A regional-based newborn hearing screening program: the Emilia-Romagna model after ten years of legislation.

Background: Hearing loss, occurring in 1-3/1,000 newborns in the well-babies population, is one of the most common congenital diseases, and hearing screening at birth still represents the only means for its early detection. Since 2011 the Emilia Romagna Regional Health Agency has recommended Newborn Hearing Screening for all babies at its birth points and for newborns moving to the region. The aims of this study are to analyze the results of this regional-based Newborn Hearing Screening program and to discuss the impact of the legislative endorsement on the organization. Material and methods: This is an observational retrospective chart study. The recordings of well-babies and babies at Neonatal Intensive Care Units were collected during the period from January 1st 2015 to December 31st 2020. The following data were included: Newborn Hearing Screening coverage, percentage of refer at otoacoustic emissions, prevalence and entity of hearing loss, unilateral/bilateral rate, presence of audiological risk factors. Results: More than 99% of a total of 198,396 newborns underwent the Newborn Hearing Screening test during the period January 1st 2015 to December 31st 2020, with a coverage ranging between 99.6% and 99.9%. Overall, the percentage of confirmed hearing loss cases was about 17-30 % of refer cases, 745 children received a diagnosis of hearing loss (prevalence 3.7/1,000). Considering profound hearing loss cases, these represent 13% of bilateral hearing loss. Conclusion: A regional-based Newborn Hearing Screening program is valuable and cost-effective. In our experience, the centralization of the data system and of the data control is crucial in order to implement its efficiency and effectiveness. Healthcare policies, tracking systems and public awareness are decisive for a successful programme implementation.

Smell and taste dysfunction during the COVID-19 pandemic: an infodemiological analysis.

OBJECTIVE: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection can cause smell and taste dysfunction. We aimed to investigate the general community's interest in smell dysfunction (SD) and taste dysfunction (TD) using Google Trends to compare results with more common symptoms associated with SARS-CoV-2 infection, such as fever and cough. MATERIALS AND METHODS: Relative Search Volumes (RSVs) for the English terms "Smell", "Taste", "Fever" and "Cough", filtered by the category "Health", were collected from 2018 through 2020. Moreover, RSVs using synonyms of "Taste" and "Smell" in 5 European languages were analyzed. RESULTS: The worldwide mean RSVs for "Fever", "Cough", "Smell", and "Taste" during 2020 were 49%, 34%, 8% and 9%, respectively. RSVs associated with the search terms "Fever" and "Cough" showed a peak between February and March 2020, as did "Smell" and "Taste". Even though RSVs were much lower, they were highly correlated (r=0.890). RSVs obtained from "Smell" and "Taste" in five European languages (German, English, French, Italian and Spanish) had similar temporal trends. CONCLUSIONS: Our findings show the level of the general population's interest for early symptoms, suggesting that their interest in SARS-CoV-2 infection symptoms, such as SD and TD, was scarce but peaked during the pandemic outbreak.

Ion concentrations in nasal airway surface liquid: a prediction model for the identification of cystic fibrosis carriers.

BACKGROUND: Cystic fibrosis (CF) carriers seem to have a higher risk to develop chronic rhino-sinusitis (CRS), although the full underlying mechanisms are unknown. Ion concentrations in nasal airway surface liquid (ASL) may be influenced by the heterozygosity for CF gene mutation, with possible impacts on the development of CRS. METHODS: A cheap and feasible standardized technique was designed to measure the ion levels in nasal ASL. With this purpose we collected, under basal conditions, samples from the nasal cavity of 165 adults: 14 homozygous for CF, 83 carriers and 68 healthy controls. Sodium (Na) and Chlorine (Cl) concentrations were then evaluated among different groups. RESULTS: Statistical analysis revealed a significant difference of Na and Cl values between controls and carriers and between controls and homozygotes. Receiver operating characteristic (ROC) curves and derived indicators (Youden's index and Area Under the Curve, AUC) were used to further evaluate the diagnostic capability of Na and Cl concentrations to differentiate heterozygotes from controls. ROC curves demonstrated that the optimal diagnostic cut-off value of Na is at 124, and the optimal cut-off value of Cl is at 103,2. CONCLUSION: ASL sampling can be considered a new diagnostic tool for providing quantitative information on nasal ion composition. According to our findings, Na and Cl concentrations of nasal ASL could represent a useful tool to assess heterozygotes and healthy controls.

Surgical management of acquired anterior glottic web: a systematic review.

BACKGROUND: Acquired anterior glottic web poses a significant challenge to laryngologists given its propensity to recur following treatment, and there are a wide variety of described techniques. METHODS: A systematic review of the medical literature was undertaken in order to identify all articles pertaining to the management of acquired anterior glottic web. RESULTS: Thirteen studies meeting the inclusion criteria were identified and analysed. All were retrospective series, with varying surgical techniques and outcome measures. Only two studies reported on the use of topical mitomycin C. CONCLUSION: Mucosal graft techniques and keel placement appear to improve success rates, but both carry risks and disadvantages. Based on the available evidence, the use of topical agents such as mitomycin C cannot be recommended in the management of acquired anterior glottic web.

Hearing threshold estimation by auditory steady state responses (ASSR) in children.

Hearing threshold identification in very young children is always problematic and challenging. Electrophysiological testing such as auditory brainstem responses (ABR) is still considered the most reliable technique for defining the hearing threshold. However, over recent years there has been increasing evidence to support the role of auditory steady-state response (ASSR). Retrospective study. Forty-two children, age range 3-189 months, were evaluated for a total of 83 ears. All patients were affected by sensorineural hearing loss (thresholds ≥ 40 dB HL according to a click-ABR assessment). All patients underwent ABRs, ASSR and pure tone audiometry (PTA), with the latter performed according to the child's mental and physical development. Subjects were divided into two groups: A and B. The latter performed all hearing investigations at the same time as they were older than subjects in group A, and it was then possible to achieve electrophysiological and PTA tests in close temporal sequence. There was no significant difference between the threshold levels identified at the frequencies tested (0.25, 0.5, 1, 2 and 4 kHz), by PTA, ABR and ASSR between the two groups (Mann Whitney U test, p < 0.05). Moreover, for group A, there was no significant difference between the ASSR and ABR thresholds when the children were very young and the PTA thresholds subsequently identified at a later stage. Our results show that ASSR can be considered an effective procedure and a reliable test, particularly when predicting hearing threshold in very young children at lower frequencies (including 0.5 kHz).

Enlarged vestibular aqueduct: Audiological and genetical features in children and adolescents.

BACKGROUND: Enlarged Vestibular Aqueduct (EVA) is one of the most common congenital malformations associated with sensorineural or mixed hearing loss. The association between hearing loss and EVA is described in syndromic (i.e. Pendred Syndrome, BOR, Waardenburg) and non-syndromic disorders, as isolate or familiar mutations of the SLC26A4 gene. The audiological phenotype of the EVA syndrome is heterogeneous, the type and entity of hearing loss may vary and vertigo episodes might also be present. OBJECTIVE: The aim of this retrospective study was to describe the clinical and genetic features of a group of adolescent subjects presenting an EVA clinical profile, considering the presence of SLC26A4 gene mutations. METHODS: 14 Caucasian patients were assessed (24 ears in total; 4 patients presented a monolateral EVA), 10 females and 4 males. Their age at the time of diagnosis was between 1 and 6 years (mean age 2.5 years). Subjects were assessed by an ENT microscopy evaluation with a complete audiometric assessment, CT & MRI scans and genetic tests for the evaluation of the pendrin gene mutations (SLC26A4). RESULTS: Considering the presence of SLC26A4 mutations and thyroid function, we could identify three sub-groups of patients: group 1, non syndromic EVA (ns EVA, no SLC26A4 mutation and no thyroid dysfunction); group 2, EVA with DFNB4 (single SLC26A4 gene mutation and no thyroid dysfunction); group 3, EVA with Pendred Syndrome (two pathological mutation of SLC26A4 and thyromegaly with thyroid dysfunction). Patients of group 1 (ns-EVA) showed various degrees of hearing loss from mild (55%) to severe-profound (45%). In groups 2 (DFNB4) and 3 (PDS), the degree of hearing loss is severe to profound in 70-75% of the cases; middle and high frequencies are mainly involved. CONCLUSIONS: The phenotypic expressions associated with the EVA clinical profile are heterogeneous. From the available data, it was not possible to identify a representative audiological profile, in any of the three sub-groups. The data suggest that: (i) a later onset of hearing loss is usually related to EVA, in absence of SLC26A4 gene mutations; and (ii) hearing loss is more severe in patients with SLC26A4 gene mutations (groups 2 and 3 of this study).

Transnasal endoscopic management of anterior cerebrospinal fluid (CSF) leak: experience from a large case series.

Transnasal endoscopic management of anterior cerebrospinal fluid (CSF) leak: experience from a large case series. OBJECTIVES: Anterior cerebrospinal fluid (CSF) leak is a consequence described after head trauma, skull base surgery or inflammatory diseases, but may also occur spontaneously. Prompt recognition and management is crucial in order to avoid complications and to achieve successful surgical repair. The purpose of this study is to present the clinical features of a large group of patients with anterior CSF leak and the results of their surgical management. METHODOLOGY: The study approach was a retrospective review of 110 consecutive patients treated by the same surgeon for CSF leak of the anterior skull base via a transnasal endoscopic approach between 2003 and 2013. Patients with anterior skull base surgery due to cancer or pituitary adenoma were excluded from this study. The diagnostic workup included nasal endoscopy, beta2-transferrin and -trace protein assay in the rhinoliquorrhoea and radiological imaging. The intrathecal fluorescein test was performed in selected cases. The surgical techniques used to repair dural defects were primarily middle turbinate placement and fascia lata grafting. RESULTS: Primary endoscopic repair of the CSF fistula was successful in 106/110 (96.4%) patients. Four patients (3.6%) required a revision procedure due to early failure of the repair. After a minimum follow-up of 24 months, no recurrent CSF leaks were observed in the study group. None of the patients developed meningitis as a surgical complication. CONCLUSIONS: The endoscopic transnasal approach is a reliable technique for treating CSF leaks. The intrathecal fluorescein test is safe and helpful in locating the defect. The fascia lata proved the best graft available in our experience.

Outcomes of long-term audiological rehabilitation in charge syndrome.

The aim of this paper is to assess the long-term audiological features and outcomes of hearing rehabilitation in a large group of individuals with CHARGE Syndrome. The study has been conducted retrospectively, on a paediatric patient database, at the Audiology Department of the University Hospitals of Ferrara and Padua. The study sample included 31 children presenting with different degrees of hearing impairment associated with CHARGE syndrome. Hearing was assessed using auditory brainstem responses (ABRs) and/or electrocochleography, or conditioned audiometry (visual reinforcement audiometry [VRA] or play audiometry). Auditory-perceptual outcomes in terms of communication skills and expressive language were also recorded. The effects of hearing rehabilitation (with hearing aids or cochlear implants) in this group of children and language outcomes after rehabilitation were monitored during long-term follow-up. The outcomes of rehabilitation measures differed in relation to the heterogeneous and often severe disabilities associated with CHARGE syndrome, e.g. developmental delay, intellectual delay, visual impairment, thin 8(th) nerve with retrocochlear auditory dysfunction (as described in cases of auditory neuropathy/dyssynchrony). Oral expressive language was severely impaired in most cases, even after lengthy follow-up, suggesting the need for alternative augmentative communication modes. The early identification of sensorineural hearing loss, and carefully planned rehabilitation treatments, can be of some benefit in children with CHARGE syndrome.

Post-operative pain management in head and neck cancer patients: predictive factors and efficacy of therapy.

There is increasing interest about all aspects of pain sensation for patients undergoing head and neck surgery, and efforts have been made to better assess, monitor and reduce the occurrence of pain. The aetiology of pain is considered to be "multifactorial", as it is defined by several features such as personal experience, quality perception, location, intensity and emotional impact. The aim of this paper is: (i) to evaluate the efficacy of analgesic treatment in patients with head and neck cancer treated by surgery, and (ii) to study the variables and predictive factors that can influence the occurrence of pain. A total of 164 patients, affected by head and neck cancer and surgically treated, between December 2009 and December 2013, were included in this study. Data collected include age, gender, assessment of anaesthetic risk, tumour localisation, pathological cancer stage, TNM stage, type of surgery performed, complexity and duration of surgery, post-operative complications, postoperative days of hospital stay and pain evaluation on days 0, 1, 3 and 5 post-surgery. We studied the appropriateness of analgesic therapy in terms of incidence and prevalence of post-operative pain; we also related pain to patient characteristics, disease and surgical treatment to determine possible predictive factors. The population studied received adequate pain control through analgesic therapy immediately post-surgery and in the following days. No associations between gender, age and post-operative pain were found, whereas pathological cancer stage, complexity of surgery and tumour site were significantly associated with the risk of post-operative pain. Adequate pain control is essential in oncological patients, and particularly in head and neck cancer patients as the prevalence of pain in this localisation is reported to be higher than in other anatomical sites. Improved comprehension of the biological and psychological factors that characterise pain perception will help to enhance its control in the future.

Listeria Monocytogenes: an uncommon pathogen of cervical necrotizing fasciitis.

The aim of this paper is to present a unique case of neck-necrotizing fasciitis caused by Listeria Monocytogenes in a young woman, successfully treated by surgery and IV antibiotic therapy. Necrotizing fasciitis is a rare, rapidly progressing and potentially life-threatening infection that infrequently occurs in the head and neck region. Pathogens involved in necrotizing fasciitis are heterogeneous and include aerobic and anaerobic bacteria. To the best of our knowledge, this is the only case of neck necrotizing fasciitis caused by Listeria Monocytogenes studied in literature so far.

Sudden hearing loss and the risk of subsequent cerebral ischemic stroke.

OBJECTIVE: Very few studies have investigated the risk of ischemic stroke after an episode of sudden sensorineural hearing loss (SSNHL), and findings have been controversial. The aim of this study was to estimate the risk of ischemic stroke among SSNHL patients within the province of Ferrara and compare the findings with data available in the literature. METHODS: This was a cohort study using hospital discharge records. The observation period was from 1 January 2001 to 31 December 2012. The study cohort consists of all adult patients hospitalized with a principal diagnosis of SSNHL (n = 484) and all adult patients with a diagnosis of ischemic stroke (n = 9985) among the resident population of the province of Ferrara, Emilia-Romagna, Italy. We calculated the incidence of SSNHL and ischemic stroke, as well as the incidence of ischemic stroke within the group of patients who experienced SSNHL. RESULTS: During the period 2001-2012 in Ferrara, the average annual crude incidence of SSNHL was 11.4/100,000 (95% CI 10.4-12.4), while the average annual crude incidence of the first ischemic stroke was 235.3/100,000. During the mean observation time period of 6 years, the expected and observed cases of ischemic stroke among the 484 patients with SSNHL did not differ significantly (6.8 expected vs. 9 observed [95% Poisson Confidence Interval 4.11-17.08]). CONCLUSIONS: Our findings suggest that SSNHL does not significantly increase the risk of ischemic stroke.

Simultaneous nasopharyngeal and parotid gland Warthin's tumour: a case report.

Herein, a rare case of synchronous cystoadenolymphoma (Warthin's tumour) of the right parotid gland and the nasopharyngeal space is described. Although Warthin's tumour (WT) of the parotid gland is a common benign pathology, the occurrence of extra-parotid cystoadenolymphoma is rare. Extra-parotid WT have been mainly localised in the submandibular gland, periparotid region and occasionally in other sites, such as the oral cavity, hard palate and nasopharynx. The simultaneous occurrence of an intra-parotid and extra-parotid WT localisation, as in the case presented, is extremely uncommon.

Idiopathic sudden sensorineural hearing loss: cardiovascular risk factors do not influence hearing threshold recovery.

Previous studies have suggested that risk factors for ischaemic vascular disease, such as cigarette smoking, hypertension and hyperlipidaemia, can also be considered risk factors for the development of idiopathic sudden sensorineural hearing loss (ISSNHL). In this study, we have evaluated the hypothesis that these factors can influence hearing threshold recovery in patients affected by ISSNHL. A total of 141 subjects who suffered an episode of ISSNHL were included. All subjects were assessed with tonal audiometry, auditory brainstem responses and MRI to exclude retrocochlear pathology. Hearing tests were conducted at ISSNHL onset (t = 0) and after 30 days. Patients were divided into three classes according to the presence/absence of one or more cardiovascular risk factors including: history of smoking, total serum cholesterol/triglycerides, history of hypertension and diabetes mellitus. Values of hearing threshold recovery were estimated and comparisons were conducted across the three risk factor classes. 75% of patients affected by ISSNHL showed a threshold recovery. However, the threshold recovery was found to be class-independent (average recovery value of 18 dB HL per classes) and also independent of age and gender. Even if cardiovascular risk factors have been found to be involved in the pathogenesis of ISSNHL, the present study suggests that these factors do not have any significant influence on the threshold recovery in ISSNHL.

Analysis of Il-10 gene sequence in patients with sinonasal polyposis.

Sinonasal polyposis (SNP) is a chronic inflammatory disease of nasal and paranasal cavities. Human leukocyte antigen-G molecules (HLA-G) are non-classic HLA-I molecules with anti-inflammatory and tolerogenic properties. HLA-G production is mainly induced by interleukin (IL)-10. IL-10 is an anti-inflammatory cytokine that inhibits the production of proinflammatory cytokines and induces HLA-class II down-modulation. Recent studies suggest that HLA-G could play a role in SNP pathogenesis; in SNP patients physiological levels of IL-10 (produced by activated peripheral blood CD14+ monocytes) are not able to induce production of HLA-G. Different mechanisms could justify these findings: genomic or amino-acidic sequence alterations in IL-10 lower IL-10 receptor expression, lower IL-10 receptor affinity, or alterations of the intracellular signal transmission. This study analyzes nucleotidic sequence of IL-10 gene in SNP patients. Sequencing of IL-10 gene shows that the lack of HLA-G production by peripheral blood CD14+ monocytes is not related to alterations in IL-10 gene nucleotidic sequence.

Genetics of presbycusis and presbystasis.

Presbycusis and presbystasis represent relevant problems of aging, caused by the increase in life expectancy in developed countries. As such, it is advantageous to better understand the physiopathological mechanisms of these age-related inner ear diseases. The hypothesis that presbycusis and presbystasis have a genetic background was proposed some years ago. Several studies (in humans and animals) are available in the literature, and possible genes involved in the physiopathology of both diseases have been identified. The aim of this paper is to present an overview of the information available in the current medical literature on presbycusis and presbystasis.

Orofacial pain and Eagle Syndrome: cues from a clinical series.

AIM: Watt Eagle, firstly described the Elongated Stylohyoid Process Syndrome (ESPS), or Eagle Syndrome, in 1937. Since then, several authors have further studied this syndrome and some classifications have been proposed in relation to signs, symptoms and etiopathogenesis. Aim of this paper was to present the clinical features of a cohort of patients affected by Eagle syndrome that underwent surgical treatment. METHODS: Retrospective study. A cohort of ten patients that underwent surgical intervention for Eagle syndrome from January 2000 to December 2012 has been selected. For each subject, medical history, clinical features, treatment and follow-up after surgery were evaluated. RESULTS AND CONCLUSIONS: The surgical treatment resulted effective in 8 of 10 patients. Two patients are still complaining neck pain, although the discomfort has a lower grade and is pharmacologically controllable. Although rare, Eagle's Syndrome should be always considered in the differential diagnosis in patients with chronic orofacial pain refractory to conventional treatments.

Auricular involvement of a multifocal non-AIDS Kaposi's sarcoma: a case report.

Kaposi's sarcoma (KS) is a multicentric, malignant neoplastic vascular disease, mainly involving skin and mucosae, characterised by the proliferation of endothelial cells. The aetiology of KS still is unknown. Nonetheless, it has been reported that several epidemiological and environmental factors may play a role in its pathogenesis. Viral factors (i.e. human herpes virus 8, HHV-8) have also been claimed to play a role in the onset of KS. Four main clinical presentations of KS have been described: classic (sporadic), African (endemic), iatrogenic (immunosuppression-associated) and AIDS-associated (epidemic). The authors present a case of KS involving the external ear of a HIVnegative patient with a history of non-Hodgkin lymphoma and tuberculosis.

Quantitative enhancement of speech in noise through a wireless equipped hearing aid.

The development of new hearing aid technology can improve speech understanding in complex listening environments. The purpose of this study is to evaluate the benefit offered by the use of wireless technology applied to hearing aids. Participants were fit with binaural hearing instruments and underwent speech-in-noise tests. The signal was transmitted either by a speaker or wirelessly directly to the hearing aid. In our experience, hearing aids with wireless systems have an advantage in two particular conditions. The first can be achieved while listening wirelessly with microphones excluded (recommended when listening in noisy environments), while the second is in conditions of asymmetric listening; the wireless signal perception remains effective, but at the same time it allows the patient to receive environmental signals. Hearing aids equipped with wireless systems may be particularly useful when listening to people talking even in noisy environments and/ or receiving other sound sources such as TV and landline or cell phones.