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Query: UMLS:C0030552 (paresis)
 5,831 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sclerosteosis is a rare, potentially lethal, autosomal recessive, progressive craniotubular sclerosing bone dysplasia with characteristic facial and skeletal features. The temporal bone changes include a marked increase in overall size, extensive sclerosis, narrowing of the external auditory canal, and severe constriction of the internal auditory meatus, fallopian canal, eustachian tube, and middle ear cleft. Attenuation of the bony canals of the 9th, 10th, and 11th cranial nerves, reduction in size of the internal carotid artery, and severe obliteration of the sigmoid sinus and jugular bulb also occur. Loss of hearing, generally bilateral, is a frequent symptom. It often manifests in early childhood and initially is expressed as sound conduction impairment. Later, a sensorineural hearing loss and loss of vestibular nerve function often develop. Impairment of facial nerve function is another feature occasionally present at birth. In the beginning, a unilateral intermittent facial weakness may occur which eventually progresses to a bilateral permanent facial paresis. The histologic examination of the temporal bones from a patient with sclerosteosis explains the mechanisms involved in the progressive impairment of sound conduction and loss of cochlear, vestibular, and facial nerve function. There is a decrease of the arterial blood supply to the brain and an obstruction of the venous drainage from it. The histopathology reveals the obstacles to decompression of the middle ear cleft, ossicular chain, internal auditory and facial canals, and the risks, and in many instances the contraindications, to such procedures. On the other hand, decompression of the sigmoid sinus and jugular bulb should be considered as an additional life-saving procedure in conjunction with the prophylactic craniotomy recommended in all adult patients.
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PMID:Sclerosteosis involving the temporal bone: histopathologic aspects. 395 65

Six episodes of facial baroparesis in 5 divers are reported. Three of the divers experienced left-sided paresis and 2 right-sided. In 2 of the subjects (3 episodes) the paresis disappeared spontaneously when the relative middle ear overpressure cleared, and in the remaining 3 subjects the paresis disappeared on recompression to less than 2 m. No residual effects were found, but the subject who experienced 2 episodes stopped diving. Available evidence favors the theory of ischemic compression neurapraxia.
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PMID:Facial baroparesis: a report of five cases. 408 48

Sclerosteosis is one of the rare, potentially lethal, autosomal recessive, progressive, craniotubular sclerosing bone dysplasias. Syndactyly of the second and third or other fingers is evident at birth. Hyperostosis and sclerosis are most prominent in the skull and tubular bones, and are frequently associated with excessive height and weight. The typical facial deformity is apparent by the age of 5 years. The changes involving the temporal bone include a marked increase in overall dimensions, extreme sclerosis, and narrowing and constriction of the external ear canal, middle ear cleft, internal acoustic meatus, and falloppian canal. Impairment of hearing, as a rule bilateral, is a frequent presenting symptom which may manifest in early childhood. Initially it is an expression of interference with sound conduction; later it may become associated with a loss of sound perception. Impairment of facial nerve function is another salient feature which occasionally is present at birth. As a rule, it manifests initially as a unilateral, recurrent paresis, eventually progressing to a bilateral permanent partial loss of facial nerve function. Since impairments of hearing and facial nerve function are two of the salient features, present at birth or in early childhood, the responsibility for recognizing the disease often falls upon the otolaryngologist. The clinical and radiologic features permit not only early recognition of the disorder but also differentiation from similar bony dysplasias. Hyperosteosis and sclerosis of the skull lead to thickening and distortion of the calvaria, cranial base, and foramen magnum resulting in reduction of the intracranial volume, interference with the cerebral blood flow, resorption of cerebrospinal fluid, and gradual increase of intracranial pressure. Severe headaches resulting from this mechanism often develop in early adulthood, and several patients have died suddenly from impaction of the medulla oblongata in the foramen magnum. Decompression of the transverse sigmoid sinus and jugular bulb may be lifesaving, combined with a posterior, and if necessary, an anterior, craniectomy for decompression. Early decompression of the internal acoustic meatus and falloppian canal may help in the preservation of cochlear and facial nerve function.
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PMID:Sclerosteosis involving the temporal bone: clinical and radiologic aspects. 667 95

The congenital cholesteatoma of the petrous bone is a rarity. The topographic situation corresponds to a congenital dermoid. The evolution is typic. On the beginning the ear drum is intact, very early one will find a loss of the labyrinth function, often accompanied by a facial paresis. The suppuration of the middle ear comes in a later state. The drainage operation is insufficient. The subtotal petrosectomy with permanent anterior dislocation of the facial nerve gives the chance for a definitive healing.
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PMID:[On the problem of the congenital (?) cholesteatoma of the petrous bone (author's transl)]. 708 56

Patients with adenocarcinoma of the ceruminous gland arising in the middle ear have a typical syndrome consisting of unilateral hearing loss, otalgia, facial paresis, and a middle-ear mass. Adjacent cranial nerves also may be affected. Some patients may have an ipsilateral cerebellar ataxia if the lesion extends into the cerebellopontine angle and compresses the cerebellar hemisphere. Obstructive hydrocephalus may occur secondary to obstruction of the aqueduct or fourth ventricle. The initial clinical findings may be those of a jugular foramen syndrome. These lesions are usually slow-growing and may be associated with a very prolonged clinical course. Although the tumors are rare, the physician should be aware of their existence if proper care is to be given.
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PMID:Intracranial ceruminous gland adenocarcinoma. 729 69

It is generally recognized that surgery for congenital aural atresia is difficult. The success or failure of the operation is often directly related to the degree of development of the middle ear. In poorly developed middle ears, the facial nerve may overhang and conceal the oval window niche, making this area inaccessible to inspection, let alone manipulation. The criteria for transposing the facial nerve are; (1) the atresia must be bilateral, (2) there must be preoperative imaging evidence of a stapes and/or patent oval window, (3) there must be no large blood vessels feeding or draining the facial nerve, and (4) facial nerve monitoring must be available. Over the past 2 years, 6 of 94 patients undergoing surgery for atresia were operated with an intent to transpose the facial nerve in order to access the oval window. In all patients, it was impossible to see the oval window niche due to a displaced nerve. In four of six cases, the facial nerve was transposed. The ossicular chain was reconstructed with a total ossicular replacement prosthesis. In no case was there a postoperative facial paralysis or paresis. Facial nerve transposition allows a final chance of achieving serviceable hearing through surgery. The lack of facial nerve injury and the potential for hearing restoration make this procedure feasible in otherwise marginal or poor surgical candidates.
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PMID:Transposition of the facial nerve in congenital aural atresia. 858 21

Cholesteatomas are found almost exclusively in the middle ear and mastoid. Occasionally this disease is seen in the external auditory canal. Cholesteatoma of the external auditory canal is a rare condition. Severe pain and profuse discharge associated with a normal eardrum and normal hearing are essential clinical features. In addition, we found facial paresis and conductive hearing loss in our case. Smaller cholesteatomas can be managed by frequent debridement in the office; larger lesions require surgical intervention. Surgery is successful in resolving otorrhea and relieving pain. In addition, our own experience has shown that surgery is successful in relieving facial paresis.
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PMID:Giant cholesteatoma of the external auditory canal. 919 5

Craniofacial malformations cause great human suffering. The purpose of the experimental studies was to investigate teratogenically induced craniofacial malformations in the rat, and to study if vitamin B6 could prevent the teratogenically induced malformations in the rat. The aim of the clinical investigation was to compare mandibulofacial dysostosis (MFD) with hemifacial microsomia (HFM) and thalidomide-induced malformations restricted to the first and second branchial arches. In the experimental studies we used two different teratogenic agents, etretinate and BAPN (beta-aminoproprionitrile). Vitamin B6 was administered one day prior to and simultaneously with the teratogenic agent. The induced malformations were observed by direct microscopy, histology, differential staining, microdissection and enzyme histochemistry. Knowledge of isoenzymic differentiation was obtained by isoelectric focusing and polyacrylamide gel electrophoresis. The clinical features of 29 patients with MFD, 26 with HFM and seven with thalidomide-induced malformations were investigated. The patients underwent clinical investigations, radiography, tomography, computed tomography, surgical exploration and audiograms. The etretinate-induced syndrome in the rat shows similarities to first and second branchial arch syndromes in man. Defective formation of Meckel's cartilage and the cartilaginous skull base, the zygoma and the middle ear ossicles were prominent features of the observed malformations. The induced malformations were accompanied by increased staining for alkaline phosphatase (APase) in the skull and skull base cartilages and Meckel's cartilage. BAPN induced cleft palate in 95% of the cases and the teratogenically induced cleft palate was accompanied by a pathological differentiation pattern that could be traced by determination of isoenzymes in the palatal shelves as well as in amniotic fluid. Vitamin B6 could prevent the teratogenic malformations induced by etretinate and BAPN in the rat. Comparing MFD, HFM and thalidomide-induced malformations, all syndromes included patients with external, middle and inner ear malformations. Cranial nerve palsy/paresis was only seen in HFM and thalidomide-induced malformations. A relationship between disturbed neural crest cell migration and defects of the first and second branchial arches seems possible.
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PMID:Teratological studies on craniofacial malformations. 920 Mar 51

Chronic diseases of the ear and their surgical treatment can endanger the delicate structures of the temporal bone in close relationship with the middle ear. The experience and expertise of the otologic surgeon, based on perfect knowledge of the complex anatomy of the whole temporal bone, are unfortunately built up partly through the management of complications encountered in tympanomastoid surgery and may require knowledge of basic neuro-otologic procedures. Paralysis and paresis of the facial nerve and labyrinthine, and dural and vascular injuries may have consequences not only in the final result of surgery but also in endangering the patient's life. All major complications are discussed and some possible treatments are proposed. Minor complications affecting the temporomandibular joint, the dura, the external auditory canal, and the bony canal wall are also covered, along with some suggestions on how to keep these consequences within reasonable percentages.
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PMID:Complications and their management in tympanomastoid surgery. 1039 87

We present a retrospective study on 22 operations of exostosis of the external auditory canal in 20 patients. 8 patients were passionated by water sports. The most frequent indication for surgery (13 operations) was recurrent external otitis or ceruminal obstruction. In 7 cases the need for a wider access to the middle ear indicated surgery. Surgery was usually performed as an outpatient procedure, maximum hospitalization was 3 days. The mean healing period was 6 (3-10) weeks. Mean follow up was 43 (3-110) months. There were no severe intraoperative complications such as facial paresis, lesions of the ossicles or of the inner ear. As intraoperative complications we found 2 perforations of the tympanic membrane, 2 expositions of the capsule of the mandibular joint, one of which was followed by chronic pain. As postoperative complications we found an early soft tissue stenosis of the external auditory canal and one late soft tissue stenosis which recurred after revision surgery. No recurrence of exostosis was seen. We describe an up to now unknown complication: the appearance of bilateral petrositis caused by staphylococcus epidermidis after bilateral surgery in an otherwise healthy patient. This study confirms that severe complications are rare, minor ones however relatively common. And that also minor complications may have a troublesome follow. Therefore and because of the potential of severe complications indication for surgery must be made cautiously and risks of the operation must not be underestimated.
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PMID:[Results and extraordinary complications of surgery for exostoses of the external auditory canal]. 1066 60


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