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1/12. Intraoperative mitomycin C in endolymphatic sac surgery for Meniere's disease: A pilot study.

    The long-term results of endolymphatic sac (ES) shunt surgery in Meniere's disease have been reported to be unsatisfactory compared to the short-term results, probably because of fibrosis and/or reclosure of the incised ES. To solve this problem, we tried to apply mitomycin C (MMC) intraoperatively to the incised ES as used in trabeculectomy for glaucoma. MMC has an antiproliferative effect as well as an antineoplastic effect. Thus, it is expected that MMC would have a benefit to prevent rapid fibrosis and/or reclosure of the ES. MMC did not show any ototoxicity in our animal study and we started a clinical trial after being approved by the ethics Committee for Human research at our university. Fourteen patients with Meniere's disease underwent ES mastoid shunt surgery with intraoperative application of MMC and they were followed up more than 6 months. No patients experienced vertigo, although some complained of slight dizziness. Five patients out of 14 showed remarkable hearing improvement by more than 10 dB, accompanied with decrease of tinnitus.
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2/12. Cerebro-oculo-facio-skeletal syndrome as a human example for accelerated cochlear nerve degeneration.

    BACKGROUND: Cerebro-oculo-facio-skeletal (COFS) syndrome is a rare autosomal-recessive disorder that includes microcephaly, severe mental retardation, and multiple congenital anomalies. Otologic findings are usually limited to descriptions of the auricles. PATIENT AND methods: The authors report inner ear histopathologic findings of a deceased 13-year-old patient with COFS. A histologic study of the inner ear in COFS syndrome has not yet been described. This patient was documented as having a profound bilateral sensorineural hearing loss at the age of 2 years. RESULTS: Histologic evaluation revealed accelerated neural and neuronal degeneration at the cochlear and retrocochlear levels. Remaining myelinated nerve fibers, counted in the spiral lamina, had degenerated by up to 97% when compared with normal innervation densities. Afferent nerve fibers innervating inner hair cells were completely absent, whereas medial efferent fibers to outer hair cells were found. vestibular nerve fibers were less affected. CONCLUSION: The authors report inner ear findings that differ from animal models of primary cochlear neural degeneration and that resemble the pattern of hereditary cochlear nerve degeneration reported in Friedreich's ataxia.
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3/12. Tympanic electrocochleography: normal and abnormal patterns of response.

    Electrocochleography has been widely used in human and animal studies of endolymphatic hydrops. A variety of response patterns have been reported in normal and hydropic ears. Recent clinical studies have focused almost exclusively on the amplitude ratio of the summating potential (SP) and action potential (AP) derived from alternating polarity click responses. In this report normal response patterns are described with a tympanic membrane electrode employing condensation, rarefaction and alternating polarity clicks and tone burst stimulation. A variety of response abnormalities are described in patients with suspected endolymphatic hydrops. The exclusive use of alternating polarity clicks is not adequate to reveal the nature of these abnormalities.
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4/12. Transient hemiageusia in cerebrovascular lateral pontine lesions.

    knowledge of human central taste pathways is largely based on textbook (anatomical dissections) and animal (electrophysiology in vivo) data. It is only recently that further functional insight into human central gustatory pathways has been achieved. magnetic resonance imaging studies, especially selective imaging of vascular, tumoral, or inflammatory lesions in humans has made this possible. However, some questions remain, particularly regarding the exact crossing site of human gustatory afferences. We present a patient with a pontine stroke after a vertebral artery thrombosis. The patient had infarctions in areas supplied by the anterior inferior cerebellar artery and showed vertical diplopia, right sided deafness, right facial palsy, and transient hemiageusia. A review of the sparse literature of central taste disorders and food preference changes after strokes with a focus on hemiageusia cases is provided. This case offers new evidence suggesting that the central gustatory pathway in humans runs ipsilaterally within the pons and crosses at a higher, probably midbrain level. In patients with central lesions, little attention has been given to taste disorders. They may often go unnoticed by the physician and/or the patient. Central lesions involving taste pathways seem to generate perceptions of quantitative taste disorders (hemiageusia or hypogeusia), in contrast to peripheral gustatory lesions that are hardly recognised as quantitative but sometimes as qualitative (dysgeusia) taste disorders by patients.
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5/12. Degeneration of cochlear neurons as seen in the spiral ganglion of man.

    A light and electron microscopic study of the spiral ganglion cells of a normal individual, a patient with Meniere's syndrome, and a patient with long-standing unilateral profound deafness was done to evaluate patterns of neural degeneration in the human inner ear. Parametric data for the normal spiral ganglion are presented and compared with the pathologic ganglia. In the ear with Meniere's syndrome, the nuclear area and axonic diameter of spiral ganglion cells were significantly smaller than in the contralateral and the normal ear. This was interpreted as evidence of neuronal degeneration in Meniere's syndrome. In the spiral ganglion of the long-standing deaf ear, there appeared to be selective preservation of large cells with no dendritic processes, contrary to the pattern of degeneration seen in the spiral ganglion of the animal.
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6/12. Inner ear autoantibodies in patients with rapidly progressive sensorineural hearing loss.

    Recognition of immune-mediated sensorineural deafness that responds to immunosuppressive therapy has led to a search for a diagnostic assay to identify inner ear autoantibodies. Without a confirmed diagnosis of autoimmune disease, many patients have undergone inappropriate immunosuppressive treatment or developed irreversible inner ear damage. serum from patients with progressive sensorineural hearing loss (n = 54), ulcerative colitis (N = 5), normal controls (N = 14), and animals with experimental autoimmune sensorineural hearing loss (EASNHL) were analyzed by Western blot against fresh bovine inner ear antigen preparations. The hearing loss group (19 [35%]) showed a single-or double-band migrating at 68,000 molecular weight (MW), differing from the normal group (1 of 14 [7%]) which showed a similar band (P = .031). Upon analysis by two-dimensional gel electrophoresis both the EASNHL guinea pigs and a patient reacted against identical components of inner ear antigen. These results suggest an autoimmune basis for disease in patients reacting against the 68,000 MW antigen.
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7/12. Quantitative evaluation of sensorineural structures following stapes surgery.

    Sensorineural hearing loss may occur following stapes surgery. The reason for this remains unclear in spite of experimental studies in animals and postmortem temporal bone evaluations in humans. In this study, we quantitatively evaluated the sensorineural elements of six human temporal bones after stapes surgery. Outer hair cell loss in the upper basal turn was present in two cases. This lesion has not previously been described in human temporal bones after stapes surgery. In temporal bones with preoperative sensorineural hearing loss, evidence of presbycusis was also present. The importance of quantification in evaluating these structures must be stressed.
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8/12. Treatable sensorineural hearing loss.

    Sensorineural hearing loss is generally felt to be an untreatable medical condition. However, in some cases, prompt diagnosis and treatment of the underlying condition may reverse the deafness. This article summarizes various treatable forms of sensorineural hearing loss and provides illustrative cases histories of patients who have had sensorineural hearing losses that were improved by medical or surgical intervention. patients with reversible sensorineural deafness due to inadvertent aminoglycoside over-dosage, congenital cholesteatoma, Meniere's syndrome, blood coagulopathy, and perilymphatic fistula all had improvements in auditory function after medical or surgical intervention. Recent experimental studies on animals may explain the basic mechanisms behind hearing loss and recovery. Aminoglycoside ototoxicity appears to have an initial reversible step, followed by a permanent process. Early endolymphatic hydrops and fistulas may cause mechanical effects in the cochlea which can be corrected. Coagulopathy may cause hypoxia which reverses after anticoagulation. These observations reveal that animal experiments can be useful in explaining human auditory dysfunction of the reversible type.
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9/12. Cochleosaccular degeneration of the inner ear and progressive cataracts inherited as an autosomal dominant trait.

    A new syndrome of cataracts and progressive sensorineural hearing loss, inherited as an autosomal dominant trait, is described. The histopathology of the inner ears in the proband demonstrated severe degeneration limited to the cochlea and saccule. This case represents the second reported example of genetically determined cochleosaccular degeneration in man. The cochleosaccular pattern of histopathology is found in both man and animals and may be the end result of prenatal dysplasia or postnatal degeneration. It also appears that cochleosaccular dysplasia and degeneration may be the result of genetically determined or acquired defects. Previously reported examples of cochleosaccular dysplasia and degeneration are reviewed including human cases in which the histopathology is similar to, but not characteristic of, the cochleosaccular pattern.
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10/12. pathology of vascular sensorineural hearing impairment.

    Circulatory disorders, which are well documented in most parts of the human body, are not well documented in the inner ear, although they are expected to occur. It has been previously shown that experimental occlusion of the labyrinthine artery in animals results in severe degenerative changes, fibrosis, and new bone formation in the cochlea. Accordingly, this paper presents presumptive evidence that depriving the human cochlea of its blood supply after surgical removal of an acoustic tumor results in severe degenerative changes that progress to total ossification of the cochlear spaces. This paper also discusses similar changes seen in temporal bones of two patients with sudden sensorineural hearing impairment. Based on these observations, the author concludes that occlusive arterial disease, whether thrombotic, embolic, or spastic, plays a role in some sudden sensorineural hearing losses. Cochlea ossification, detected by polytomography, suggests a vascular etiology of the hearing impairment. Treatment with vasodilator drugs and anticoagulants is justified in these cases until a better diagnostic protocol is developed to eliminate other causes.
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