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Three-Dimensional Histopathologic Correlates of Cochlear Hypoplasia Type II Versus Incomplete Partition Type II
Chloe Swords1,2, Christopher Giardina2,3, MengYu Zhu2,3
1Department of Physiology, Development and Neuroscience, University of Cambridge, Cambridge, UK.
Hypothesis:
Cochlear hypoplasia type-II (CH-II) and incomplete partition type-II (IP-II) may display measurable differences in modiolar: cochlear area ratios and interscalar ridge angles correlating with spiral ganglion neuron (SGN) counts.
Background:
Inner ear malformations (IEM) can cause variable sensorineural hearing loss and cochlear implant outcomes. CH-II and IP-II share overlapping imaging features. Subtle differences in bony cochlear architecture may underlie distinct SGN patterns.
Methods:
IP-II (n=11), CH-II (n=5), and normal controls (n=16) were identified from a digitised human temporal bone histologic collection. Radiologically relevant assessments of midmodiolar 2-dimensional (2D) morphology, including interscalar ridge angles and modiolar: cochlear area ratio, were compared with controls. SGNs were quantified. Digital 3-dimensional (3D) reconstructions were created to appreciate the difference in anatomy further.
Results:
On midmodiolar sections, interscalar septum deficiency, indicating fusion of perilymph-containing scalae, occurred between upper basal and upper middle turns (CH-II, IP-II) and lower middle and apical turns (IP-II only) of the cochlea. Compared with normal cochleae, both CH-II and IP-II cases had a smaller modiolar: cochlear area ratio (P<0.01), and flattened interscalar ridge angles (attachment of interscalar septum to inner cochlea lumen; P<0.01). In CH-II, SGN counts were markedly reduced, ranging from 0% to 20% (mean 8%) of SGN counts in age-matched normally formed cochleae (normal hearing ears); IP-II ranged from 5% to 95% (mean 57%) of age-matched normal cochleae.
Conclusions:
Given the reduced SGN counts in CH-II compared with normal and IP-II, accurate radiologic subclassification of CH-II versus IP-II is necessary to optimise candidacy selection and counselling for cochlear implantation. A predictive algorithm is suggested based on histologic criteria that might help distinguish between IP-II and CH-II cochleae.
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