Acute mastoiditis in cochlear implanted children: A single-centre experience / La mastoiditis aguda en niños con implante coclear: experiencia de un solo centro

Background Acute mastoiditis (AM) is the most common complication of acute otitis media and primarily affects children under the age of two; current data on its prevalence in paediatric patients with cochlear implant (CI) are still scant. Proper management of AM in CI children is crucial in order to avoid the implications (financial and emotional) of an explant. Aim of this paper is to describe the cases of AM occurred among young patients with CI in follow up at our department, also in order to evaluate its prevalence, potential predisposing factors, clinical course and therapeutic strategies. Patients and methods Retrospective study. Medical records of all paediatric patients with CI, who had at least one year of follow-up, were searched aiming to identify those who developed AM, from January 1st 2002 to January 31st 2022. The following data were collected and analysed: demographic features, implant type and side, interval between CI surgery and AM, treatment, laboratory tests, clinical course, vaccination history, associated diseases. Results AM was developed by six (1.3%) of the 439 children with CI (541 implanted ears). In total, 9 episodes (2.05 %) were recorded, as three patients reported two consecutive infections. Average time interval between CI surgery, to the first or only AM diagnosis, was 13.8 months (range 3–30 months). Furthermore, 3/6 of patients had a history of recurrent acute otitis media; 2/6 an autism spectrum disorder, associated to a combined immune deficiency in one case. All patients were hospitalized and promptly treated by intravenous antibiotic therapy; 4/6 also underwent a mastoidectomy. CI was not explanted in any cases of this series. Conclusions Over a 20-year period, AM rate in CI children was 1.3%, which is consistent with the current literature rates of 1–4.7%. All cases were successfully treated, preserving the integrity of the device. ... (AU)

Introducción La mastoiditis aguda (MA) es la complicación más común de la otitis media aguda y afecta principalmente los niños menores de dos años; los datos actuales sobre su prevalencia en pacientes pediátricos con implante coclear (IC) son aún escasos. El manejo adecuado de la MA en los niños con IC es crucial para evitar las implicaciones (económicas y emocionales) de un explante. El objetivo de este articulo es describir los casos de MA ocurridos en pacientes jóvenes con IC en seguimiento en nuestro servicio, también para evaluar su prevalencia, posibles factores predisponentes, curso clínico y estrategias terapéuticas. Pacientes y métodos Estudio retrospectivo. Se realizaron búsquedas en las historias clínicas de todos los pacientes pediátricos con IC, que tenían al menos un año de seguimiento, con el objetivo de identificar a aquellos que desarrollaron la MA, desde el 1 de enero de 2002 hasta el 31 de enero de 2022. Se recopilaron y analizaron los siguientes datos: características demográficas, tipo de implante y lado, intervalo entre cirugia del IC y MA, tratamiento, exámenes de laboratorio, evolución clínica, antecedentes vacunales, enfermedades asociadas. Resultados La MA fue desarrollada por seis (1,3%) de los 439 niños con IC (541 oídos implantados). En total se registraron 9 episodios (2,05 %), ya que tres pacientes reportaron dos infecciones consecutivas. El intervalo de tiempo promedio entre la cirugía del IC y el primer o único diagnóstico de la MA fue de 13,8 meses (rango 3-30 meses). Además, 3/6 de los pacientes tenían antecedentes de otitis media aguda recurrente; 2/6 un trastorno del espectro autista, asociado a una inmunodeficiencia combinada en un caso. Todos los pacientes fueron hospitalizados y tratados de inmediato con terapia antibiótica intravenosa; 4/6 también se sometieron a una mastoidectomía. El IC no fue explantado en ningún caso de esta serie. Conclusiones ... (AU)

Acute mastoiditis in cochlear implanted children: A single-centre experience.

BACKGROUND: Acute mastoiditis (AM) is the most common complication of acute otitis media and primarily affects children under the age of two; current data on its prevalence in paediatric patients with cochlear implant (CI) are still scant. Proper management of AM in CI children is crucial in order to avoid the implications (financial and emotional) of an explant. Aim of this paper is to describe the cases of AM occurred among young patients with CI in follow up at our department, also in order to evaluate its prevalence, potential predisposing factors, clinical course and therapeutic strategies. PATIENTS AND METHODS: Retrospective study. Medical records of all paediatric patients with CI, who had at least one year of follow-up, were searched aiming to identify those who developed AM, from January 1st 2002 to January 31st 2022. The following data were collected and analysed: demographic features, implant type and side, interval between CI surgery and AM, treatment, laboratory tests, clinical course, vaccination history, associated diseases. RESULTS: AM was developed by six (1.3%) of the 439 children with CI (541 implanted ears). In total, 9 episodes (2.05 %) were recorded, as three patients reported two consecutive infections. Average time interval between CI surgery, to the first or only AM diagnosis, was 13.8 months (range 3-30 months). Furthermore, 3/6 of patients had a history of recurrent acute otitis media; 2/6 an autism spectrum disorder, associated to a combined immune deficiency in one case. All patients were hospitalized and promptly treated by intravenous antibiotic therapy; 4/6 also underwent a mastoidectomy. CI was not explanted in any cases of this series. CONCLUSIONS: Over a 20-year period, AM rate in CI children was 1.3%, which is consistent with the current literature rates of 1-4.7%. All cases were successfully treated, preserving the integrity of the device. In our experience, the early parenteral antibiotic therapy and, when necessary, surgical treatment were adequate to eradicate the infection.

Characterization of the Notch pathway in nasal polyps of patients with chronic rhinosinusitis: A pilot study.

Chronic rhinosinusitis with nasal polyps is a widespread pathology characterized by persistent inflammation of nasal and paranasal mucosa. Although it represents one of the most frequent diseases of the nasal cavities, its etiology is still not completely elucidated. There is evidence suggesting that the Notch signaling, a highly conserved intercellular pathway known to regulate many cellular processes, including inflammation, is implicated in nasal polyps formation. The purpose of this study was to investigate the expression of genes of the Notch pathway in nasal polyps from patients with chronic rhinosinusitis. Nasal polyps and adjacent mucosa tissue were obtained from 10 patients. RNA was analyzed by quantitative reverse transcriptase-polymerase chain reaction for the expression level of (1) Notch pathway components such as receptors (NOTCH1-4), ligands (DLL4, JAGGED-1), and target genes (HEY1, 2, and HES1) and (2) genes providing information on the pathogenesis of polyposis (C-MYC and SCGB1A1) and on eosinophils content (CCL26, IL5, and SAA2). We report a Notch-driven gene expression pattern in nasal polyps which correlates with the expression of genes highly expressed in eosinophils, whose presence is an important parameter to define the pathophysiologic diversity characterizing nasal polyps. Taken together, our results suggest a role for Notch signaling in the pathophysiology of polyposis. Further studies are needed to elucidate the role of Notch in nasal polyps formation and to establish whether it could represent a novel therapeutic target for this pathology.

Serum HPV16 E7 Oncoprotein Is a Recurrence Marker of Oropharyngeal Squamous Cell Carcinomas.

Despite improved prognosis for many HPV-positive head and neck squamous cell carcinomas (HNSCCs), some cases are still marked by recurrence and metastasis. Our study aimed to identify novel biomarkers for patient stratification. Classical HPV markers: HPV-DNA, p16 and HPV mRNA expression were studied in HNSCC (n = 67) and controls (n = 58) by qPCR. Subsequently, ELISA tests were used for HPV16 L1 antibody and HPV16 E7 oncoprotein detection in serum at diagnosis and follow-up. All markers were correlated to relapse-free survival (RFS) and overall survival (OS). HPV-DNA was found in HNSCCs (29.85%), HPV16-DNA in 95% of cases, HPV16 E7 mRNA was revealed in 93.75%. p16 was overexpressed in 75% of HPV-positive HNSCC compared to negative samples and controls (p < 0.001). Classical markers correlated with improved OS (p < 0.05). Serological studies showed similar proportions of HPV16 L1 antibodies in all HNSCCs (p > 0.05). Serum E7 oncoprotein was present in 30% HPV-positive patients at diagnosis (p > 0.05) and correlated to HNSCC HPV16 E7 mRNA (p < 0.01), whereas it was associated to worse RFS and OS, especially for oropharyngeal squamous cell carcinoma (OPSCC) (p < 0.01). Detection of circulating HPV16 E7 oncoprotein at diagnosis may be useful for stratifying and monitoring HPV-positive HNSCC patients for worse prognosis, providing clinicians a tool for selecting patients for treatment de-escalation.

Association between oncogenic human papillomavirus type 16 and Killian polyp.

BACKGROUND: Killian polyp (KP) is a benign lesion that arises from the maxillary sinus. The etiology of KP is unknown. The aim of this study was to investigate the potential involvement of human papilloma- (HPV) and polyoma-viruses (HPyV) infections in the onset of KP. METHODS: DNA from antral (n = 14) and nasal (n = 14) KP fractions were analyzed for HPV and HPyV sequences, genotypes, viral DNA load and physical status along with expression of viral proteins and p16 cellular protein. RESULTS: The oncogenic HPV16 was detected in 3/14 (21.4%) antral KPs, whilst nasal KPs tested HPV-negative (0/14). The mean HPV16 DNA load was 4.65 ± 2.64 copy/104 cell. The whole HPV16 episomal genome was detected in one KP sample, whereas HPV16 DNA integration in two KPs. P16 mRNA level was lower in the KP sample carrying HPV16 episome than in KPs carrying integrated HPV16 and HPV- negative KPs (p< 0.001). None of the antral and nasal KP samples tested positive for HPyV DNA (0/28). CONCLUSIONS: A fraction of KP tested positive for the oncogenic HPV16. HPV16 detection in the KP antral portion may be consistent with HPV16 infection derived from the maxillary sinus. HPV16 DNA integration represents a novel finding. Altogether, these data improve our knowledge on the association between KP and HPV infection, whereas it indicates that the KP onset is heterogeneous.

Outcome of cochlear implantation in children with congenital Cytomegalovirus infection: A retrospective case control study.

INTRODUCTION: To date, cCMV represents the most frequent non-genetic congenital cause of permanent sensorineural hearing loss (SNHL) in childhood and the leading infectious cause of developmental and neurologic disabilities. The aim of this paper is to describe the outcome of cochlear implantation in children affected by severe-to-profound sensorineural hearing loss, due to a symptomatic or asymptomatic cCMV infection, particularly comparing their performance results to that of matched mutated Connexin 26 (Cx26) implanted patients. METHODS: Retrospective case control study. The clinical data of symptomatic cCMV and asymptomatic cCMV patients were collected and compared to those of Cx26 patients matched for age and pre-CI (cochlear implant) linguistic category; all subjects were affected by bilateral severe-to-profound SNHL and were treated by CI and speech therapy rehabilitation. The Speech Perception Category, the language stage and the linguistic level scores, at 6 months, 1 year, and 3-4 years after CI of the three groups (symptomatic cCMV, asymptomatic cCMV and Cx26 mutation) were collected and compared. RESULTS: Statistical analysis did not show any significant difference in pre-CI perception category and linguistic level among the three groups; the symptomatic cCMV group showed a statistically worse performance of the language stage over time (p = 0.017). CONCLUSIONS: Our data support that children affected by cCMV have improved language abilities over time, although the symptomatic cCMV group achieved a lower language stage 3-4 years after CI compared to the asymptomatic cCMV and Cx26 mutation groups. Nonetheless, to date, CI supported by speech therapy can be considered an effective intervention for children affected by cCMV-related severe-to-profound hearing loss.

Lymphoscintigraphy and radio-guided neck dissection in oral cavity squamous cell carcinoma.

PURPOSE: The development of erratic distribution of cervical metastases from oral cavity squamous cell carcinoma (OSCC) bypassing the typical metastatic pattern can possibly challenge the role of the classic neck dissection. The purpose of this study was to assess the role of lymphoscintigraphy (LS) and radio-guided neck dissection as a simple and widely accessible method with a favorable cost/benefit ratio, able to improve the OSCC staging and possibly to tailor the surgical approach to cervical lymph node dissection. METHODS: From June 2015 to December 2018, 16 patients (5 women, 11 men, median age 59.5±12.5 years) with cN0 (10) and cN+ (6) OSCC were enrolled. The day before surgery all patients underwent LS with acquisition of planar and SPECT (Single Photon Emission Computed Tomography)/CT images, after a peritumoral injection of 99mTc-Nanocoll® (median 74±1.2 MBq). Patients underwent tumor excision and a radioguided neck dissection, using a portable gamma camera. The sentinel lymph nodes (SLNs) were isolated and separately analyzed in 200-micron sections and pancytokeratin immunohistochemistry assessment, looking for micrometastases. RESULTS: A homolateral lymphatic spread on LS was observed in all cases, whereas in 5 cases (31.3%) lymphatic drainage was contralateral to the OSCC site. In one cN0 patient, a skip micrometastasis has been identified in a SLN. CONCLUSION: The results of the present study may suggest a role of LS and radioguided neck dissection in detecting the real lymphatic spread of OSCC, in order to improve the oncological assessment and to tailor the neck dissection.

High Human Papillomavirus DNA loads in Inflammatory Middle Ear Diseases.

Background. Previous studies reported human papillomaviruses (HPVs) in middle ear tumors, whereas these viruses have been poorly investigated in chronic inflammatory middle ear diseases. We investigated HPVs in non-tumor middle ear diseases, including chronic otitis media (COM). Methods. COM specimens (n = 52), including chronic suppurative otitis media (CSOM) (n =38) and cholesteatoma (COMC) (n = 14), as well as normal middle ear (NME) specimens (n = 56) were analyzed. HPV sequences and DNA loads were analyzed by quantitative-PCR. HPV genotyping was performed by direct sequencing. Results. HPV DNA was detected in 23% (12/52) of COM and in 30.4% (17/56) of NME (p > 0.05). Specifically, HPV DNA sequences were found in 26.3% (10/38) of CSOM and in 14.3% (2/14) of COMC (p > 0.05). Interestingly, the HPV DNA load was higher in COMC (mean 7.47 copy/cell) than in CSOM (mean 1.02 copy/cell) and NME (mean 1.18 copy/cell) (P = 0.03 and P = 0.017 versus CSOM and NME, respectively). HPV16 and HPV18 were the main genotypes detected in COMC, CSOM and NME. Conclusions. These data suggest that HPV may infect the middle ear mucosa, whereas HPV-positive COMCs are associated with higher viral DNA loads as compared to NME.

Is Peripheral Oxygen Saturation a Reliable Predictor of Upper Airways Air-Flow Limitation?

BACKGROUND: Dyspnea secondary to acute upper airways airflow limitation (UAAFL) represents a clinical emergency that can be difficult to recognize without a suitable history; even when etiology is known, parameters to assess the severity are unclear and often improperly used. OBJECTIVES: The aim of this study was to assess the role of peripheral oxygen saturation (SpO2) as a predictor of severity of upper airway obstruction. METHODS: The authors propose an experimental model of upper airway obstruction by a progressive increase of UAAFL. Ten healthy volunteers randomly underwent ventilation for 6 min with different degrees of UAAFL. SpO2, heart rate, respiratory rate (RR), tidal volume, accessory respiratory muscle activation, and subjective dyspnea indexes were measured. RESULTS: In this model, SpO2 was not reliable as the untimely gravity index of UAAFL. Respiratory rate, visual analogue scale (VAS), and Borg dyspnea scale were statistically correlated with UAAFL (p < 0.0001 for RR and p < 0.05 for VAS and Borg scale). No significant changes were observed on heart rate (p > 0.05) and tidal volume (p > 0.05); a RR ≤ 7 breaths/min; VAS and Borg scale showed statistically significant parameters changes (p < 0.05). CONCLUSIONS: RR, VAS, and Borg dyspnea scales are sensitive parameters to detect and stage, easily and quickly, the gravity of an upper airways impairment, and should be used in emergency settings for an early diagnosis of a UAAFL. SpO2 is a poorer predictor of the degree of upper airways flow limitation.

Combined Endoscopic and Trans Palpebral Orbital Reconstruction for Silent Sinus Syndrome.

Silent Sinus Syndrome is defined as a painless spontaneous and progressive enophthalmos and hypoglobus with maxillary sinus hypoplasia and orbital floor resorption. It is caused by maxillary sinus atelectasis in a setting of ipsilateral chronic maxillary sinus hypoventilation. The syndrome was first described in 1964 by Montgomery, but the term "Silent Sinus Syndrome" was not coined until 1994 by Soparkar. The aetiology is still controversial: some authors postulate a basal hypoplastic sinus, other suggest an acquired process due to an obstruction of the ostium in the medium meatus. Silent Sinus Syndrome presents in the third to fifth decades of life, very rarely in childhood with no gender predilection and it is usually a unilateral disorder. The symptoms are not shown to be related to chronic sinuses disease. The clinical signs are: enophthalmos, hypoglobus, upper lid retraction secondary to dystopia of the globe, sinking of the eye and orbital asymmetry, deepened upper lid sulcus, disappearance of the palpebral fold line, lagophthalmos, vertical diplopia, malar depression, and facial asymmetry. Extraocular muscle function is generally preserved and usually there is no visual impairment. The diagnosis is confirmed by computed tomography scan of the orbits and paranasal sinuses. The treatment consists of orbital reconstruction and functional rehabilitation of the maxillary sinuses.

Supracricoid partial laryngectomy: oncological and functional results.

PURPOSE: The purpose of this study was to evaluate the oncological and functional results of patients affected by laryngeal squamous cell carcinoma (SCC) and surgically treated by supracricoid partial laryngectomy (SCPL) at the ENT Department of the University Hospital of Ferrara. METHODS: In this retrospective study a total of 155 patients (149 males/l96.1% and 6 females/3.9%), have been included. All patients were treated between January 1st 1998 and December 31st 2010, by SCPL, including 126 cricohyoidopexies (CHP) and 29 cricohyoidoepiglottopexies (CHEP). RESULTS: The overall survival (OS) at 3 and 5 years was 88.77 and 83.24%, respectively and the disease-free survival (DFS) at 3 and 5 years was 84.4 and 81.55%, respectively.The recurrence rate was 17.5%, with local recurrences in 12.1% of the cases, regional in 4.7% and distant metastasis in 0.7% of the cases. Synchronous second primary tumors were 0.7% and metachronous second primary cancers (MSPCs) 5.4%. Removal of nasogastric feeding tube (NGT) or percutaneous endoscopic gastrostomy (PEG) was performed in 98.7% of the patients and lasted 22 days on average after SCPL (range 9-60), while decannulation was performed in all of patients at the 27th day on average after surgery. CONCLUSIONS: Oncological outcomes of this series are consistent with those of the literature, showing that SCPL is an effective and safe procedure in terms of survival rate and disease control. Functional outcomes confirmed that SCPL allows a good organ preservation and recovery of laryngeal functions.

Infection and HLA-G molecules in nasal polyposis.

Sinonasal polyposis (SNP) is a chronic inflammatory pathology with an unclear aetiopathogenesis. Human papillomavirus (HPV) infection is one candidate for the development of SNP for its epithelial cell trophism, hyperproliferative effect, and the induction of immune-modulatory molecules as HLA-G. We enrolled 10 patients with SNP without concomitant allergic diseases (SNP-WoAD), 10 patients with SNP and suffering from allergic diseases (SNP-WAD), and 10 control subjects who underwent rhinoplasty. We analyzed the presence of high- and low-risk HPV DNA and the expression of membrane HLA-G (mHLA-G) and IL-10 receptor (IL-10R) and of soluble HLA-G (sHLA-G) and IL-10 by polyp epithelial cells. The results showed the presence of HPV-11 in 50% of SNP-WoAD patients (OR:5.5), all characterized by a relapsing disease. HPV-11 infection was absent in nonrelapsing SNP-WoAD patients, in SNP-WAD patients and in controls, supporting the hypothesis that HPV-11 increases risk of relapsing disease. HPV-11 positive SNP-WoAD patients presented with mHLA-G and IL-10R on epithelial cells from nasal polyps and showed secretion of sHLA-G and IL-10 in culture supernatants. No HLA-G expression was observed in HPV negative polyps. These data highlight new aspects of polyposis aetiopathogenesis and suggest HPV-11 and HLA-G/IL-10 presence as prognostic markers in the follow-up of SNP-WoAD.

Synchronous papillary carcinoma and hemangiopericytoma with lung metastases.

Hemangiopericytomas (HPC) are uncommon tumors that originate from perivascular cells of capillary vessels. HPC are about 1% of all vascular tumors and can be found in the head-neck region with an incidence between 16% and 33%. HPC is a neoplasm of uncertain malignant potential; it can behave as an aggressive tumor with metastases and increased mitotic activity or as a relatively benign neoplasm with only local development. In this paper we describe a case of hemangiopericytoma with uncertain malignant potential with cervical location associated with a concomitant papillary thyroid carcinoma and lung metastasis of unknown origin; this case led us to follow a specific and uncommon diagnostic and therapeutic strategy.

Decreased production of human leukocyte antigen G molecules in sinonasal polyposis.

BACKGROUND: Sinonasal polyposis (SNP) is a chronic inflammatory pathology of nasal and paranasal cavities. Human leukocyte antigen (HLA) G molecules are nonclassic class I antigens with anti-inflammatory and tolerogenic properties. As most theories consider polyps to be the manifestation of chronic inflammation, there could be a possible implication of HLA-G molecules in SNP. The purpose of this study was to investigate the possible correlation between SNP and the production of soluble HLA-G (sHLA-G) by peripheral blood mononuclear cells (PBMCs). METHODS: The study involved 22 SNP patients (11 with no evidence of disease [NED] after surgery and 11 with relapse [RE]) and 20 healthy subjects. The presence of sHLA-G in PBMC lipopolysaccharide (LPS)-stimulated culture supernatants was analyzed. The levels of interleukin (IL) 10, one of the main up-regulators of sHLA-G production, were determined. Exogenous IL-10 was added to the SNP PBMC cultures to reconstitute the impairment in sHLA-G production. RESULTS: Increased IL-10 levels in LPS-activated PBMC culture supernatants were found in NED patients in comparison with healthy subjects (p = 0.0184). No sHLA-G production was observed in either of the patient subgroup supernatants (p < 0.0001). The addition of exogenous IL-10 showed the reconstitution of sHLA-G production in NED and in a lower amount in RE patients. CONCLUSION: The results show a defect in sHLA-G production in SNP patients mainly related to the IL-10/HLA-G pathway. Given the anti-inflammatory functions of HLA-G molecules, this impairment could increase the susceptibility to the disease. The different sHLA-G production after exogenous IL-10 addition between NED and RE SNP could represent a marker of disease severity.