Dynamic Heterogeneity Shapes Patterns of Spiral Ganglion Activity

Objective To perform an otopathologic analysis of temporal bones (TBs) with CHARGE syndrome. Study Design Otopathologic study of human TB specimens. Setting Otopathology laboratories. Methods From the otopathology laboratories at the University of Minnesota and Massachusetts Eye and Ear Infirmary, we selected TBs from donors with CHARGE syndrome. These TBs were serially sectioned at a thickness of 20 µm, and every 10th section was stained with hematoxylin and eosin. We performed otopathologic analyses of the external ear, middle ear (middle ear cleft, mucosal lining, ossicles, mastoid, and facial nerve), and inner ear (cochlea, vestibule, internal auditory canal, and cochlear and vestibular nerves). The gathered data were statistically analyzed. Results Our study included 12 TBs from 6 donors. We found a high prevalence of abnormalities affecting the ears. The most frequent findings were stapes malformation (100%), aberrant course of the facial nerve (100%) with narrow facial recess (50%), sclerotic and hypodeveloped mastoids (50%), cochlear (100%) and vestibular (83.3%) hypoplasia with aplasia of the semicircular canals, hypoplasia and aplasia of the cochlear (66.6%) and vestibular (91.6%) nerves, and narrowing of the bony canal of the cochlear nerve (66.6%). The number of spiral ganglion and Scarpa’s ganglion neurons were decreased in all specimens (versus normative data). Conclusions In our study, CHARGE syndrome was associated with multiple TB abnormalities that may severely affect audiovestibular function and rehabilitation.

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Meta-Analysis—Correlation between Spiral Ganglion Cell Counts and Speech Perception with a Cochlear Implant

Audiology Research ◽

10.3390/audiolres11020020 ◽

2021 ◽

Vol 11 (2) ◽

pp. 220-226

Author(s):

Yew-Song Cheng ◽

Mario A. Svirsky

Keyword(s):

Speech Perception ◽

Cochlear Implant ◽

Ganglion Cells ◽

Meta Analysis ◽

Spiral Ganglion ◽

Spiral Ganglion Cell ◽

Cell Counts ◽

Temporal Bones ◽

The Relationship ◽

Existing Data

The presence of spiral ganglion cells (SGCs) is widely accepted to be a prerequisite for successful speech perception with a cochlear implant (CI), because SGCs provide the only known conduit between the implant electrode and the central auditory system. By extension, it has been hypothesized that the number of SGCs might be an important factor in CI outcomes. An impressive body of work has been published on findings from the laborious process of collecting temporal bones from CI users and counting the number of SGCs to correlate those numbers with speech perception scores, but the findings thus far have been conflicting. We performed a meta-analysis of all published studies with the hope that combining existing data may help us reach a more definitive conclusion about the relationship between SGC count and speech perception scores in adults.

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Complex distribution patterns of voltage-gated calcium channel α-subunits in the spiral ganglion

Hearing Research ◽

10.1016/j.heares.2011.01.016 ◽

2011 ◽

Vol 278 (1-2) ◽

pp. 52-68 ◽

Cited By ~ 26

Author(s):

Wei Chun Chen ◽

Hui Zhong Xue ◽

Yun (Lucy) Hsu ◽

Qing Liu ◽

Shail Patel ◽

...

Keyword(s):

Calcium Channel ◽

Spiral Ganglion ◽

Distribution Patterns ◽

Voltage Gated ◽

Voltage Gated Calcium Channel ◽

Α Subunits ◽

Complex Distribution

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Vascular Supply of the Human Spiral Ganglion: Novel Three-Dimensional Analysis Using Synchrotron Phase-Contrast Imaging and Histology

Scientific Reports ◽

10.1038/s41598-020-62653-0 ◽

2020 ◽

Vol 10 (1) ◽

Cited By ~ 2

Author(s):

Xueshuang Mei ◽

Rudolf Glueckert ◽

Annelies Schrott-Fischer ◽

Hao Li ◽

Hanif M. Ladak ◽

...

Keyword(s):

Blood Supply ◽

Phase Contrast ◽

Spiral Ganglion ◽

Three Dimensional ◽

Vascular Anatomy ◽

Helical Structure ◽

Vascular Supply ◽

Phase Contrast Imaging ◽

Contrast Imaging ◽

Temporal Bones

AbstractHuman spiral ganglion (HSG) cell bodies located in the bony cochlea depend on a rich vascular supply to maintain excitability. These neurons are targeted by cochlear implantation (CI) to treat deafness, and their viability is critical to ensure successful clinical outcomes. The blood supply of the HSG is difficult to study due to its helical structure and encasement in hard bone. The objective of this study was to present the first three-dimensional (3D) reconstruction and analysis of the HSG blood supply using synchrotron radiation phase-contrast imaging (SR-PCI) in combination with histological analyses of archival human cochlear sections. Twenty-six human temporal bones underwent SR-PCI. Data were processed using volume-rendering software, and a representative three-dimensional (3D) model was created to allow visualization of the vascular anatomy. Histologic analysis was used to verify the segmentations. Results revealed that the HSG is supplied by radial vascular twigs which are separate from the rest of the inner ear and encased in bone. Unlike with most organs, the arteries and veins in the human cochlea do not follow the same conduits. There is a dual venous outflow and a modiolar arterial supply. This organization may explain why the HSG may endure even in cases of advanced cochlear pathology.

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