OBJECTIVE:To propose a comprehensive audiological-vestibular test battery for characterising age-related hearing loss, enabling precise phenotyping of suprathreshold functional, physiological, and vestibular factors beyond audibility. To establish age- and audibility-dependent reference data in age-appropriate normal-hearing controls. DESIGN:Multidisciplinary consensus about test battery composition; Statistical analysis of centre effects to assess comparability of the test battery measured at two centres (Germany and France); Statistical analysis of age and pure-tone average (PTA) effects per test to identify potential covariates. SAMPLE:n = 55 (39 German and 16 French) participants with hearing thresholds better than the age-dependent median of the PTA, aged 40 years or older. RESULTS:Due to negligible centre effects, all data were pooled across centres. Age- and PTA-dependent reference data were derived. Age and PTA effects were identified for some tests, especially for audiological-functional tests. No age effects were found for vestibular tests. CONCLUSIONS:Normative values for a clinically feasible, multidimensional audiological-vestibular test battery were provided, including several measures whose age and PTA dependencies were previously unclear. Age and PTA should be considered as covariates for interpretation of these tests in future applications such as, e.g. phenotype-genotype relations in specified cohorts. Extensive data documentation and verification are essential for cross-centre comparability.
Abstract The vestibular system is critical for early motor development, yet the respective roles of its two subsystems, the semicircular canals and otolith organs, remain poorly defined. Here we analyse 411 children with comprehensive vestibular assessment to determine whether a functional hierarchy underlies their contributions to the acquisition of four postural and motor milestones during the first two years of life. Using Type III ANOVA to account for the frequent co-occurrence of canal and otolith dysfunction, we show that canal areflexia is associated with a 7.0-month delay in independent walking, three times larger than the otolith effect. Canal function is the only component reaching significance after Bonferroni correction across four milestones. Canal function alone predicts walking delay (>18 months) with an area under the curve of 0.83. Canal areflexia carries a positive predictive value of 80.2% for walking delay, while normal canal function effectively rules out a walking delay of vestibular origin (negative predictive value 93.5%). These findings establish a functional hierarchy of vestibular contributions to motor development and identify canal function as a powerful developmental biomarker.
INTRODUCTION:Hearing aid (HA) outcomes vary substantially despite similar hearing loss severity. We tested whether audiometric configuration improves outcome prediction beyond traditional measures. METHODS:We conducted a retrospective analysis of 22,694 adults (aged 50-90 years) fitted with HAs across 780 French audiology centers (2018-2021). Eight audiometric profiles were defined a priori based on pathophysiological classifications. Machine learning models identified predictors of speech reception threshold improvement in quiet (SRTQ, ≥6 dB) and noise (SRTN, ≥2 dB). RESULTS:Overall, 93% of participants showed demonstrated meaningful improvement in SRTQ and/or SRTN. Adding profile membership variable increased prediction accuracy by 6% for SRTN and 5% for SRTQ. Profile-stratified analysis revealed distinct optimization pathways: Profile A (moderate presbycusis) benefited from 4 kHz amplification and follow-up appointments; Profile C (ski slope) required premium technology and high compliance; Profile D (advanced presbycusis) responded exclusively to frequency-specific precision, resisting behavioral interventions. Mid-to-high frequencies (2-6 kHz) drove noise outcomes; low frequencies (250-1,000 Hz) and clinical support predicted quiet performance. Globally, the amplification of both low and high frequencies are beneficial for both SRTQ and SRTN, and high frequencies especially for SRTN. CONCLUSION:Audiometric profiling adds significant predictive value beyond traditional severity measures, identifying profile-specific intervention levers (amplification strategy, technology; choice, compliance, follow-up appointments) for personalized optimization. This configuration-aware approach supports precision audiology, where treatment is guided by an audiometric profile and expected outcomes.
OBJECTIVE:To investigate the relationship between daily manual abilities, as measured by the ABILHAND questionnaire in hearing aid users, and their hearing aid handling skills, independently assessed by a panel of Hearing Care Professionals (HCPs) using a dedicated questionnaire. The study aimed to assess the potential of the ABILHAND questionnaire as a tool to guide HCPs in hearing aid choices. DESIGN:A cross-sectional study design was conducted. STUDY SAMPLE:Two hundred and twenty-one adult patients who had been wearing hearing aids for at least one year were recruited during routine follow-up visits. Participants completed the self-administered ABILHAND questionnaire, evaluating manual dexterity through everyday manual tasks. Twenty HCPs completed a non-standardised questionnaire assessing patients' manipulation abilities related to hearing aid use. RESULTS:ABILHAND Light-7, a shortened version of the ABILHAND questionnaire, was developed. The two ABILHAND questionnaires showed a negative correlation with age and a positive correlation with the HCP questionnaire. Threshold scores were established for both ABILHAND questionnaires to guide recommendations on hearing aid type and battery size. CONCLUSIONS:Both ABILHAND and ABILHAND Light-7 are valuable tools for prosthetic orientation, as they help identify devices, batteries, or earpieces that may be too complex for patients to manage.
Vestibular paroxysmia (VP) is a disabling medical condition characterized by a high recurrence rate of vertigo. VP is classically associated with the presence of contact between a vascular structure and the cochleovestibular nerve, a neurovascular cross compression (NVCC). Antiepileptic drugs are the first-line treatment for disabling symptoms. We conducted a systematic review to evaluate their efficacy in patients with VP, and whose imaging shows NVCC. All published studies in PubMed databases until October 2024 were included. A total of seven studies were selected. Carbamazepine and oxcarbazepine are by far the most widely used drugs, but there is still a lack of data showing the efficacy of antiepileptic drugs in a large sample of patients. This suggests that a future randomized controlled trial with a satisfactory sample size of VP patients with NVCC and comparing several drugs with neuroprotective properties is needed. Further, as imaging in some of the patients with obvious clinical signs does not always support this diagnosis, we discussed VP physiopathology and the different types of VP published in the literature, including those with no radiological support for NVCC.
Background/Objectives: Objective: To discuss therapeutic outcomes in patients with symptomatic near-narrow internal auditory canal (NNIAC). Methods: We retrospectively analyzed the records of 26 symptomatic patients diagnosed with NNIAC, who had been treated with anti-epileptic drugs. In addition to clinical and radiological data, we recorded I–III latencies of auditory brainstem responses prior to and after medical therapy. Results: Among a total of 48 patients with NNIAC, 26 patients were included. Oxcarbazepine, Gabapentin, and Lamotrigine were prescribed among 19 (73%) patients, 6 (23%) patients, and 1 patient (4%), respectively. After treatment, 24 (92.3%) patients described improvement of vestibular symptoms, and 16 (76.2%) reported improvement of auditory symptoms. After treatment with antiepileptic drugs, ipsilateral IPL I-III latencies decreased (less than 2.3 ms) in 16 (84.2%) patients (23 ears out of 42). Conclusions: A low dose of anti-epileptic monotherapy for NNIAC could be effective over the long term and is generally well-tolerated. Further studies are needed to provide more solid evidence of the efficacy and safety of anti-epileptic drugs on a larger number of patients with NNIAC.
The vestibular atelectasis (VA) is a very rarely reported clinical and radiological condition characterized by the collapse of the labyrinthine membrane. Clinical features include symptoms which mimic endolymphatic hydrops; bilateral forms are rarely reported in the literature. Tullio's sign was reported in a few cases. In this paper, we report an original case of bilateral fluctuating VA associated with a rare posterior semicircular canal (SSC) dehiscence variant. A 54-year-old patient presented with evolving and fluctuating bilateral vestibulopathy associated with a pressureinduced nystagmus. Vestibular assessment revealed a bilateral vestibulopathy affecting low and mid velocities, with fluctuating high-velocity impairment. Exploration of the otolithic system showed preserved saccular activity and an absence of utriculo-ocular response. Radiological evaluation demonstrated bilateral VA and a dehiscence of the right posterior SSC. We provide clinical elements to better understand this entity, and propose a new hypothesis for the presence of a previously reported Tullio sign in VA.
Function word (FW) is a reliable marker of Developmental Language Disorder (DLD), yet their profile in European French (EF) preschoolers remains poorly understood. This corpus study compared FW use in 73 monolingual EF-speaking children with DLD (39-84 months, M = 54, SD = 10.93) and 76 typically developing peers (24-36 months, M = 30, SD = 5.97) matched on mean length of utterance in words (M = 2.5). Six FW categories-articles, prepositions, demonstrative pronouns, subject pronouns, and modal and auxiliary verbs-were analysed using chi-square tests and log-linear regression. Performance was comparable for articles and prepositions, but children with DLD showed impairments of FW use related to verbal contexts-reduced pronoun use, skewed FW distribution, and frequent omission or agreement errors with modal and auxiliary verbs. These patterns indicate processing limitations beyond developmental variability, implicating working memory, procedural learning, and sensitivity to input regularities. The findings refine cross-linguistic models of DLD and highlight FW assessment as a sensitive diagnostic tool for EF-speaking children.
Background/Objectives: As vestibular paroxysmia (VP) has recently been described in children, with an incidence of up to 4% of vertigo, and a promising therapeutic response to sodium-channel-blocking drugs has also been reported, the aim of this paper is to review the available literature on this topic and to provide the best possible guidance for diagnosis and treatment. Methods: PubMed, Medline, Cochrane, and Crossref databases were searched, and all studies on VP in children and sodium channel blockers were selected. Results: Only five articles reporting small case series or single case reports were identified. To date, oxcarbazepine (OXC) and carbamazepine (CBZ) are the only two molecules prescribed. The recommended doses were 300 to 360 mg/day and 50 to 200 mg/day for OXC and CBZ, respectively, for a total duration of 6 weeks. Fast efficacy (one week) was reported. Conclusions: VP has been identified in pediatric patients and appears to respond to sodium channel blockers in a manner similar to adults. Only a limited number of cases have been reported to date; thus, there is a need to raise awareness about this treatable cause of episodic vertigo in children.
Background: Superior semicircular canal dehiscence (SSCD) is characterized by a bony defect of the superior semicircular canal (SSC), leading to vestibular and auditory symptoms. A process of spontaneous "auto-plugging," in which the overlying dura mater progressively occludes the SSC, may replicate the effects of surgical canal plugging but remains under-recognized. The present study reports diverse clinical, instrumental, and 3d High Resolution MRI findings in patients with SSCD and subsequently confirmed to present with spontaneous complete or partial auto-plugging. Methods: We retrospectively reviewed 11 patients with SSCD diagnosed on high-resolution CT and suspected auto-plugging based on clinical atypia and large dehiscence (>4 mm). Patients underwent comprehensive neurotological assessment, including pure-tone audiometry, vestibular testing, and HR MRI with 3D labyrinthine reconstructions to identify partial or complete auto-plugging. Auto-plugging was classified as partial (Canalis semicircularis superior depressus) or complete (absence of endolymph fluid signal; Canalis semicircularis superior obturatus). Results: Among 13 ears with auto-plugging, 6 were partial and 7 complete. The mean SSCD size in auto-plugged ears was 5.5 mm. Most ears had normal or near-normal vestibular function on VHIT, with minimal air-bone gaps and preserved VEMP responses. Imaging demonstrated varying degrees of dural contact with the SSC, confirming partial or complete canal occlusion. Conclusions: Spontaneous auto-plugging of the SSC is a plausible, under-recognized phenomenon that may reproduce functional effects of surgical plugging. Dedicated 3D labyrinthine MRI enhances detection and characterization. Prospective multimodal studies are needed to clarify the pathophysiology, progression, and clinical implications, optimizing patient selection for surgical versus conservative management.
OBJECTIVE:This report presents for the first time a case of triple otic capsule dehiscence (OCD) of the labyrinthine-vascular type, with 2 dehiscences treated simultaneously and effectively by a recently described endovascular approach. STUDY DESIGN:Case report. SETTING:Single otology/neurotology clinic. SUBJECT:A 47-year-old male with triple otic capsule dehiscence (OCD) and associated symptoms. MAIN OUTCOME MEASURES:Improvement in the Tinnitus Handicap Inventory (THI) and Dizziness Handicap Inventory (DHI) scores. RESULTS:The first OCD was located between the internal jugular vein (IJV) and the posterior semicircular canal (SCC), and the second OCD was located between the vestibular aqueduct (VA) and the IJV. The third OCD, situated between the superior SCC and the superior petrosal sinus, remained untreated since it was considered less contributive to the patient's symptoms. The procedure involved (1) stenting of the right jugular vein combined with (2) coiling of a small portion of the vein in contact with the homonymous vestibular aqueduct. Early clinical signs, along with the audio-vestibular assessment, also suggested the diagnosis of right-sided endolymphatic hydrops. The outcome was uneventful, and the long-term follow-up showed promising results as the menieriform-like vertigo attacks disappeared after the procedure. THI and DHI scores were significantly improved. Auditory symptoms, including tinnitus, also progressively improved, especially after the fitting of a hearing aid. CONCLUSIONS:Multiple OCD (not only dehiscence of the superior SCC) should be systematically looked for on high-resolution computed tomography, given that a combined endovascular approach could be carried out in multiple labyrinthine-vascular forms.
BackgroundWhile there is no cure for tinnitus, research has shown that cognitive behavioral therapy (CBT) is effective in managing clinical sequelae. Although traditional CBT is labor-intensive and costly, new online consultations may improve accessibility. Moreover, there is promise in an engaging conversational agent, or a “chatbot,” delivering CBT in a conversation-like manner and allowing users to work through complex situations with the guidance of a virtual coach. Currently, there is little research examining a possible hybrid model using iCBT and tele consultation with a psychologist.MethodsA randomized, 2 parallel-group trial was conducted to compare the clinical effectiveness of (1) iCBT delivered through a chatbot mobile app (i.e., Tinnibot only group) (2) Tinnibot combined with telepsychology (i.e., hybrid-intervention group). A total of 30 eligible adults with tinnitus were included. After an 8-week intervention period, participants were followed up for 2 months. The primary outcome measure, the Tinnitus Functional Index (TFI), and the secondary outcome measures, Hyperacusis Questionnaire (HQ), Generalized Anxiety Disorder 7-item (GAD-7), and Patient Health Questionnaire (PHQ-9), were assessed before treatment, post-treatment, and at follow-up.ResultsThe TFI decreased significantly over time in both groups, with a trend for a larger improvement in the group that received telepsychology. At post-treatment, a clinically significant improvement was observed in 42% of the Tinnibot-only group and 64% of the hybrid-intervention group. At follow-up, this was 64% for both groups. The secondary outcome measures, PHQ-9 and GAD-7 improved significantly over time, but the HQ did not.DiscussionInternet-based delivery of CBT is effective in decreasing tinnitus distress, and levels of anxiety and depression, which is more relevant today than ever in the context of a global pandemic that has challenged the delivery of face-to-face intervention. The addition of telepsychology might be beneficial, but not essential for the effectiveness of treatment. There is a need for further research to determine whether there is any relationship between the characteristics of tinnitus patients and the success of the different modes of delivery of therapy.
Sensorineural hearing loss (SNHL) is the most common type of hearing loss. Causes include degenerative changes in the sensory hair cells, their synapses and/or the cochlear nerve. As human inner ear hair cells have no capacity for regeneration, their destruction is irreversible and leads to permanent hearing loss. SNHL can be genetically inherited or acquired through ageing, exposure to noise or ototoxic drugs. Ototoxicity generally refers to damage to the structures and functions of the inner ear following exposure to specific drugs. Ototoxicity can be multifactorial, causing damage to cochlear hair cells or cells with homeostatic functions that modulate cochlear hair cell function. Clinical strategies to limit ototoxicity include identifying patients at risk, monitoring drug concentrations, performing serial hearing assessments and switching to less ototoxic therapy. This review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, using the PubMed® database. The search terms "ototoxicity", "hearing loss" and "drugs" were combined. We included studies published between September 2013 and June 2023, and focused on medicines and drugs used in hospitals. The review highlighted a number of articles reporting the main drug classes potentially involved: namely, immunosuppressants, antimalarials, vaccines, antibiotics, antineoplastic agents, diuretics, nonsteroidal anti-inflammatory drugs and analgesics. The presumed ototoxic mechanisms were described, together with the therapeutic and preventive options developed over the last ten years.
Objectives: Investigate factors contributing to the effective management of age-related hearing loss (ARHL) rehabilitation. Methods: A systematic review was conducted following PRISMA guidelines. The protocol was registered in PROSPERO (CRD42022374811). Articles were identified through systematic searches in the Scopus, PubMed, Web of Science, and Cochrane databases in May 2024. Only articles published between January 2005 and May 2024 were included. Studies were assessed for eligibility by two independent researchers and evaluated using the Crowe Critical Appraisal Tool v1.4 (CCAT). Results: Of the 278 articles identified, 54 were included. Three factors explain effective HA use. First, hearing aid signal processing, with directional microphones and noise reduction, improves user comfort and understanding regarding noise. Second, there is hearing aid fitting, with the NAL prescription rules as the gold standard, and bilateral, high-level HA performance for spatial localization and noise comprehension. Third, there is a patient-centered approach, using patient-related outcome measures (PROMs), questionnaires, counseling, and regular follow-up to involve patients in their therapeutic rehabilitation. Conclusions: Reaching a consensus on acoustic parameters is challenging due to variability in audiological results. Involving patients in their rehabilitation, addressing their needs and expectations, and offering individualized care are crucial.
Sudden sensorineural hearing loss (SSNHL), a rare audiological condition that accounts for 1% of all cases of sensorineural hearing loss, can cause permanent hearing damage. Soon after the launch of global COVID-19 vaccination campaigns, the World Health Organization released a signal detection about SSNHL cases following administration of various COVID-19 vaccines. Post-marketing studies have been conducted in different countries using either pharmacovigilance or medico-administrative databases to investigate SSNHL as a potential adverse effect of COVID-19 vaccines. Here, we examine the advantages and limitations of each type of post-marketing study available. While pharmacoepidemiological studies highlight the potential association between drug exposure and the event, pharmacovigilance approaches enable causality assessment. The latter objective can only be achieved if an expert evaluation is provided using internationally validated diagnostic criteria. For a rare adverse event such as SSNHL, case information and quantification of hearing loss are mandatory for assessing seriousness, severity, delay onset, differential diagnoses, corrective treatment, recovery, as well as functional sequelae. Appropriate methodology should be adopted depending on whether the target objective is to assess a global or individual risk.
Trop d’articles sont encore refusés dans les revues médicales scientifiques, en raison du manque de préparation du manuscrit et de la méconnaissance des règles rédactionnelles modernes qui régissent la rédaction médicale scientifique. Aussi le comité de rédaction des European Annals of Otorhinolaryngology Heads & Neck Diseases a synthétisé les travaux publiés par ses membres depuis 2020 dans les colonnes de la revue scientifique de la société française d’oto-rhino-laryngologie et de la Société internationale francophone d’oto-rhino-laryngologie ainsi que les données de la littérature indexée PubMed dédiées à la rédaction médicale scientifique en oto-rhino-laryngologie au XXIe siècle. Avec cette mise au point construite sur le mode « Faites ! » et « Ne Faites Pas ! », les auteurs espèrent fournir aux auteurs un guide pratique facilitant la publication de travaux scientifiques rigoureux, reproductibles et transparents, en accord avec l’évolution pour une meilleure science souhaitée par la société depuis le début de ce siècle.
Importance Children with profound hearing loss (HL) and vestibular impairment have worse cochlear implant outcomes compared with those without vestibular impairment. However, the decision for cochlear implantation is rarely based on vestibular function assessment as a complement to audiologic testing. Objectives To identify the prevalence of vestibular impairment according to HL origin and to assess the association between vestibular impairment and delayed posturomotor development in children with profound HL. Design, Setting, and Participants This cohort study was conducted in a pediatric referral center for cochlear implantation in Paris, France, using medical records data on HL origin, vestibular assessment, and ages of developmental milestone achievement. The cohort included children with profound HL (loss >90 dB HL) who completed vestibular assessment prior to cochlear implantation between January 1, 2009, and December 31, 2019. Data analyses were conducted between January and June 2023. Main Outcomes and Measures The primary outcome was prevalence of vestibular impairment according to HL origin. Children were classified into 3 groups according to their responses to vestibular testing: normal vestibular function (NVF), partially impaired vestibular function (PVF), and complete bilateral vestibular loss (CBVL). Generalized logit models were performed to evaluate the association between vestibular impairment and causes of HL as well as posturomotor development delay. Results A total of 592 children were included (308 males [52.0%]; mean [SD] age, 38 [34] months). In children with documented HL origin (n = 266), 45.1% (120) had HL with genetic origin, 50.0% of which were syndromic (mainly Usher and Waardenburg syndromes) and 50.0% were nonsyndromic (mainly associated with connexin 26). Among patients with infectious HL origin (n = 74), 70.3% (52) had cytomegalovirus (CMV) infection. Vestibular impairment was found in 44.4% (263 of 592) of the children; it was mostly symmetrical in 88.9% (526) and was CBVL in 5.7% (34) of the cases. Vestibular impairment was present in 78.3% (47) of children with genetic syndromic HL (56.7% [34] with PVF; 21.7% [13] with CBVL) and in 69.2% (36) of children with CMV infection (57.7% [30] with PVF; 11.5% [6] with CBVL). Genetic syndromic HL origin was found to be more often associated with both PVF and CBVL than other HL causes. The odds of having delays in 4 developmental milestones (head holding, sitting, standing with support, and independent walking) were higher in both PVF and CBVL (eg, head-holding odds ratios: 2.55 and 4.79) compared with NVF, and the age of achieving these milestones was higher in CBVL than PVF (eg, head holding: 7.33 vs 4.03 years; P < .001). All 4 developmental milestones were associated with the degree of vestibular impairment. Conclusions and Relevance This cohort study found that among children with profound HL, vestibular impairment was prevalent, varied according to HL origin, and associated with posturomotor development; while all developmental milestones were associated with vestibular impairment severity, not all HL causes were associated with vestibular impairment severity. Children with profound HL may benefit from complete vestibular assessment before cochlear implantation, which would support early and adapted management, such as physical therapy for CBVL and cochlear implantation strategy.
Improving adverse events following immunisation (AEFI) detection is vital for vaccine safety surveillance, as an early safety signal can help minimize risks. In February 2022, the World Health Organization reported a preliminary signal on sudden sensorineural hearing loss (SSNHL) following coronavirus disease 2019 (COVID-19) vaccination, 54 million persons in France received at least one dose, covering 78.8
We read, with great interest, two recent articles by Castellucci and al [...]