True hemangiopericytoma of the nasal cavity.

Two cases of nasal tumors with pericytic myoid differentiation are reported. The tumors occurred in a 77-year-old woman and a 60-year-old man as polypoid lesions covered by normal mucosa. Histologically, the tumors were composed of uniform short spindle or stellate cells with indistinct cell borders arranged in narrow and short fascicles. Numerous blood vessels of various sizes were common in both cases. The tumor cells of both cases stained intensely with anti-vimentin and anti-actin antibodies, but not with anti-desmin, CD34, or anti-high-molecular-weight caldesmon antibodies. Ultrastructural examination revealed well-developed actin thin filaments with dense bodies, subplasmalemmal plaques, intercellular junctions, and irregular discontinuous basement membranes. These histopathologic features suggest true pericytic differentiation of the tumors (true hemangiopericytoma), unlike soft tissue-type hemangiopericytoma. Generally, sinonasal hemangiopericytomas are subdivided into soft tissue-type hemangiopericytomas and true hemangiopericytomas identical to the cases presented here. Soft tissue-type hemangiopericytomas are frequently highly aggressive, whereas true hemangiopericytomas show localized benign behavior. Sinonasal true hemangiopericytomas should be strictly differentiated from soft tissue-type hemangiopericytomas.

Temporal bone pathology in Cornelia de Lange syndrome.

OBJECTIVE: This study aimed to demonstrate the temporal bone histopathology of Cornelia de Lange syndrome. STUDY DESIGN: Histopathologic examination of an infant with this syndrome. SETTING: This study was performed at the temporal bone laboratory. Fukushima Medical University, Fukushima. RESULTS: The authors describe interesting temporal bone findings of this syndrome: anomalies of the middle and inner ear and the facial nerves. CONCLUSIONS: This is the second case report describing the histologic findings of the temporal bone in Cornelia de Lange syndrome in the English literature.

Monitoring of the thromboxane A2/prostacyclin ratio in the urine of patients with retinal vascular occlusion through the low-dose-aspirin therapy using the gas chromatography/selected ion monitoring method.

We determined the levels of the stable urinary metabolites of thromboxane A2 and prostacyclin, 11-dehydro-thromboxane B2 (11-dehydro-TXB2) and 2,3-dinor-6-keto-prostaglandin F1alpha (2,3-dinor-6-keto-PGF1alpha) in patients with retinal vascular occlusion (RVO) to elucidate the change of the thromboxane A2/prostacyclin (TX/PGI) ratio with this disease and the effect of low-dose-aspirin therapy. 11-Dehydro-TXB2 and 2,3-dinor-6-keto-PGF1alpha were converted to 1-methyl ester-propylamide-9,12,15-tris-dimethylisopropylsilyl ether derivative and 1-methyl ester-6-methoxime-9,12,15-tris-dimethylisopropylsilyl ether derivative, respectively, and applied to a gas chromatography/selected ion monitoring. The average level of 11-dehydro-TXB2 in 30 patients with RVO was 1038 +/- 958 pg/mg creatinine. It was significantly higher than that of 27 healthy volunteers, which was 616 +/- 294 pg/mg creatinine (p < 0.05 with unpaired t-test). However, 2,3-dinor-6-keto-PGF1alpha levels were not significantly different between these two groups. The average ratio of TX/PGI in the RVO patients was 32 +/- 26 and it was significantly higher than that of healthy volunteers, 17 +/- 10 (p < 0.01). Patients with central retinal artery occlusion or branch retinal artery occlusion showed greatly high 11-dehydro-TXB2 levels and TX/PGI ratios, although the number of patients was limited in the current study. After the administration of low-dose aspirin (40 mg/day) for about 1 month, the TX/PGI ratio decreased to around the normal level. Following the levels for up to 10 months, they also remained at the normal level. These observations suggested that the 11-dehydro-TXB2 levels and the TX/PGI ratio reflect the pathological conditions of RVO and are useful markers of the treatment.

[An experimental study of ototoxicity induced by deferoxamine mesilate].

We investigated the possibility of ototoxicity induced by deferoxamine mesilate (DFO). DFO was administered to guinea pigs intraperitoneally at 200 or 400 mg/kg body weight/day, 6 days/week for 5 weeks, or 600 mg/kg body weight/day, 6 days/week for 4 weeks. Physiological saline instead of DFO was given to animals as a control. After the course of administration was completed, we measured the threshold of the compound action potential (CAP) and the endocochlear potential (EP). We also examined the cochlear histology by scanning electron microscopy. The CAP threshold of animals treated with 200 or 400 mg of DFO per kg was not elevated. In the animals treated with 600 mg/kg , however, the CAP threshold was clearly elevated when compared with that of control animals. The EP values of animals treated with 200 or 400 mg of DFO per kg were comparable to those of control animals. In some animals treated with 600 mg of DFO per kg, however, the EP values were decreased. Histological examination revealed that the outer hair cells of the cochlea were missing. DFO presumably acts on hair cells, resulting in elevation of the CAP threshold. We concluded that hair cell damage is one of the contributing factors to CAP threshold elevation.

Analysis of urinary 11-dehydrothromboxane B2 in patients with occluded retinal vein using GC/SIM.

We describe variations of 11-dehydrothromboxane B2(11-dehydro-TXB2) levels in human urine samples. Retinal vein occlusion (RVO) is a thrombotic disease in which the retinal vein is blocked by blood aggregations. We considered the possibility that 11-dehydro-TXB2 plays an important role in the formation of RVO. Thus, we determined the 11-dehydro-TXB2 levels in patients with RVO using gas chromatography/selected ion monitoring (GC/SIM) and compared them with those of healthy volunteers. The thromboxane levels in patients with RVO, who did not also have diabetes, were significantly higher than those in healthy volunteers. One cause of RVO may be the variation of thromboxane production. Furthermore, this GC/SIM method can be applied to the prevention and treatment of not only RVO, but also of general thrombosis.

Inner ear autoantibodies in patients with sensorineural hearing loss.

In order to identify the inner ear autoantibodies, sera of 195 patients with sensorineural hearing loss and/or vertigo were reached with bovine inner ear antigen by western blotting method. Positive reaction was seen at several molecular weights, in which 33-35 kD, 42 kD and 68 kD were highly reactive. In moderate to severe hearing loss patients, positive reactions with 33-35 kD, 42 kD, 68 kD and others were 25.8%, 26.8%, 26.8% and 71.5% respectively. On the other hand, a positive rate did not clearly correlate with diagnostic entities. Further study to purify inner ear antigen may evaluate inner ear autoimmune disease.

Visual outcome after vitrectomy for diabetic retinopathy. A five-year follow-up.

For 5 years after surgery we measured visual acuity in 56 eyes that had undergone pars plana vitrectomy for proliferative diabetic retinopathy. A total of 44.7% eyes maintained improved visual outcome, 19.6% had unchanged visual acuity, and 35.7% were considered failures, as compared to preoperative measurements. Visual results obtained from each patient's visual acuity measured at 3 months, 6 months, 1 year, and 3 years after surgery were statistically compared with that obtained from the visual acuity at 5 years using the chi 2 test. Results showed that visual results recorded at 3 months and 6 months were significantly different from that recorded at the 5-year follow-up (p < 0.02, respectively). We found a tendency for the visual acuity to change during the initial 6 months after surgery and to stabilize by 1 year. Further analysis showed that the initial instability was due to the presence of preoperative retinal detachment. Postoperative visual functions also worsened with complications such as postoperative retinal detachment, glaucoma, and cataract formation.

Inner ear-specific autoantibodies.

The sera of patients with idiopathic sensorineural hearing loss (SNHL) were examined, using qualitative immunoblotting (Western blotting), for the presence of inner ear (IE)-specific autoantibodies. The water-soluble extracts and the sodium dodecyl sulfate-soluble extracts were prepared as the antigens from bovine IE tissues and several other organs in order to determine the specificity of autoantibodies in their sera to the IE. Some patients (n = 46) tested had autoantibodies that reacted with 68-, 62-, 55-, 50-, or 47-kd antigenic determinants found in all tissues, while others (n = 13) showed IE-specific autoantibodies which reacted with 220-, 60-, 58-, 33-35-, or 32-kd determinants. The presence of these autoantibodies from patients with progressive SNHL may have important implications with regard to their etiology and possibly their sensitivity to therapeutic intervention. It is now necessary to purify these IE-specific antigens and determine their clinical usefulness.

Evidence of direct communication of bone marrow cells with the endolymphatic sac in experimental autoimmune labyrinthitis.

In experimental autoimmune labyrinthitis, we found that the guinea pigs sensitized with bovine inner ear antigen (IEAg) developed cellular infiltration within the endolymphatic sac (E. sac). In this study, we investigated the distribution of immune mediated cells within bony vascular channels. The channels, which are normally found around the E. sac, are tiny and contain few immune-mediated cells. In contrast, in guinea pigs sensitized with IEAg bony vascular channels were large and contained immune-mediated cells. We hypothesize that immune-mediated cells in these channels are the result of the immune response directed against inner ear antigen, and that some immune-mediated cells seen in the E. sac during inner ear inflammatory events have migrated from the adjacent bone marrow.

Extraction of inner ear antigens for studies in inner ear autoimmunity.

The search for a diagnostic assay in patients with autoimmune inner ear disease has led to the preparation of antigens from the inner ear, a presumed target in this disorder. In order to standardize the antigen preparations currently being used in the Western blot immunoassay, we have examined several distinct extraction procedures that employ well-known solutions and detergents. Results of this investigation clearly show that antigens of interest (68 kd, 33 to 35 kd, and 32 kd) are optimally extracted with a detergent (0.5% sodium dodecyl sulfate) and that bovine serum albumin, a potential contaminant, can be removed in the water-soluble fractions. Purification of inner ear antigens by these methods will lead to more reproducible results in immunoblotting, as well as a greater opportunity to identify the mechanisms involved in autoimmune inner ear disease.

Spontaneous remission in experimental autoimmune labyrinthitis.

We investigated the time course of hearing impairment and cellular infiltration into the inner ear after systemic sensitization of guinea pigs with a single intradermal injection of bovine inner ear antigen (IEAg) in complete Freund's adjuvant (CFA). Lymphocytes and polymorphonucleocytes appeared mainly in the scala tympani on days 7 to 14, and in addition there was thickening and cellular infiltration of the round window membrane at day 14. These cellular infiltrations resolved after day 28. The auditory brain stem response thresholds from IEAg-sensitized animals were significantly elevated after day 7. Some sensitized animals (n = 5) had spontaneous remissions after day 28; however, the hearing thresholds did not completely recover. These results demonstrate that experimental autoimmune labyrinthitis can be induced by a single inoculation of IEAg-CFA and that remission, as evidenced by clearing of the cochlear cellular infiltration and improved hearing thresholds, can occur spontaneously.

Three-component analysis of caloric nystagmus in humans.

Three-component analysis of caloric nystagmus, focusing on the horizontal, vertical, and torsional, using a computerized eye movement analysis system, was carried out in 10 normal human subjects. The caloric response was induced by cold stimulation to the right ear of the subjects in the supine and prone positions. In the supine position, the three components of nystagmus were toward the left (10 subjects), upward (eight subjects) or downward (two subjects), and clockwise (10 subjects). In the prone position, on the other hand, the three components were directed toward the right (10 subjects), downward (five subjects), upward (three subjects), and counterclockwise (10 subjects); there was no vertical direction in two subjects. These findings indicate that caloric stimulation activates the three semicircular canals simultaneously. Also the changes in the nystagmus direction in the supine and prone positions could be explained, at least partially, by the nonconvective component of caloric stimulation.

Relationship between auditory brainstem response waveform and head size.

The relationship between the head size and the latency and amplitude of the auditory brainstem response (ABR) was investigated in 60 normal-hearing adults (30 males and 30 females). We found a strong positive correlation between the latency of ABR waves and the head size and a strong negative correlation surfaced between the ABR amplitude and head size. These results indicate that the head size is one of the most important factors influencing the waveform of the ABR.

Potentiation of inner ear damage following electron beam irradiation with CDDP administration.

This study was designed to examine the combined ototoxic effects of cisplatin, an antitumor platinum compound, administration and electron beam irradiation on the inner ear of guinea pigs, histopathologically. One group received saline solution 4 ml/kg/day and the other group received cisplatin of 2 mg/4 ml/kg/day for five consecutive days. The right temporal bones of all animals of both groups were exposed to the electron beam of 14 Gy/day 3 hours after the daily injection of saline or cisplatin. All animals were sacrificed for inner ear histopathological findings 21 days after the last injection. A combination of cisplatin administration and electron beam irradiation produced a severe potentiation of the outer hair cell damage with no inner hair cell damage. Therefore, in treatment of head and neck carcinomas, it is important to pay attention to inner ear damage caused by combination therapy of cisplatin and electron beam irradiation which involves the inner ear.

Eye movement analysis system using computerized image recognition.

A new technique for an eye movement analysis system utilizing infrared video recording and a computerized image recognition method is presented. The system consists of an infrared lighting apparatus, a very small infrared video charge-coupled device camera, a video tape recorder, an analogue-digital converter, and microcomputers. This system makes it possible to simultaneously analyze the slow-phase velocity quantitatively not only of the horizontal and vertical but also of the rotatory components of the energy-induced nystagmus. The maximum slow-phase velocity of the rotatory component of energy-induced nystagmus was found to be 4.1 degrees per second on an average in this study.

[Auditory brainstem response in dead on arrival cases].

The relationship between ABR on the one hand and acid-base balance, EEG findings and prognosis on the other was studied in 47 cases of DOA. As for the acid-base balance, the balance tipped to acidosis in almost all cases. The ABR showed wave I to wave V in 25 cases, wave I to wave III in 2 cases and only wave I or no response in 20 cases on the admission day. The EEG was isoelectric in 27 out of 35 cases. All the patients who showed some activity in EEG had ABR which showed waves I through V. However, 11 of the 27 patients who were isoelectric EEG showed wave I through V potentials in ABR. By the relationship between EEG and ABR, the cases could be classified into 4 types, namely, 1) there are EEG activities with ABR showing up to wave V and the other types EEG are isoelectric with ABR findings that 2) wave V appears, 3) wave V disappears to leave wave I-III and 4) only I wave or becomes no response. The acid-base balance of the cases was inclined to acidosis; therefore, the effect of acidosis on ABR, EEG and disturbance of the brain tissue due to anoxic state wave suspected. Prognosis of DOA cases was poor and 87.2% died. All the patients who exhibited no response in ABR and isoelectric EEG at anytime of their course, died eventually. These findings suggest that combination of EEG and ABR enables one to learn more exactly the central function in those with disturbance of consciousness.