Corrigendum: Current phenotypic and genetic spectrum of syndromic deafness in Tunisia: paving the way for precision auditory health.

[This corrects the article DOI: 10.3389/fgene.2024.1384094.].

Predictors of Speech-in-Noise Understanding in a Population of Occupationally Noise-Exposed Individuals.

Understanding speech in noise is particularly difficult for individuals occupationally exposed to noise due to a mix of noise-induced auditory lesions and the energetic masking of speech signals. For years, the monitoring of conventional audiometric thresholds has been the usual method to check and preserve auditory function. Recently, suprathreshold deficits, notably, difficulties in understanding speech in noise, has pointed out the need for new monitoring tools. The present study aims to identify the most important variables that predict speech in noise understanding in order to suggest a new method of hearing status monitoring. Physiological (distortion products of otoacoustic emissions, electrocochleography) and behavioral (amplitude and frequency modulation detection thresholds, conventional and extended high-frequency audiometric thresholds) variables were collected in a population of individuals presenting a relatively homogeneous occupational noise exposure. Those variables were used as predictors in a statistical model (random forest) to predict the scores of three different speech-in-noise tests and a self-report of speech-in-noise ability. The extended high-frequency threshold appears to be the best predictor and therefore an interesting candidate for a new way of monitoring noise-exposed professionals.

Non-invasive intracranial pressure monitoring for high-grade gliomas patients treated with radiotherapy: results of the GMaPIC trial.

Introduction: Patients with high-grade gliomas are at risk of developing increased intracranial hypertension (ICHT) in relation to the increase in volume of their tumor. ICP change cannot be measured by invasive method but can be estimated by using routine clinical signs, in combination with a standard imaging method, magnetic resonance imaging (MRI). A non-invasive monitoring of ICP could be of interest in high-grade glioma, in particular after radiotherapy treatment with as major side effect a cerebral oedema. Patients and Methods: This prospective clinical study aimed to compare the ICP changes (estimated by a non-invasive method based upon distortion product otoacoustic emissions (DPOAE) monitoring) with volume changes observed on MRI in patients with high-grade gliomas treated with radiotherapy. DPOAE measurements were performed one month after the end of radiotherapy and then every 3 months for one year. At each visit, the patient also underwent MRI as well as an evaluation of clinical signs. Results: The variation in the estimate of intracranial pressure readout measured at each follow-up visit (in absolute value with respect to the baseline measurements) was significantly associated with the variation of T2/FLAIR volume (n=125; p<0.001) with a cut off value of change ICP readout of 40.2 degrees (e.i. an estimated change of 16 mm Hg). Discussion: The GMaPIC trial confirm the hypothesis that the ICP change estimated by DPOAEs measurement using a non-invasive medical device is correlated with the change of the tumor or edema in high grade glioma after radiotherapy. The device could thus become an easy-to-use and non-invasive intracranial pressure monitoring tool for these patients. Clinical Trial Registration: Clinicaltrials.gov, identifier (NCT02520492).

Current phenotypic and genetic spectrum of syndromic deafness in Tunisia: paving the way for precision auditory health.

Hearing impairment (HI) is a prevalent neurosensory condition globally, impacting 5% of the population, with over 50% of congenital cases attributed to genetic etiologies. In Tunisia, HI underdiagnosis prevails, primarily due to limited access to comprehensive clinical tools, particularly for syndromic deafness (SD), characterized by clinical and genetic heterogeneity. This study aimed to uncover the SD spectrum through a 14-year investigation of a Tunisian cohort encompassing over 700 patients from four referral centers (2007-2021). Employing Sanger sequencing, Targeted Panel Gene Sequencing, and Whole Exome Sequencing, genetic analysis in 30 SD patients identified diagnoses such as Usher syndrome, Waardenburg syndrome, cranio-facial-hand-deafness syndrome, and H syndrome. This latter is a rare genodermatosis characterized by HI, hyperpigmentation, hypertrichosis, and systemic manifestations. A meta-analysis integrating our findings with existing data revealed that nearly 50% of Tunisian SD cases corresponded to rare inherited metabolic disorders. Distinguishing between non-syndromic and syndromic HI poses a challenge, where the age of onset and progression of features significantly impact accurate diagnoses. Despite advancements in local genetic characterization capabilities, certain ultra-rare forms of SD remain underdiagnosed. This research contributes critical insights to inform molecular diagnosis approaches for SD in Tunisia and the broader North-African region, thereby facilitating informed decision-making in clinical practice.

Psychometric Validation of a Hearing Screening Questionnaire for Preschoolers Based on Language Development Evaluation by Caregivers.

INTRODUCTION: This study aimed to validate three age-adjusted versions of a Hearing Screening Questionnaire for Preschoolers, in Brazilian Portuguese, based on parents' perception of their children's hearing and oral language. METHODS: Psychometric validation was conducted on three questionnaires, each comprising nine items with yes/no responses. Three items focused on hearing screening at birth, and six assessed hearing and oral language. The study included 152 parents and their children, who attended daycare centers in Belo Horizonte, Brazil. The children were categorized into three age bands: 12-18 months, 19-35 months, and 36-48 months. Audiological assessments, including tympanometry, transient-evoked otoacoustic emissions (TEOAE), and pure-tone audiometry (when applicable), were performed on the children. In case of abnormal findings in the previous exams, auditory brainstem response (ABR) testing was conducted. Descriptive data, false alarm, and false-negative analyses were carried out. RESULTS: Considering any type of hearing loss, whether unilateral or bilateral, the questionnaires showed a false-negative rate of 41.17% (7/17 children). However, when considering only bilateral hearing loss, the questionnaire showed a false alarm rate of 31.69% (45/142) and a false-negative rate of 30.0% (3/10). When focusing exclusively on sensorineural hearing loss, the questionnaire identified two children (1.31%), with a false-negative rate of 0% but a false-positive rate of 33.33%. CONCLUSION: Language-development-oriented questionnaires allowed quick screening of potential hearing loss in preschoolers. This study found a robust hit rate with these questionnaires. Their validation signifies a promising and cost-effective tool for conducting hearing screenings in preschool children, especially in nations lacking a comprehensive school screening policy. The validated questionnaire affords an easy-to-apply, low-cost, and effective instrument for preschool hearing screening.