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Cases reported "Hearing Loss, Conductive"

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191/323. A child with noonan syndrome.

    The speech, language, and hearing characteristics of a child with noonan syndrome are described in this report. The physical characteristics of this disorder are presented. Also included is a description of a pragmatic language analysis completed to provide a description of social-linguistic communication and a basis for treatment. (+info)

192/323. incidence and type of otopathology associated with congenital palatopharyngeal incompetence.

    Based upon the known association of cleft palate and middle ear disease, a study was undertaken to determine the incidence and type of middle ear pathology associated with velar anomalies exclusive of cleft palate which may produce congenital palatal pharyngeal incompetence. The range of velar anomalies encountered was subdivided into congenital palatal incompetence Type 1 (clinically manifested by one or more of a triad of visible palatal anomalies including submucous deficiency of the hard palate, bifid uvula, and a diastasis of velar musculature) and congenital palatal incompetence Type 2 (no visible velar anomalies but radiographic anomalies of the velopharyngeal region such as short or thin velum and/or enlarged nasopharyngeal dimensions consequent to vertebral and skull base anomalies). Middle ear disease was assessed separately in CPI Types 1 and 2 in order to differentiate the effects upon middle ear function between overt and occult velar anomalies. Middle ear disease was more frequent in CPI Type 1 than in CPI Type 2. The predominant otopathologic finding was serous otitis media, paralleling the type associated with cleft palate. Tympanic membrane atrophy, tympanosclerosis and tympanic membrane perforation, often considered sequelae of chronic serous otitis media, were noted infrequently. This investigation supports the concept that middle ear disease frequently occurs with congenital palatal incompetence as it does with cleft palate. (+info)

193/323. Osteopathia striata with cranial sclerosis. Report of a case and review of the literature.

    This article describes a case of osteopathia striata with cranial sclerosis. The patient also has multiple sclerosis. The symptomatology includes a right sided conduction deafness and a left maxillar nerve deficit, which were both attributed to the bone disorder. The authors review the literature of this rare genetic syndrome and pay special attention to the neurological manifestations. These mainly consist of hearing loss, mental subnormality and occasionally the involvement of other cranial nerves. The bone scan in their patient shows hyperactivity in the left skull base region. This finding provides further evidence that, at least in some instances, the bone disorder has a progressive course. (+info)

194/323. Beta blockers and loss of hearing.

    Loss of hearing in a 43 year old man during treatment with metoprolol was dose related and disappeared within a few months after the drug had been stopped. The hearing impairment was of mixed type, with an air bone gap without any disorder of the middle ear observable by conventional clinical methods. Similar scattered reports from international sources on loss of hearing during treatment with beta blockers are also presented. (+info)

195/323. Conductive hearing loss, middle ear ossicular anomalies, malformed thickened lop auricles, and micrognathia. A rare autosomal dominant congenital syndrome.

    A rare congenital autosomal dominant syndrome of thickened bilateral lop auricles, conductive hearing loss, ossicular anomalies, and micrognathia is reported. The anomaly is presumably a defect in the first and second branchial arch development during the sixth and seventh weeks of gestation with an auricle abnormality (first and second arch), abnormal incus and malleus (first and second arch), abnormal stapes (second arch), and micrognathia (first arch). Recognition of low-set or malformed auricles with a unilateral or bilateral conductive hearing loss should alert the otolaryngologist to possible middle ear abnormalities and other associated branchial cleft anomalies. Surgical correction of the congenital conductive hearing loss may include prosthetic ossicular reconstruction and otoplasty. The possibility of associated congenital anomalies of other systems (for example, vestibular, cardiac, genitourinary, reproductive, and so on) should be evaluated. An accurate pedigree and family medical and genetic history should be obtained to screen for other involved family members and for assessment of genetic passage of the trait. (+info)

196/323. arthritis of the middle ear in ankylosing spondylitis.

    A case of ankylosing spondylitis with aortic valve disease and hearing loss is described. A series of radiographic and audiometric investigations showed the hearing loss to be of a conductive type. It seemed most likely that the hearing loss was related to an inflammatory involvement of the ossicular joints due to the primary disease. No other case of conductive hearing loss has previously been reported due to otoarthritis in ankylosing spondylitis. This is important both theoretically and practically. (+info)

197/323. otosclerosis and Meniere's syndrome: diagnosis and treatment.

    Occasionally a patient with otosclerosis and a conductive hearing loss will develop typical findings of Meniere's syndrome in the involved ear, years later. A review of clinical and pathological studies in the literature and in our laboratory and clinic indicates a likely cause-and-effect relationship for these cases. The pathology and pathogenesis of the syndrome of otosclerosis and Meniere's syndrome is discussed. A stapedectomy/sacculotomy was used to treat 17 patients, 13 of whom acquired a satisfactory result in terms of improvement of hearing and control of vertigo. This technique and findings are described and discussed. (+info)

198/323. stapes surgery in patients with osteogenesis imperfecta.

    osteogenesis imperfecta is not in itself a contraindication to stapedectomy. Thirty stapedectomies were performed on 24 patients with osteogenesis imperfecta. Thin ossicles, crural fractures, and thick, mushy, granular footplates predominate in this condition. Deficient, short crura that did not contact the footplate were noted in three patients; this is possibly a new clinical observation. Three times in this small series the endosteum was so thick that it was possible to fenestrate the soft, granular, mush-like footplate without invading the vestibule. Extreme caution in handling the incus is necessary. Conductive hearing loss can be relieved through stapedectomy in patients with osteogenesis imperfecta with about the same level of predictability as in those with otosclerosis. (+info)

199/323. An unusual aetiology of unilateral glue ear in a child.

    A case of unilateral glue ear in a child of fourteen due to amyloid deposit of the post nasal space is presented. A grommet was inserted through a myringotomy incision. Hearing improved to normal limits post-operatively. Investigations excluded systemic and familial amyloidosis. Extrusion of the grommet was followed by recurrence of the middle ear effusion. The patient's parents refused admission for removal of the amyloid deposit and replacement of the grommet. (+info)

200/323. Conductive deafness, symphalangism, and facial abnormalities: the WL syndrome in a Japanese family.

    We report on a family in which four relatives were affected with an autosomal dominant syndrome of unusual facial appearance, nasal abnormality, conductive deafness, pectus carinatum, and symphalangism. This appears to be only the second report of the WL symphalangism syndrome of Herrmann and the first studied in a Japanese family. (+info)
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Last update: April 2009
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