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Results: 3

FIG. 1

FIG. 1. Audio-cytocochleograms of the right and left ears. From: TEMPORAL BONE FINDINGS IN A CASE OF SUSAC'S SYNDROME.

The audiogram was done 2 weeks before death and showed a bilateral sensorineural hearing loss with reduced speech discrimination as shown. Graphic reconstruction of the cochlear was performed in each ear according to the method described by Schuknecht (Merchant, 2010). Missing or abnormal elements are shown in black. Vertical axes of cytocochleogram boxes for the stria and neurons show percentage of loss. Neuronal loss was compared with age-matched controls. Both ears showed severe atrophy and degeneration of outer and inner hair cells, tectorial membrane, and stria vascularis in the apical halves of the cochleae. The cochlear neurons were unaffected. IHC, inner hair cell; OHC, outer hair cell; IPC, inner pillar cells; OPC, outer pillar cells.

Howard W. Francis, et al. Otol Neurotol. ;32(8):1198-1204.
FIG. 3

FIG. 3. Word recognition analysis of contributing regions. From: TEMPORAL BONE FINDINGS IN A CASE OF SUSAC'S SYNDROME.

Standard pure tone audiograms are seen on the right, with speech intelligibility boxes for each ear on the left. The horizontal axis of these boxes is word level (dB HL), and the vertical axis is percent correct (standard monosyllable tests). The patient’s score is the central tic in the rectangular data symbol (score and level), with the rectangle enclosing the 95% confidence interval. The continuous curves in each box are scores across level predicted by the Speech Intelligibility Index. Low frequencies were removed from this calculation by removing these thresholds from the audiograms. The result shows that the scores may be predicted (particularly on the left) on the basis of a lack of contribution from the apical regions of these cochleae. Also see text.

Howard W. Francis, et al. Otol Neurotol. ;32(8):1198-1204.
FIG. 2

FIG. 2. Otopathologic findings in the right ear. From: TEMPORAL BONE FINDINGS IN A CASE OF SUSAC'S SYNDROME.

A, Low power view of the midmodiolar section of the cochlea. Cochlear neurons, Reissner’s membrane, and fluid spaces seem unremarkable. There is no endolymphatic hydrops. Note cellular debris within the saccule. IAC, internal auditory canal. B, Lower basal turn showing the healthy-appearing organ of Corti (OoC), tectorial membrane, spiral limbus, stria vascularis, and spiral ligament. C, Lower middle turn showing widespread atrophy and degeneration. The OoC was missing. The tectorial membrane was retracted and partly covered by cells. The spiral limbus showed patchy atrophy, and dendrites were missing within the osseous spiral lamina. The stria vascularis was completely atrophic. There was severe loss of fibrocytes within the spiral ligament. D, Upper middle turn showing widespread abnormalities, similar to C. The OoC was represented by a mound of cells. The tectorial membrane was detached from the limbus and encapsulated by cells. The stria was severely atrophic. E, Distal modiolus showing intact cochlear neurons and several branches of the main cochlear artery (black arrows). These blood vessels seemed unremarkable. F, Lateral cochlear wall of the middle turn showing near-total atrophy of the stria vascularis. Arrows pointing to the left depict 3 capillaries within the stria, which seemed to be partly occluded. Arrow pointing to the right shows a capillary within the spiral ligament that seemed more normal, containing red blood cells. G, Middle turn showing partial atrophy of the stria vascularis and an occluded strial capillary (white arrows). H, IAC. Inset shows a higher power view of the boxed area. Black arrows point to 4 arterioles within the leptomeninges showing occlusion of their lumen.

Howard W. Francis, et al. Otol Neurotol. ;32(8):1198-1204.

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