ArticleBrain : a journal of neurology2013
Loudness adaptation accompanying ribbon synapse and auditory nerve disorders.
Article in Brain : a journal of neurology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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Who cites it
28 citing papers in PubMed, 58 citations in OpenAlex.
- Distinct genetic patterns and natural history of OPA1-related auditory neuropathy in Chinese population.Orphanet journal of rare diseases · 2025Article
- Cochlear Synaptopathy in Full-Term Neonates: A Case-Control Study of NICU Hospitalization Impact.Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India · 2025Article
- Auditory brainstem responses as a biomarker for cognition.Communications biology · 2024Article
- Tone Decay Reconsidered: Preliminary Results of a Prospective Study in Hearing-Aid Users with Moderate to Severe Hearing Loss.Journal of clinical medicine · 2024Article
- The natural history, clinical outcomes, and genotype-phenotype relationship of otoferlin-related hearing loss: a systematic, quantitative literature review.Human genetics · 2023Review
- Auditory neuropathy in mice and humans with Friedreich ataxia.Annals of clinical and translational neurology · 2023Article
- Temperature-Sensitive Auditory Neuropathy: Report of a Novel Variant of OTOF Gene and Review of Current Literature.Medicina (Kaunas, Lithuania) · 2023Article
- Current Advances in Gene Therapies of Genetic Auditory Neuropathy Spectrum Disorder.Journal of clinical medicine · 2023Review
- A Home-Based Approach to Auditory Brainstem Response Measurement: Proof-of-Concept and Practical Guidelines.Seminars in hearing · 2022Review
- Auditory Dysfunction Among Individuals With Neurofibromatosis Type 1.JAMA network open · 2021Article
- Sound level context modulates neural activity in the human brainstem.Scientific reports · 2021Article
- Familial Temperature-Sensitive Auditory Neuropathy: Distinctive Clinical Courses Caused by Variants of theFrontiers in cell and developmental biology · 2021Article
- The Many Faces of DFNB9: RelatingGenes · 2020Review
- Effect of Quiet and Noise on P300 Response in Individuals with Auditory Neuropathy Spectrum Disorder.International archives of otorhinolaryngology · 2020Article
- Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports.Journal of clinical medicine · 2020Review
- High Frequency ofNeural plasticity · 2020Article
- Auditory synaptopathy, auditory neuropathy, and cochlear implantation.Laryngoscope investigative otolaryngology · 2019Review
- Hearing Dysfunction in a Large Family Affected by Dominant Optic Atrophy (OPA8-Related DOA): A Human Model of Hidden Auditory Neuropathy.Frontiers in neuroscience · 2019Article
- A dual-AAV approach restores fast exocytosis and partially rescues auditory function in deaf otoferlin knock-out mice.EMBO molecular medicine · 2019Article
- Assessment of auditory functions in chronic hepatitis C patients treated by sofosbuvir.Journal of otology · 2018Article
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
Abstract
Abnormal auditory adaptation is a standard clinical tool for diagnosing auditory nerve disorders due to acoustic neuromas. In the present study we investigated auditory adaptation in auditory neuropathy owing to disordered function of inner hair cell ribbon synapses (temperature-sensitive auditory neuropathy) or auditory nerve fibres. Subjects were tested when afebrile for (i) psychophysical loudness adaptation to comfortably-loud sustained tones; and (ii) physiological adaptation of auditory brainstem responses to clicks as a function of their position in brief 20-click stimulus trains (#1, 2, 3 … 20). Results were compared with normal hearing listeners and other forms of hearing impairment. Subjects with ribbon synapse disorder had abnormally increased magnitude of loudness adaptation to both low (250 Hz) and high (8000 Hz) frequency tones. Subjects with auditory nerve disorders had normal loudness adaptation to low frequency tones; all but one had abnormal adaptation to high frequency tones. Adaptation was both more rapid and of greater magnitude in ribbon synapse than in auditory nerve disorders. Auditory brainstem response measures of adaptation in ribbon synapse disorder showed Wave V to the first click in the train to be abnormal both in latency and amplitude, and these abnormalities increased in magnitude or Wave V was absent to subsequent clicks. In contrast, auditory brainstem responses in four of the five subjects with neural disorders were absent to every click in the train. The fifth subject had normal latency and abnormally reduced amplitude of Wave V to the first click and abnormal or absent responses to subsequent clicks. Thus, dysfunction of both synaptic transmission and auditory neural function can be associated with abnormal loudness adaptation and the magnitude of the adaptation is significantly greater with ribbon synapse than neural disorders.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.