Fabrizio Ottaviani

Autoimmunity in sudden sensorineural hearing loss: possible role of anti-endothelial cell autoantibodies.

In order to verify whether anti-endothelial cell autoantibodies (AECAs) can be used as serological markers of inner ear vasculitis in sudden sensorineural hearing loss (SSHL), 32 patients affected by idiopathic SSHL were investigated. All patients underwent a routine general physical examination and extensive audiovestibular, microbiological and immunological investigations. Fourteen normal subjects without a history of HL, autoimmune or metabolic disease served as controls. Detection of AECAs was performed using an indirect immunofluorescence technique. AECA-positive patients were treated with methylprednisone, while AECA-negative patients were treated with a combined regimen of steroids, plasma expander and aspirin. The average hearing recovery for 5 frequencies (0.25-4 kHz) was analyzed in each subject 1 month after treatment and every 3 months thereafter; median follow-up was 12 months (range 9-18 months). A total of 15/32 patients (46.8%; 11/19 females, 4/13 males) were AECA-positive and thus differed significantly from the normal population in whom only 2/14 tested cases were positive ( p =0.03). Severe hearing loss was associated with being AECA-positive in 8/11 cases. During follow-up, 25/32 patients improved their hearing and 17 of these patients were AECA-negative. The seven cases without hearing improvement were all AECA-positive. In patients with SSHL, immune-mediated vascular damage may have a pathogenetic role and AECAs may represent a serological marker of vasculitis even if they are not inner ear-specific and even if they represent an epi-phenomenon rather than the only cause of SSHL.

Rhinoscintigraphy: A Simple Radioisotope Technique To Study the Mucociliary System

PURPOSE This was a radioisotope study of nasal mucociliary clearance of total and subtotal nasal obstruction. METHODS Rhinoscintigraphy was performed by insufflating 1.85 MBq (69 mCi) Tc-99m MAA in 20 patients. Six cases were regarded as the control group, because the presence of small spurs does not affect nasal patency. The remaining 14 patients had various rhinopathic conditions. Two regions of interest were selected, one in the nasal cavity and one in the pharynx. Mucociliary transport speed was calculated. RESULTS This parameter appeared to be a sensitive index for the assessment of the degree of mucociliary alteration. It showed that polyposis impairs mucociliary transport most severely, thus confirming the results of other published studies. CONCLUSIONS Rhinoscintigraphy proved to be a reliable, easily reproducible, and harmless method, so it may be used for follow-up examinations in patients who have had surgery of the nose and paranasal sinuses, and for drug therapy of rhinopathic conditions.

Clinical associations of serum antiendothelial cell antibodies in patients with sudden sensorineural hearing loss

Objectives/Hypothesis The role of antiendothelial cell antibodies in systemic vasculitis has been reported. The aim of the study was to define the clinical associations of serum antiendothelial cell antibodies in patients with sudden sensorineural hearing loss.

Tonotopic Organization of Human Auditory Cortex Analyzed by SPET

Single photon emission tomography (SPET) was used to map blood flow increases in the temporal and parietal cortex of 16 normally-hearing subjects after auditory stimulation. Eight subjects were stimulated with a multifrequency 40 dB HL pure tone at 250, 500, 1000, 2000, 4000 Hz, each frequency varying every 30 s. Single 500 Hz pure tones at 40 dB HL were delivered to the remainder of the subjects. Five bilaterally deaf subjects were used as controls. Marked cerebral flow increase following acoustic stimulation with a significantly prevalent activation of the contralateral temporal cortex was achieved (p < 0.001). According to the tonotopic organization of the human auditory cortex, low monofrequency stimulation activated the most lateral sagittal tomograms (from 48.75 to 56.25 mm laterally to the brain midline) only, while multifrequency stimuli activated all sagittal tomograms (from 18.75 to 56.25 mm). On the basis of these results, it is likely that SPET is able to give real information on the cortical distribution of the auditory frequency range, taking into account the number and position of the activated slices. Further clinical investigations in order to define the relationships among blood flow cortical increases, stimulus intensity and auditory threshold, are in progress.

Large Nasal Septal Perforation Repair by Closed Endoscopically Assisted Approach

Options for the surgical closure of large symptomatic perforations are limited and consist of an open or closed approach using skin or mucosal flaps, with or without different grafts. The aim of this study is to review our experience in treating large nasal perforations using a closed approach with endoscopic assistance, undertaking a 3-layer reconstruction of the septum. We reviewed 14 consecutive patients with large (2–4 cm) nasal septal perforations, who were treated using an endonasal/endoscope-assisted approach. In these cases, the mucosal defect was reconstructed through a horizontal advancement of the bipedicled mucoperichondrial flaps and sutured using absorbable sutures. The cartilagineous defect was consistently reconstructed using autogenous auricular conchal grafts. Pre- and postoperative nasal symptom scores were used for the study; a decline in the number of Nasal Obstruction Symptom Evaluation Scale symptoms were recorded in 12 of 14 patients (85.7%), and visual analogue scale scores for crusting, bleeding, nasal discharge, whistling, headache, nasal pain, snoring, olfactory loss, and overall discomfort levels also decreased. It was concluded that bipedicled mucoperichondrial flaps with the insertion of auricular cartilage for a 3-layer septal reconstruction seem to give reasonably good results. The use of nasal endoscopy is an endonasal approach, which offers superior precision in all surgical steps and provides a way to obtain excellent closure of the perforation without external incisions.

A Case of PANDAS Treated With Tetrabenazine and Tonsillectomy

PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) is a rare clinical syndrome characterized by the presence of tics, Tourette syndrome, obsessive-compulsive disorder, or chorea in the context of an immediately precedent streptococcal infection. In this report, we describe the case of an 11-year-old boy who developed PANDAS with severe choreic movements. The criteria for PANDAS diagnosis were met. Moreover, serum antibrain antibodies were present. The patient was initially treated with tetrabenazine 12.5 mg twice daily with remission of the neurological symptoms. Subsequently, the patient underwent tonsillectomy and has been asymptomatic since, with antistreptolysin O titer levels in range.

Vestibulo-Ocular Reflex Modification after Virtual Environment Exposure

Immersion in an illusory world is possible by means of virtual reality (VR), where environmental perception is modified by artificial sensorial stimulation. The application of VR for the assessment and rehabilitation of pathologies affecting the vestibular system, in terms of both diagnosis and care, could represent an interesting new line of research. Our perception of reality is in fact based on static and dynamic spatial information perceived by our senses. During head movements in a virtual environment the images on the display and the labyrinthine information relative to the head angular accelerations differ and therefore a visuo-vestibular conflict is present. It is known that mismatches between visual and labyrinthine information may modify the vestibulo-oculomotor reflex (VOR) gain. We studied the post-immersion modifications in 20 healthy subjects (mean age 25 years) exposed to a virtual environment for 20 min by wearing a head-mounted display. VOR gain and phase were measured by means of harmonic sinusoidal stimulation in the dark before, at the end of and 30 min after VR exposure. A VOR gain reduction was observed in all subjects at the end of VR exposure which disappeared after 30 min. Our data show that exposure to a virtual environment can induce a temporary modification of the VOR gain. This finding can be employed to enable an artificial, instrumental modification of the VOR gain and therefore opens up new perspectives in the assessment and rehabilitation of vestibular diseases.Immersion in an illusory world is possible by means of virtual reality (VR), where environmental perception is modified by artificial sensorial stimulation. The application of VR for the assessment and rehabilitation of pathologies affecting the vestibular system, in terms of both diagnosis and care, could represent an interesting new line of research. Our perception of reality is in fact based on static and dynamic spatial information perceived by our senses. During head movements in a virtual environment the images on the display and the labyrinthine information relative to the head angular accelerations differ and therefore a visuo-vestibular conflict is present. It is known that mismatches between visual and labyrinthine information may modify the vestibulo-oculomotor reflex (VOR) gain. We studied the post-immersion modifications in 20 healthy subjects (mean age 25 years) exposed to a virtual environment for 20 min by wearing a head-mounted display. VOR gain and phase were measured by means of harmonic sinusoidal stimulation in the dark before, at the end of and 30 min after VR exposure. A VOR gain reduction was observed in all subjects at the end of VR exposure which disappeared after 30 min. Our data show that exposure to a virtual environment can induce a temporary modification of the VOR gain. This finding can be employed to enable an artificial, instrumental modification of the VOR gain and therefore opens up new perspectives in the assessment and rehabilitation of vestibular diseases.

Retropharyngeal lipoma causing sleep apnea syndrome.

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Inactivation of Human Salivary Glutathione Transferase P1-1 by Hypothiocyanite: A Post-Translational Control System in Search of a Role

Glutathione transferases (GSTs) are a superfamily of detoxifying enzymes over-expressed in tumor tissues and tentatively proposed as biomarkers for localizing and monitoring injury of specific tissues. Only scarce and contradictory reports exist about the presence and the level of these enzymes in human saliva. This study shows that GSTP1-1 is the most abundant salivary GST isoenzyme, mainly coming from salivary glands. Surprisingly, its activity is completely obscured by the presence of a strong oxidizing agent in saliva that causes a fast and complete, but reversible, inactivation. Although salivary α-defensins are also able to inhibit the enzyme causing a peculiar half-site inactivation, a number of approaches (mass spectrometry, site directed mutagenesis, chromatographic and spectrophotometric data) indicated that hypothiocyanite is the main salivary inhibitor of GSTP1-1. Cys47 and Cys101, the most reactive sulfhydryls of GSTP1-1, are mainly involved in a redox interaction which leads to the formation of an intra-chain disulfide bridge. A reactivation procedure has been optimized and used to quantify GSTP1-1 in saliva of 30 healthy subjects with results of 42±4 mU/mg-protein. The present study represents a first indication that salivary GSTP1-1 may have a different and hitherto unknown function. In addition it fulfills the basis for future investigations finalized to check the salivary GSTP1-1 as a diagnostic biomarker for diseases.

Lack of contralateral suppression in transient-evoked otoacoustic emissions in multiple chemical sensitivity: a clinical correlation study.

Multiple chemical sensitivity (MCS) is a chronic disorder characterized by a variety of symptoms associated with the exposure to chemicals at a concentration below the toxic level. Previous studies have demonstrated peculiar responses in brain activity in these patients with respect to sensory stimuli while the association between chemical sensitivity and other environmental intolerances such as noise sensitivity has been questioned by researchers. In this study, a cohort of 18 MCS patients underwent transient-evoked otoacoustic emission (TEOAE) testing with and without contralateral suppression to evaluate the functionality of the medial olivocochlear (MOC) reflex involved in speech-in-noise sensitivity. Results were compared with an age- and gender-matched control group (n = 20) and correlation analysis with disease onset and quick environmental exposure sensitivity inventory (qEESI) symptom severity scale was performed. Subjects affected by MCS showed statistically significant impairment of MOC reflex, and the onset of the disease and several symptom subscales showed to be correlated to such reduction in some of the frequencies tested. These data suggest that alterations of MOC reflex could be part of the complex features of this disease although more studies are needed to further explore auditory perception disorders in environmental intolerances.