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Among patients who receive cochlear implants, those with Cogan's syndrome make a unique group. On one hand they are part of the post-lingual patients and good results can be anticipated. On the other hand, their basic illness is thought to have an autoimmune aetiology and for that reason more susceptible to complications, especially flap problems. In a series of 60 patients who were implanted at the Sheba Medical Center, three had Cogan's syndrome. No post-operative complications, including flap problems, were observed (followed-up for at least 18 months). Subjective and objective hearing results were very good.
The authors study the different parameters of the stapedius reflex on a 55 patients group with retrocochlear and non tumoral diagnostic is assess by electrophysiologic (BERA, Electrocochleography) and neuroradiologic explorations (TAC), Meato-cisternography, NMR). We realise an comparative study between the results of this group and the results of 2 other groups (98 acoustic neuromas and 104 endocochlear diseases). The test has a moderate sensibility and none specificity in this kind of pathology, because the high percent of false negative (30%), and the positivity on 95% of acoustic neuromas and on 74% of endocochlear diseases.
Microcystic lesions are a persistent final stage in a neurodegenerative disorder characteristic of the cochlear nuclei of gerbils. When gerbils of various ages raised under known acoustic conditions were examined, the volume density and number of lesions increased with age, however, the affected region was restricted to the posteroventral cochlear nucleus and adjacent portions of the dorsal cochlear nucleus, interstitial nucleus, and posterior anteroventral cochlear nucleus. Lesions were also noted in a separate locus in the auditory nerve trunk associated with the acoustic nerve nucleus. The fusiform and molecular layers of the dorsal cochlear nucleus were spared at all ages observed. The spherical cell region of the arteroventral cochlear nucleus was also largely spared. A good correlation was observed between the cumulative input from the auditory nerve fibers caused by the ambient acoustic environment acting over the life of the animal and the number of lesions in tonotopic subdivisions of the cochlear nuclei. The earliest microcysts formed in regions receiving auditory nerve fibers most strongly stimulated by the ambient noise. Thereafter, short exposures to higher levels of input or long exposures to lower levels of input were quantitatively equivalent in producing microcystic lesions.
Vestibular neurectomy (VN) results in a high success rate in the control of vertigo in Meniere's disease, although the subsequent fate of auditory function is fairly unpredictable. The present investigation reports the postoperative results obtained in a group of 30 subjects with a clinical diagnosis of Meniere's disease and vascular cross-compression of cranial nerve VIII. All subjects underwent VN using a retrosigmoid approach, and in half of them microvascular decompression (MVD) of the cochlear nerve with interposition of autogenous muscle was performed at the same time. All patients had complete relief from vertigo. Hearing was significantly improved in the VN-MVD group (46.7% of subjects). In this group tinnitus and aural fullness also improved significantly, with values of 62.6% and 66.6%, respectively.
The early results of hyperosmolar perfusion of the scala tympani for advanced Meniere's disease were reported in 1987. Thirty patients were treated in this way between March 1981 and March 1986; follow-up is now 2 to 7 years. The rationale of cochlear dialysis is to reduce the endolymphatic hydrops of the pars inferior by filing the scala tympani with a sterile solution of mannitol in 5% dextrose that has an osmolality of 350 m.osm per liter, which compares with a scala media osmolality of circa 300 m.osm per liter. The technique is a qualitative and quantitative extension of the "salt in the round window niche" used as far back as 1972 by Arslan.
Evoked otoacoustic emissions (EOAE) are active mechanical responses from the cochlea which provide information about the integrity of the preneural cochlear receptor mechanisms. It may be hypothesised, therefore, that if a hearing impairment is neural in origin, normal EOAEs may be obtained from the cochlea, which, although dissociated, is functioning normally. This study examined the status of the cochlea with EOAE in patients with cochlear (Meniere's disease) and neural (surgically proven acoustic neuroma) disease. In patients with presumed cochlear lesions, no emissions were present with mean hearing worse than 40 dB across a frequency range of 0.5 to 4 kHz. Similarly, an EOAE was not present in any of the 26 acoustic neuroma patients studied when the average (0.5 to 4 kHz) hearing was greater than 40 dB. We conclude that dissociation of the cochlea in patients with acoustic neuroma appears to be rare and, in fact, cochlear involvement occurs in most cases. Possible mechanisms responsible for the effect on the cochlea in this group include degenerative changes due to chronic partial obstruction of the blood supply by the tumour, biochemical alterations in the inner ear fluids, loss of efferent control of active mechanical tuning, and hair cell degeneration secondary to neuronal loss in the eighth nerve.
The aim of this study was to investigate the involvement of oxidative stress and apoptosis in an animal model of Meniere's disease. Endolymphatic hydrops (ELH) is generally accepted as the decisive histological characteristic of Meniere's disease. Closure of the endolymphatic duct (Kimura's method) was used to induce endolymphatic hydrops in guinea pigs. Sham-operated animals served as controls. After 4 weeks the animals operated showed a significant elevation of the hearing thresholds as measured by audiometric brainstem responses (ABR) pre- and postoperatively. Immediately after the second ABR measurement, the animals were sacrificed for further immunohistological examinations of the inner ear with specific antibodies to active caspase-3 (cas-3) as a marker for apoptosis and antibodies to 8-isoprostane (8-iso) and nitrotyrosine (NT) as indicators of oxidative stress. Compared with the sham-operated controls, hydropic cochleae showed strong immunostaining for both oxidative stress markers in spiral ganglion cells, in the blood-vessels and fibrocytes of the lateral wall, as well as in supporting cells of the organ of Corti. Activation of cas-3 in spiral ganglion cells and the lateral wall was found exclusively in hydropic cochleae. Our findings suggest that oxidative stress is involved in the development of endolymphatic hydrops and may lead to cellular damage which induces apoptosis by activation of cas-3. Apoptotic cell death might contribute to the sensorineural hearing loss found in later stages of Meniere's disease.
Progress has been made in the diagnosis and treatment of inner ear disorders. Autoimmune inner ear disorders and Ménière's disease (MD), the prototype inner ear disease, are highlighted in this review of current knowledge and contemporary dietary, medical, surgical, and vestibular rehabilitation therapy. A number of other peripheral vestibular disorders are presented and contrasted with MD.
Hearing in patients with Menière's disease improved when they were treated in an underpressure chamber. In this study 46 patients with neurotological diseases, including 32 cases of Menière's disease, were tested in an overpressure chamber. As a result, hearing in 32% of the patients with Menière's disease improved, although this was lower than the 50% rate of improvement in Menière's disease patients tested in an underpressure chamber.
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Clinical, electrophysiological investigations, evaluation of autonomic nervous system reactivity with estimation of Kerdo vegetative and stability indices were carried out in 58 patients with cochleovestibulopathy (CVP) and 10 normal controls. CVP patients with compensated disease had sympathicotonia, those with decompensated CVP exhibited parasympathicotonia. Functional loading tests and the stability index values revealed compensatory nature of sympathicotonia. Quantitative and qualitative characteristics of the compensation were related to age (under 40, over 41) and pathogenesis of the disease (central or peripheral). In CVP decompensation poor tolerance of mental work load was reported.
Intensive industrial noise is an adequate stimulus of the hearing organ. Obviously, it can cause occupation-related hypoacusis in noise exposed workers. Pathological changes in other organs and systems that accompany cochlear neuritis are poorly studied; however, it seems that they have no noise-determined specific features. Clinical and provocative examinations have shown that noise, which exceeds maximal allowable levels, produces a significant effect on the development of the symptom-complex of pathological disorders, including hypoacusis, neurodynamic and neurocirculatory changes, hypertensive disease. Combination of disorders in the cardiovascular and central nervous function should be viewed as a pathology induced by intensive industrial noise, i. e. as an independent nosological form of pathology termed noise disease.
Relapsing polychondritis is an infrequently recognized disease. Its etiology is unknown. It is characterized by recurrent and potentially destructive inflammatory lesions involving cartilaginous structures, the cardiovascular system and some sensory organs. We report a patient with important tracheal stenosis, vestibulo cochlear dysfunction and polyarthritis. We present a literature review.
PURPOSE: Fabry disease is an inborn error of metabolism due to a deficient activity of the lysosomal enzyme alpha-galactosidase A. The enzyme defect leads to the systemic accumulation of neutral glycosphingolipids in tissues, mainly in the vascular endothelium. STRONG POINT: The aim of this paper is to present a review of the auditory manifestations in Fabry disease, and to discuss hypothesis on the vascular origin of deafness. PERSPECTIVES: Sensorineural hearing loss in Fabry disease could be the first documented vascular pathology of the inner ear.
In a series of 360 patients who underwent cochlear implantation at our center, four patients (five procedures) had cochlear implantation with obliteration of the mastoid cavity and management of cholesteatoma as a single-staged procedure. Three patients were bilaterally deaf secondary to CSOM and had bilateral mastoid cavities, and in one patient congenital cholesteatoma was identified during cochlear implantation. A mastoidectomy or revision mastoidectomy with obliteration of the mastoid cavity and cochlear implantation was performed as a single stage procedure. Cholesteatoma reoccurred in one patient 9 years after cochlear implantation. Surgical procedures, complications, follow-up and outcomes are discussed.