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Clinical Note |

Histopathologic Features of the Temporal Bone in Usher Syndrome Type I

Mariette Wagenaar, MD, PhD; Harold Schuknecht, MD, PhD; Joseph Nadol Jr, MD, PhD; Meeke Benraad-van Rens, MD; Sandra Pieke-Dahl; William Kimberling, PhD; Cor Cremers, MD, PhD
Arch Otolaryngol Head Neck Surg. 2000;126(8):1018-1023. doi:10.1001/archotol.126.8.1018.
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Temporal bones of 2 patients with Usher syndrome type I were examined using light microscopy. In both patients, findings from histopathologic examination of the cochlea were characterized by degeneration of the organ of Corti, which was most marked in the basal turn, atrophy of the stria vascularis, and a decrease in the number of spiral ganglion cells. The cochlear nerve appeared to be diminished. The sensory epithelium of the saccular and utricular maculae of patient 1 was normal for age. The left temporal bone of patient 2, classified as Usher syndrome genetic subtype USH1D or USH1F, demonstrated the typical signs of severe cochleosaccular degeneration. Present cases and cases from the literature were reviewed in search of an explanation for the above-described differences in histologic findings.

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Figure 1.

Pathological findings from the left cochlea of patient 1. Severe degeneration of the organ of Corti, atrophy of the stria vascularis, and loss of spiral ganglia cells and nerve fibers (hematoxylin-eosin, original magnification × 61).

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Figure 2.

Pathological findings in the left cochlea of patient 1.

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Figure 3.

Pathological findings from the left cochlea of patient 2. The organ of Corti is severely degenerated; inner and outer hair cells are missing. Severely disorganized stria vascularis, basophilic masses, and acidophilic globules can be seen (hematoxylin-eosin, original magnification × 110).

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Figure 4.

Pathological findings of the left cochlea of patient 2.

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Figure 5.

Pathological findings from the left vestibule of patient 2. There is a severely degenerated saccular macula. The saccular wall collapsed onto remnants of the otolithic membrane (hematoxylin-eosin, original magnification × 43).

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Figure 6.

Pedigree/haplotypes for unlinked genetic subtype USH1B (top) and linked genetic subtype USH1D or USH1F.

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