1/10. Fluctuant, progressive hearing loss associated with Meniere like vertigo in three patients with the Pendred syndrome.OBJECTIVE: To evaluate vestibular and long-term audiometric findings in patients with Pendred syndrome. Study design: Retrospective analysis of long-term clinical data. SETTING: University hospital department. patients: Three patients with Pendred syndrome caused by a mutation in the SLC26A4 gene. methods: Perchlorate discharge test, mutation analysis of the SLC26A4 gene, MR imaging of temporal bones, vestibular function test (in two cases) and serial audiometry. A saturation hyperbola with onset age was fitted to the audiometric threshold-on-age data using a nonlinear regression method. The residues remaining after regression were analyzed in a correlation analysis to detect significant ipsilateral or contralateral cofluctuation. RESULTS: All three patients had a mutation in the SLC26A4 gene and bilateral enlarged vestibular aqueduct; two of them had a positive perchlorate discharge test but in one of two siblings this test was negative. hearing loss was significantly progressive with significant ipsilateral and contralateral cofluctuation in all evaluable cases, combined with episodes of Meniere like vertigo in two cases. The episodes of vertigo are as seen in meniere disease. One case had unilateral caloric areflexia and one had bilateral vestibular hyporeflexia, proven to be progressive in a repeat examination. CONCLUSIONS: patients with Pendred syndrome may exhibit progressive and fluctuant hearing loss with episodes of vertigo.- - - - - - - - - - ranking = 1keywords = caloric (Clic here for more details about this article) |
2/10. Neurotological findings in a patient with narrow internal auditory canal: a case report.We report neurotological findings in a patient with unilateral narrow internal auditory canal, as confirmed by computed tomography. The patient presented no auditory brainstem response on the affected side. Vestibular tests including vestibular-evoked myogenic potentials (VEMP) and caloric test revealed normal function of both inferior and superior vestibular neural pathways.- - - - - - - - - - ranking = 1keywords = caloric (Clic here for more details about this article) |
3/10. Labyrinthine anomalies with normal cochlear function.Three cases of labyrinthine anomaly confirmed by polytomography and CT scan are reported. They showed similar dysplasia of the bony labyrinth: dilation and fusion of the lateral semicircular canal (SCC) and of the vestibule with a normally shaped cochlea and other SCCs. One side was involved in 2 cases and both sides in 1 case. The 1st case showed normal hearing levels with markedly reduced response to caloric stimulation in the affected ear. The 2nd case showed conductive hearing loss due to cholesteatoma with normal bone conduction hearing levels and normal caloric response. The 3rd case showed bilateral conductive hearing loss of unknown cause. The classification of labyrinthine anomalies and labyrinthine functions is discussed. Labyrinthine anomaly detected by CT scan and polytomography can be present in patients with normal cochlear and/or vestibular function.- - - - - - - - - - ranking = 2keywords = caloric (Clic here for more details about this article) |
4/10. Neurotological and neuroanatomical changes in the connexin-26-related HID/KID syndrome.The phenotype of the HID (hystrix-like ichthyosis, deafness)/KID (keratitis, ichthyosis, deafness) syndrome is primarily characterized by skin changes. However, the connexin 26 (Cx 26) autosomal dominant mutation underlying this syndrome is of special neurotological interest. In the present paper, the clinical pattern, audiovestibular and neuroimaging findings and the detailed genetic analysis of 4 patients with identical HID/KID-associated mutation D50N of Cx 26 are reported. The audiological test results demonstrated profound sensorineural hearing loss in all of the patients. Neurotological testing revealed inconsistent abnormalities in dynamic posturography (sensory organization test), but the vestibular ocular reflex upon caloric irrigation was normal in all patients. Vestibular-evoked myogenic potential testing for otolith function (saccule) showed a regular response in 1 patient and pathologic responses in 3 patients, while subjective haptic vertical (utricular function) testing was normal in all of the patients. CCT showed an extended (in length), but very thin (in diameter) bony lining between the basal portion of the internal auditory canal and the vestibule in the 3 scanned patients. Our study provides evidence for functionally intact semicircular canals and normal utricular function in subjects with the autosomal dominant D50N mutation of Cx 26, in contrast to saccular function which was generally compromised and hearing loss which was profound.- - - - - - - - - - ranking = 1keywords = caloric (Clic here for more details about this article) |
5/10. Waardenburg's syndrome: a case report with CT scanning and cochleovestibular evaluation.The Waardenburg's syndrome, a congenital ectodermal germ layer defect of autosomal dominant inheritance with variable phenotypic expressivity consists of 6 major characteristics: dystopia canthorum, broad nasal root, hypoplasia of the medial eyebrow, heterochromia irides, white forelock, and congenital deafness. Twelve additional characteristics have been reported including meningocele, atresia of the esophagus, and Hirshsprung's disease supporting an early hypothesis that the neural crest is the embryonic site linking defects of the cervical sympathic system with pigmentary abnormalities and inner ear anomalies. Congenital deafness reported in 20% of Waardenburg patients, and the most disabling characteristic, has been associated with ENG abnormalities in 30% of these deaf patients. A congenitally deaf young person with Waardenburg's syndrome is examined for basic cause of episodic vertiginous symptoms. Diagnostic assistance from previous investigations is lacking because they have not involved state-of-the-art functional evaluations or correlations of behavioral and radiographic findings. In this patient, high-resolution computerized tomography failed to demonstrate bony labyrinthine anomalies. Functional cochleovestibular analysis revealed a bilateral profound sensorineural hearing loss and a unilateral weakness in oculomotor responses to caloric stimulations. Repeated measurements of ocular counterroll show variations compatible with intermittent otolithic dysfunctioning. These findings are consistent with a diagnosis of an acquired active unilateral peripheral vestibular disorder. Some of the technical issues underlying the derivation of this conclusion are discussed.- - - - - - - - - - ranking = 1keywords = caloric (Clic here for more details about this article) |
6/10. Pitfalls in the diagnosis of acoustic neuroma.The patient with an acoustic neuroma may present to the otologist with a variety of clinical features. Classically these include a retrocochlear pattern of sensorineural hearing loss, reduced vestibular response on caloric testing and radiological asymmetry of the internal auditory canals (IAC). The absence of any or all of these features, however, does not exclude the presence of tumour. Five cases are presented to illustrate the potential for diagnostic delay unless a routine battery of investigations is undertaken in patients with neuro-otological disorders. All patterns of subjective audiometry are encountered, auditory brain stem response testing may be unreliable where hearing loss is profound, vestibular testing is normal in half of small tumours and the intracanalicular tumour may be radiologically undetectable unless IAC meatography is employed.- - - - - - - - - - ranking = 1keywords = caloric (Clic here for more details about this article) |
7/10. 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.- - - - - - - - - - ranking = 1keywords = caloric (Clic here for more details about this article) |
8/10. temporal bone pathology in patients without caloric response.Pathologies of serial sections of 9 temporal bones from 6 patients without caloric response were studied. The patients died of epipharyngeal cancer, middle ear cancer, chronic renal failure with DIC, pancreatic cancer with DIC, multiple brain metastasis of pancreatic cancer, and leukemia. Under light microscopy, 8 ears showed pathologies in the vestibular end organs and the nerves. Possible causes of the lack of caloric responses in the 8 ears were endolymphatic hemorrhage, labyrinthitis, leukemic infiltration in the labyrinth, degeneration or disappearance of the vestibular sensory cells, reduction in the number of vestibular nerve fibers, and/or brainstem lesions. The remaining one ear did not show abnormal findings in the vestibular end organs or the nerves, but the patient had brainstem lesions. Four types of temporal bone pathologies were observed according to the lesion site responsible for the lack of caloric response; i) sensory type, ii) neural type, iii) mixed type and iv) central type.- - - - - - - - - - ranking = 7keywords = caloric (Clic here for more details about this article) |
9/10. Pendred's syndrome.Although 5% of all cases of congenital deafness are caused by Pendred's syndrome, there are few reports in the literature. Seven patients with Pendred's syndrome in three families living in the same village were detected. For that reason, the syndrome is reviewed in light of the literature. The sex distribution of the patients with Pendred's syndrome and their families was recorded. We tested for thyroxine, triiodothyronine, thyroid-stimulating hormone, triiodothyronine resin uptake, and perchlorate, and performed caloric testing. In one patient, subtotal thyroidectomy was performed. In the histopathologic study, a thyroid nodule filled with colloid was found. Chromosome studies showed no anomalies in any patient. Five of the patients were deaf-mutes. We observed that the parents were cousins in all three families. These families also had healthy children, and the existence of the syndrome in both sexes points to an autosomal recessive trait.- - - - - - - - - - ranking = 1keywords = caloric (Clic here for more details about this article) |
10/10. Sudden deafness from vertebrobasilar artery disorder.Two cases are presented of sudden deafness with vertigo and/or dizziness, a 19-year-old male and a 54-year-old female. Their onset condition was considered to be caused by vascular disorders in the area of the vertebrobasilar artery, but they showed no signs of the central nervous system disorders. Case 1 suffered right sudden deafness just after a super selective embolization for the peripheral area of the right vertebral artery, and case 2, just after the accidental cutting of the left vertebral artery during the procedure of neurovascular decompression surgery. The neurotologic findings in these cases were almost the same and the common characteristics were (1) irreversible total deafness of the affected ear, (2) canal paresis in caloric test, (3) retrolabyrinthine disorder of the vestibular system detected by the galvanic test, and (4) no obvious findings of central nervous system disorders, such as eye movement disorder or ataxy. These findings indicate that the sudden deafness was attributable to localized embolism in the inner ear artery caused by thrombosis in the area of the vertebrobasilar artery.- - - - - - - - - - ranking = 1keywords = caloric (Clic here for more details about this article) |
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