Sudden Sensorineural Hearing Loss in Patients Aged from 15 to 40 Years.

Objectives: The purpose of this study was to investigate the hearing characteristics and causes of sudden sensorineural hearing loss (SSNHL) in patients aged from 15 to 40 years, focusing on audiological outcomes one year after the diagnosis. Methods: The medical records of individuals with SSNHL who were referred to our tertiary-level audiologic center were reviewed. All patients had undergone comprehensive diagnostic evaluations, including high-resolution 3D-FLAIR delayed magnetic resonance imaging (MRI), cone beam computed tomography (CBCT), and screening for coagulation, infectious, and autoimmune diseases. Results: Overall, 56 patients (mean age 28.1 ± 7.6 years) were included in the study. The hearing threshold in the affected ear improved significantly from 56.0 ± 18.0 dB at the diagnosis to 46.9 ± 22.3 dB after one year (p = 0.02). The degree of hearing loss, audiometric configurations, hearing improvements, and adherence to hearing treatments showed considerable variability among patients. Aural fullness, tinnitus, and hyperacusis were the predominant symptoms associated with SSNHL, and their prevalence decreased significantly over time. The diagnostic protocol led to the identification of the specific cause of SSNHL in 75% (42/56) of patients. The known etiology was found to be otological (39.3%), infectious (21.4%), autoimmune (7.1%), vascular (5.4%), or neoplastic (1.8%). In particular, Menière's disease (n = 12), isolated cochlear endolymphatic hydrops (n = 6), HSV-1 (n = 5), and EBV (n = 4) infections were the most frequent causes of SSNHL. Conclusions: The identification of the specific etiology of SSNHL may facilitate a more personalized approach to management and treatment.

Otitis Media With Effusion After the COVID-19 Pandemic: Return to the Past and New Lessons.

OBJECTIVE: To assess the prevalence of otitis media with effusion (OME) among children who attended a tertiary level audiologic center 2 and 3 years after the COVID-19 lockdown, and to determine the impact of temporary interruption of day care center attendance on chronic OME. STUDY DESIGN: Retrospective study. SETTING: Tertiary level referral audiologic center. METHODS: We assessed the prevalence of OME among children aged 6 months to 12 years in 3 different periods (May-June 2022, January-February 2023, and May-June 2023) and compared the results with those of the corresponding periods before the COVID-19 lockdown. We also compared the disease resolution rates between a subgroup of children with chronic OME who interrupted day care center attendance for a 2-month period (Subgroup A) and a similar subgroup who continued attending day care centers (Subgroup B). RESULTS: The prevalence of OME was 38.5% (138/358) in May-June 2022, 51.9% (193/372) in January-February 2023, and 40.9% (149/364) in May-June 2023. No significant prevalence differences were observed between the periods May-June 2019, May-June 2022, and May-June 2023 (P = .78), and between the periods January-February 2020 and January-February 2023 (P = .93). At the May-June 2023 assessment, the children belonging to Subgroup A presented a greater rate of disease resolution (85.7%, 18/21) than the children belonging to Subgroup B (32%, 8/25, P < .001). CONCLUSION: This study suggests that the prevalence of OME has returned to prelockdown levels, and that interrupting day care center attendance for a 2-month period could be effective in resolving most cases of chronic OME.

Unilateral Sensorineural Hearing Loss in Children: Etiology, Audiological Characteristics, and Treatment.

The aim of this study was to evaluate audiological characteristics and parents' opinions on hearing device use in children with unilateral sensorineural hearing loss (USNHL) who attended a tertiary-level audiologic center. The medical charts of 70 children aged 6 to 12 years with USNHL were reviewed. In 51.4% of cases, the children were diagnosed with USNHL after the age of 2 years. The main causes of USNHL were congenital cytomegalovirus infection (21.4%) and unilateral cochlear nerve hypoplasia (12.9%). The percentage of patients wearing a hearing device was 45.7% (32/70); of these, 28 (87.5%) wore a conventional hearing aid, 2 (6.3%) a CROS device, and 2 (6.3%) a cochlear implant. Regarding the choice to use a hearing device, no significant differences were found between the subcategories of hearing loss degree (p = 0.55) and audiometric configuration (p = 0.54). Most parents of children with mild-to-severe USNHL observed improved attention (90.9%), and reduced fatigue and restlessness (86.4%) using the hearing aid. These children performed significantly better on all audiological tests (speech perception in quiet and in noise conditions, and sound localization) while wearing the hearing aid (p < 0.001). More efforts should be made to raise awareness among professionals and parents about the negative consequences of uncorrected USNHL.

Acute otitis media diagnosis in childhood: still a problem in 2023?

BACKGROUND: Diagnosis of acute otitis media (AOM) in children can be challenging, given that symptoms are often non-specific or absent, and that the direct observation of the tympanic membrane in its entirety through otoscopy can sometimes be difficult. The aim of this study is to assess the diagnostic concordance in detection of AOM episodes between primary care paediatricians and physicians especially trained in paediatric otoscopy, and to characterize the most misleading elements in diagnostic failure. METHODS: Consecutive clinical charts of children regularly followed for recurrent AOM (RAOM, i.e.: >3 episodes in 6 months or > 4 episodes in 1 year) at our Otitis Media paediatric outpatient clinic were retrospectively screened, in order to collect any diagnosis of AOM episode (and the related clinical findings/middle ear complaints) performed by primary care paediatricians/emergency room paediatricians. Diagnosis of AOM episode was validated by the same experienced physician (FF) in case of otoscopic relief of a bulging eardrum with at least one of the following: hyperaemia or yellow-like colour. The diagnostic concordance in detection of AOM episodes between primary care/emergency room paediatricians and our internal validator was expressed as the percentage of matching diagnosis. RESULTS: One hundred and thirty-four single AOM episodes occurring in 87 children (mean age: 26.9 +/- 18.9 months) were included in the analysis. Diagnostic concordance in detection of AOM episodes between primary care/emergency room paediatricians and our internal validator was reported in 72.4% of cases. The most common pitfall found in our study was the misleading diagnosis of AOM in case of hyperaemic tympanic membrane without bulging (32/37 out of non-validated diagnoses). CONCLUSIONS: AOM diagnosis still represents a relevant issue among paediatricians in our country, and the presence of tympanic membrane hyperaemia without concomitant bulging can be confusing.

Effects on hearing after long-term use of iron chelators in beta-thalassemia: Over twenty years of longitudinal follow-up.

OBJECTIVE: The role of iron chelation in causing hearing loss (HL) is still unclear. The present study assessed the prevalence of HL among transfusion-dependent thalassemia (TDT) patients who underwent audiological follow-up over a 20-year period. METHODS: We retrospectively analyzed clinical records and audiological tests from January 1990 (T0) to December 2022 (T22) of a group of TDT patients who received iron chelation therapy with deferoxamine (DFO), deferiprone (DFP) or deferasirox (DFX), in monotherapy or as part of combination therapy. RESULTS: A total of 42 adult TDT patients (18 male, 24 female; age range: 41-55 years; mean age: 49.2 ± 3.7 years) were included in the study. At the T22 assessment, the overall prevalence of sensorineural HL was 23.8 % (10/42). When patients were stratified into two groups, with and without ototoxicity, no differences were observed for sex, age, BMI, creatinine level, pre-transfusional hemoglobin, start of transfusions, cardiac or hepatic T2 MRI; only ferritin serum values and duration of chelation were significantly higher (p = 0.02 and p = 0.01, respectively) in patients with hearing impairment in comparison to those with normal hearing. CONCLUSION: This study with long-term follow-up suggests that iron chelation therapy might induce ototoxicity; therefore, a long and accurate audiological follow-up should be performed in TDT patients.

Audiological findings in children aged 2-6 years with isolated speech-language delay.

BACKGROUND: The present study aimed to assess the prevalence and characteristics of hearing loss (HL) among preschoolers with speech-language delay who had passed the universal newborn hearing screening (UNHS). METHODS: This retrospective study included children aged 2-6 years with isolated speech-language delay who were referred to a third-level audiological center for a comprehensive audiological assessment, including otomicroscopy, tympanometry, and click-evoked auditory brainstem responses. RESULTS: A total of 375 speech-language delayed children (285 male and 90 female) were assessed. The mean age was 41±12.4 months. Overall, 133 children (35.5%) were diagnosed with HL: 16 children (12.0%) had sensorineural HL (six unilaterally and 10 bilaterally), six (4.5%) had mixed HL (one unilaterally and five bilaterally), and 111 (83.5%) had conductive HL (19 unilaterally and 92 bilaterally). Regarding the presence of HL, no significant differences were found between males and females (P=0.60) or age groups (P=0.29). Among children with HL, 24 (18.0%) underwent tympanostomy tube placement (all bilaterally), one (0.8%) underwent unilateral cochlear implantation, eight (6.0%) were fitted with hearing aids (one unilaterally and seven bilaterally), two (1.5%) received a bimodal hearing solution (a cochlear implant on one ear and a hearing aid on the other ear), and 98 (73.7%) received no treatment. CONCLUSIONS: Although UNHS and audiological surveillance programs have been implemented over the years, all children with speech-language delay should undergo audiological evaluation. Indeed, the prevalence of undetected HL is not negligible among preschoolers with speech-language delay and requires early diagnosis and prompt treatment.

Comment on Manchaiah et al. Social Representations of "Tinnitus" and "Health" among Individuals with Tinnitus Seeking Online Psychological Interventions. Audiol. Res. 2023, 13, 207-220.

We read with pleasure the interesting paper titled "Social Representations of "Tinnitus" and "Health" among Individuals with Tinnitus Seeking Online Psychological Interventions" by Vinaya Manchaiah et al. [...].

Congenital Cytomegalovirus and Hearing Loss: The State of the Art.

In developed countries, congenital cytomegalovirus (cCMV) infection is the most common congenital viral infection, representing the leading non-genetic cause of sensorineural hearing loss (HL). Diagnosis of cCMV infection can be performed by detection of CMV DNA in urine or saliva within 2-3 weeks after birth, or later in dried blood samples on the Guthrie card. Currently, there are many controversies regarding the preventive, diagnostic, and therapeutic approaches to cCMV infection. HL secondary to cCMV is highly variable in onset, side, degree, audiometric configuration, and threshold changes over time. Therefore, it is of paramount importance to perform a long and thorough audiological follow-up in children with cCMV infection to ensure early identification and prompt treatment of progressive and/or late-onset HL. Early cochlear implantation appears to be a valid solution not only for children with bilateral profound HL, but also for those with single-sided deafness, improving localization ability and understanding speech in noisy environments. Moreover, the decision to apply a unilateral cochlear implant in children with cCMV is strengthened by the non-negligible possibility of hearing deterioration of the contralateral ear over time.

Sudden hearing loss and early hyperbaric oxygen therapy: A preliminary study.

Purpose: Sudden sensorineural hearing loss (SSNHL) is a time-sensitive urgent condition. The aim of this study was to evaluate the frequency of hearing improvement in patients with idiopathic SSNHL who only received hyperbaric oxygen (HBO2) therapy within three days of symptom onset, instead of conventional corticosteroid treatment. Methods: The medical charts of patients who experienced SSNHL between January 1, 2012, and December 31, 2021, were reviewed. The present study included all adult patients who were diagnosed with idiopathic SSNHL and started HBO2 therapy within 72 hours of symptom onset. These subjects did not take corticosteroids due to contraindications or because they were concerned about possible side effects. The HBO2 therapy protocol consisted of at least 10 sessions of 85 minutes each with pure oxygen inhalation at 2.5 atmospheres absolute pressure. Results: Overall, 49 subjects (26 males and 23 females) met the inclusion criteria, with a mean age of 47 (± 20.4) years. The mean initial hearing threshold was 69.8 dB (±18.0). After HBO2 therapy, complete hearing recovery was observed in 35 patients (71.4%), and the mean hearing threshold improved significantly (p≺0.001) to 31.4 dB (±24.5). In patients with complete hearing recovery, no significant differences were found between males and females (p=0.79), right and left ears (p=0.72) or initial grades of hearing loss (p=0.90). Conclusion: This study suggests that, in the absence of the confounding effect of concurrent steroid therapy, starting HBO2 therapy within three days of symptom onset could have a positive impact on patients with idiopathic SSNHL.

Autosomal Dominant Non-Syndromic Hearing Loss (DFNA): A Comprehensive Narrative Review.

Autosomal dominant non-syndromic hearing loss (HL) typically occurs when only one dominant allele within the disease gene is sufficient to express the phenotype. Therefore, most patients diagnosed with autosomal dominant non-syndromic HL have a hearing-impaired parent, although de novo mutations should be considered in all cases of negative family history. To date, more than 50 genes and 80 loci have been identified for autosomal dominant non-syndromic HL. DFNA22 (MYO6 gene), DFNA8/12 (TECTA gene), DFNA20/26 (ACTG1 gene), DFNA6/14/38 (WFS1 gene), DFNA15 (POU4F3 gene), DFNA2A (KCNQ4 gene), and DFNA10 (EYA4 gene) are some of the most common forms of autosomal dominant non-syndromic HL. The characteristics of autosomal dominant non-syndromic HL are heterogenous. However, in most cases, HL tends to be bilateral, post-lingual in onset (childhood to early adulthood), high-frequency (sloping audiometric configuration), progressive, and variable in severity (mild to profound degree). DFNA1 (DIAPH1 gene) and DFNA6/14/38 (WFS1 gene) are the most common forms of autosomal dominant non-syndromic HL affecting low frequencies, while DFNA16 (unknown gene) is characterized by fluctuating HL. A long audiological follow-up is of paramount importance to identify hearing threshold deteriorations early and ensure prompt treatment with hearing aids or cochlear implants.

Chronic Rhinosinusitis: T2r38 Genotyping and Nasal Cytology in Primary Ciliary Dyskinesia.

OBJECTIVES: Chronic rhinosinusitis (CRS) is a major hallmark of primary ciliary dyskinesia (PCD). We investigated the possible correlation between some severity markers of CRS and several clinical features of the disease. We further studied the bitter taste receptor TAS2R38 polymorphisms to identify the genotypes associated with more severe disease. METHODS: We included 39 adult PCD patients with (CRSwNP) and without nasal polyposis (CRSsNP); a sample for nasal cytology was obtained and clinical cytological grading (CCG) was determined. The SNOT-22 and Lund-Mackay scores were recorded. A sample of DNA was extracted from peripheral blood to investigate TAS2R38 polymorphisms. RESULTS: CRSwNP patients had features of more severe disease: indeed, they had statistically significantly higher frequency of previous sinus surgery, higher SNOT-22, LM scores, and CCG than CRSsNP patients. Upon genotyping of TAS2R38 polymorphisms, we observed that the AVI-AVI genotype, associated to homozygous nonfunctional bitter TAS2R38 receptor, was more prevalent among CRSwNP (100%) than in CRSsNP patients (0%); furthermore, AVI-AVI subjects showed statistically significantly worse SNOT-22 and CCG scores than PAV-PAV and PAV-AVI subjects. The group of AVI-AVI patients also had more frequent respiratory exacerbations, Gram-negative infections, and Pseudomonas aeruginosa colonization than PAV-PAV and PAV-AVI patients. CONCLUSION: Our findings indicate for the first time that PCD patients with CRSwNP display a more severe disease than those with CRSsNP. Genotyping of TAS2R38 polymorphisms demonstrated that in PCD patients, the AVI-AVI genotype is strikingly more prevalent among CRSwNP than in CRSsNP, while the PAV-PAV genotype might be protective against Gram-negative infections and respiratory exacerbations. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:248-254, 2023.

Audiological and vestibular symptoms following SARS-CoV-2 infection and COVID-19 vaccination in children aged 5-11 years.

PURPOSE: The present study assessed the prevalence of audio-vestibular symptoms following SARS-COV-2 infection or COVID-19 vaccination among children, comparing the two groups. A further aim was to evaluate whether children with pre-existing unilateral hearing loss were more prone to adverse events. MATERIALS AND METHODS: This retrospective study included children aged 5-11 years with normal hearing or a proven history of unilateral hearing loss who contracted SARS-CoV-2 or received two doses of COVID-19 vaccine. Tinnitus, hyperacusis, aural fullness, otalgia, otorrhea, new-onset hearing loss, vertigo and dizziness were investigated as possible complications of SARS-CoV-2 infection or the COVID-19 vaccine. RESULTS: This study included 272 children (143 boys, 129 girls), with a mean age of 7.8 ± 2.3 years. Among these, 120 were affected by pre-existing unilateral hearing loss. The most common audio-vestibular symptoms reported by children following SARS-CoV-2 infection and COVID-19 vaccination were aural fullness (33/132, 25 %) and dizziness (5/140, 3.6 %), respectively. All symptoms following COVID-19 vaccination resolved within 24 h. Compared to children who received the COVID-19 vaccine, those infected with SARS-CoV-2 had a higher prevalence of tinnitus (p = 0.009), hyperacusis (p = 0.003), aural fullness (p < 0.001), otalgia (p < 0.001), otorrhea (p < 0.001), and vertigo (p = 0.006). Two girls also experienced new-onset unilateral sensorineural hearing loss following SARS-CoV-2 infection. Children with a known history of unilateral hearing loss did not have a higher prevalence of audio-vestibular symptoms than children with normal hearing. CONCLUSIONS: Our results suggest that the COVID-19 vaccine is safe and can be recommended for children with unilateral hearing loss without fear of possible audio-vestibular sequelae.

Prevalence of Dysphonia in Non hospitalized Patients with COVID-19 in Lombardy, the Italian Epicenter of the Pandemic.

INTRODUCTION: Dysphonia has been described in patients affected by coronavirus disease 2019 (COVID-19). The aim of this study was to evaluate the prevalence of dysphonia, and its severity and extent, of voice fatigue and of dysphagia in non hospitalized patients affected by COVID-19 in Lombardy, the Italian region most hit by the first explosive outbreak of COVID-19 in Europe. METHODS: Demographic and clinical data of 160 consecutive patients, with COVID-19 diagnosis confirmed by nasal swabs processed by reverse transcription polymerase chain reaction, were gathered by means of telephone interviews performed by physicians in charge of daily follow-up. General and specific symptoms concerning voice and swallowing impairment were investigated. Dysphonia grade and duration were graded on 4-point scales, while voice fatigue was graded on a 5-point scale. RESULTS: Dysphonia was reported by 70 (43.7%) patients and was positively associated with voice fatigue (P < 0.001), cough (P = 0.005), rhinitis (P = 0.01), and dyspnea (P = 0.06); it was mild and/or moderate in 69 patients, but its duration was > 2 weeks in 33/70 (47.1%) patients and >1 month in 11/70 (15.7%). Grade and duration of dysphonia were positively associated with cough and rhinitis (all P values < 0.01). Voice fatigue was reported by 43/160 patients (26.8%) and its severity was correlated with dysphonia (P < 0.0001), cough (P = 0.02), rhinitis (P = 0.02), dyspnea (P < 0.001), and loss of appetite (P = 0.01). Dysphagia was encountered in 27/160 patients (16.9%) and was associated with dysgeusia, cough, arthralgia, myalgia and loss of appetite but not with dysphonia. CONCLUSIONS: Dysphonia was a highly prevalent and long-lasting symptom in this series; it has been underestimated to date. Further studies might shed light on the pathophysiology of voice disorders in COVID-19 patients.