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The course of COVID-19 infection may be complicated by a variety of neurological manifestations. Since the inner ear is vulnerable to viruses, sensorineural hearing loss (SNHL) has been reported to occur following the SARS-CoV-2 infection, often resulting in long-term morbidity and worsening the quality of life. The interest in how the virus affects the inner ear has gradually increased since the pandemic’s spread, but little is still known about the SNHL potentially caused by SARS-CoV-2. The aim of this paper is to evaluate the possible association between SNHL and COVID-19 infection, through a systematic literature review. Currently available data suggest that SARS-CoV-2 may hamper cochlear function; however, available reports are still limited. Large cohort and prospective studies are necessary to evaluate the long-term effects of this viral infection in the inner ear.

Background. Defects of mitochondrial DNA (mtDNA) involved in the function of the mitochondrial electron transport chain can result in primary mitochondrial diseases (PMDs). Various features can influence the phenotypes of different PMDs, with relevant consequences on clinical presentation, including the presence of hearing impairment. This paper aims to describe the hearing loss related to different PMDs, and when possible, their phenotype. Methods. A systematic review was performed according to PRISMA guidelines, searching Medline until December 2022. A total of 485 papers were identified, and based on specified criteria, 7 were included in this study. Results. A total of 759 patients affected by PMDs and hearing loss were included. The age of patients ranged from 2 days to 78 years old, and the male-to-female ratio was 1.3:1. The percentage of subjects affected by hearing loss was 40.8%, (310/759), and in most cases, hearing impairment was described as sensorineural, bilateral, symmetrical, and progressive, with different presentations depending on age and syndrome severity. Conclusions. PMDs are challenging conditions with different clinical phenotypes. Hearing loss, especially when bilateral and progressive, may represent a red flag; its association with other systemic disorders (particularly neuromuscular, ocular, and endocrine) should alert clinicians, and confirmation via genetic testing is mandatory nowadays.

Children with single-sided deafness (SSD) may experience delays in language and speech development. Reduced speech discrimination and poor sound localization abilities in young SSD patients may result in greater cognitive efforts required to focus and process auditory information, as well as increased listening-related fatigue. Consequently, these children can have a higher risk of academic failure and are often in need of extra help at school. Recently, cochlear implants (CIs) have been introduced as a rehabilitative option for these children, but their effectiveness is still a topic of debate. A literature review was performed according to PRISMA guidelines, searching the Medline database from inception to October 2023. The research identified nine papers that met the inclusion criteria. Data extracted from the selected studies included 311 children affected by SSD and cochlear implants. The reported audiological outcomes were further analyzed. Overall, a high level of satisfaction was described by parents of children with SSD and CI, and those who received a CI under the age of 3 presented better results. However, a proportion of patients did not use the device daily. Our review highlights the possible, and still controversial, role of CI for the hearing rehabilitation of children with unilateral deafness, underlining the need for further research in this field. To date, careful and comprehensive counseling with the child and the family is necessary before considering this option.

Background and aim: Several viruses have previously been reported to be responsible for congenital hearing loss; therefore, since the beginning of the SARS-CoV-2 infection pandemic, various reports have investigated a possible link. The aim of this review is to assess the possible link between maternal COVID-19 infection and congenital hearing loss. Methods: This systematic review was performed using PRISMA criteria, searching Medline and Embase databases from March 2020 to February 2023. A total of 924 candidate papers were identified; however, considering the specific selection criteria, only nine were selected for additional analysis. Results: The overall number of children born from mothers infected with COVID-19 during pregnancy identified through this review was 1687. The confirmed cases of hearing loss were 0.7% (12/1688); a description of its nature (sensorineural vs. conductive) is missing in the selected studies, and the follow-up period is variable across the analyzed papers. Surprisingly, a large proportion of false positives were recorded at the first stage of screening, which resulted normal at the re-test. Conclusions: Currently, a correlation between congenital hearing loss and SARS-CoV-2 infection cannot be definitively established. Further studies are desirable to provide additional evidence on this topic.

The facial nerve (FN) plays a pivotal role in human life; apart from its sensory and parasympathetic functions, it innervates the facial muscles, and it is therefore involved in non-verbal communication, allowing us to express emotions and reactions. Especially in the case of childhood onset, FN dysfunction can severely affect the quality of life. The aim of this review is to analyze the most recent literature, focusing on the acute onset of peripheral FN palsy among pediatric patients, discussing the different etiologies, prognoses, and management strategies. A total of 882 papers were initially identified, but only 7 met the selection criteria. Therefore, data on 974 children in total were pooled and analyzed. According to the findings of this review, FN palsy is idiopathic in most cases, while an infective etiology was identified as the second most common. The main pathogen agents identified were Borrelia Burgdorferi, especially in endemic areas, and Herpesviridae. Respiratory tract infections and/or ear infections were also described. Head trauma or direct injury of the FN accounted for 2% of all cases. The overall FN recovery rate is high, even though the etiology remains unknown for most patients. Therapeutic indications are still lacking, especially in the case of non-recovering FN palsy. In our opinion, large, prospective studies are necessary for improving our knowledge of this disorder and establishing evidence-based approaches.

Currently, the guidelines for audiological and vestibular follow-up in children with congenital cytomegalovirus (CMV) are not well-defined. The general recommendation is to evaluate hearing in all children with congenital CMV at the same intervals: once every 3–6 months up to 1 year of age, once every 6 months from 1 to 3 years of age, and once a year from 3 to 6 years of age. Additionally, there are no universally accepted protocols for the vestibular follow-up of children with congenital CMV, although video head impulse test (v-HIT) and cervical vestibular-evoked myogenic potentials (cVEMPs) are sometimes used. This narrative review critically evaluates existing audiological and vestibular follow-up approaches for children with congenital CMV, highlighting the need for personalized protocols. Tailoring follow-up schedules with different timing and methods based on risk factors, such as the trimester of maternal infection, CMV PCR results in amniotic fluid, and valganciclovir use, would indeed allow for more precise evaluations, timely interventions, and optimized resource allocation. This strategy would also alleviate the logistical and emotional burdens on families by ensuring that high-risk children receive more frequent and appropriate assessments and early interventions, while lower-risk children avoid unnecessary testing.

Background: Vertigo and dizziness are relatively infrequent in paediatric patients, but specific data on the prevalence of these disorders are limited and influenced by various factors, including the age of the examined population. These conditions often have a significant impact on patients’ and parents’ quality of life. The aim of this paper is to investigate the prevalence of different aetiologies of vertigo in the paediatric population through a systematic review. Methods: According to PRISMA guidelines, a systematic review of the literature was performed. Medline and Embase were searched from January 2011 through to 10 September 2021. The search yielded 1094 manuscripts, which were reduced to 7 upon the application of inclusion criteria. Results: A total of 2470 paediatric patients were evaluated by the selected papers. Vestibular Migraine was the most frequently diagnosed condition, occurring alone or in association with other diseases. Overall, audio-vestibular disorders represented the second cause of vertigo, and the prevalence appears to increase according to age growth. Over the years, even though we assisted in the amelioration of diagnostic rates, partially related to an improvement in diagnostic tools, the aetiology of vertigo remains still unclear in a variable percentage of patients. Conclusion: Vertigo in children, despite being an uncommon symptom, requires a multidisciplinary approach, often involving Paediatricians, Neurologists and Otorhinolaryngologists. A comprehensive evaluation of children suffering from vertigo is crucial for establishing a successful therapy and reducing parental worries.

Background: While gender differences of several diseases have been already described in the literature, studies in the area of hyperacusis are still scant. Despite the fact that hyperacusis is a condition that severely affects the patient’s quality of life, it is not well investigated; a comprehensive understanding of its features, eventually including gender differences, could be a valuable asset in developing clinical intervention strategies. Aim: To evaluate gender differences among subjects affected by hyperacusis. Methods: A literature search was conducted focused on adult patients presenting hyperacusis, using the MedLine bibliographic database. Relevant peer-reviewed studies, published in the last 20 years, were sought. A total of 259 papers have been identified, but only 4 met the inclusion criteria. The review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Results: The four selected papers included data from 604 patients; of these, 282 subjects resulted as affected by hyperacusis (125 females and 157 males). Questionnaires for analyzing factors affecting the attentional, social and emotional variance of hyperacusis (such as VAS, THI, TSCH, MASH) were administered to all included subjects. The data suggest that there are no hyperacusis gender-specific differences in the assessed population samples. Conclusions: The literature data suggest that males and females exhibit a similar level of hyperacusis. However, in light of the subjective nature of this condition, the eventual set up of further tests to assess hyperacusis features could be very helpful in the near future.

Background. Otosclerosis can occur during childhood, resulting in the early onset of conductive hearing loss. The approach to a child with otosclerosis can present some difficulties in terms of diagnosis and treatment, and the literature on juvenile otosclerosis (JO) is still relatively limited. Aim. To explore the current approaches to JO, in order to clear the management of this condition and evaluate the outcomes and the possible complications of surgical treatment. Methods. A systematic review was performed according to PRISMA guidelines, searching Medline and Embase from January 2002 through to 30 September 2022. A total of 759 papers were identified but based on specified criteria, nine were included in this study. Results. There were 94 children affected by JO and treated by stapes surgery. According to the available data, Male: Female ratio was 1:3–4, whilst the mean ages ranged from 10 to 16.3 years at the time of stapes surgery. After stapes surgery, the target of ABG < 10 dB was achieved in most of the patients. Overall, the 4 complications were reported (4/94= 4%): stenosis of the external ear canal, deterioration of hearing, anacusis with vertigo, tinnitus. Conclusions. The heterogeneity of the available studies does not allow us to draw straight conclusions on this topic, currently. More data about the natural history of the disease in children could help in approaching the treatment correctly, and possibly in drawing guidelines. Studies with a prolonged follow-up could be helpful for assisting clinicians and families in taking the most favorable decision about treatment.

White Sutton Syndrome is a rare autosomal dominant disorder resulting from a de novo mutation of Pogo Transposable Element Derived with Zinc Finger domain gene. The phenotype is characterized by a wide spectrum of cognitive dysfunction and developmental delays. Hearing loss is frequently mentioned as one of the symptoms of this rare disease, but details are usually scant. We report a case of a male child affected by White Sutton Syndrome and sensorineural hearing loss, with audiological findings of an auditory neuropathy spectrum disorder, a dysfunction of the auditory pathway with preserved cochlear outer hair cell function. Up to date, the present case is the first description of hearing loss due to an auditory neuropathy spectrum disorder in White Sutton Syndrome. A comprehensive audiological assessment is therefore mandatory in all White Sutton Syndrome patients in order to recognize a possible auditory neuropathy disorder and then avoid misdiagnosis, or erroneous clinical management.