.619, 95% CI: 0.567-0.670, difference between AUCs 0.0916, p=0.084) and for PE-related mortality (AUC sPESI 0.764, 95% CI: 0.717-0.808 versus ESC 0.650, 95% CI: 0.598-0.700, difference between AUCs 0.114, p=0.11). Fatal or major bleedings occurred in 4.30% of high risk, 1.60% of intermediate risk and 2.50% of low risk patients according to 2008 ESC model, whereas these occurred in 1.80% of high risk and 1.45% of low risk patients according to sPESI, respectively. Predictive performance for fatal or major bleeding between two models was not significantly different (AUC sPESI 0.658, 95% CI: 0.606-0.707 versus ESC 0.512, 95% CI: 0.459-0.565, difference between AUCs 0.145, p=0.34). In hemodynamically stable patients, the combined endpoint in-hospital PE-related mortality and/or fatal or major bleeding (adverse events) occurred in 0% of patients with low risk ESC model and sPESI score 0, whilst it occurred in 5.5% of patients with low-risk ESC model but sPESI ≥1. In intermediate risk patients according to ESC model, adverse events occurred in 3.6% of patients with sPESI score 0 and 6.65% of patients with sPESI score ≥1.Conclusions: In real world, predictive performance of sPESI and the hemodynamic/biomarkers-based ESC model as prognosticator of in-hospital mortality and bleedings is similar. Combination of sPESI 0 with low risk ESC model may identify patients with very low risk of adverse events and candidate for early hospital discharge or home treatment.