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Purpose: Sinonasal nuclear protein in testis carcinoma (SNUTC) is a rare, aggressive malignancy caused by genetic rearrangements in the NUTM1 gene. The prognosis of SNUTC ranks among the most unfavorable within the naso-sinusal district, with an overall survival of 9.7 months. This systematic review aimed to determine the best therapeutic strategy for SNUTC., Methods: We reviewed eligible articles for patient demographics, TNM and stage at presentation, best response after primary treatment, disease-free survival and overall survival (OS) times, other following therapy lines, and final outcomes., Results: Among 472 unique citations, 17 studies were considered eligible, with reported treatment data for 25 patients. Most studies (n = 12) were case reports. The most frequently administered treatment regimen was surgery as primary treatment and combined radiochemotherapy as second-line or adjuvant treatment. Four patients were alive at follow-up., Conclusion: Basing on the existing literature, a standardized line in the treatment of SNUTC is not yet well delineated. A self-personalized strategy of therapy should be drawn on each patient affected by SNUTC., (© 2024. The Author(s).)

Purpose: We compared our historical medium-term data obtained with an active semi-implanted bone conduction device and the hearing results of a new passive bone conduction hearing device to determine its predictive value for the hearing results with the semi-implanted device., Methods: The study sample was 15 patients with an active bone conduction implant (mean follow-up 26 months). Pure tone audiometry was performed with headphones, sound field speech audiometry was conducted unaided, and free-field speech audiometry was carried out with both the active bone conduction system and the passive device switched off., Results: As compared with the unaided condition, speech reception was significantly improved with both devices. Comparison of speech reception threshold at 100% of word recognition showed no difference between the active and the passive device. At lower intensity the difference in speech perception was significant in the patients with monaural fitting (group A) and was non-statistically significant in those with binaural fitting (group B); the speech reception threshold at 50% of word recognition was 26.00 dB (± 10.22) with the active implant and 30.50 dB (± 7.98) with the passive device in group A (p = 0.047) and 24.00 dB (± 5.48) and 29.00 dB (± 2.24) in group B (p = 0.052), respectively., Conclusions: The hearing outcome after active bone conduction implant was comparable to published data. Compared with the unaided condition, speech recognition was significantly improved with the passive device. The device may also provide value to predict the hearing outcome with the implanted device, especially at higher intensities., Level of Evidence: IV.

Aim of this work was to assess the role of polymorphisms belonging to genes involved in the regulation of ionic homeostasis in Caucasian patients with Ménière Disease (MD). We recruited 155 patients with definite Ménière Disease and 186 controls (Control Group 1) without a lifetime history of vertigo, overlapping with patients for age and rate of hypertension. We validated the positive results on 413 Caucasian subjects selected from a European general population (Control Group 2). The clinical history for migraine and hypertension was collected; genomic DNA was characterized for a panel of 33 SNPs encoding proteins involved in ionic transport. We found a higher rate of migraineurs in MD subjects compared to Group 1 (46.8 vs 15.5%, p = 0.00005). Four SNPs displayed differences in MD patients compared to Group 1 controls: rs3746951 and rs2838301 in SIK1 gene, rs434082 and rs487119 in SLC8A1; the p values of Chi-squared test for genotype frequencies are 0.009, 0.023, 0.009 and 0.048, respectively. SLC8A1 gene encodes for Na + -Ca ++ exchanger, while SIK1 gene encodes for Salt Inducible Kinase 1, an enzyme associated with Na + -K + ATPase function. The validation with Control Group 2 displayed that only rs3746951 and rs487119 are strongly associated to MD (p = 0.001 and p = 0.0004, respectively). These data support the hypothesis that a genetically induced dysfunction of ionic transport may act as a predisposing factors to develop MD.

The aim of the study is to investigate acoustic reflex testing in amyotrophic lateral sclerosis patients. Amplitude, latency, and rise time of stapedial reflex were recorded for 500 and 1000 Hz contralateral stimulus. Statistical analysis was performed by the Wilcoxon test and the level of significance was set at 5 %. Fifty-one amyotrophic lateral sclerosis patients and ten sex- and age-matched control subjects were studied. Patients were further divided in two groups: amyotrophic lateral sclerosis-bulbar (38 cases, with bulbar signs at evaluation) and amyotrophic lateral sclerosis-spinal (13 cases, without bulbar signs at evaluation). Stapedial reflex was present in all patients. There was a statistically significant difference in the mean amplitude, latency, and rise time between the amyotrophic lateral sclerosis patients as compared with the controls. Amplitude was lower in both the amyotrophic lateral sclerosis-bulbar and the amyotrophic lateral sclerosis-spinal patients than in the controls (p < 0.05) and rise time was longer in both patient groups compared with the controls (p < 0.05). These results confirm the presence of abnormal acoustic reflex patterns in amyotrophic lateral sclerosis cases with bulbar signs and, moreover, suggesting a possible subclinical involvement of the stapedial motor neuron even in amyotrophic lateral sclerosis-spinal patients. Amplitude and rise time seem to be good sensitive parameters for investigating subclinical bulbar involvement.

Hearing loss in Menière's disease has been described to affect above all low frequencies (upward curve) with a tendency to become irreversible and non-fluctuating at the higher frequencies (peaked curve) over time. The aim of the study was to determine the effects of MD on hearing function on the basis of differences existing between the affected and the unaffected ear in a group of patients affected by definite unilateral MD and whose contralateral ear was not affected by any disease other than age-related hearing loss (ARHL). Following this procedure we have also evaluated the possible effects of age and disease duration on hearing loss in MD. The study group consisted of 86 subjects affected by definite unilateral MD. In our sample a peaked audiometric curve characterized the affected ears; however, the result after subtracting the normal ear hearing threshold was an upward sloping curve, which highlighted the greater suffering at the lower frequencies. On the basis of differences existing between affected and unaffected ear, our data suggest that threshold evolution is more related to disease duration rather than to age.

The aim of this study was to compare oval and round window vibroplasty. Eighteen (18) patients implanted with Vibrant Soundbridge (VSB) were enrolled. Two groups were formed depending on FMT placement: on round window in ten cases (RW group) and on oval window in eight (OW group). Pre and postoperative audiological tests were performed both under headphones and free-field settings, VSB on and off. One (1) RW patient experienced sudden hearing loss at the operated side after 4 months from surgery and was excluded from the analysis. Both groups showed good hearing results. Significant differences were measured at free-field pure-tone test with VSB on at 0.5 kHz (RW better than OW, p = 0.026) and 4 kHz (OW better than RW, p = 0.043). Both techniques share similar good results and are considered safe. However, we had one failure with deep and sudden hearing threshold worsening after some months of good results. From a surgical point of view OW vibroplasty is easier and safer to perform, when the stapes suprastructure is absent, as it does not require any drilling and should be preferred in such cases. More reports are needed to explain if RW vibroplasty is risky in a mid to long term.

To assess the reliability of Blackman windowed tone burst auditory brainstem response (ABR) as a predictor of hearing threshold at low frequencies. Fifty-six subjects were divided in to three groups (normal hearing, conductive hearing loss, sensorineural hearing loss) after pure tone audiometry (PTA) testing. Then they underwent tone burst ABR using Blackman windowed stimuli at 0.5 kHz and 1 kHz. Results were compared with PTA threshold. Mean threshold differences between PTA and ABR ranged between 11 dB at 0.5 kHz and 14 dB at 1 kHz. ABR threshold was worse than PTA in each but 2 cases. Mean discrepancy between the two thresholds was about 20 dB in normal hearing, reducing in presence of hearing loss, without any differences in conductive and sensorineural cases. Tone burst ABR is a good predictor of hearing threshold at low frequencies, in case of suspected hearing loss. Further studies are recommended to evaluate an ipsilateral masking such as notched noise to ensure greater frequency specificity.

The aim of this study was to evaluate the frequency of surgical approach in a population of patients affected by definite Menière's disease (MD). In the majority of patients, relief from vertigo attacks can be achieved by means of medical therapy (MT). In cases in which MT fails surgery may offer relief to vertigo. The most applied surgical procedures are intratympanic gentamicin (ITG) and vestibular neurectomy (VN), based on vestibular deafferentation. Until now, the real incidence of the different therapeutic approaches for MD has not been evaluated. The study design was a retrospective study. The study was performed in 177 patients affected by definite MD. Subjects referred directly for surgery by other centers were excluded from the study. All the patients were medically treated with salt restriction and diuretics. In case of MT failure, surgical therapy, ITG or retrosigmoid VN were proposed. In the 75% of cases, the only therapeutic approach was MT, while in 20% of cases we carried out ITG and in 5% VN. In 33% of VN group, this operation was carried out after ITG failure and in 67% as the first surgical approach. The VN group was characterized by younger age and higher disability degree. The primary therapy in definite MD seems to be MT. Ablative therapy represents the second choice: ITG was carried out in 80% of cases, while VN was performed in 20%.

The aims of this study were to evaluate the influence of age and exposure to noise in determining the evolution of hearing loss after noise-induced hearing loss has been already established and to define the further evolution of presbycusis. This is a cross-sectional study based on the evaluation of pure-tone audiometry threshold on 568 subjects affected by noise-induced hearing loss and exposed to noise for at least 10 years; noise exposure at testing was 85-90 dB Leqd(A). The further evolution of hearing loss was found to be more related to age than to noise exposure and was significantly less than expected for presbycusis. In conclusion our data support the hypothesis that once NIHL has manifested, it tends to worsen slightly with continued noise exposure and that progressive hearing loss is chiefly due to aging. However, in individuals with NIHL, age-related hearing loss is significantly less at frequencies damaged by noise than in non-noise-exposed individuals.

The auditory steady-state responses to single continuous tones modulated in amplitude have been proposed as an alternative to objective frequency-specific audiometry. The aim of this study was to compare thresholds obtained by pure-tone audiometry (PTA) and by auditory steady-state responses in normal hearing or affected by hearing loss in adults and in order to evaluate the applicability of this objective test in no collaborative hearing-impaired subjects. Eleven people, 6 normal hearing and 5 with hearing loss, underwent PTA and multiple frequency auditory steady-state responses; simultaneous carrier tones (0.5, 1, 2 and 4 KHz) modulated in amplitude at different rates (77-105 Hz) were presented monaurally (TDH 49 earphones) at variable intensities (110-20 dB SPL). The mean threshold difference between PTA and multiple frequency auditory steady-state responses was 28 dB (standard deviation=14.2) and R correlation value at 0.5-1-2-4 kHz was 0.71 (P=0.0012) at the Pearson's test. These differences were significantly smaller considering the hearing-impaired separately (11.7 dB, standard deviation=2.9). The results of this study confirm previous reports showing that the multiple auditory steady-state response method is an accurate predictor of the behavioural audiogram in patients with sensory-neural hearing impairments and can be used as a valid support for behavioural evaluations.

Low frequency sudden hearing loss (LFSHL) is a frequent finding in the otological practice. Several prognostic indicators have been suggested concerning the prediction of the outcome of sudden hearing loss, but so far there are no proven factors to establish the prognosis. The aim of this study was to assess whether OAEs could be considered as a reliable prognostic test in LFSHL. The study group consisted of 20 patients presenting with a unilateral LFSHL. Each patient was submitted to spontaneous otoacoustic emissions (SOAEs), transient otoacoustic emissions (TEOAEs) and distortion products (DPOAEs) recording and then treated with glycerol administrated intravenously in 3-h intervals for 4 days. Pure tone audiometry (PTA) threshold was evaluated again 1 h after the last administration of glycerol. After osmotic therapy 12 patients (60%) showed a significant PTA improvement with a mean improvement of 11 dB; modifications were significant at the Student's t test for paired data (P<0.0001). The relationship between the pretherapy presence or absence of SOAEs, TEOAEs and DPOAEs and PTA modification was not significant at the exact Fisher's test. In conclusion, even if our study supports that OAEs could be an indicator of the inner ear functional state, they cannot be utilized as a prognostic test in LFSHL in relation to the efficacy of osmotic therapy. Among the other parameters evaluated, only the precocity of therapy seems to be related to prognosis in LFSHL.