1/76. The leaking labyrinthine lesion resulting from direct force through the auditory canal: report of five cases.The leaking labyrinthine lesion is treated by conservative methods or surgical procedures. With respect to the stapes, the surgical treatment is controversial. Five cases of middle ear injuries accompanying oval window rupture are reported herein. In each case, direct force through the auditory canal damaged not only the ossicular chain but also the oval window. Initial symptoms were sudden hearing loss with significant conductive disturbance and various degrees of unsteadiness. Spontaneous horizontal nystagmus directed toward the uninvolved ear was observed in each case. Tympanic cavities were promptly explored under general anesthesia and oval window injuries were confirmed. In each case, the damaged stapes was temporally removed from the oval window. Perilymphatic leakage was recognized in each case. Two patients had subluxation of the stapes with a paucity leakage. Three had complete luxation of the stapes with a relatively huge oval window fistula. Disrupted oval windows were repaired with temporalis muscle fascial grafts that were inserted under the middle ear mucosae surrounding the oval windows. The stapes were replaced in the repaired oval windows, and the ossicular chains were reconstructed without artificial grafts. Vestibular dysfunctions disappeared within 7 days, and satisfactory audiologic results were obtained in each case.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
2/76. Fibrous dysplasia of the temporal bone and maxillofacial region associated with cholesteatoma of the middle ear.Fibrous dysplasia of the temporal bone is a rare disease which may lead to progressive stenosis of the external auditory canal and the development of cholesteatoma. We present a case in which minimal symptoms were present despite a massive temporal bone fibrous dysplasia. cholesteatoma resulted most probably secondary to external auditory canal stenosis. Retroauricular fistula developed as a result of destructive effect of cholesteatoma, that influenced previous diagnosis and treatment of this clinically silent disease.- - - - - - - - - - ranking = 0.4keywords = canal (Clic here for more details about this article) |
3/76. External acoustic meatus cholesteatoma.cholesteatoma is a condition usually affecting the middle ear and mastoid, but in certain cases it can be found in the external ear canal. It usually affects elderly people and presents with chronic otorrhoea and dull otalgia. The disease can progress to extensive mastoid destruction. Small lesions can be treated with regular microscopic debridement in out-patients while larger lesions require surgery to eradicate the cholesteatoma sac. We present 2 cases of external ear cholesteatoma to add to the less than 50 cases reported to date.- - - - - - - - - - ranking = 0.2keywords = canal (Clic here for more details about this article) |
4/76. Inner ear malformations and hearing loss in linear nevus sebaceous syndrome.OBJECTIVE: Linear nevus sebaceous syndrome (LNSS) is a rare disorder comprising of nevus sebaceous, seizures, and mental retardation. While extensive literature describe the dermatologic, neurologic, as well as ophthalmologic manifestations of this LNSS, otologic problems have not been previously described. The objective of this report is to describe the otologic manifestation of a patient with LNSS. methods: A child with LNSS was referred to the Otologic clinic for evaluation of hearing loss. Pertinent findings on history, physical findings, audiometric testing, and imaging studies are discussed. RESULTS: Audiometric testing showed bilateral conductive hearing loss. Computerized tomography of the temporal bone demonstrated widened internal auditory canals and dysplastic lateral semicircular canals. CONCLUSION: LNSS can be associated with hearing impairment and inner ear malformations. The evaluation of a child with LNSS should include otologic and audiologic testing.- - - - - - - - - - ranking = 0.4keywords = canal (Clic here for more details about this article) |
5/76. otitis externa sicca/fibrotising external otitis (FEO) as a complication of sjogren's syndrome.sjogren's syndrome (SS) is a condition characterized by sicca symptoms and by autoimmune features. We describe two SS patients with otitis externa fibroticans/sicca. One of these 2 patients developed a lesion of the tympanic membrane making it necessary to perform a tympantomy and meatoplasty. Our findings suggest firstly that the epithelial cell-mediated secretion of lamellar bodies and the production of the permeability barrier are defective in SS. Secondly, local moisturing and/or topical corticosteroid treatment in SS patients with sicca symptoms in the auditory canal could help to avoid reconstructive surgical treatment.- - - - - - - - - - ranking = 0.2keywords = canal (Clic here for more details about this article) |
6/76. Goldenhar's syndrome: congenital hearing deficit of conductive or sensorineural origin? temporal bone histopathologic study.BACKGROUND: Oculoauriculovertebral dysplasia (OAVD) (Goldenhar's syndrome) is a congenital syndrome with ipsilateral deformity of the ear and face, epibulbar lipodermoids, coloboma, and vertebral anomalies. Goldenhar's anomaly has often been associated with a degree of congenital hearing deficits, almost always of a conductive origin, but a sensorineural component is also suspected in some cases, evident through malformations of the inner ear. patients AND methods: Both temporal bones of a 10-day-old deceased patient with oculoauriculovertebral dysplasia were examined by light microscopy. RESULTS: The ear deformities included deformity of the auricle, atresia of the external auditory canal, and malformation of the tympanic cavity and ossicles. Abnormalities of the stria vascularis and the semicircular canals were also demonstrated. Further inner ear deformities were not identified in this case. CONCLUSION: These histopathologic findings appear to confirm the conductive component of the congenital hearing deficit, but a sensorineural component could not be omitted. The ear alterations favor early developmental field defects. The causes of this condition are controversial. Recent results in genetic research pertaining to the MSX class genes permit better understanding of the variety, variability, and different degrees of severity of the anomalies described here.- - - - - - - - - - ranking = 0.4keywords = canal (Clic here for more details about this article) |
7/76. Vestibular-evoked myogenic potentials in the diagnosis of superior canal dehiscence syndrome.patients with superior canal dehiscence (SCD) syndrome have vertigo and oscillopsia induced by loud noises and by stimuli that result in changes in middle ear or intracranial pressure. We recorded vestibular-evoked myogenic potentials (VEMP responses) in 10 patients with SCD syndrome. The diagnosis had been confirmed in each case by evoked eye movements and by high-resolution CT scans of the temporal bones that showed a dehiscence overlying the affected superior canal. For the 8 patients without prior middle ear disease, the VEMP threshold from the dehiscent ears measured 72 /- 8 dB NHL (normal hearing level) whereas the threshold from normal control subjects was 96 /- 5 dB NHL (p < 0.0001). The VEMP threshold measured from the contralateral ear in patients with unilateral dehiscence was 98 /- 4 dB NHL (p > 0.9 with respect to normal controls). Two patients with apparent conductive hearing loss from middle ear disease, and SCD, had VEMP responses from the affected ears. In the absence of dehiscence, VEMP responses would not have been expected in the setting of conductive hearing loss. These findings confirm earlier studies demonstrating that patients with SCD syndrome have lowered VEMP thresholds. Conditions other than SCD syndrome may also lead to lowered VEMP thresholds. Rather than being based upon a single test, the diagnosis of SCD syndrome is best established when the characteristic symptoms, signs, VEMP response, and CT imaging all indicate SCD.- - - - - - - - - - ranking = 1.2keywords = canal (Clic here for more details about this article) |
8/76. Familial microtia in four generations with variable expressivity and incomplete penetrance in association with type I syndactyly.Familial microtia with external ear canal atresia and conductive deafness is rarely reported. Autosomal dominant and recessive inheritance have been suggested depending on various family reports. Cases with other malformations in addition to microtia have been described, although the microtia generally is an isolated finding. Here we report a family with microtia, external auditory canal atresia and conductive deafness in four generations. The mode of inheritance of the disease was autosomal dominant within this family. Also, variable expressivity, incomplete penetrance and generation skipping are evident in the pedigree. association of microtia with type I syndactyly, which has never been reported previously, was present in the index case.- - - - - - - - - - ranking = 0.4keywords = canal (Clic here for more details about this article) |
9/76. The Wildervanck syndrome: cervico-oculo-acoustic dysplasia.The Wildervanck syndrome consists of the Klippel-Feil deformity of the spine, eyeball retraction, lateral gaze weakness, and hearing loss. Conductive hearing loss, as well as a more frequently occurring sensorineural hearing loss, is caused by dysplasia of the inner ear. Typically, polytomograms demonstrate a bulbous vestibule and dilated lateral semicircular canals with basilar impression of the skull. The Duane's eyeball retraction phenomenon completes the syndrome; this may be explained either by misdirected innervation of the extraocular muscles or by atrophy or fibrosis of the lateral rectus muscles.- - - - - - - - - - ranking = 0.2keywords = canal (Clic here for more details about this article) |
10/76. Familial lateral semicircular canal malformation with external and middle ear abnormalities.We report a family with inner ear lateral semicircular canal (LSC) malformation and external and middle ear abnormalities. The family had no history of known syndromes or toxic exposures. Distinct phenotypic manifestations were found in three family members. A young girl exhibited bilateral LSC malformation with a right-sided preauricular tag, a mildly deformed auricle, a stenotic external auditory canal, and a constricted middle ear cavity. She had moderate conductive hearing loss in the right ear but normal hearing in the left ear. Her younger brother exhibited right-sided LSC malformation, microtia, external auditory canal atresia, a malformed middle ear cavity, and abnormal auditory ossicles. He had severe mixed hearing loss in his right ear. Their mother exhibited left-sided LSC malformation without external and middle ear abnormalities, and the hearing was normal in her left ear. None of the three cases had vestibular symptoms, and their results of balance tests were appropriate for the corresponding ages. In contrast, significantly decreased LSC function was revealed by caloric tests in an ear with LSC malformation. Previously, LSC malformation may have been underdiagnosed in patients presenting with external and middle ear abnormalities and their relatives, since this malformation is frequently associated with normal hearing and balance or conductive hearing loss only. To our knowledge, this condition has not been described previously. This condition supports a genetic basis for the combination of LSC malformation and external and middle ear abnormalities and may represent an autosomal dominant condition with variable expressivity.- - - - - - - - - - ranking = 1.4keywords = canal (Clic here for more details about this article) |
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