Severe chronic rhinosinusitis treated with dupilumab, a real-life analysis of early effectiveness.

OBJECTIVE: The present study evaluated the safety and efficacy of dupilumab in severe uncontrolled type 2 chronic rhinosinusitis with nasal polyps (CRSwNP). PATIENTS AND METHODS: A retrospective analysis was conducted on a cohort of adult patients affected by severe CRSwNP treated with dupilumab. Maxillofacial computed tomography, evaluation of blood eosinophils and serum IgE levels, measurement of nasal polyp score (NPS), smell identification test (SSIT-16), sinonasal outcome test-22 (SNOT-22) and asthma control test (ACT) were performed. Follow-up was conducted at 2 weeks, and at 1, 3, and 6 months. Adverse events and the efficacy of treatment were monitored. RESULTS: 23 patients were enrolled. After 15 days, scores of the SNOT-22, NPS and SSIT-16 significantly improved. These outcomes were also maintained after 1, 3, and 6 months (p < 0.001). At this latter follow-up time, SNOT-22 showed a change of -33.10 (p < 0.001), NPS -3.36 (p < 0.001) and SSIT-16 +5.60 (p < 0.001). In all, 26.1% of patients experienced early minor complications. CONCLUSIONS: In the present study, dupilumab was effective in the treatment of severe uncontrolled CRSwNP, demonstrating a quick significant improvement in both questionnaires and endoscopic evaluation. Only minor complications were observed.

Sinonasal and rhinopharyngeal solitary fibrous tumour: a case report and review of the literature.

Solitary fibrous tumours are rare neoplasms that arise mostly from the pleura. Much more rarely they can also be found in extrapleural sites, including the head and neck. We report a rare case of a sinonasal and rhinopharyngeal solitary fibrous tumour. The tumour, measuring 67 x 28 x 55 mm, was first embolised and then successfully removed through endonasal endoscopic surgery. Histopathologic analysis confirmed the nature of the lesion, which was positive for CD34 and vimentin. A post-operative CT scan and endoscopic follow-up demonstrated total resection and absence of recurrence after 13 months.

Paroxysmal positional vertigo: short- and long-term clinical and methodological analyses of 794 patients.

Between 1995 and 2001, eight Italian clinical centres used the same diagnostic and therapeutic protocol in order to assess the clinical progress of paroxysmal positional vertigo and the benefits of an appropriate follow-up in prevention of relapse. The study population comprises 794 patients affected by paroxysmal positional vertigo. The study protocol comprised diagnostic staging including a complete otoneurological test, an anamnestic questionnaire aimed at identifying any possible risk factor, a blood test in basal conditions and monitoring of blood pressure. If necessary, more specific instrumental tests have been carried out. Appropriate rehabilitative manoeuvres were performed from 1 to 3 times within the same session. The patient was checked 3-5 days later: in the presence of a positive result, the treatment was repeated; if negative, patients were seen at clinical follow-up 7, 30, 180 and 365 days after recovery. Wherever possible, patients have been contacted 2 years after the first treatment and asked to answer a questionnaire and to attend for a clinical check-up. The incidence of paroxysmal positional vertigo appeared to be higher in females and in patients aged 50-70 years, being low in patients under 30. In 88.8% of cases posterior semicircular canals showed a significant involvement; in 6.8% of cases, only involvement of lateral semicircular canals; monolateral (2.7%) and bilateral (1.7%) multicanalar forms were rare. Paroxysmal positional vertigo forms involving posterior semicircular canals have been treated with Semont (simplified by Toupet), Epley, Parnes Price-Jones manoeuvres; those, involving lateral semicircular canals with Vannucchi-Vicini forced position and "barbecue" or Gufoni manoeuvre. Whilst all these manoeuvres were equally effective, longer recovery times have been observed in paroxysmal positional vertigo forms involving lateral semicircular canals when the Vannucchi-Vicini forced position was ineffective. Any relapses have been evaluated at least 15 days after a negative clinical pattern. Possible involvement of other semicircular canals (recurrence) some time after the first onset has been considered separately. Follow-up at 6 months showed recurrence in 12.4% of cases, while being chronic in 1.5% of cases. Only 9.3% of cases showed recurrence at 6 months, no statistically significant difference being observed between vertical (8.9%) and lateral canal (9.6%), forms. Relapses occurred in 3.1% of cases, in one third of which at least two risk factors were detected.

Evaluation of functionality of cochlear outer hair cells in patients with retinitis pigmentosa and in their relatives.

Hearing functionality was studied in 36 patients with retinitis pigmentosa (R P) and 29 of their relatives. These patients were defined as having bilateral normal hearing on the basis of tonal-threshold audiometric and acoustic-immittance tests. The transiently evoked otoacoustic emissions (T E O A Es) were studied in these normal hearing patients. T E O A Es represent an extremely sensitive method to study the functionality of the outer hair cells of the organ of Corti. When the values of T E O A E amplitude, intensity, and frequency in R P patients and in their relatives were compared with those in control subjects, they were found to be significantly reduced. The T E O A Es were clearly pathological in 52.8% of patients with R P and in 24.1% of their relatives. During embryologic development, there is one transitory axoneme in the outer hair cells of the organ of Corti; this transitory axoneme is important for the organization of the stereocilia. Axonemes are found in mature hair cells, including photoreceptors. The alteration of cochlear outer hair cells in a high percentage of patients with R P and in some of their relatives corroborates the hypothesis that, in some instances, retinitis pigmentosa may be due to a structural anomaly of the ciliated cells.

Study of functionality of cochlear outer hair cells in patients with retinitis pigmentosa.

Hearing functionality was studied in 30 patients affected by retinitis pigmentosa (RP) and 25 of their relatives. These patients were defined as normal-hearing on the basis of pure-tone threshold audiometric and acoustic immittance tests. The evoked otoacoustic emissions (EOEs) were studied in these normal hearing patients. EOEs are an extremely sensitive method to study the functionality of the outer hair cells of the organ of Corti. In RP patients and in their relatives the values of EOE (amplitude, intensity and frequency) were found to be statistically reduced compared to those of control subjects. The EOEs turned out to be clearly pathological in 60% of patients with RP and in 24% of their relatives. During the embryologic development there is one transitory axoneme in the outer hair cells of the organ of Corti. This axoneme is important for the organization of the stereocilia. The axonemes are found inside a few mature hair cells, including the photoreceptors. The alteration of cochlear outer hair cells in a high percentage of patients with RP and in some of their relatives seems to corroborate the hypothesis that, in many cases, retinitis pigmentosa may be due to a structural anomaly of the ciliated cells.

Evoked otoacoustic emissions behaviour in retinitis pigmentosa.

The hearing function was studied in 26 patients affected by retinitis pigmentosa (RP) and in their relatives. Sixteen patients showed bilateral normal hearing when examined with traditional audiometric methods. In these normoacusic patients evoked otoacoustic emissions (EOE) have been studied. The EOE offer a unique opportunity to measure objectively the function of outer hair cells: they record the amplitude of the energy produced by the outer hair cells of the coclea following an acoustic stimulation. The data have been statistically compared, using the Student's t-test, with those obtained in a homogeneous control-group of normal subjects. In normoacusic subjects with RP the average values of EOE intensity are statistically lower than those of normal subjects in 64 of the 127 frequency bands examined. Moreover, the distribution of the EOE in patients with retinitis pigmentosa proved to be more discontinous than that observed in the normal subjects. The EOE recorded in 14 normoacusic relatives show in some cases small anomalies but the data, on account of the limited sample group, cannot be statistically evaluated. Therefore a subclinical alteration of the Organ of Corti is found in 100% of the patients affected by RP, although they appear to be normoacusic to usual audiometric tests.

[Short-term reproducibility of acoustic oto-emissions induced by clicks in adults]. / Reproductibilité à court terme des oto-émissions acoustiques provoquées par des clics chez des sujets adultes.

Otoacoustic emissions (OAE) are sounds emitted by the cochlea and recordable in the external ear canal by a miniature microphone. The OAE reflect the existence of an active mechanism within the cochlea. Development of the OAE has spurred much interest because they may used as a valid test for screening and monitoring cochlear changes. The OAE were recorded in 8 normal hearing subjects (15 ears) during nine test sessions under similar conditions. The aim of this work was to study the short-term variability in the amplitude of the emissions. Results of the study indicate that response levels are stable over time in the same ear therefore changes in the response level can be associated with changes in the cochlea.

[Epistaxis and climatic events]. / Epistassi e fattori meteorologici.

Data concerning daily climatic conditions in the area of Perugia during 1987-1990 have been gathered and correlated to daily incidence of idiopathic nose-bleeding observed in the same area and during the same period (1064 patients on the whole). The aim was to verify the relation between climatic events and "epistaxis" phenomenon, relation that has been assumed since a long time but has never been scientifically demonstrated before. Following factors have been estimated: highest and lowest daily temperature, thermal gradient, atmospheric pressure and pressure gradient, relative humidity. Results of statistic correlations show that every climatic factors has a narrow range related to higher incidence of epistaxis and that nose-bleeding is likely conditioned by combination of critical values, as a low temperature with 4 degrees C daily gradient, a 716 mm Hg atmospheric pressure with 2 mm Hg negative gradient, a close to 100% relative humidity.

[Evoked acoustic oto-emissions in patients with diabetes mellitus]. / Etude des oto-émissions acoustiques évoquées chez des patients atteints de diabète sucré.

The purpose of this study was to evaluate the cochlear function in patients with diabetes mellitus by analysis of evoked otoacoustic emissions (EOAE). EOAE were studied in 20 diabetic patients with normal hearing. The parameters used for analysis were the EOAE intensity and amplitude measured per 100 Hz frequency bands between 700 and 4000 Hz These data were compared to the data obtained in a group of non-diabetic control subjects with normal hearing using a Student's t test. The mean EOAE intensity and amplitude by 100 Hz frequency band was significantly lower in diabetic patients than in the control group. This seems to indicate the existence of an alteration in cochlear micromechanics in diabetic patients possibly due to changes in the functioning of the hair cells.

[Evoked oto-acoustic emissions in patients with retinitis pigmentosa]. / Le otoemissioni acustiche evocate in pazienti affetti da retinite pigmentosa.

Evoked otoacoustic emissions (EOE) offer a unique opportunity to measure objectively the function of outer hair cells in human beings. In this paper the Authors report a study carried out employing EOE evaluation on cochlear function in subjects with retinitis pigmentosa. Nine patients with retinitis pigmentosa with normal hearing in both ears upon traditional audiometric evaluation were studied. The frequency spectrum of the EOE was taken into consideration, evaluated in frequency bands of 50 Hz, in order to ascertain the presence or absence of the emissions and, when present, their amplitude. These data were compared statistically, using the Student's T Test, to those obtained in a homogeneous control group of normal subjects. In subjects with retinitis pigmentosa, average EOE intensity values were statistically lower than those found in normal subjects. In fact, the Student's T Test evaluation revealed significant differences in EOE intensity in 64 of the 127 frequency bands examined. Moreover, the distribution of the EOE in patients with retinitis pigmentosa proved to be more discontinuous than that observed in the normal subjects. These data appear to indicate authentic alterations in the cochlear mechanics in patients with retinitis pigmentosa. Such alterations may well be related to outer hair cell lesions. It seems likely that the auditory system is involved in retinitis pigmentosa degenerative processes more frequently than traditional audiometric tests have been able to show.