Light and electron microscopic studies of a case with simultaneous Mondini and Michel deformities of the inner ears

We performed light and electron microscopic studies on the temporal bones of a patient with genetic aplastic deafness, in which the right ear had a Mondini-type defect and the left ear a Michel-type anomaly. The round window of each ear was absent. The stapedial foot plate of the right ear was depressed at its central part and was covered by thin membrane. The course of the internal auditory meatus of the left ear was deviated ventrally. There was total aplasia of the cochlea in the left ear, while that of the right ear showed only three-quarters of a turn. In this ear, three sites of spherical bulging were found and were believed to represent outer hair cells. Other structures of the normal cochlear duct were not present. The modiolus of the left ear was round and dome-shaped, contained sparse nerve fibers and a primitive spiral ganglion. The saccule, utricle and semicircular canals of each ear were missing, so that a common cavity of the vestibular system was present. Several otoliths could be seen under the surface of the membrane covering the common cavity. No sensory cells or their related tissues could be found in either ear.     

Light and electron microscopic studies of localized laryngeal amyloidosis

Summary We performed light and electron microscopic studies in a case of localized supraglottic laryngeal amyloidosis involving a 55-year-old woman. Tissue specimens were stained with hematoxylin and eosin, and alkaline Congo red with or without permanganate (KMnO₄) treatment, and were also examined with peroxidase-antiperoxidase immunohistochemistry. Biopsy specimens from the epiglottis showed diffuse replacement of the subepithelial region by amyloid, which showed a typical green birefringence under polarized light. Tissue affinity for Congo red was persistent after the KMnO₄ treatment. However, the tissue sections showed a positive reaction only to the anti-P component antibody. Electron microscopic studies revealed some disorders of the plasma cells, suggesting that these cells play an important role in focal production of amyloid fibrils.     

Novel strategy for treatment of inner ears using a biodegradable gel

OBJECTIVE: The present study aimed to evaluate the efficacy of a biodegradable hydrogel as a drug-delivery medium for the inner ear. Brain-derived neurotrophic factor (BDNF) was chosen as the agent to be administered. METHOD: First, we used an enzyme-linked immunosorbent assay to measure BDNF concentrations in the cochlear fluid after placing a hydrogel containing this agent onto the round-window membrane of the ear. Second, the functional and histologic protection of the auditory primary neurons (spiral ganglion neurons [SGNs]) by BDNF applied through the hydrogel was examined using an animal model of SGN degeneration. RESULTS: The results revealed sustained delivery of BDNF into the cochlear fluid by way of the hydrogel. Second, the functional and histologic protection of the auditory primary neurons (SGNs) by BDNF applied through the hydrogel was examined using an animal model of SGN degeneration. The measurement of electrically evoked auditory-brainstem responses demonstrated that BDNF delivered by way of the hydrogel significantly reduced the threshold elevation. Immunohistochemistry for neurofilament 200 kD demonstrated increased survival of SGNs because of BDNF application through the hydrogel. CONCLUSION: These findings indicate that biodegradable hydrogels can be used for drug delivery to the inner ear.     

Cerebrospinal fluid otorrhea in a patient with congenital inner ear dysplasia

A case of a 10-month-old boy with a cerebrospinal fluid (CSF) fistula in his right ear is reported. In the same ear, the patient also showed congenital inner ear dysplasia. The CSF fistula was obstructed surgically. Surgical exploration showed a fistula superior to the oval window and a normally shaped stapes. The stapes was removed and the fistula was closed by obliteration of the vestibulum with the temporal fascia and fat tissue. The location of the fistula was very rare; to our knowledge, this is the first reported case of CSF fistula superior to the oval window. The relationship between perilymphatic fistula and the microfissure revealed by temporal bone study is discussed.     

The fine structure of lipofuscin in the human inner ear

Summary Lipofuscin inclusions in the human membranous labyrinth were studied by electron microscopy. Lipofuscin is morphologically an irregularly shaped, membrane-bound inclusion consisting of an electron-dense structure. The most common component was a fine, granular, osmiophilic substance which was always associated with a homogenous, spherical structure resembling a lipid droplet. The combination of these two components was frequently observed in the human inner ear. Distended inclusions containing lipofuscin components were also observed within the supporting cells, saccular, utricular and ampullar wall, the epithelial cells of the transitional zone and in the dark cells. Lipofuscin is closely associated with lysosome and is known to accumulate in the tissue as a result of aging. The high lysosomal activity possibly may result in lipofuscin formation in the human inner ears. Also some other unknown metablic conditions may provide the deposits of lipofuscin.I wish to express my thanks to Professor Nobuo Ueda of the Biochemistry Department for his advice and to Mr. Dominic W. Hughes for English editing of this paper.     

45例先天性内耳畸形的高分辨率CT特征

目的：探讨先天性内耳畸形的高分辨率CT(HRCT)特征及其在先天性内耳畸形诊治中的意义。方法：总结45例先天性内耳畸形的临床资料．分析颞骨HRCT所见。结果：45例耳聋多起于幼儿．呈缓慢进行性感音神经性听力减退，波动性者15耳；17耳半规管麻痹。HRCT显示：Michel型3例(4耳)；Mondini型25例(39耳)；不伴内耳畸形的前庭水管扩大13例(23耳)；内耳道发育畸形4例(5耳)。伴中、外耳畸形13耳。结论：HRCT对先天性内耳畸形具有重要的诊断价值，并为临床治疗、特别是人工耳蜗植入术适应证的选择提供了重要的依据。     

Light and electron microscopic studies of a case of middle ear adenoma

An adenomatous neoplasm from the middle enr was studied. Two distinct groups of cells were present. Electron microscopy suggested that one group was epithelial and the other neuroendocrine in origin. The features supported the hypothesis that such tumors arise from middle ear mucosa.     

An immunohistochemical and electron microscopic study of a central venous hemangioma of the mandible

Summary To date, 96 cases of central hemangiomas of the jawbones have been described in the literature. Of these cases, the mandible has been more affected than the maxilla, while approximately 50% of all cases occur in the first and second decades of life. Histopathologically, cavernous and capillary types are often found, in contrast to the central venous type, which is rare. We have recently treated a 44-year-old man, who was found to have a central venous hemangioma in his mandible. Immunohistochemical studies were performed using factor-VIIIrelated antigen as well as various lectins as markers for tumor endothelial cells. Our results showed that four kinds of lectins (UEA-I, PNA, ConA, and DBA) were useful for the marker as factor-VIII-related antigen. Scanning electron microscopic findings of this tumor demonstrated that the lumina of the blood vessels were more rough, while the lining endothelial cells were more irregular when compared with those of the normal vascular system.     

Light and transmission electron microscopic studies following frontal sinus obliteration with ionomer cement in cats

Osteoplastic frontal sinus surgery in combination with sinus obliteration can be performed for various indications, including chronic sinusitis, frontal sinus trauma and removal of osteomas. In an experimental study using cats, the mucous lining of the frontal sinus was removed, the nasofrontal duct sealed with semifluid ionomer cement and the cavity filled with Ionogran, a solid and porous bone substitute based on ionomer cement. Histological investigations up to 1 year after surgery showed increasing sinus obliteration by regenerating bone, starting from the sinus wall, and formation of connective tissue between the cement grains. There was no evidence for mucosal regeneration. Electron microscopic examination of the interface between the cement and connective tissue revealed mesenchymal cells, collagen fibers and areas of mineralization in close contact with the implant material. Newly formed connective tissue matrix in intimate contact with the cement was a good indication for biocompatibility of the material and is a possible explanation for the implant's solid adhesion to bone. The present findings indicate that lonogran is a suitable alloplastic material for experimental frontal sinus obliteration in cats.Adapted from a presentation of preliminary results reported at the 65th annual meeting of the German Society for Otorhinolaryngology — Head and Neck Surgery (Deutsche Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie), Chemnitz (Germany), 14–18 May 1994     

Ultrastructural findings of the macula utriculi in a case of a petrous apex cholesteatoma: a comparison with findings in a patient with an acoustic neuroma

The morphological characteristics of the vestibular sensory cells of the macula utriculi obtained during surgery in a patient with a petrous apex cholesteatoma were examined using scanning and transmission electron microscopy. Findings were compared to cells studied in a patient with acoustic neuroma. Scanning electron microscopy showed that compared to the apparently normal cells in the acoustic neuroma case, most sensory cells in the cholesteatoma case had large cuticular plates, irregular locations of cilia and no clear polarizations. Supporting cells showed profuse short microvilli on the whole surface. With transmission electron photomicrographs, type I hair cells were not seen and certain morphological changes were observed in type-1I-like cells and supporting cells. We presume that the degenerative changes in the vestibular epithelia were due to circulatory disturbances and/or direct pressure applied to the vestibular nerve at the internal auditory canal, with subsequent involvement of the macula utriculi.     

Cytochemical localization of adenylcyclase in the lateral wall of the inner ear

Summary The cytochemical localization of adenylcyclase in the lateral cochlear wall was studied using a modification of the Reik-Howell method. All the cell membranes of the stria vascularis and spiral prominence cells, except the vascular endothelium, showed a low enzyme activity. In the marginal cell and spiral prominence epithelium facing the endolymph, this activity was limited to the perilymphatic membrane sections. The results are discussed.Supported by a grant of the Deutsche Forschungsgemeinschaft (Me 783/1-3); presented in part at the 54th Annual Meeting of the German Society of Otorhinolaryngology, Head and Neck Surgery, 17 May 1983, Travemünde     

Auditory function in women with autoimmune inner ear diseases and their offspring

Objective

The precise cause of congenital sensorineural hearing loss (CSNHL) is unclear in many cases. In a previous study we found that offspring from guinea pigs with autoimmune sensorineural hearing loss (ASNHL) exhibited signs of SNHL. Here we studied women with autoimmune inner ear diseases (AIED) and their offspring. Our aim was to determine if autoimmune damage may be one of the causes of CSNHL.

Methods

Thirty-eight pregnant women with AIED were recruited. Thirty-three had ASNHL; one with autoimmune delayed endolymphatic hydrops (ADEH) and four with autoimmune Meniere's disease (AIMD). The following were assessed in all women: audiogram, auditory brain stem response (ABR), otoacoustic emission (OAE), vestibular function test and presence of inner ear antigens. The following were assessed in offspring from these women: OAE, ABR and presence of inner ear antigens.

Results

Five of the 38 children born to women with AIED had SNHL (an incidence much higher than normal). OAEs were not inducible in these children shortly after birth or within 46-100 days after birth. Abnormal ABR findings were apparent in these five children and inner ear antigens were detected in three of the five children (the mother's of these children were also positive for inner ear antigens).

Conclusions

These preliminary findings suggest that the prevalence of congenital ASNHL may be increased in offspring born to women with AIED.     

Hearing in patients with congenital deformity of the inner ear

Congenital abnormalities of the bony labyrinth and internal auditory meatus range from complete absence to minor anomalies compatible with normal cochlea function. A ‘follow-up’ survey to assess the hearing of 56 patients with abnormalities of the inner ear shown by tomography was made. The hearing levels are discussed in relation to the tomographic appearances and comparison made with other series of inner ear abnormalities demonstrated both radiographically and histologically.     

Scanning electron microscopic studies of the nasal blood vessels

Summary Current histological and transmission electron microscopic techniques are insufficient for studying all different aspects of vascular morphology. As a result, we have used scanning electron microscopy (SEM) to examine nasal blood vessels. In our present studies the nasal septa of 15 rabbits were fixed by vascular perfusion, dried by the critical point method and coated with gold for SEM studies. Lower magnifications of specimens using this method show the tissue arrangement in the nasal septum. In higher magnifications the different vessels can be distinguished by their morphological features. The course of the vascular structures can be obverved as well as the junctions between the different vessels. Our results indicate that different morphological techniques must be combined to understand the endonasal vasculature.     

Scanning electron microscopic studies of the capillaries crossing the scala tympani

Summary Scanning electron microscopic examinations were carried out on the perilymphatic space bordering the round window in guinea pigs. A number of small vessels were found crossing free through the lumen of the scala tympani in this area. The larger number (5–10) of these capillaries are suspended between the bony cochlear wall and the terminal net of fibrocytes which covers the cochlear opening of the cochlear aqueduct and spreads onto the basal third of the round window membrane. Other capillaries (3–5) cross the lumen of the scala tympani from the outer cochlear wall to the modiolus. All of these capillaries have a thin endothelium and a very thin pericytic covering. These findings suggest that the capillaries crossing the perilymphatic space may give rise to a different chemical composition of the perilymph bordering the round window in comparison with the more upper parts of the scala tympani.Support for this work was provided by the Deutsche Forschungsgemeinschaft (PL 79/3). Computerization of the literature was sponsored by Breuninger Stiftung GmbH, Stuttgart     

Surgery of the inner ear with hearing preservation: serial histological changes

OBJECTIVES/HYPOTHESIS: Surgery of the inner ear can result in hearing preservation under certain conditions, but the mechanisms responsible for hearing preservation or loss are not well understood. The specific aim of the study is to examine histological sections obtained at different time intervals after varying degrees of surgical entry into the inner ear, to understand how the cochlea is protected. The hypothesis is that internal partitioning occurs. STUDY DESIGN: Histologic examination of guinea pig inner ears by light microscopy. METHODS: Guinea pigs underwent lateral semicircular canal transection and plugging, ampullectomy, or vestibulotomy, and tone-burst auditory brainstem response thresholds at 2, 8, and 24 kHz were measured at intervals before and after surgery. Animals were killed after 1, 3, 7, or 21 or more days, and temporal bones were examined histologically. RESULTS: The histological response to surgical trauma consists of fibrosis and varying amounts of inflammation near the site of surgical entry. Cochlear hair cells are nearly always preserved, even when hearing loss occurs. Extension of the inflammatory response to the cochlea is associated with greater degrees of hearing loss. CONCLUSION: The guinea pig inner ear is capable of withstanding surgical trauma to the semicircular canals and vestibule without complete loss of cochlear function. Fibrosis creates an effective partition between the site of surgical entry and the rest of the inner ear. Cochlear preservation might be enhanced if the inflammatory response can be contained.