ArticleJournal of clinical medicine2024
Diffusion-Weighted Magnetic Resonance Imaging (dMRI) and Cochlear Implant Outcomes in Axonal Auditory Neuropathy: A Case Report.
Article in Journal of clinical medicine, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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Who cites it
1 citing paper in PubMed.
- Diffusion-Weighted Magnetic Resonance Imaging: A Diagnostic Tool for Auditory (Axonal) Neuropathy.European journal of neurology · 2025Article
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4 authors.
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Abstract
backgroundProgressive auditory dysfunction is common in patients with generalized neurodegenerative conditions, but clinicians currently lack the diagnostic tools to determine the location/degree of the pathology and, hence, to provide appropriate intervention. In this study, we present the white-matter microstructure measurements derived from a novel diffusion-weighted magnetic resonance imaging (dMRI) technique in a patient with axonal auditory neuropathy and consider the findings in relation to the auditory intervention outcomes.
methodsWe tracked the hearing changes in an adolescent with Riboflavin Transporter Deficiency (Type 2), evaluating the sound detection/discrimination, auditory evoked potentials, and both structural- and diffusion-weighted MRI findings over a 3-year period. In addition, we explored the effect of bilateral cochlear implantation in this individual.
resultsBetween the ages of 15 years and 18 years, the patient showed a complete loss of functional hearing ability. The auditory brainstem response testing indicated an auditory neuropathy with evidence of normal cochlear function but disrupted auditory neural activity. While three structural MRI assessments across this period showed a clinically normal cochleovestibular anatomy, the dMRI evaluation revealed a significant loss of fiber density consistent with axonopathy. The subsequent cochlear implant function was affected with the high levels of current required to elicit auditory sensations and concomitant vestibular and facial nerve stimulation issues.
conclusionsThe case study demonstrates the ability of dMRI technologies to identify the subtle white-matter microstructure changes in the auditory pathway, which may disrupt the neural function in patients with auditory axonopathy.
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