Otopathology of Vasculitis in Granulomatosis With Polyangitis

Abstract

Objective: To describe the temporal bone histopathology of vasculitis in granulomatosis with polyangitis.

Background: Granulomatosis with polyangitis is an autoimmune disease that presents as granulomatosis and vasculitis. Otologic findings, including otitis media, hearing loss, vertigo, and facial paralysis are common in this condition.

Material and methods: The temporal bones of four subjects with manifestations of vasculitis attributed to granulomatosis with polyangitis were studied under light microscopy.

Results: The four subjects had manifestations of vasculitis including hemorrhage within the cochlea and vestibule, and inflammation and occlusion of vessels in the lateral cochlear wall and the vasa nervorum of the facial nerve.

Conclusions: We infer that sensorineural hearing loss, vestibulopathy, and facial nerve paresis in granulomatosis with polyangitis can be the results of vasculitis.

Figure 1

Audiogram of the right ear 1 week prior to death shows a mixed hearing loss

Figure 2

Case I. Photomicrograph of the right middle ear. There was granulation tissue filling the mesotympanum. The ossicles are anatomically intact but surrounded by inflammatory tissue. Malleus (a), Incus (b).

Figure 3

Case I. A. The vasa nervorum in the proximal vertical segment of the facial nerve is occluded. There was a marked lymphocyte infiltration (arrows). B. Distal to the vasculitis there was vacuolization of the vertical segment of the facial nerve.

Figure 3

Case I. A. The vasa nervorum in the proximal vertical segment of the facial nerve is occluded. There was a marked lymphocyte infiltration (arrows). B. Distal to the vasculitis there was vacuolization of the vertical segment of the facial nerve.

Figure 4

Case I. A. The scala media in the basal turn of the cochlea. B. Vessels in the stria vascularis and the spiral ligament were thickened and in some instances occluded (arrows). Punctate areas of hemosiderin deposition suggest prior hemorrhage (arrow heads.)

Figure 4

Case I. A. The scala media in the basal turn of the cochlea. B. Vessels in the stria vascularis and the spiral ligament were thickened and in some instances occluded (arrows). Punctate areas of hemosiderin deposition suggest prior hemorrhage (arrow heads.)

Figure 5

Case I. In the hook region of the cochlear duct there was hemorrhage. Arrows point to erythrocytes.

Figure 6

Case II. The audiogram shows an asymmetric down sloping sensorineural hearing loss with a more pronounced loss of speech discrimination on the right.

Figure 7

Case II. There was ischemic necrosis of the lateral crista with detached sensory epithelium and cupula. These changes are attributable to ischemia in the distribution of the ampullary branch of the anterior vestibular artery (not shown.)

Figure 8

Audiocytocochleogram for case III. There is a severe sensorineural hearing loss prior to death. Speech discrimination scores were not available. In the cytocochleogram black bars indicate loss. Overall the hair cells and spiral ganglion cells are well preserved with predominant loss of the stria vascularis.

Figure 9

Case III. Hemosiderin deposition (arrow heads) in the stria vascularis.

Figure 10

Case IV. Vasculitis (arrow) of the vasa nervorum in the tympanic segment of the facial nerve.