Yoshihiko Terayama

P-ANCA-positive Wegener's granulomatosis presenting with hypertrophic pachymeningitis and multiple cranial neuropathies: case report and review of literature.

An autopsy case of hypertrophic pachymeningitis and multiple cranial neuropathies is reported. A 53‐year‐old woman with paraplegia and various neurological signs which developed over a 2 year period was diagnosed as having an epidural mass with thickened dura mater extending from the lower cervical to the thoracic spinal cord. In addition, bilateral episcleritis, blephaloptosis, and blindness of the right eye with various cranial nerve deficits were found to be caused by the mass lesions involving the paranasal sinuses, orbit, and the cavernous sinus. Perinuclear antineutrophil cytoplasmic antibody (p‐ANCA) was positive, but cytoplasmic antineutrophil cytoplasmic antibody (c‐ANCA) was negative by enzyme‐linked immunosorbent assay. The partially removed epidural mass with hypertrophied dura mater and biopsy of the paranasal lesions showed chronic granulomatous inflammation with vasculitis. The remaining lesions resolved with steroid therapy with remarkable neurological improvement. The positive p‐ANCA test, paranasal involvement, the report of a similar histopathological case and a review of the literature on granulomatous pachymeningitis suggest the presence of p‐ANCA‐positive Wegener’s granulomatosis with central nervous system involvement characterized by hypertrophic pachymeningitis and/or multiple cranial neuropathies.

Ultrastructural Changes of the Nerve Elements Following Disruption of the Organ of Corti

3-137 days after disruption of the guinea pig organ of Corti by perilymphatic perfusion with 20% streptomycin (SM), ultrastructural changes of the nerve fibers in the organ were observed. Most of nerve fibers began to degenerate after a latent period of 4 days. On the other hand, a number of fibers survived reactively enlarged and later developed into myelinated and unmyelinated fibers by becoming enclosed in Schwann cells which entered the organ of Corti through the habenula perforata. Regeneration and sprouting of the surviving nerve fibers also occurred. The fibers became mature, but atrophied after 60 days and then gradually disappeared. The regenerating fibers were mainly of the myelinated and unmyelinated efferent type. Retrograde degeneration occurred in both afferent and efferent fibers. In the less damaged organ of Corti perfused with 2% SM or Ringers solution, Schwann cell invasion was not found.

Ultrastructural changes of the nerve elements following disruption of the organ of Corti. II. Nerve elements outside the organ of Corti.

Various stages of changes in the nerve fibers, spiral ganglion cells, and satellite cells from the guinea pig cochlea 3 to 137 days after perilymphatic perfusion with streptomycin solution (2 and 20%) were observed electron microscopically. Initially, the axoplasms of the cochlear nerve fibers became swollen or pyknotic. Then, the axons disappeared and myelin lamellae disrupted. The Schwann cells shrank and degenerated, though their basement membranes survived for a time. Regeneration of the cochlear nerve fibers began with extension of axonal sprouts into the tube of the basement membrane and surviving Schwann cells, which still contained myelin debris. Only one of the axonal sprouts matured for myelination. These regenerating cochlear nerve fibers were found in the osseous spiral lamina, modiolus and internal auditory meatus, but these fibers atrophied and disappeared afterward. Retrograde degeneration occurred in the olivo-cochlear bundle. Some of the efferent myelinated fibers also showed temporary regeneration.

Organ of Corti in the Human Fetus Scanning and Transmission Electronmicroscope Studies

The organ of Corti in the five-month human fetus was studied by transmission and scanning electronmicroscopy. Differentiation of the surface organization of the organ of Corti into a single row of inner and three to four rows of outer hair cells was complete at this stage except at the apical end. The morphological aspects of the hair bundles changed with maturation of the sensory cells; the inner hair cells preceded the outer hair cells in cytodifferentiation at a given location.

The efficacy of Burow's solution as an ear preparation for the treatment of chronic ear infections.

Objective To determine the efficacy of Burows solution as an otologic preparation for the treatment of chronic ear infection. Study Design Two studies were included: 1) a prospective clinical study and 2) a laboratory study on antibacterial and antifungal effects. Setting A private otology practice and a laboratory study. Patients Fifty-eight patients with refractory otorrhea. Intervention Diagnosis by otoscopy, audiometry, and bacteriology. Methods Burows solution was mixed in solutions with four organisms: methicillin-resistant Staphylococcus aureus, penicillin-resistant Streptococcus pneumoniae, Candida albicans, and Aspergillus. Soon after Burows solution was mixed in the solutions with organisms, and 5, 10, and 20 minutes thereafter, the mixtures were cultured on agars. The numbers of the bacterial or fungal colonies were counted to evaluate the effect of Burows solution. Main Outcome Measures Changes in the clinical findings of the ears, the symptom of otorrhea, and side effects were assessed. Results Thirty-five (70%) of the 50 ears assessed were “cured” and 10 (20%) ears assessed were “improved.” No significant side effect was observed. Regarding the laboratory study, the four organisms disappeared within 20 minutes after Burows solution was mixed. Conclusion Burows solution was considered to be an effective otologic preparation.

Olivo-cochlear bundle in the guinea pig cochlea after central transsection of the crossed bundle. Electron microscopic study on origin and distribution of unmyelinated efferent fibers by axonal degeneration.

In order to clarify origin and distribution of unmyelinated efferent fibers in the guinea pig cochlea, the olivo-cochlear bundle (OCB) was electron microscopically traced from the internal auditory meatus to the organ of Corti at various intervals after central transsection of the crossed bundle. In othx animals the organ of Corti was destroyed in order to induce retrograde degeneration of the co-chlear nerve fibers. The resultant Wallerian degeneration occurred in a majority of myelinated and unmyelinated fibers in the fascicles of OCB after nerve section, whereas the fascicles survived after destruction of the organ of Corti. These findings indicate that both the crossed and uncrossed OCBs consist of myelinated and unmyelinated fibers. Both the myelinated and unmyelinated fibers of the crossed OCB originate in the brain stem. Both the crossed and uncrossed fibers are intermingled in the fascicles distal to the internal auditory meatus. It is probable that the unmyelinated efferents would be different fr...

Distribution And Origin Of Adrenergic Nerve Fibers In The Vestibular Apparatus And Their Arterial Supply In The Guinea Pig: A Fluorescent Microscopic Study

Fine adrenergic nerve fibers which are distributed to the saccular and utricular maculae, ampullar capsules and ducts of the semicircular canals and vestibular nerve of the guinea pig were observed by fluorescent microscopy. These fibers were divided into three groups according to their course and location: perivascular, nerve trunk, and independent groups. The arteries leading to the vestibular organs contained many adrenergic fibers. The uni- and bilateral cervical sympathetic denervations at various levels showed that the adrenergic fibers in the vestibular organs originated in the ipsilateral superior cervical ganglion. Regarding the arteries leading to the vestibular organs, the basilar, anterior inferior cere-bellar, labyrinthine and common cochlear arteries receive adrenergic fibers from the bilateral superior cervical ganglia, while the peripheral branches of the labyrinthine artery receive their adrenergic fibers only from the ipsilateral superior cervical ganglion. These adrenergic fibers come f...

Experimental Mumps Virus-induced Labyrinthitis: Immunohistochemical and Ultrastructural Studies

Guinea pigs were inoculated with mumps virus (Torii strain) by the intralabyrinthine or intravascular route and their cochleas were examined by immunofluorescence and electron microscopy. In these animals, viral antigen was detected in the cochleas, most often in the stria vascularis. However, viral infection was produced only in those with intralabyrinthine inoculation. The cochlear lesion commonly observed in this study was severe degeneration of the organ of Corti, which was usually found in the basal turn. Morphological evidence of viral infection, as depicted by intracellular strands of nucleocapsids and budding of the virus at the endolymphatic surface, was prominent in both the stria vascularis and Reissners membrane. The cochlear location of mumps infection correlated closely with that of the viral antigen formerly reported in newborn hamsters.

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.

Electron microscope study of bone lesion in cholesteatoma otitis

ZusammenfassungDie elektronenmikroskopische Untersuchung von Knochenbiopsien bei Cholesteatomoperationen zeigt Veränderungen der kollagenen Knochenmatrix sowie eine ausgedehnte Degeneration von Knochenzellen. Dabei findet sich die Oberfläche des veränderten Knochens in direktem Kontakt mit dem subepithelialen Bindegewebe oder mit Granulationen, nicht aber mit dem Plattenepithel selbst. Die Untersuchungen zeigen, daß entzündliche Prozesse in den dem Knochen benachbarten Bindegewebslagen vorwiegend für die Knochenzerstörung bei Cholesteatom verantwortlich sind.SummaryBiopsy specimens were obtained at ear surgery of patients with cholesteatoma otitis and were studied by electron microscopy. The osseous tissue decalcified in EDTA demonstrated degradation of collagen matrix as well as extensive degeneration of bone cells. The surface of the eroded bone was found to be in direct contact with the subepithelial connective tissue or the granulation, but not with the keratinizing squamous epithelium of matrix. These results indicate that the bone destruction by cholesteatoma is of non-osteoclastic type and lend support to the view that the keratinizing squamous epithelium do not participate in bone destruction. On the ultrastructural basis, the inflammatory process in the soft tissue adjacent to bone and extensive degeneration of bone cells are considered as cardinal factors for bone destruction in this disease.