ObjectiveTo investigate the temporal bone histopathology of vasculitis, especially in the vestibular organs, in granulomatosis with polyangitis (GPA).MethodsUsing light and differential interference contrast microscopy, we examined 12 human temporal bones from six deceased GPA patients and 12 histopathologically normal human temporal bones from six deceased age‐matched patients.ResultsIn the GPA group, three patients had undergone tympanostomy tube placement. Two of them had suffered mixed hearing loss; one, sensorineural hearing loss; and one, conductive hearing loss. Of the 12 specimens in the GPA group, the granulation tissue invaded the round window niche in seven; cochlear hair cells were not preserved in five. Hemosiderin was deposited in the stria vascularis in eight specimens, in the ampulla or semicircular duct in 10, and in the vestibule in three. The spiral ligament showed severe loss of cellularity in two specimens. In the GPA group, type I vestibular hair cell density was significantly decreased; however, type II vestibular hair cell density did not significantly differ between the GPA group and the control group.ConclusionOur histopathologic findings in human temporal bone specimens of GPA patients delineated changes in the tympanic membrane, middle ear cavity, round window membrane, organ of Corti, stria vascularis, spiral ligament, ampulla, semicircular duct, and vestibule. Type I vestibular hair cell density significantly decreased in the GPA group, as compared with the control group.Level of EvidenceN/A
Rationale: IgG4-related diseases cause lesions in various organs throughout the body. In otorhinolaryngology, IgG4-related Mikulicz’s disease is suspected and diagnosed based on the presence of lesions of the head and neck, salivary and lacrimal gland enlargement, and bilateral sinus opacity concentrated on the maxillary sinuses. However, in some cases, it is necessary to consider about differentiation between IgG4-related Mikulicz’s disease and Sjögren syndrome. Patient concerns and diagnosis: A 75-years-old male patient visited our hospital with bilateral otitis media with effusion, which was resistant to conservative treatment. Other symptoms at presentation included enlarged bilateral submandibular and sublingual glands marked oral dryness, severe decrease in saliva secretion (1 mL/10 minutes), and dry eyes. We conducted a Schirmer’s and fluorescent dye tests, both of which were positive. High serum IgG4 levels were observed, and although the Sjögren syndrome (SS)-A/SS-B antibodies were negative, marked hypolacrimation and tear secretion were observed. Therefore, a detailed examination considering both IgG4-related Mikulicz’s disease and SS was conducted. Salivary gland scintigraphy performed prior to the salivary gland biopsy revealed a marked decrease in uptake, which satisfied the diagnostic criteria for SS; however, it was difficult to diagnose IgG4-related disease based on the diagnostic definition. Intervensions: Although a definitive diagnosis of SS was made, the persistent otitis media with effusion that was resistant to conservative treatment and bilateral mixed hearing loss were confirmed. As mixed hearing loss is considered an otological symptom of IgG4-related disease, oral steroid treatment was administered. Outcome: Thereafter, marked recovery of hearing and reduced swelling and induration of the bilateral parotid and submandibular glands were observed. Clinically, IgG4-related Mikulicz’s disease was strongly suspected, but a definite diagnosis of SS was made. lessons: In the absence of an IgG4-related Mikulicz’s disease diagnosis, careful differentiation between IgG4-related Mikulicz’s disease and 2 diseases and their diagnostic criteria was essential.
The position, configuration, and size of the tensor tympani muscle and tendon do not seem to play a role in the pathogenesis of Menière's disease. Because the tympanic isthmus and protympanum in Menière's disease are not smaller than controls and that none of the temporal bones had otitis media or cholesteatoma, it is unlikely that there was dysventilation in the middle ear.
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