(Auditory symptoms absent)
1,619 results
  • Comprehensive analysis of hearing function in Morquio A syndrome: Implications for diagnosis and management. [Journal Article]
    Mol Genet Metab. 2026 May 13; 148(3):110139. [Online ahead of print]Shih A, Morlet T, … Tomatsu SMG
  • Morquio A syndrome (mucopolysaccharidosis IVA) is a lysosomal storage disorder characterized by systemic skeletal dysplasia and multisystem involvement. Hearing loss is frequently reported but remains insufficiently characterized, with few studies incorporating comprehensive behavioral and electrophysiologic assessments. In this prospective study, the largest Morquio A auditory cohort to date, pa…
  • Voluntary Attention Selectively Modulates Omission Responses. [Journal Article]
    Hum Brain Mapp. 2026 Apr 01; 47(5):e70518.Dercksen TT, Widmann A, Wetzel NHB
  • Predictive coding conceptualizes attention as a weighting of prediction error signals. However, empirical findings on how attention influences common markers of prediction error have been inconsistent, likely because these markers are typically derived from stimulus-evoked responses. To avoid stimulus-related confounds and isolate effects related purely to prediction, we investigated how attentio…
  • Pre-treatment audiological and vestibular assessment in adults starting platinum-based chemotherapy. [Journal Article]
    Support Care Cancer. 2026 Apr 02; 34(4).Van Der Biest H, Verhulst S, … Dhooge ISC
  • CONCLUSIONS: Audiological assessments were largely feasible, even in older adults, and revealed pre-existing audiovestibular deficits including high-frequency hearing loss and BPPV. Given risk factors such as advanced age, baseline hearing deficits, and planned type of platinum-based chemotherapy, this population is particularly vulnerable to cochleotoxicity. These findings support implementing a pre-treatment audiovestibular protocol that can serve as a baseline for subsequent longitudinal ototoxicity monitoring, with individualized adaptations to optimize feasibility and to support early detection of ototoxic changes and timely intervention during follow-up.
  • ABR findings in inner ear anomaly subgroups: Influence of cochlear nerve deficiency. [Journal Article]
    Eur Arch Otorhinolaryngol. 2026 May; 283(5):2975-2983.Eşdoğan A, Kaya ES, … Sennaroğlu GEA
  • CONCLUSIONS: This study delineates ABR characteristics specific to inner ear anomaly subgroups and highlights the influence of CND on ABR outcomes. The findings underscore the potential of detailed ABR analysis to serve as a valuable tool for guiding auditory rehabilitation decisions and emphasize its relevance in shaping future clinical practice guidelines.
  • Pre-Neural Source of the Envelope-Following Response Revealed in Cases of Auditory Neuropathy. [Journal Article]
    Ear Hear. 2026 Feb 13. [Online ahead of print]Díaz M, Lucchetti F, … Nonclercq AEH
  • CONCLUSIONS: The EFR-H observations suggested a pre-neural origin, with the asymmetrical mechanoelectrical transduction process at the sensory hair cells as the main contributor. These findings provide further evidence of a pre-neural potential explaining the discrepancy between ABR and ASSR threshold estimations in ANSD. In addition, the results from the contralateral ears of unilateral ANSD cases may reveal subtle deficits typically undetected by standard audiological techniques. Overall, the study of noninvasive EFR recordings in ANSD may contribute to refining their diagnosis, ultimately improving their treatment.
  • Adrenoleukodystrophy in adults: phenotypic characterisation and natural history in a large cohort. [Journal Article]
    J Neurol Neurosurg Psychiatry. 2026 May 14; 97(6):483-492.Benzoni C, Moscatelli M, … Salsano EJN
  • CONCLUSIONS: Adult adrenoleukodystrophy comprises distinct phenotypes with variable prognosis. Recognition of ALMN as an intermediate form and the low cerebral progression rate in Italian AMN refine disease classification. Sex-related VLCFA differences may influence severity, although standard assays lack sensitivity. Neurophysiological testing, particularly BAEPs, can support differential diagnosis in patients with hereditary spastic paraplegias.