Shiroh Maguchi

Otologic Manifestations of Wegener's Granulomatosis

Objective/Hypothesis To evaluate the clinical features, treatment, and outcomes of otologic manifestations in Wegeners granulomatosis (WG) treated at Hokkaido University Graduate School of Medicine, Sapporo, Japan.

Myeloperoxidase-antineutrophil cytoplasmic antibody-associated sensorineural hearing loss

A 36-year-old female with hyperthyroidism that had been treated with propilthiouracil (PTU) complained of tinnitus and hearing loss in both ears. She was treated with steroid administration by an otolaryngologist; however, hearing continued to fluctuate when the steroids were tapered. Laboratory evaluation revealed a decreased complement level and elevated levels of immunoglobulin M (IgM) and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). With the withdrawal of PTU and high-dose methylprednisolone, she had excellent return of right-sided hearing. In recent years, there have been many reports about MPO-ANCA-associated small vessel vasculitis. Although any organ may be affected by this disease, there are no reports about MPO-ANCA-associated progressive hearing loss without any other organ involvement. The present case suggests the possibility that inner ear blood flow impairment due to ANCA-associated small vessel vasculitis induces the so-called autoimmune sensorineural hearing loss.

Histological findings in biopsies from patients with cytoplasmic-antineutrophil cytoplasmic antibody (cANCA)-positive Wegener's granulomatosis

OBJECTIVE Currently Wegeners granulomatosis (WG) is diagnosed from clinical features, a test for antineutrophil cytoplasmic antibody (ANCA) and histological confirmation. The purpose of our study was to evaluate histological findings, in particular those in the biopsy samples of head and neck region. METHODS Fifty-one biopsy specimens from 24 WG patients who were confirmed to be positive for cANCA were examined retrospectively. RESULTS Only 12 biopsies out of 51 biopsies (24%) were diagnosed as typical of WG, and diagnosis could be confirmed in only 10 patients (42%) as typical of WG from the results of multiple biopsy specimens. CONCLUSION In the diagnosis of WG. biopsy can be utilized restrictedly only in the cases when cANCA is negative or clinical symptoms are atypical of WG.

Clinical features of bilateral progressive hearing loss associated with myeloperoxidase-antineutrophil cytoplasmic antibody.

In this study, we analyzed the clinical features, diagnostic criteria, treatment, and outcome of atypical bilateral progressive hearing loss associated with myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). The evaluation was made by audiogram and laboratory data in 6 cases treated at Hokkaido University Hospital. We measured MPO-ANCA by enzyme-linked immunosorbent assay; the result was positive in all cases. The hearing loss developed bilaterally and progressed to total deafness within several weeks. The onset of hearing loss was not simultaneous in the 2 ears. Methylprednisolone pulse therapy was effective in 9 ears. Three ears showed no improvement; 2 of the 3 ears had already exhibited total deafness at the first visit. After the treatment. the titer of MPO-ANCA decreased in all patients. The level of MPO-ANCA should be tested in patients with bilateral progressive hearing loss of unknown origin and can be used as a marker of the diseases activity.

Relapsing polychondritis: a report of eight cases.

OBJECTIVE To investigate clinical manifestations of relapsing polychondritis and to clarify the significance of type II collagen antibody in the disease. METHODS Clinical manifestations and antibody titers were examined in eight cases of relapsing polychondritis which had been treated at Otolaryngology Department, Hokkaido University during the eight years from 1991 to 1998. Anti-type II collagen antibody titer was measured by ELISA method. RESULTS The most frequent symptom was auricular chondritis; it was seen in 88% (7/8) of the cases. Ocular symptom, nasal chondritis, arthritis, respiratory tract chondritis, and audio-vestibular symptom were also common in the cases. Compared with previous reports, no difference was recognized in the manifestation frequency. Considering none of the samples from the controls was positive for anti-type II collagen antibody, two samples from the disease group were positive. The antibody positive rate was 25% (2/8). CONCLUSION Though the measurement of type II collagen antibody titer is not a decisive factor for detection of relapsing polychondritis, it is useful as one of the complementary factors for the diagnosis, since there is no specific test for this disease.

Springtime pollinosis and oral allergy syndrome in Sapporo

OBJECTIVE The purpose of this present study was to investigate pollinosis in the spring and oral allergy syndrome (OAS) in Sapporo to utilize it for future treatment. MATERIALS AND METHODS Of the patients referred to our out-patient clinic during April and May in 1999. all those that were suspected to have pollinosis were asked to fill in a questionnaire. A 101 patients (30 males and 71 females) with a mean age of 33 years (range 7-74 years) answered the questionnaire. In all, the patients specific IgE tests were performed. RESULTS The most common allergen was birch, affecting 54 of 87 patients (62%). In 61% of patients with birch allergy, we observed fruit and vegetable allergy. Among them, apple was the most prevalent allergen (97%), followed by peach (67%), cherry (58%), pear (40%), plum (40%) and melon (33%). The sensitivity of skin prick test with commercial extract to apple was low (11%). CONCLUSION Patients with birch pollen allergy had OAS much more frequently than ever reported in Japan, although OAS has not widely been known in Japan. Patients with birch pollen allergy should be informed about the possibility of hypersensitivity to certain fruits.

Glycolipids in Rat Cochlea

In recent years, the functions of glycolipids have been intensively studied. Before the research of the roles of glycolipids in the inner ear, it seemed to be necessary to demonstrate the composition of glycolipids at first. Therefore, rat cochlea has been examined for glycolipid composition in the present study. Glycolipids extracted from 200 cochlea samples were separated into neutral and acidic glycolipid fractions. Each fraction was analyzed by thin-layer chromatography. The neutral glycolipid was almost exclusively galactosylceramide (cerebroside) with trace amounts of globoside and unidentified glycolipids. In acidic glycolipids, sulfated galactosylceramide (cerebroside sulfate) was most abundant. Of the gangliosides (sialic acid-containing glycolipids), ganglioside GM3 (51.8%) was the predominant component, and GM2 (6.0%), GM1 (8.8%), GD3 (10.7%), GD1a (8.7%), GD1b (5.9%), and GT1b (3.6%) were also detected. In addition to these, rat cochlea also contained several, minor, unidentified gangliosides. This pattern of the cochlear acidic glycolipids is very similar to that of the renal acidic glycolipids. This finding seems to be very interesting when considering that the toxic side effects of aminoglycosides occur only in the kidney and the inner ear.

Correlation between the levels of circulating adhesion molecules and PR3-ANCA in Wegener's granulomatosis.

OBJECTIVE The adhesion of neutrophils to the vascular endothelial cells appears to be important for the pathogenesis of vasculitis in Wegeners granulomatosis (WG). To determine the pathological significance of the neutrophil adhesion, we have examined and evaluated the relationships between the levels of adhesion molecules and proteinase-3 anti-neutrophil cytoplasmic antibodies (PR3-ANCA), and between the levels of TNF-alpha and disease activities in WG. METHODS sELAM-1 and sICAM-1 in sera from WG and healthy donors were detected by ELISA. SLex and LFA-1 on neutrophils were detected by flow cytometry. RESULTS The level of sICAM-1 was significantly higher in active WG than in inactive WG, tending to reflect individual disease activities. There was no difference in the level of sELAM-1 between WG patients and healthy donors. CONCLUSION Our study suggests that the adhesion of neutrophils to the vascular endothelium is significant for the pathogenesis of the vasculitis in WG.

Taxus cuspidata (Japanese yew) pollen nasal allergy

We have investigated the Taxus cuspidata pollinosis that has never been studied before. We applied immunoblotting method in order to detect specific IgE antibody against T. cuspidata using its pollen itself. Out of 18 patients with seasonal allergic rhinitis suffering mainly in April and May, 5 patients were sensitized with this pollen. Furthermore, we were able to diagnose one case as T. cuspidata pollinosis from the clinical and laboratory findings. While the birch pollen is the main causative pollen in the spring, T. cuspidata is one of the minor allergens causing pollinosis in our district.