Cases reported "Ear Diseases"

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1/6. What inner ear diseases cause benign paroxysmal positional vertigo?

    Benign paroxysmal positional vertigo (BPPV) originating from the posterior semicircular canal (pSCC) is a common vestibular disorder that is easy to diagnose and usually easy to treat. The majority of patients with BPPV have no known inner ear disease; they have "primary" or "idiopathic" BPPV. However, a minority does have objective evidence of an inner ear disease on the same side as the BPPV and this group has "secondary" or "symptomatic" BPPV. Previous publications differ on the prevalence of secondary BPPV and about the types of inner ear diseases capable of causing it. In order to determine what proportion of patients have secondary as opposed to primary BPPV and which inner ear diseases are capable of causing secondary BPPV, we searched our database for the 10-year period from 1988 to 1997 and found a total of 2847 patients with BPPV. Of these, 81 (3%) had definite pSCC-BPPV secondary to an ipsilateral inner ear disease. Sixteen had Meniere's disease, 24 had an acute unilateral peripheral vestibulopathy, 12 had a chronic unilateral peripheral vestibulopathy, 21 had chronic bilateral peripheral vestibulopathy and 8 had unilateral sensorineural hearing loss. It seems that any inner ear disease that detaches otoconia and yet does not totally destroy pSCC function can cause BPPV and that a case can be made for audiometry and caloric testing in all patients with BPPV.
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2/6. Acute vestibular paralysis in herpes zoster oticus.

    A case of herpes zoster oticus is presented in which the lateral and superior semicircular canals of the labyrinth were affected unilaterally. The results of several electronystagmographic examinations are described and correlated with the patient's description of symptoms. This case study indicates that disease affecting the lateral semicircular canal is reliably detected by the conventional caloric test. However, the fact that the posterior semicircular canal remained intact could not be inferred from the results of the caloric test in this case. Also the appearance of nystagmus upon eye closure appears to have been a more sensitive index of the state of the disease process than was the caloric test.
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3/6. An algorithm for neurotologic disorders.

    In the evaluation of patients with complaints of dizziness, hearing loss, and/or tinnitus, the primary objective is to determine the site of the lesion. An accurate localization of the lesion is important for selecting the most appropriate radiologic study. The information obtained from the vestibular and audiometeric evaluations identifies the site of the lesion accurately. For the vestibular evaluation we use photoelectric nystagmography (PENG) and the Torok monothermal differential caloric test. The initial audiometeric evaluation consists of a pure-tone audiogram and speech discrimination scores. Additional audiometric site-of-lesion tests and ABR are used selectively as the diagnostic yield of these tests does not significantly add to the information already obtained from the vestibular evaluation. If the lesion is suspected to lie in the middle ear or internal auditory canals, we use pluridirectional tomography. For further delineation of lesions confined to the internal auditory canal we use pneumo-CT. For suspected morphologic lesions of the posterior fossa we use thin-section CT with enhancement. review has proved useful in defining petrous apex and skull base lesions. CT-rBBC studies have proved valuable in objectively demonstrating a deficient perfusion of the brain. The vascularity of certain lesions such as glomus tumors can be satisfactorily confirmed by this technique.
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4/6. Otoneurological observation and classification of Harada's disease presenting with aural symptoms, especially vertigo.

    Harada's disease is an uncommon autoimmune disease affecting melanocytes located in the uvea, inner ear, skin, hair, and meninges. A classification of Harada's disease presenting with aural symptoms, especially vertigo, was devised on the basis of otoneurological findings. Peripheral vestibular dysfunction, such as staggering in the stepping test, rotato-horizontal or horizontal nystagmus, and diminished labyrinthine function in the caloric nystagmus test, was observed in the vast majority of these patients. Harada's disease with such features was designated as the peripheral type, and was further divided into vestibular, cochleo-vestibular, and cochlear subtypes. patients only rarely presented with symptoms or signs of central nervous system involvement, and Harada's disease with such features was designated as the central type.
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5/6. Fibroinflammatory pseudotumor of the ear. A locally destructive benign lesion.

    We describe three cases of a fibroinflammatory pseudotumor (tumefactive fibroinflammatory lesion) of the middle and inner ear. The patients presented with total deafness in the affected ear and no response to caloric stimulation. The computed tomographic pattern showed destruction of inner ear structures and a typical widening of parts of the labyrinth. magnetic resonance imaging performed in all three patients showed an extension greater than expected based on computed tomographic images of both areas of destruction, as well as areas of radiologic normality. An enhancing mass was seen in the inner ear with a characteristic extension into both the internal auditory canal and the middle ear. A transotic approach or subtotal petrosectomy was used to remove the tumor in all three cases. Although histologically benign, these tumors are locally destructive and, as such, behave like a neoplastic lesion. They are composed of fibrovascular tissue admixed with chronic inflammatory cells. To our knowledge, this is the first report on pseudotumors of the middle ear, inner ear, and internal auditory canal. Inflammatory pseudotumor used to be a somewhat confusing term for a recognized entity of unknown origin. It is likely that infection is an important contributing factor in the development of these lesions. Although surgical removal seems to be the treatment of choice, no clear judgment of its prognosis can be made owing to the rarity of this tumor.
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keywords = caloric
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6/6. Bilateral Mondini dysplasia with normal hearing.

    A 37-year-old man with both Mondini dysplasia and normal hearing is reported. The patient visited our clinic with a complaint of unsteadiness. Pure-tone audiometry showed a normal hearing level in both ears. Polytomography and computed tomography (CT) revealed enlargement of the vestibules and lateral semicircular canals in both ears but a normal shape of the cochlea and other semicircular canals. The caloric test indicated severe canal paresis (CP) in the left ear and moderate CP in the right ear, whereas the active head rotation test demonstrated that head-eye coordination was preserved at frequencies of 0.33 and 0.67 Hz but slightly deteriorated at 1.0 Hz. The patient's unsteadiness seemed to be attributable to a hypofunction of the bilateral semicircular canals, which may be due to insidious, repeated cerebrospinal fluid otorrhea caused by judo wrestling. Although Mondini dysplasia with normal hearing has been believed to be rare, phylogenetic consideration suggests that more such patients exist than has been assumed.
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