Late-onset, progressive sensorineural hearing loss in the paediatric population: a systematic review.

PURPOSE: To review possible risk factors for permanent delayed-onset, progressive sensorineural hearing loss (SNHL) in the paediatric population to recommend follow-up protocols for early detection. METHODS: PRISMA-compliant systematic review was performed, including observational studies on the paediatric population up to 16 years old who have passed the newborn hearing screening programme (NHSP), investigating the development of late-onset, progressive SNHL. Electronic searches were performed through Medline, Embase, Cochrane, and Emcare. RESULTS: 37 studies were included. 21 showed an association between late-onset SNHL and congenital cytomegalovirus (cCMV) infection (age at hearing loss diagnosis 0.75 to 204 months, mean 45.6 ± 43.9), while 16 between late-onset SNHL and other congenital or perinatal factors, namely Neonatal Intensive Care Unit (NICU) stay, prematurity, neonatal respiratory failure, mechanical ventilation, extracorporeal membrane oxygenation (ECMO) support, hypocapnia, hypoxia, alkalosis, seizure activity, congenital diaphragmatic hernia (CDH), inner ear malformation, and gene mutations (age at hearing loss diagnosis 2.5 to 156 months, mean 38.7 ± 40.7). CONCLUSIONS: cCMV infection may cause late-onset SNHL, which can be missed on standard NHSP. There is, therefore, evidence to support universal screening programmes to enable detection in even asymptomatic neonates. Ongoing audiological follow-up for all children with cCMV is advisable, to enable timely treatment. In the paediatric population presenting conditions such as NICU stay > 5 days, prematurity ≤ 34 weeks gestation, severe neonatal respiratory failure, mechanical ventilation, ECMO support, and CDH surgery, an audiological follow-up from 3 months of age up to at least 3-4 years of age, and at least annually, should be recommended.

Basal cell adenoma and pleomorphic adenoma of the parotid gland: a single center experience.

BACKGROUND: Basal cell adenoma (BCA) and pleomorphic adenoma (PA) are among the most common benign neoplasms of the salivary glands. The aim of this study was to analyze and compare the diagnosis, treatment, and recurrence rate of these two different types of parotid benign tumors. METHODS: A retrospective analysis of all cases of parotid gland BCA and PA surgically treated between January 1, 1990, and December 31, 2019, was performed at our university. RESULTS: A total of 349 patients were enrolled in the present study, 311 of which (89.1%) were affected by PA, and 38 patients (10.9%) by BCA. The most frequently performed surgery was partial parotidectomy for both groups (85.9% in PA and 65.8% in BCA). Perioperative complications - often transient and of short duration - occurred within 48 hours of surgery and were observed in 30.6% of PA patients and in 18.4% of BCA patients; furthermore, recurrences were noticed in 19 PA patients (6.2%) and in 3 BCA patients (7.9%) (rates in range with the available literature data). CONCLUSIONS: To the best of our knowledge, this study is one of the largest single-center series in the literature comparing diagnosis, treatment, recurrence rate and clinical-pathological features of two different types of benign parotid gland tumors, BCA, and PA.

Hearing Loss and Blood Coagulation Disorders: A Review.

A relationship between microvascular disorders and sensorineural hearing loss (SNHL) has been widely proposed. The vascular hypothesis, theorized for the onset of sudden SNHL (SSNHL), is among the most acknowledged: a localized acute cochlear damage, of ischemic or haemorrhagic nature, could be considered a causative factor of SSNHL. The aim of this review is to assess (i) the effect on hearing in patients affected by blood coagulation disorders (prothrombotic or haemorrhagic) and (ii) the possible etiopathogenetic mechanisms of the related hearing loss. A PRISMA-compliant review was performed. Medline, Embase, and Cinahl databases were searched from inception to 31 January 2023, and a total of 14 studies have been included in the review. The available data suggest that it is possible to consider clotting disorders as a potential condition at risk for sensorineural hearing loss; in particular, coagulation tests and eventually the assessment of genetic and acquired prothrombotic factors should be recommended in patients with SSNHL. Also, an audiological evaluation should be recommended for patients with blood coagulation disorders presenting cochlear symptoms, especially in those suffering from clotting diseases.

Single centre analysis of perioperative complications in trans-oral robotic surgery for oropharyngeal carcinomas.

Trans Oral Robotic Surgery (TORS) is a modality in the management of oropharyngeal squamous cell carcinoma(OPSCC). This study was conducted to show the rates of peri-operative complications after TORS for OPSCC in our experience. Single centre retrospective analysis of consecutive OPSCC treated with TORS. The surgical complication severity was recorded according to Clavien-Dindo criteria (CDC). Eighty-seven OPSCC were operated with TORS. According to CDC, grade I, grade II and IIIb were registered in 8%, 4.6% and 11.5% of cases, respectively. The postoperative pain, registered with visual-analogue scale (VAS) score, was 8 ± 1.2 for the secondary healing wounds and 6.2 ± 1.5 for the flap reconstructions (p < 0.01). The impact on swallowing function was not significant between secondary healing and flap reconstructions(p = 0.96). Any major or life-threatening intraoperative complications have not been recorded. Only one patient had postoperative bleeding into the neck whilst 13.3% of patients had postoperative bleeding from the primary tumor. No total local or free flap failure were registered. The mean duration of tracheostomy use was 7.4 ± 2.6 days, and nasogastric tube 14.3 ± 6.9 days. Only one patient, who had also reconstruction with flap, experienced a postoperative severe dysphagia with severe aspiration, needing a permanent tracheostomy tube and percutaneous endoscopic gastrostomy feeding. TORS for OPSCC showed less morbidity, lower risk of severe complication and mortality. Thus, this treatment modality could be offered as first line treatment in selected cases.

Cerebral Outflow Discrepancies in Recurrent Benign Paroxysmal Positional Vertigo: Focus on Ultrasonographic Examination.

This prospective pilot study aimed to evaluate whether cerebral inflow and outflow abnormalities assessed by ultrasonographic examination could be associated with recurrent benign paroxysmal positional vertigo (BPPV). Twenty-four patients with recurrent BPPV, affected by at least two episodes, and diagnosed according to American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) criteria, evaluated at our University Hospital, between 1 February 2020 and 30 November 2021, have been included. At the ultrasonographic examination, 22 of 24 patients (92%) reported one or more alterations of the extracranial venous circulation, among those considered for the diagnosis of chronic cerebrospinal venous insufficiency (CCSVI), although none of the studied patients were found to have alterations in the arterial circulation. The present study confirms the presence of alterations of the extracranial venous circulation in recurrent BPPV; these anomalies (such as stenosis, blockages or regurgitation of flow, or abnormal valves, as per the CCSVI) could cause a disruption in the venous inner ear drainage, hampering the inner ear microcirculation and then possibly causing recurrent otolith detachment.

Role of 18F-FDG PET/CT in evaluating lymph node status in patients with head and neck squamous cell carcinoma.

Objective: The presence of cervical lymph node metastases (CLNM) at diagnosis is one of the most relevant negative prognostic factors in patients with head and neck squamous cell carcinoma (HNSCC). The aim of this study was to analyse 2-deoxy-2[18F]fluoro-D-glucose (FDG) PET/CT findings for the identification of primary tumours and CLNM in a sample of patients affected by HNSCC. Moreover, a maximum standardised uptake value (SUVmax) threshold for the detection of CLNM was estimated. Clinical variables (i.e. smoking and alcohol habits), and tumour features (i.e. EBV and HPV positivity) were also evaluated in relation to FDG PET/CT findings. Methods: We retrospectively analysed patients who underwent FDG PET/CT for HNSCC staging between 2015-2020 at the University Hospital of Ferrara. All patients had cytological or histological confirmation of suspected cervical lymph nodes. Results: In total, 65 patients were enrolled (53 males, 12 females, median age 65.7 years). CLNM of patients with smoking habit had significantly higher SUVmax values than those of patients with previous smoking habit and non-smokers (p = 0.04). p16 positive HNSCC demonstrated a trend for higher SUVmax values on CLNM, in comparison to p16 negative tumours (p = 0.089). ROC curve analysis identified 5.8 as the best cut-off value of SUVmax for the detection of CLNM (AUC = 0.62, sensitivity 71.4% and specificity 72.7%). Conclusions: FDG PET/CT is a useful tool to evaluate CLNM in patients with HNSCC, particularly in those with smoking habit and p16 positive disease. A SUVmax cut-off of 5.8, combined with the use of conventional radiological investigations, may represent a useful tool in the identification of CLNM.

Dizziness, psychological disorders and cognitive decline.

INTRODUCTION: Dizziness is a common disorder, particularly among the elderly population. Aim of this paper was to revise the current concepts surrounding the relationship between dizziness, psychological disorders and cognitive decline. EVIDENCE ACQUISITION: This is a PRISMA-compliant systematic review, including observational studies in people with dizziness. Database inception, Medline/Cochrane/Embase/Web of Science/Scopus/NHS evidence were searched until October 30, 2019. EVIDENCE SYNTHESIS: Overall 22 studies, and 65,730 participants were included. Eleven studies were cross-sectional, 7 cross-sectional controlled, 2 prospective case-control, 1 retrospective case series, and 1 cohort study. The persistence of vestibular impairment (for 6 months or more) was correlated to the presence of psychological disorders affecting patient's Quality of Life and causing social anxiety, particularly in some conditions such as Ménière's disease. Interestingly, vestibular loss has been also correlated to cognitive impairment, with certain vestibular dysfunctions reported to be more prevalent in cognitive impaired individuals. CONCLUSIONS: The current literature suggests that there is an association between vestibular function, psychological disorders and cognitive functions. The findings from this review could be useful in informing on the need for a multidimensional diagnostic and rehabilitative programs for patients with dizziness. More studies could explore the role of counseling or behavioral therapy with an aim to reduce the perceived dizziness-related disability.

The Effects of Barbed Repositioning Pharyngoplasty in Positional and Non-Positional OSA Patients: A Retrospective Analysis.

PURPOSE: The aim of our retrospective study is evaluating the effectiveness of barbed repositioning pharyngoplasty (BRP) in a consecutive cohort of patients and assessing its impact on positional indexes in order to potentially identify specific obstructive sleep apnea (OSA) phenotypes for patients who might benefit more significantly from this intervention. METHODS: A single-center retrospective study with baseline and follow-up type III sleep tests evaluating the Apnea Hypopnea Index (AHI), supine AHI, non-supine AHI, oxygen desaturation index (ODI), mean SaO2, percentage of time spent at SaO2 below 90% (CT90), and lowest oxygen saturation (LOS) were performed. The patients were then divided into groups according to Sher's criteria and Amsterdam Positional OSA Classification (APOC). Parametric and non-parametric tests and univariate and multivariate analyses were conducted. RESULTS: The study finally included 47 patients. The statistical analysis showed significant improvement in AHI, supine AHI, non-supine AHI, and ODI after surgery. The linear regression showed that high values of baseline AHI, AHI supine, and AHI non supine predict more significant postoperative reductions in AHI, AHI supine, and AHI non supine, respectively. Therapeutic success was achieved in 22 patients out of 47. The logistic regression did not find any independent risk factors for success. The most significant reduction in AHI, supine AHI, and non-supine AHI was observed in the APOC 3 group while the APOC 1 patients experience a substantially lower improvement. CONCLUSIONS: BRP appears to be an effective surgical procedure for the treatment of OSA. The non-positional patients might benefit more from BRP in comparison with positional patients. Moreover, OSA severity should not be considered an absolute contra-indication for this surgical procedure.

Tapia's Syndrome: keep it in mind!

INTRODUCTION: The aim of this study was to revise the etiologic features about Tapia's Syndrome (TS), a condition to particularly consider in the era of the COVID-19 pandemic. EVIDENCE ACQUISITION: A systematic review was performed according to the PRISMA criteria. The Medline and Embase databases were searched from January 1, 1990, to December 31, 2020. Initially the search yielded 399 manuscripts, which were reduced to 50, upon the application of inclusion criteria. EVIDENCE SYNTHESIS: A total of 65 patients were included in the present review. Mean age was 44±17.5 (DS) years (15-95); M:F ratio was 2.3:1. TS involved mainly the left side (3:2) and was rarely bilateral. Only 2 TS reported cases were due to central causes. Peripheral causes were mainly due to postintubation edema (77%), extrinsic compression (15%), vascular disease (3%), other/not defined (5%). CONCLUSIONS: TS is a rare syndrome that has been related to a combined cranial nerve palsy; while TS due to central causes is very rare, it is mainly related to peripheral causes. A particular attention to TS should be given during the SARS-CoV-2 pandemic, either since the correlation between Tapia's syndrome, airway management and anesthetic procedures, since the possible implication of the viral infection itself.

Transoral robotic surgery for oropharyngeal cancer in the era of chemoradiation therapy.

Over time, the primary treatment modality of oropharyngeal squamous cell cancers (OPSCC) is intensity-modulated radiation therapy (IMRT), eventually associated with chemotherapy (CHT). This treatment modality is preferred for reduced impact on the quality of life rather than aggressive open surgical approaches. The advent of TransOral Robotic Surgery (TORS) has rediscovered the role of surgery in the treatment of OPSCC. Although the primary chemoradiation therapy (CRT) and transoral robotic surgery (TORS) are competing for similar oncologic results, CRT may result in significant functional complaints such as severe dysphagia and feeding tube dependence and, on the other hand, TORS might result in surgical defects depending on the size of the resection and anatomic location. Nevertheless, TORS may intercept the locoregionally advanced population through pathologic downstaging as well as the potential for improvement in oncologic outcomes. The available reconstructive options allow an expanding role of this minimally invasive surgery, even in locally advanced tumours. Finally, the surgical management of OPSCC with TORS identifies areas of opportunity in shortening the overall treatment package time compared to traditional surgery.

Vestibular and postural impairment in congenital Cytomegalovirus infection.

OBJECTIVES: To describe the vestibular and postural impairment related to the congenital Cytomegalovirus infection (cCMV), including the inner ear damages CMV-induced. METHODS: A PRISMA systematic review was performed, with the PubMed, Embase, and Cinahl databases searched from inception through to March 2021; after the application of inclusion and exclusion criteria a total of 12 papers were included in this review. RESULTS: Vestibular and postural disorders have been reported in cCMV children, with a high variability of clinical manifestation. Presently, the available reported data on vestibular and postural impairment in cCMV children differ in terms of the sample size and the features of the studied populations. CONCLUSION: At present, the vestibular and postural impairment in cCMV cases is underestimated; a complete vestibular assessment, a follow-up and management of all children affected by cCMV or, at least, of all cCMV patients showing sensorineural hearing loss, is recommended.

Laryngeal Taping as a Supportive Tool to Relieve Phonasthenia in Singers: A Preliminary Report.

AIM: This report aims to perform a preliminary evaluation of the role of taping in the improvement of phonasthenia among professional voice users. The larynx is a vital organ but also a work instrument for many people. Prevention of erroneous use of the vocal mechanism is essential for an optimal voice performance. METHODS: Nineteen singers complaining voice fatigue underwent taping application in peri-laryngeal areas of the anterior neck for 10 days. Taping effects were estimated by the evaluation of voice analysis parameters (jitter, shimmer, noise to harmonic ratio and singing power ratio) performed in M1 and M2 mechanisms with PRAAT software before and after treatment. In addition, a subjective assessment of phonasthenia was performed using a self-administrated questionnaire. RESULTS: Statistically significant reduction of Jitter% in M1 (P = 0.021) was reported at the end of treatment; also, an improvement of NHR in M2 was found immediately after the application (P = 0.012) and after 10 days (P = 0.002). These outcomes suggest possible beneficial effects of laryngeal taping on relieving voice fatigue, and therefore improving voice quality. Self-assessment results are consistent with this finding. CONCLUSION: Since the application of muscular taping in phoniatrics is still in its early stage, further studies and in particular a standardized protocol of application, could facilitate the spread of this technique, also offering help in comparing results.

SARS-CoV-2 (COVID-19) and audio-vestibular disorders.

To describe the audio-vestibular disorders related to the newly SARS-CoV-2 infection, including the possible ototoxicity side-effects related to the use of drugs included in the SARS-CoV-2 treatment protocols. A systematic review was performed according to the PRISMA protocol. The Medline and Embase databases were searched from March 1, 2020 to April 9, 2021. Initially the search yielded 400 manuscripts, which were reduced to 15, upon the application of inclusion criteria. Sensorineural hearing loss (SNHL) is the most frequent audio-vestibular symptom described, occurring alone or in association with tinnitus and vertigo. The etiopathogenesis of the inner ear disorders related to COVID-19 infection is still poorly understood. The number of reports of COVID-19 infections associated to audio-vestibular disorders is increasing; even if the quality of the studies available is often insufficient, audio-vestibular disorders should be considered as possible manifestations to be included among the symptoms of this infection.

The risk of recurrence in surgically treated head and neck squamous cell carcinomas: a conditional probability approach.

BACKGROUND: Over 50% of patients with head-and-neck squamous cell carcinoma (HNSCC) experience locoregional recurrence, which is associated with poor outcome. In the course of follow-up for patients surviving primary surgery for HNSCC, one might ask: What is the probability of recurrence in one year considering that the cancer has not yet recurred to date? MATERIALS AND METHODS: To answer this question, 979 patients surgically treated for HNSCC (i.e. cancer of the oral cavity, oropharynx, hypopharynx or larynx) between March 2004 and June 2018 were enrolled in a multicenter retrospective cohort study, followed up for death and recurrence over a 5 year period. The conditional probability of recurrence in 12 months - i.e. the probability of recurrence in the next 12 months given that, to date, the patient has not recurred - was derived from the cumulative incidence function (Aalen-Johansen method). RESULTS: Overall, the probability of recurrence was the highest during the first (17.3%) and the second years (9.6%) after surgery, declining thereafter to less than 5.0% a year thereafter. The probability of recurrence was significantly higher for stage III-IV HNSCCs than for stage I-II HNSCCs in the first year after surgery (20.4% versus 10.0%; p < 0.01), but not thereafter. This difference was most pronounced for oral cavity cancers. No significant differences were observed across different tumor sites. CONCLUSION: This dynamic evaluation of recurrence risk in patients surgically treated for HNSCC provides helpful and clinically meaningful information, which can be useful to patients in planning their future life, and to clinicians in tailoring post-treatment surveillance according to a more personalized risk stratification.

Non-Syndromic Sensorineural Prelingual and Postlingual Hearing Loss due to COL11A1 Gene Mutation.

This paper aims to present a third world case of Non-Syndromic sensorineural hearing loss (NSHL) due to a novel missense variant in COL11A1 gene, defined as DFNA37 non-syndromic hearing loss. The clinical features of a 6-year-old boy affected by a bilateral moderate to severe down-sloping sensorineural hearing loss are presented, as well as the genetic analysis, the latter identifying a heterozygous missense variation in the COL11A1 gene. In addition, in families with autosomal dominant transmission, COL11A1 gene should be considered in the genetic workup of the NSHL with prelingual onset.

The Pathogenesis of secondary forms of Autoimmune Inner Ear Disease (AIED): advancing beyond the audiogram data.

INTRODUCTION: Autoimmune Inner Ear Disease (AIED) can be of a primary or secondary type. To date, a clear pathogenesis of the disease is still not available. Focusing on the secondary forms of AIED, the aim of this review is to (i) assess and describe the hearing involvement in patients affected by autoimmune diseases, (ii) describe the possible association between clinical features (among serological/laboratory data and disease activity/duration) and hearing impairment, (iii) show evidence connecting the AIED types with various etiopathogenetic mechanisms. AREAS COVERED: A PRISMA-compliant systematic review was performed. Medline, Cochrane, Embase, and Cinahl were searched from 1 January 2015 through to 5 August 2020. Overall, 16 studies (involving 1043 participants) were included in the review. The data in the literature suggested that bilateral mild-to-moderate sensorineural hearing loss is a commonly reported clinical symptom of AIED. EXPERT OPINION: Patients with systemic autoimmune disorders present a cochlear injury which might be associated with the humoral and/or cellular immune response against the inner ear. To date, AIED pathogenesis remains an open issue, due to the rarity of these clinical entities and due to the difficulties in investigating the inner ear immunology, considering the inner ear inaccessibility for tissue sampling.

Genetic Polymorphisms in Sudden Sensorineural Hearing Loss: An Update.

OBJECTIVE: To investigate the association between genetic polymorphisms and sudden sensorineural hearing loss (SSNHL). Most of the SSNHL cases still remain idiopathic, and several etiopathogenetic hypotheses, including a genetic predisposition, have been proposed. METHODS: A literature review was conducted using different databases: Medline/PubMed, EMBASE, and CINAHL, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. All databases have been searched from May 2016 to April 2020. RESULTS: Genetic susceptibility could represent a key element in the pathogenesis of SSNHL. A number of genetic polymorphisms related to (1) inner ear microvascular disease and endothelial dysfunction and (2) to inner ear oxidative stress and inflammation have been addressed in the current literature. CONCLUSIONS: The potential identification of a genetic profile related to SSNHL could provide a more accurate prognostic evidence of idiopathic SSNHL (ISSNHL), offering to the patients not only early-prevention strategies but eventually information on various inheritance modalities.

The Influence of Hearing Aids on Balance Control: A Systematic Review.

AIM: To assess the current opinion on the effects of hearing loss treatment by hearing aids (HAs) and the benefits of HA use on imbalance. METHODS: PRISMA-compliant systematic review was done, including observational studies in patients affected by mild to severe sensorineural hearing loss with HAs, investigating the benefits of HAs on balance. Electronic searches were performed through Medline, Cochrane, Embase, Web of Science, and Scopus. RESULTS: A total of 200 patients in 8 studies were included in this systematic review. Four studies were cross-sectional, 3 cross-sectional controlled and 1 prospective nonrandomized study. Static and dynamic balance in the aided condition improved in patients assessed using clinical investigations including Romberg test and Functional Ambulation Performance/mini-BESTest, respectively. Variable outcomes were found measuring static and dynamic balance during the aided condition with objective tests (computerized posturography, Mobility Lab device). Improved quality of life outcomes and self-confidence were noted, while subjective measurements of balance had conflicting results. CONCLUSION: Although an improvement in balance in patients with HAs has been shown in certain conditions, the overall benefit is still unclear and it is only possible to speculate that HAs may also improve static, dynamic, or subjective perception of balance function in adults affected by hearing loss.

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.