Audiological Phenotypes of Connexin Gene Mutation Patterns: A Glance at Different GJB2/GJB6 Gene Mutation Profiles.

GJB2 mutations are the most common cause of autosomal-recessive non-syndromic sensorineural hearing loss (SNHL). The available evidence shows large phenotypic variability across different genotypes and allelic variants. The aim of this study was to investigate the clinical and audiological features of a cohort of subjects with different GJB2/GJB6 gene mutation profiles from a tertiary referral center in Northeastern Italy. We considered 57 patients with GJB2/GJB6 mutations presenting with congenital, non-syndromic SNHL, mainly coming from the Veneto region (Italy). The samples were screened for mutations in exons 1 and 2 of the GJB2 gene and for the GJB6 gene deletion del (GJB6-D13S1830). Free-field and air-conduction frequency-specific thresholds and the pure-tone average (PTA) were considered in the statistical analysis. Five patients (8.87%) had connexin gene mutations in simple heterozygosis, 15 (26.31%) in compound heterozygosis, 34 (59.64%) in homozygosis, and 3 (5.26%) with digenic patterns. The frequency-specific air-conduction thresholds showed significantly different mean values across the different genotypes (Roy's largest-root test, p = 0.0473). Despite the evidence already available on genetic SNHL, many new insights are to be expected. Further large-scale prospective studies including different populations are necessary to confirm these preliminary findings about the clinical and audiological features of patients with different GJB2/GJB6 gene mutation patterns.

Polyphenols in Inner Ear Neurobiology, Health and Disease: From Bench to Clinics.

There is substantial experimental and clinical interest in providing effective ways to both prevent and slow the onset of hearing loss. Auditory hair cells, which occur along the basilar membrane of the cochlea, often lose functionality due to age-related biological alterations, as well as from exposure to high decibel sounds affecting a diminished/damaged auditory sensitivity. Hearing loss is also seen to take place due to neuronal degeneration before or following hair cell destruction/loss. A strategy is necessary to protect hair cells and XIII cranial/auditory nerve cells prior to injury and throughout aging. Within this context, it was proposed that cochlea neural stem cells may be protected from such aging and environmental/noise insults via the ingestion of protective dietary supplements. Of particular importance is that these studies typically display a hormetic-like biphasic dose-response pattern that prevents the occurrence of auditory cell damage induced by various model chemical toxins, such as cisplatin. Likewise, the hormetic dose-response also enhances the occurrence of cochlear neural cell viability, proliferation, and differentiation. These findings are particularly important since they confirmed a strong dose dependency of the significant beneficial effects (which is biphasic), whilst having a low-dose beneficial response, whereas extensive exposures may become ineffective and/or potentially harmful. According to hormesis, phytochemicals including polyphenols exhibit biphasic dose-response effects activating low-dose antioxidant signaling pathways, resulting in the upregulation of vitagenes, a group of genes involved in preserving cellular homeostasis during stressful conditions. Modulation of the vitagene network through polyphenols increases cellular resilience mechanisms, thus impacting neurological disorder pathophysiology. Here, we aimed to explore polyphenols targeting the NF-E2-related factor 2 (Nrf2) pathway to neuroprotective and therapeutic strategies that can potentially reduce oxidative stress and inflammation, thus preventing auditory hair cell and XIII cranial/auditory nerve cell degeneration. Furthermore, we explored techniques to enhance their bioavailability and efficacy.

Cochlear Implantation in Children with Additional Disabilities: A Systematic Review.

This study examines the last 10 years of medical literature on the benefits of cochlear implantation in children who are deaf or hard of hearing (DHH) with additional disabilities. The most recent literature concerning cochlear implants (CIs) in DHH children with additional disabilities was systematically explored through PubMed, Embase, Scopus, PsycINFO, and Web of Science from January 2012 to July 2023. Our two-stage search strategy selected a total of 61 articles concerning CI implantation in children with several forms of additional disabilities: autism spectrum disorder, cerebral palsy, visual impairment, motor disorders, developmental delay, genetic syndromes, and intellectual disability. Overall, many children with additional disabilities benefit from CIs by acquiring greater environmental sound awareness. This, in turn, improves non-verbal communication and adaptive skills, with greater possibilities to relate to others and to be connected with the environment. Instead, despite some improvement, expressive language tends to develop more slowly and to a lesser extent compared to children affected by hearing loss only. Further studies are needed to better appreciate the specificities of each single disability and to personalize interventions, not restricting the analysis to auditory and language skills, but rather applying or developing cross-culturally validated instruments able to reliably assess the developmental trajectory and the quality of life of DHH children with additional disabilities before and after CI.

The Audiological Follow-Up of Children with Symptomatic Congenital Cytomegalovirus Infection: An Experience in Two Italian Centers.

BACKGROUND: Congenital cytomegalovirus (cCMV) infection is the leading cause of non-hereditary sensorineural hearing loss in children. While about 10% of children reportedly display symptoms at birth, 85-90% of cCMV infection cases are asymptomatic. However, 10-15% of these asymptomatic infants may later develop hearing, visual, or neurodevelopmental impairments. This study aimed to evaluate the impact of cCMV infection on newborns' hearing function with a particular emphasis on progressive and late-onset cases. METHODS: This study is a retrospective chart analysis with longitudinal character and was conducted in two Italian centers: Center 1 (from 1 November 2007 to 31 December 2021) and Center 2 (from 1 January 2012 to 31 December 2021). Data collected included newborn hearing screening results, characterization of hearing loss (unilateral/bilateral, degree of impairment), and audiological follow-up. RESULTS: The cohort consisted of 103 children (42% males, 58% females). In total, 28 children presented with hearing impairment; 71.4% (20 out of 28) of the cases of hearing loss were severe/profound, with 35.7% of the cases due to unilateral hearing loss. Out of twenty-eight, six experienced progression of hearing loss and four had late-onset hearing loss. CONCLUSIONS: In the absence of universal cCMV screening, hearing screening at birth for cCMV remains a critical factor for early diagnosis. A significant percentage of children affected by cCMV with normal audiological evaluations at birth is easily lost to follow-up. Close collaboration between neonatologists, pediatricians, and audiological services is fundamental to ensure timely diagnosis and treatment of cCMV-related hearing loss.

Italian validation of the Hearing Handicap Inventory for Elderly - Screening version (HHIE-S-It).

Objective: Validate the Italian version of the Hearing Handicap Inventory for Elderly - Screening version (HHIE-S-It). Methods: After translation, psychometric properties and attributes were analysed by administering the HHIE-S-It to 167 elderly outpatients together with the Psychological General Well-Being Index (PGWBI). Results: The Cronbach's α coefficient was 0.908 for the total score, and 0.832 and 0.816 for its two subscales. Significant test-retest reliability was observed (p < 0.001). Moderate to high correlations were found between HHIE-S-It and pure tone average in the better ear (p < 0.001). The ANOVA test confirmed the significant difference in HHIE-S-It scores across groups according to the degree of hearing loss (p < 0.001). Only very low and low significant correlations were observed between HHIE-S-It and PGWBI. The criterion HHIE-S-It > 11 was observed as the best cut-off with highest sensitivity (86.4%), specificity (72.4%), positive predictive value (52.8%), negative predictive value (93.7%) and likelihood ratios (3.12 and 0.19). Conclusions: Since the HHIE-S-It presented acceptable psychometric properties, its adoption is justified for both clinical and research purposes. Acceptable diagnostic attributes allow its use as a screening tool for age-related hearing loss.

The Effect of Alpha-Lipoic Acid in the Treatment of Chronic Subjective Tinnitus through the Tinnitus Handicap Inventory Scores.

BACKGROUND AND OBJECTIVES: Tinnitus affects millions of adults. Many therapies, including complementary and alternative medicine and tinnitus retraining therapies, have been trialed, but an effective option, particularly for chronic subjective tinnitus (CTS), is still lacking. MATERIALS AND METHODS: This study investigated the effects of alpha-lipoic acid (600 mg. per day for two months) on two groups of patients using a questionnaire. One group (A) was affected by tinnitus associated with likely cochlear dysfunction and metabolic syndrome, and the other (B) was composed of subjects with acoustic nerve lesions. All the patients were asked to complete the Italian version of the tinnitus handicap inventory (THI) to determine the overall degree of perceived annoyance at the beginning and end of therapy. Pure tone averages for speech frequencies and for high frequencies were computed, and psychoacoustic pitch and loudness matches were determined for each subject before and after treatment. RESULTS: The pure tone audiometry, pitch, loudness, and THI scores of both groups were reported. In group A, statistically significant differences were observed for the "functional" and "emotional" subscales. The total score of THI and the loudness of tinnitus were also significantly reduced. No statistically significant differences were observed in group B. CONCLUSIONS: These findings suggest a possible contribution of the antioxidant effect to the organ of Corti in subjects with metabolic syndrome and CST.

Why Are Causal and Temporal Connectives Difficult to Understand? A Comparison Between Italian Hearing Good and Poor Comprehenders and Deaf Poor Comprehenders.

PURPOSE: Both hearing poor comprehenders (PCs) and deaf or hard-of-hearing (DHH) PCs have problems in understanding causal and temporal coherence relations signaled by connectives. The study examined whether hearing and DHH PCs' problems with connective understanding are similar and mainly related to their limited vocabulary, including knowledge of connective words, or to their poor reading comprehension abilities more generally. METHOD: Three groups of 7- to 10-year-old readers, matched on grade level (hearing PCs, DHH PCs, and hearing good comprehenders [GCs]) performed a reading comprehension task, a vocabulary task, and causal and temporal connective understanding tasks. Hearing and DHH PCs were also matched on reading comprehension and decoding abilities. RESULTS: The DHH PCs performed significantly worse than both the hearing GCs and PCs in temporal and causal connective understanding. Significant differences between hearing PCs and GCs were found only in causal connective understanding. DHH readers' difficulties in causal connective understanding were significantly associated with poorer vocabulary knowledge. In contrast, vocabulary knowledge did not uniquely contribute to hearing PCs' difficulties with causal connective understanding, once their reading comprehension skills were controlled for. CONCLUSIONS: The results suggest that despite a similar reading profile, DHH PCs' difficulties with causal connective understanding are more closely related to their vocabulary delay, whereas hearing PCs' difficulties are more strongly influenced by their poor text integration processes (as indexed by their reading comprehension skills). Neither vocabulary knowledge nor reading comprehension skills contributed to the explanation of DHH readers and hearing PCs' temporal connective understanding.

Vertebrobasilar and Basilar Dolichoectasia Causing Audio-Vestibular Manifestations: A Case Series with a Brief Literature Review.

Audio-vestibular symptoms can arise from vertebrobasilar dolichoectasia (VBD) and basilar dolichoectasia (BD). Given the dearth of available information, herein we reported our experience with different audio-vestibular disorders (AVDs) observed in a case series of VBD patients. Furthermore, a literature review analyzed the possible relationships between epidemiological, clinical, and neuroradiological findings and audiological prognosis. The electronic archive of our audiological tertiary referral center was screened. All identified patients had a diagnosis of VBD/BD according to Smoker's criteria and a comprehensive audiological evaluation. PubMed and Scopus databases were searched for inherent papers published from 1 January 2000 to 1 March 2023. Three subjects were found; all of them had high blood pressure, and only the patient with high-grade VBD showed progressive sensorineural hearing loss (SNHL). Seven original studies were retrieved from the literature, overall including 90 cases. AVDs were more common in males and present in late adulthood (mean age 65 years, range 37-71), with symptoms including progressive and sudden SNHL, tinnitus, and vertigo. Diagnosis was made using different audiological and vestibular tests and cerebral MRI. Management was hearing aid fitting and long-term follow-up, with only one case of microvascular decompression surgery. The mechanism by which VBD and BD can cause AVD is debated, with the main hypothesis being VIII cranial nerve compression and vascular impairment. Our reported cases suggested the possibility of central auditory dysfunction of retro-cochlear origin due to VBD, followed by rapidly progressing SNHL and/or unnoticed sudden SNHL. More research is needed to better understand this audiological entity and achieve an evidence-based effective treatment.

Auditory Screening in Newborns after Maternal SARS-CoV-2 Infection: An Overview.

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 Role of Primary Mitochondrial Disorders in Hearing Impairment: An Overview.

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.

Monitoring of Auditory Function in Newborns of Women Infected by SARS-CoV-2 during Pregnancy.

BACKGROUND: Gestational SARS-CoV-2 infection can impact maternal and neonatal health. The virus has also been reported to cause newborn sensorineural hearing loss, but its consequences for the auditory system are not fully understood. OBJECTIVE: The aim of this study was to evaluate the impact of maternal SARS-CoV-2 infection during pregnancy on newborn' hearing function during the first year of life. METHODS: An observational study was conducted from 1 November 2020 to 30 November 2021 at University Modena Hospital. All newborns whose mother had been infected by SARS-CoV-2 during pregnancy were enrolled and underwent audiological evaluation at birth and at 1 year of age. RESULTS: A total of 119 neonates were born from mothers infected by SARS-CoV-2 during pregnancy. At birth, five newborns (4.2%) presented an increased threshold of ABR (Auditory Brainstem Evoked Response), but the results were confirmed only in 1.6% of cases, when repeated 1 month later, while the ABR thresholds in all other children returned to normal limits. At the 1-year follow-up, no cases of moderate or severe hearing loss were observed, while concomitant disorders of the middle ear were frequently observed. CONCLUSIONS: Maternal SARS-CoV-2 infection, regardless of the trimester in which it was contracted, appears not to induce moderate or severe hearing loss in infants. It is important to clarify the possible effect of the virus on late-onset hearing loss and future research is needed.

Development of a New Self-Assessment Tool for Laryngopharyngeal Reflux Screening in Singers (SVHI-12-LPR).

INTRODUCTION: The present study aimed to develop a new tool for the evaluation of singers with self-reported symptoms suggestive of laryngopharyngeal reflux (LPR) (the SVHI-12-LPR), by correlating RSI with SVHI in a population sample of 163 subjects (both professional and amateur singers), evaluated also by videolaryngostroboscopy. This study was a cross-sectional, double-observational study. METHODS: RSI and SVHI were administered to 159 singers (amateurs, singing students, and professional singers). All subjects underwent videolaryngostroboscopy to objectively identify four subgroups: normal subjects (41.5%), subjects with organic lesions occupying the glottic space (17.6%), subjects with functional dysphonia (18.2%), and subjects presenting solely signs suggestive of LPR (22.6%). Using the validated RSI threshold, 33.9% of participants presented an RSI total score >13, suggestive of LPR. RESULTS: Subjects with a suspected diagnosis of LPR at videolaryngostroboscopy presented a mean RSI significantly higher than other subgroups (p < 0.001). Moreover, the SVHI-36 score did not statistically differ between pathological subgroups. A significant positive relationship was observed between RSI and SVHI total score (Spearman's rank correlation coefficient [ρ] = 0.474, p < 0.001). 12 SVHI items (items 1, 2, 4, 5, 6, 7, 12, 20, 24, 25, 26, 30) showed a significant association with RSI pathology classification. Statistical analysis demonstrated for the 12 selected items (SVHI-12-LPR) acceptable specificity (0.691) and sensibility (0.833) for the suspected diagnosis of LPR with a cut-off of 15. CONCLUSIONS: From the SVHI-36, 12 items were extracted that correlated with the specific impact that LPR has on the singer's voice (SVHI-12-LPR), as evaluated by RSI and videolaryngostroboscopy. Such questionnaire represents a new tool that could be applied to singers with symptoms suggestive of LPR to select which patients would benefit from a further phoniatric and videolaryngostroboscopic evaluation.

Physical Therapy for Benign Positional Vertigo of Posterior Canal: The Role of Alternated Epley and Semont Maneuvers.

OBJECTIVE: To investigate if alternated Epley (EP) and Semont (ST) maneuvers could be more effective than repetition of the same in benign paroxysmal positional vertigo of posterior semicircular canal (pBPPV). DESIGN: We retrospectively reviewed the outcome of pBPPV patients treated with a second maneuver for the persistence of positional nystagmus. STUDY SAMPLE: Forty-seven patients underwent 2 STs, 64 with 2 EPs, and 71 EPs followed by ST. Videonystagmography and Dizziness Handicap Inventory (DHI) questionnaire were performed. RESULTS: Absence of positional nystagmus was achieved after 2 maneuvers in 136 patients with pBPPV: 65.9% in the ST group, 70.3% in the EP group, and 84.5% in the EP-ST group. Alternated EP and ST were significantly more effective than repeated ST (P = .03), while we found no significant difference when compared with repeated EP (P = .07). At 1-month follow-up, 12 patients showed persistent positional nystagmus without difference between groups. After 1 month, the 46 patients with negative outcome had significantly higher DHI values (P = .01) than other 136 patients with pBPPV. CONCLUSIONS: Alternated EP and ST seemed more effective than repeating the same maneuver in treating pBPPV, and this should be confirmed in prospective clinical studies. Resolution of nystagmus after maneuvers was fundamental to reduce handicap deriving from dizziness as reported in DHI.

Psychological Profile and Social Behaviors of Patients with Hyperacusis.

Increased noise sensitivity refers to the abnormal subjective response to external sounds, with a prevalence of between 8% and 15.2% in the adult population as suggested by epidemiological studies. The basic neural mechanism of hyperacusis still remains obscure, so therapies for this often-devastating symptom remain elusive. The aim of this study was to assess psychological profiles in patients with presbycusis without tinnitus in a perspective case-control design. All subjects were initially submitted to audiological evaluation (tympanometry, recordings of the acoustic reflex thresholds, pure tone audiometry) and subsequently were administered the following questionnaires: the hyperacusis questionnaire (HQ), the brief symptom inventory (BSI), and the modified somatic perception questionnaire (MSPQ). Patients with hyperacusis reported a total score and subscales (attentional, social, and emotional) of the HQ significantly higher than controls. They also reported higher scores of the MSPQ and significantly higher mean values with concern to the somatization, obsessive-compulsive, interpersonal sensitivity, depression, and anxiety subscales of the BSI. These results show that psychological distress, as expressed by higher level of somatic attention, somatization, anxiety, and depression, is a significant factor to consider for a complete diagnosis and effective treatment of hyperacusis. For a correct diagnosis of patients seeking help for hyperacusis, their psychological distress should also be assessed, regardless of their hearing abilities. Further studies are required to investigate the pathological mechanisms that are involved in the onset of hyperacusis in patients with normal hearing and those with sensorineural hearing loss.

Monitoring of Communication Precursors in Extremely Low Birth Weight (ELBW) Newborns by Video Analysis Method: Preliminary Results.

Background: The survival of extremely low birth weight infants (ELBW) has increased worldwide. Even in the absence of major disabilities, ELBW infants show difficulty in simple language functions. It is relevant to assess early abilities, which are the base of early linguistic skills, in order to implement customized intervention programs in ELBW infants. Aims: To evaluate communication precursors of language development in ELBW infants at 12 and 24 months of correct age (C.A). To investigate the correlation of linguistic and communicative prerequisites with mental development outcome at 24 months CA. Method: 52 ELBW neonates (mean gestational age 26.6 weeks, mean birth weight was 775 g) who were admitted to the neonatal intensive care unit of the University Hospital of Modena, were enrolled. Data were collected from archived audio-video recordings of neurodevelopmental follow-up visits. Video analysis of communicative and linguistic developmental was performed at 12 and 24 months CA. Neurodevelopmental outcome was evaluated with Mental Developmental Scales (GMDS-R). Results: The video-analysis showed that infants at 12 months CA used predominantly eye contacts and gestural turns, while vocal turns were scant. At 24 months CA, a significant change in eye contacts, vocal turns, gestural turns, and utterances (p < 0.001) occurred. The total number of utterances (p = 0.036) and eye contacts (p = 0.045) were significantly correlated to the Development Quotient (DQ) of Hearing and Language scale. Moreover, a significant correlation was found with the Personal-Social scale vocal turns (p = 0.009) and the total number of utterances (p = 0,02). Finally, the Global Quotient of the GMDS-R was related to the Vocal Turns (p = 0.034) and the total number of Utterances (p = 0.013). Conclusions: ELBW infants at 12 months CA use predominantly eye contacts and gestural turns to communicate with adults. At 24 months CA, the child's communicative intention evolves from gestural to verbal communication. The latter is characterized by an increase in both vocal turns and the number of utterances produced during interaction. The video analysis we implement appears to be a sensitive tool for early assessment of communication and language development and to refine early intervention

The Effect of the Use of Hearing Aids in Elders: Perspectives.

Older adults with hearing loss have difficulties during conversation with others because an elevated auditory threshold reduces speech intelligibility, especially in noisy environments. Listening and comprehension often become exhausting tasks for hearing-impaired elders, resulting in social isolation and depression. The aim of the present study was to investigate the advantages of hearing aid use in relation to relief from listening-related fatigue, which is still controversial. Participants included a sample of 49 hearing-impaired elders affected by presbycusis for whom hearing aids were prescribed. The Modified Fatigue Impact Scale was used to assess cognitive, physical and psychosocial fatigue. The vitality subscale of the Short Form Health Survey 36 and a single item of the multi-dimensional Speech, Spatial and Quality Hearing Scale ("Do you have to put a lot of effort to hear what is being said in conversation with others?") were also used. The Cognitive Failures Questionnaire was used to investigate daily errors related to lack of memory and reduced mindedness. Hearing aids rehabilitation resulted in improved speech intelligibility in competing noise, and a significant reduction in cognitive and psychosocial fatigue and listening effort in conversation. Vitality was also improved and a significant reduction in the Cognitive Failures Questionnaire scores was observed. Findings from the study indicate that the use of hearing aids in older impaired-listeners provide them not only with an increased auditory function but also with a reduction in listening-related fatigue and mindedness.

Bulging of the Oval Window in Common Cavity Deformity: A Possible Predictor of Meningitis.

OBJECTIVE: To investigate the prevalence-rate of oval window bulging in the common cavity and its association with bacterial meningitis. PATIENTS: CT and clinical files of 29 children with preliminary diagnosis of common cavity deformity were collected from 13 Italian centers. INTERVENTION: A retrospective case review study was conducted with a centralized evaluation of the temporal bone CT imaging was performed at Azienda Ospedale - Università Padova, Padova, Italy. MAIN OUTCOME MEASURE: Diagnosis of common cavity was reviewed; in addition, a fluid protrusion into the middle-ear cavity through the oval window at CT imaging was considered as oval window bulging. Its association with the history of bacterial meningitis was investigated. RESULTS: Common cavity deformity was confirmed in 14/29 children (mean-age 11.4 ±â€Š3.8; age-range 5-20; nine females) referred with this diagnosis. In 7/14 patients, the common cavity deformity was bilateral (i.e., 21 common cavities). Oval window bulging was found in 3/19 common cavities (concomitant middle-ear effusive otitis hampered the evaluation in two cases), while the internal acoustic meatus fundus was defective in 10/21 cases. History of bacterial meningitis was found in three children (21%) and two of them had oval window bulging at CT. In the case unrelated to oval window bulging, meningitis occurred late at the age of 12 during acute otitis contralateral to common cavity deformity (ipsilaterally to incomplete partition type 1). CONCLUSION: Patients harboring common cavity deformity have a high risk of meningitis in their first years of life. Oval window bulging seems to be associated with a higher risk of meningitis. This information might be important for appropriate surgical planning.

Cochlear Implantation Following Transcanal Infrapromontorial Approach for Vestibular Schwannoma: A Case Series.

Background: Cochlear implantation (CI) following endoscopic transcanal infrapromontorial vestibular schwannoma (VS) dissection is a feasible intervention in intracanalicular VS, with minimal extension into the cerebellopontine angle, but no audiologic results have ever been reported in the literature. Methods: From 2015 to 2021 in the Otorhynolaryngology Departments of Modena and Verona, three patients underwent this intervention. All were suffering from sporadic left-sided intracanalicular Koos I VS. Intraoperative electrically evoked auditory brainstem responses and electrophysiological measurements were performed before and after the placement of the electrode array, respectively. Since device activation one month after the surgery, each patient was followed up with audiometric tests, data logging, electrode impedance measurements and neural response telemetry performed at each scheduled fitting session at 15 days and 3, 6, 12 and 24 months. Results: Only in patient No. 3, an auditory benefit was observed and still evident even 36 months after activation. Impedances increased progressively in patient No. 1 and a benefit was never reported. Patient No. 2 left the follow-up for worsening comorbidities. Conclusions: CI following transcanal infrapromontorial VS resection is a beneficial intervention. The residual cochlear nerve after the tumour dissection and the course of electrophysiological measurements in the postoperative period were the main predictive factors for audiological outcomes.

Vertigo and Dizziness in Children: An Update.

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.

Genetic and Non Genetic Hearing Loss and Associated Disabilities: An Epidemiological Survey in Emilia-Romagna Region.

Hearing loss is one of the most common congenital sensory disorders. It can be associated with several comorbidities, in particular developmental disabilities (DD). In Emilia-Romagna (ER), a region in Northern Italy, Child and Adolescent Mental Health Services (CAMHS) provide the diagnostic framework and treatment for these conditions. The aim of the present study is to evaluate the prevalence of hearing loss, both isolated or in association with comorbidities, in the juvenile population. The study draws its data from the ER Childhood and Adolescent Neuropsychiatry Information System (SINPIAER), an Administrative Healthcare Database collecting the clinical data of all those who have attended CAMHS since 2010. The most frequent type of hearing loss was bilateral sensorineural hearing loss, which was present in 69-72% of the cases, while bilateral conductive hearing loss was the second most common type, ranging from 8 to 10%. Among DD, congenital malformations, mental retardation, visual impairment, and cerebral palsy were the most common. In particular, autism spectrum disorders show increasing incidence and prevalence among CAMHS users in ER region. In-depth knowledge of hearing loss epidemiology and related conditions, such as developmental disabilities, in the juvenile population is crucial for disease prevention, health planning, and resource allocation.