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.

Laryngeal Leishmaniasis: A Neglected, Emerging Disease in Northern Italy.

Leishmaniasis represents an emerging public health issue in Mediterranean countries. The incidence of this condition has progressively risen in Northern Italy due to the growing number of immunocompromised people and probably due to climate changes. We hereby describe a case of relapsing laryngeal leishmaniasis in a female immunocompetent patient, presenting as aspecific chronic laryngitis. She was affected by severe asthma treated by inhaled steroid therapy, likely responsible for the parasite's diffusion through a locus minori resistentiae. The aspecific clinical presentation led to a delayed diagnosis and the lack of guidelines for the treatment caused multiple relapses. Biopsies of laryngeal lesions in the follow-up were performed by operative flexible videolaryngoscopy, thus avoiding general anesthesia and reducing associated healthcare costs. The aim of this report is to underline the diagnostic and therapeutic challenges that patients with this condition face and to present what is, to the best of our knowledge, the first application of prophylactic aerosolized pentamidine for relapsing laryngeal leishmaniasis.

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.

Dominant VPS16 Pathogenic Variants: Not Only Isolated Dystonia.

BACKGROUND: VPS16 pathogenic variants have been recently associated with inherited dystonia. Most patients affected by dominant VPS16-related disease display early-onset isolated dystonia with prominent oromandibular, bulbar, cervical, and upper limb involvement, followed by slowly progressive generalization. CASES: We describe six newly reported dystonic patients carrying VPS16 mutations displaying unusual phenotypic features in addition to dystonia, such as myoclonus, choreoathetosis, pharyngospasm and freezing of gait. Response to bilateral Globus Pallidus Internus Deep Brain Stimulation (GPi-DBS) is reported in three of them, associated with significant improvement of dystonia but only minor effect on other hyperkinetic movements. Moreover, five novel pathogenic/likely pathogenic variants are described. CONCLUSIONS: This case collection expands the genetic and clinical spectrum of VPS16-related disease, prompting movement disorder specialists to suspect mutations of this gene not only in patients with isolated dystonia.

3D-4K Exoscope-Assisted CO2 Laser Supraglottoplasty for Severe Laryngomalacia in a Pediatric Patient.

Laryngomalacia is the most frequent congenital laryngeal anomaly. Surgery is possible by means of cold knife, carbon dioxide (CO2 ) laser, microdebrider and coblation, traditionally under microscopic view. We here describe the use of the 3D-4K exoscope assisted CO2 laser supraglottoplasty in tubeless general anesthesia in spontaneous breathing in a 5 month-old patient with severe laryngomalacia. Laryngoscope, 134:1359-1362, 2024.

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.

Consensus for voice quality assessment in clinical practice: guidelines of the European Laryngological Society and Union of the European Phoniatricians.

INTRODUCTION: To update the European guidelines for the assessment of voice quality (VQ) in clinical practice. METHODS: Nineteen laryngologists-phoniatricians of the European Laryngological Society (ELS) and the Union of the European Phoniatricians (UEP) participated to a modified Delphi process to propose statements about subjective and objective VQ assessments. Two anonymized voting rounds determined a consensus statement to be acceptable when 80% of experts agreed with a rating of at least 3/4. The statements with ≥ 3/4 score by 60-80% of experts were improved and resubmitted to voting until they were validated or rejected. RESULTS: Of the 90 initial statements, 51 were validated after two voting rounds. A multidimensional set of minimal VQ evaluations was proposed and included: baseline VQ anamnesis (e.g., allergy, medical and surgical history, medication, addiction, singing practice, job, and posture), videolaryngostroboscopy (mucosal wave symmetry, amplitude, morphology, and movements), patient-reported VQ assessment (30- or 10-voice handicap index), perception (Grade, Roughness, Breathiness, Asthenia, and Strain), aerodynamics (maximum phonation time), acoustics (Mean F0, Jitter, Shimmer, and noise-to-harmonic ratio), and clinical instruments associated with voice comorbidities (reflux symptom score, reflux sign assessment, eating-assessment tool-10, and dysphagia handicap index). For perception, aerodynamics and acoustics, experts provided guidelines for the methods of measurement. Some additional VQ evaluations are proposed for voice professionals or patients with some laryngeal diseases. CONCLUSION: The ELS-UEP consensus for VQ assessment provides clinical statements for the baseline and pre- to post-treatment evaluations of VQ and to improve collaborative research by adopting common and validated VQ evaluation approach.

A misdiagnosed laryngeal foreign body causing recurrent wheezing in a pediatric patient: Case report.

Key clinical message: The recurrence of respiratory symptoms without a clear clinical reason in children can be secondary to the presence of an unknown foreign body of the airways. In such cases, endoscopy of the airways is always necessary, regardless of the patient's age. Abstract: The management of foreign bodies of the pediatric airway can be challenging. The clinical presentation may vary and, in case of recurrent respiratory symptoms without a clear clinical cause, the presence of a foreign body of the airway should be suspected. We describe the case of a 13-month-old patient, weight 11 kg, with a misdiagnosed subglottic foreign body causing dysphonia and worsening respiratory distress, removed by means of a direct laryngotracheoscopy in tubeless general anesthesia in spontaneous breathing.

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.

Machine Learning Assessment of Spasmodic Dysphonia Based on Acoustical and Perceptual Parameters.

Adductor spasmodic dysphonia is a type of adult-onset focal dystonia characterized by involuntary spasms of laryngeal muscles. This paper applied machine learning techniques for the severity assessment of spasmodic dysphonia. To this aim, 7 perceptual indices and 48 acoustical parameters were estimated from the Italian word /a'jwɔle/ emitted by 28 female patients, manually segmented from a standardized sentence and used as features in two classification experiments. Subjects were divided into three severity classes (mild, moderate, severe) on the basis of the G (grade) score of the GRB scale. The first aim was that of finding relationships between perceptual and objective measures with the Local Interpretable Model-Agnostic Explanations method. Then, the development of a diagnostic tool for adductor spasmodic dysphonia severity assessment was investigated. Reliable relationships between G; R (Roughness); B (Breathiness); Spasmodicity; and the acoustical parameters: voiced percentage, F2 median, and F1 median were found. After data scaling, Bayesian hyperparameter optimization, and leave-one-out cross-validation, a k-nearest neighbors model provided 89% accuracy in distinguishing patients among the three severity classes. The proposed methods highlighted the best acoustical parameters that could be used jointly with GRB indices to support the perceptual evaluation of spasmodic dysphonia and provide a tool to help severity assessment of spasmodic dysphonia.

Endoscopic surgery under tubeless anesthesia and spontaneous breathing for a congenital laryngeal web in an infant: A case report.

Congenital laryngeal webs are rare and are defined as thick epithelium-covered fibrous tissue lying between the vocal folds; the anterior glottis is the most common site of involvement, with possible extension to the subglottic region. The association with chromosome 22q11.2 deletion syndrome has also been reported. Symptoms have been abnormal or absent crying and airway obstruction since birth. Management strategies range from endoscopic division using cold instruments to open surgery for severe webbing. In endoscopic surgery, the need for tubeless anesthesia and spontaneous breathing is fundamental for obtaining the best surgical outcome. Here, we describe the case of a 4-month-old female patient affected by a type II glottic web according to Cohen, who was treated by simple endoscopic division in spontaneous breathing.

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.