Reply to De Luca et al. Comment on "Marra et al. Metastatic Renal Cell Carcinoma to the Soft Tissue 27 Years after Radical Nephrectomy: A Case Report. Medicina 2023, 59, 150".

We appreciated the comments of De Luca and colleagues [...].

Metastatic Renal Cell Carcinoma to the Soft Tissue 27 Years after Radical Nephrectomy: A Case Report.

Background and Objectives: Approximately 20-40% of patients affected with renal cell carcinoma (RCC) develop either distant metastatic or locally recurring disease following radical nephrectomy. Soft tissue, skin, and the central nervous system are less common metastatic sites. We present the case of a patient who has received a diagnosis of RCC; it was found that she had no metastases at the time of nephrectomy but had metastases in the soft tissue and subcutaneous tissue of the scalp 27 years later. As far as we can tell, this is the longest period elapsed between primary renal tumor and subcutaneous/soft tissue metastasis; moreover, this case is the first report of a combined soft tissue/subcutaneous metastasis from RCC. Case presentation: A 73-year-old woman underwent right radical nephrectomy 27 years earlier for clear cell renal cell carcinoma (CCRCC). She presented at our unit because she noticed swelling in the left temporal region; after radiological exams, a benign lesion was suspected. The patient underwent surgical eradication, but the massive bleeding did not allow the removal of the lesion. A biopsy of the mass was performed and the histological examination was consistent with RCC metastases. Conclusions: Metastases from renal cell carcinoma to the subcutaneous and soft tissues are rare. It is essential to take into account RCC metastases in the differential diagnostic of the new starting mass of the head and neck, and the necessity for close and continuous surveillance of patients diagnosed with renal cancer even after a long disease-free period should be emphasized.

Cross-modal connectivity effects in age-related hearing loss.

Age-related sensorineural hearing loss (HL) leads to localized brain changes in the primary auditory cortex, long-range functional alterations, and is considered a risk factor for dementia. Nonhuman studies have repeatedly highlighted cross-modal brain plasticity in sensorial brain networks other than those primarily involved in the peripheral damage, thus in this study, the possible cortical alterations associated with HL have been analyzed using a whole-brain multimodal connectomic approach. Fifty-two HL and 30 normal hearing participants were examined in a 3T MRI study along with audiological and neurological assessments. Between-regions functional connectivity and whole-brain probabilistic tractography were calculated in a connectome-based manner and graph theory was used to obtain low-dimensional features for the analysis of brain connectivity at global and local levels. The HL condition was associated with a different functional organization of the visual subnetwork as revealed by a significant increase in global efficiency, density, and clustering coefficient. These functional effects were mirrored by similar (but more subtle) structural effects suggesting that a functional repurposing of visual cortical centers occurs to compensate for age-related loss of hearing abilities.

Longitudinal randomized study to evaluate the long-term outcome of endoscopic primary dacryocystorhinostomy with or without silicone tube.

OBJECTIVE: Dacryocystorhinostomy (DCR) is indicated for the treatment of nasolacrimal obstruction with some authors suggesting the use of a silicone tube (stent) to maintain rhinostomy patency a long time. This study aims at comparing the results of endoscopic-DCR (En-DCR) with and without silicone stenting. METHODS: A randomized prospective study was conducted from January 2013 to January 2018, following patients for up to 72 months. Sixty outbound patients suffering from chronic epiphora for primary acquired nasolacrimal duct obstruction were simply randomized and assigned to En-DCR with "silicone stent tube" (SST) or "no silicone stent tube" (NSST) group. Data about the results of the two procedures were collected using Munk' and Ali' assessments. The results were statistically compared to evaluate the differences. RESULTS: 30 patients were in the SST group and 30 in NSST. In the SST group, the tube remained in place for 3-6 months (4.1 ± 1.2 months). The follow-up period was 12-72 months (48.3 ± 6.2 months). Success rates (Junk and Javed Ali assessments) were, respectively, 97% in SST and 90% NSST group, with no statistical difference (Student's test). On a long-term follow-up, SST patients had an increased risk of re-stenosis by 14 months. CONCLUSIONS: Our results showed there were not benefit in using tube, in the opposite it increased risk of re-stenosis. Despite preliminary results, our data confirmed comparing the two methods that silicone tube should not be used.

Auditory cortex hypoperfusion: a metabolic hallmark in Beta Thalassemia.

BACKGROUND: Sensorineural hearing loss in beta-thalassemia is common and it is generally associated with iron chelation therapy. However, data are scarce, especially on adult populations, and a possible involvement of the central auditory areas has not been investigated yet. We performed a multicenter cross-sectional audiological and single-center 3Tesla brain perfusion MRI study enrolling 77 transfusion-dependent/non transfusion-dependent adult patients and 56 healthy controls. Pure tone audiometry, demographics, clinical/laboratory and cognitive functioning data were recorded. RESULTS: Half of patients (52%) presented with high-frequency hearing deficit, with overt hypoacusia (Pure Tone Average (PTA) > 25 dB) in 35%, irrespective of iron chelation or clinical phenotype. Bilateral voxel clusters of significant relative hypoperfusion were found in the auditory cortex of beta-thalassemia patients, regardless of clinical phenotype. In controls and transfusion-dependent (but not in non-transfusion-dependent) patients, the relative auditory cortex perfusion values increased linearly with age (p < 0.04). Relative auditory cortex perfusion values showed a significant U-shaped correlation with PTA values among hearing loss patients, and a linear correlation with the full scale intelligence quotient (right side p = 0.01, left side p = 0.02) with its domain related to communication skills (right side p = 0.04, left side p = 0.07) in controls but not in beta-thalassemia patients. Audiometric test results did not correlate to cognitive test scores in any subgroup. CONCLUSIONS: In conclusion, primary auditory cortex perfusion changes are a metabolic hallmark of adult beta-thalassemia, thus suggesting complex remodeling of the hearing function, that occurs regardless of chelation therapy and before clinically manifest hearing loss. The cognitive impact of perfusion changes is intriguing but requires further investigations.

Neuropsychological profile of hearing-impaired patients and the effect of hearing aid on cognitive functions: an exploratory study.

Few studies have investigated the neuropsychological profile of Hearing Loss (HL) subjects and the effects of hearing-aid on cognitive decline. We investigated the neuropsychological profile of HL patients at baseline and compared the neuropsychological profiles of patients with and without hearing-aid at 6 month. Fifty-six HL patients and 40 healthy subjects (HC) underwent neuropsychological and behavioral examination and were compared at baseline. Changes at follow-up were compared between HL patients with (N = 25) and without (N = 31) hearing-aids. At baseline, significant differences between HL and HC were found in MOCA test, Raven's Coloured Progressive Matrices (CPM) and SF-36. Among mild-HL patients, patients with hearing-aid significantly improved on the Clock Drawing Test (CDT) as compared to patients without hearing-aid. Our findings indicate that hearing loss is associated with both a reduced efficiency of the global cognitive state and a worse quality of life as compared to HC, supporting the association between HL and cognitive impairment. Moreover, only patients with mild-HL shows some cognitive improvement after using hearing-aid, suggesting that rehabilitative strategies may be more effective to delay cognitive decline in such patients. However, we cannot exclude that hearing-aids may affect cognitive decline in more severe-HL, but a longer follow-up is needed.

Long-Range Auditory Functional Connectivity in Hearing Loss and Rehabilitation.

Background: Patients with age-related sensorineural hearing loss (HL) may benefit from auditory input amplification by using hearing aids (HAs). However, the impact of both HL- and HA-based rehabilitation on central auditory functional connectivity (FC) is not clear. Methodology: Sixty-two HL (22 females, aged 64.4 ± 7.6 years, pure-tone average 50.9 ± 14.7 dB right ear, 50.7 ± 12.9 dB left ear) and 32 normal hearing (NH) subjects (22 females, aged 59.3 ± 7.3 years) were examined in a 3T magnetic resonance imaging (MRI) study. HL patients were analyzed cross-sectionally at baseline (vs. NH subjects) and longitudinally at 6-month follow-up. Between the 2 scans, 31/62 patients used the HA 9.5 ± 3.8 h a day. Arterial spin labeling and blood oxygen level-dependent resting-state functional MRI were performed to measure regional perfusion in the primary auditory cortex and, from here to the whole brain, seed-based FC was performed. Before each scan, HL patients underwent audiological and neurological assessments. Results: At baseline, the HL condition was associated with regional hypoperfusion in right Heschl's gyrus (seed) and negative seed-based FC (anticorrelation) in posterior brain regions. Long-range FC in the precuneus correlated negatively with pure-tone and speech reception average thresholds. At 6-month follow-up, HA usage was associated with seed-based FC increase in the right superior frontal gyrus (SFG) and seed-based FC reduction in the right middle temporal gyrus. Long-range FC changes in the SFG correlated positively with executive function improvements. Conclusions: These findings suggest that HA-based rehabilitation may not reverse HL-related neural effects and yet carry neurological benefits by retuning long-range FC of the auditory system. Impact statement Age-related sensorineural hearing loss (HL) affects 40% to 60% of the worldwide population and a common, viable rehabilitation strategy is to provide auditory input amplification through hearing aids (HAs). By targeting metabolically depressed, auditory cortical centers, our work reveals a possible neural link between peripheral and central vulnerability in HL patients in the form of aberrant, long-range, functional connectivity effects. Similarly, we unveil how wearing HAs for 6 months may induce neuroplastic changes that positively correlate with improved neuropsychological performances.

Left mixed laryngocele in absence of risk factors: A case report and review of literature.

Laryngocele is an uncommon benign cystic dilatation of the laryngeal saccule that communicates with the laryngeal lumen and contains air. On the basis of its localization, it can be traditionally classified in internal, external, or mixed. Usually unilateral and rarely bilateral, it may be congenital or acquired. It most often appears later in life without important symptoms except for cervical swelling. Here, together with a review of literature, we report the case of a 72-year-old man, smoker but without other specific risk factors, who presented laryngeal dyspnea for about one year. Neck CT scan performed during a previous hospitalization for respiratory failure revealed a left mixed laryngocele that was later surgically removed with cervicotomic access. The patient was discharged after one week. One month after surgery, we confirmed the absence of disease with video laryngoscopy.

Inner-Ear Disorders Presenting with Air-Bone Gaps: A Review.

Air-bone gaps (ABGs) are commonly found in patients with conductive or mixed hearing loss generally due to outer- and/or middle-ear diseases such as otitis externa, tympanic membrane perforation, interruption or fixation of the ossicular chain, and chronic suppurative otitis media. ABGs can also be found in correlation with inner-ear disorders, such as endolymphatic hydrops, enlarged vestibular aqueduct syndrome, semicircular canal dehiscence, gusher syndrome, cochlear dehiscence, and Paget disease's as well cerebral vascular anomalies including dural arteriovenous fistula. The typical clinical presentation of inner-ear conditions or cerebral vascular anomalies causing ABGs includes audiological and vestibular symptoms like vertigo, oscillopsia, dizziness, imbalance, spinning sensation, pulsatile or continuous tinnitus, hyperacusis, autophony, auricular fullness, Tullio's phenomenon, and Hennebert's sign. Establishing a definitive diagnosis of the underlying condition in patients presenting with an ABG is often challenging to do and, in many patients, the condition may remain undefined. Results from an accurate clinical, audiological, and vestibular evaluation can be suggestive for the underlying condition; however, radiological assessment by computed tomography and/or magnetic resonance imaging is mandatory to confirm any diagnostic suspicion. In this review, we describe and discuss the most recent updates available regarding the clinical presentation and diagnostic workup of inner-ear conditions that may present together with ABGs.

Impact of the COVID-19 Pandemic on Otolaryngology Residency: A Real-Life Experience.

The coronavirus disease (COVID-19) pandemic as been rapidly spreading worldwide. In our country, the entire Italian Healthcare System has been forced to adapt to this unprecedented condition in this century. The Head and Neck Department clinical and surgical activity was substantially reduced. In this situation, the Ear, Nose and Throat (ENT) residents in University Hospitals find themselves in an uncertain position; we are physicians, facing a deadly disease about which much remains unknown, but we are also trainees, and there is a high risk for our residency training to be affected. With this Letter, we would like to give a testimony of our experience and give some advices to bridge the training gap.

Cortical pattern of reduced perfusion in hearing loss revealed by ASL-MRI.

Age-related hearing loss (HL) can be related to brain dysfunction or structural damage and may result in cerebral metabolic/perfusion abnormalities. Arterial spin labeling (ASL) magnetic resonance imaging (MRI) allows investigating noninvasively brain perfusion changes. Pseudocontinuous ASL and T1-weighted MRI (at 3 T) and neuropsychological testing (Montreal Cognitive Assessment) were performed in 31 HL (age range = 47-77 years, mean age ± SD = 63.4 ± 8.4 years, pure-tone average [PTA] HL > 50 dB) and 28 normal hearing (NH; age range = 48-78 years, mean age ± SD = 59.7 ± 7.4 years) subjects. Cerebral blood flow (CBF) and gray matter volume (GMV) were analyzed in the cortical volume to assess perfusion and structural group differences. Two HL subjects showing cognitive impairment were excluded from group comparisons. No significant differences in either global or local atrophy were detected between groups but the HL group exhibited significant regional effects of reduced perfusion within the bilateral primary auditory cortex, with maximal CBF difference (-17.2%) in the right lateral Heschl's gyrus. For the whole sample of HL and NH subjects (n = 59 = 31 HL + 28 NH), the regional CBF was correlated positively to the regional GMV (p = 0.020). In HL subjects (n = 31), the regional CBF was correlated negatively to the audiogram steepness (frequency range: 2-4 kHz, right ear: p = 0.022, left ear: p = 0.015). The observed cortical pattern of perfusion reduction suggests that neuronal metabolism can be related to HL before the recognition of brain structural damage. This also illustrates the potential of ASL-MRI to contribute early functional markers of reduced central processing associated with HL.

Distraction techniques for face and smile aesthetic preventing ageing decay.

Modern concepts in the world of beauty arise from popular models, beautiful faces of actors document a bi-protrusive asset with high tension for soft tissues. Facial symmetry has been proposed as a marker of development and stability that may be important in human mate choice. For various traits any deviation from perfect symmetry can be considered a reflection of imperfect development. Additionally, bi-protrusive profile is dependent on the hormonal level regardless of male or female sex. The goal of maxillofacial surgery is to provide best results both for aesthetic and functional aspects. Following these new concepts of aesthetic of the face, new surgical procedure by osteodistraction techniques will lead to a very natural result by harmonizing the face also preventing aesthetic decay in aging faces. Ten cases with a feedback on the aesthetic results using the fivepoint scale of Likert after orthognatic surgery performed following distraction new techniques in combination with ancillary surgical procedures. The aesthetic results in all patients were highly satisfactory. All the patients accepted the new aesthetic of the face avoiding elements of discrepancy and consequently medico-legal problems.