Objective: The cochlear nucleus (CN) is the target of the auditory brainstem implant (ABI). Most ABI candidates have Neurofibromatosis Type 2 (NF2) and distorted brainstem anatomy from bilateral vestibular schwannomas. The CN is difficult to characterize as routine structural MRI does not resolve detailed anatomy. We hypothesize that diffusion tensor imaging (DTI) enables both in vivo localization and quantitative measurements of CN morphology.
Study Design: We analyzed 7 Tesla (T) DTI images of 100 subjects (200 CN) and relevant anatomic structures using an MRI brainstem atlas with submillimetric (50 μm) resolution.
Setting: Tertiary referral center.
Patients: Young healthy normal hearing adults.
Intervention: Diagnostic.
Main Outcome Measures: Diffusion scalar measures such as fractional anisotropy (FA), mean diffusivity (MD), mode of anisotropy (Mode), principal eigenvectors of the CN, and the adjacent inferior cerebellar peduncle (ICP).
Results: The CN had a lamellar structure and ventral-dorsal fiber orientation and could be localized lateral to the inferior cerebellar peduncle (ICP). This fiber orientation was orthogonal to tracts of the adjacent ICP where the fibers run mainly caudal-rostrally. The CN had lower FA compared to the medial aspect of the ICP (0.44 ± 0.09 vs. 0.64 ± 0.08, p < 0.001).
Conclusions: 7T DTI enables characterization of human CN morphology and neuronal substructure. An ABI array insertion vector directed more caudally would better correspond to the main fiber axis of CN. State-of-the-art DTI has implications for ABI preoperative planning and future image guidance-assisted placement of the electrode array.
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http://dx.doi.org/10.1097/MAO.0000000000002565 | DOI Listing |
Cochlear Implants Int
December 2024
Department of ENT and Head & Neck Surgery, Seth GS Medical College & K.E.M. Hospital, Mumbai, India.
Introduction: Wolfram syndrome, a rare autosomal recessive disorder, is characterised by diabetes insipidus, juvenile diabetes mellitus, optic nerve atrophy and deafness (DIDMOAD).
Case Report: We present a case of a 21-year-old male diagnosed with Wolfram syndrome who underwent cochlear implantation due to progressive hearing loss. The patient first complained of bilateral hearing loss at the age of 8 years.
Ear Hear
December 2024
Department of Medical Bionics, University of Melbourne, Melbourne, Australia.
Objectives: Cochlear implants (CIs) have revolutionized hearing restoration for individuals with severe or profound hearing loss. However, a substantial and unexplained variability persists in CI outcomes, even when considering subject-specific factors such as age and the duration of deafness. In a pioneering study, we use resting-state functional near-infrared spectroscopy to predict speech-understanding outcomes before and after CI implantation.
View Article and Find Full Text PDFJAMA Otolaryngol Head Neck Surg
December 2024
Department of Hearing and Speech Sciences, Vanderbilt University Medical Center, Nashville, Tennessee.
Importance: Speech recognition outcomes with a cochlear implant (CI) are highly variable. One factor suggested to correlate with CI-aided speech recognition is frequency-to-place mismatch, or the discrepancy between the natural tonotopic organization of the cochlea and the electric frequency allocation of the CI electrodes within the patient's cochlea.
Objective: To evaluate the association between frequency-to-place mismatch and speech recognition outcomes in a large cohort of postlingually deafened adult CI users, while controlling for various clinical factors known to be associated with those outcomes.
J Neurosci
December 2024
Dept. Biological Sciences, University at Buffalo, State University of New York, Buffalo, NY 14260
The mammalian auditory system encodes sounds with subtypes of spiral ganglion neurons (SGNs) that differ in sound level sensitivity, permitting discrimination across a wide range of levels. Recent work suggests the physiologically-defined SGN subtypes correspond to at least three molecular subtypes. It is not known how information from the different subtypes converges within the cochlear nucleus.
View Article and Find Full Text PDFJ Comp Neurol
December 2024
School of Biomedical Sciences, The Chinese University of Hong Kong, Shatin, Hong Kong SAR, China.
A gene cadre orchestrates the normal development of sensory and non-sensory cells in the inner ear, segregating the cochlea with a distinct tonotopic sound frequency map, similar brain projection, and five vestibular end-organs. However, the role of genes driving the ear development is largely unknown. Here, we show double deletion of the Iroquois homeobox 3 and 5 transcription factors (Irx3/5 DKO) leads to the fusion of the saccule and the cochlear base.
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