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Biomedical subjects

L Bergstrom

Publications and source records attributed to L Bergstrom.

At least 37 records · Page 2Linked to original sources

Fragile bones and fragile ears.

Conductive, sensorineural and mixed hearing loss occur in osteogenesis imperfecta in autosomal dominant inheritance pattern. Hearing loss is generally due to the middle and inner ear pathology of osteogenesis imperfecta and only occasionally to the coincidental association of otosclerosis and osteogenesis imperfecta. Two lesions cause the conductive hearing loss of osteogenesis imperfecta: (1) functional ossicular discontinuity due either to stapes superstructure fracture or fibrous replacement, or (2) thick, crumbly, lightly fixed stapes footplate. Cochlear hair cell loss, stria vascularis atrophy and calcification, tectorial membrane distortion and perilymph hemorrhage are autopsy findings that could account for sensorineural hearing loss, which occurs in a surprisingly high percentage of osteogenesis imperfecta patients. Hearing loss occurs earlier in osteogenesis imperfecta than in otosclerosis. Distinctive acoustic impedance and X-ray abnormalities occur in osteogenesis imperfecta. Other otologic findings may include lopped pinna, notching of the helix of the pinna, rosy flush of the medial wall of the middle ear and vestibular abnormalities.

Adult↗

Renal disease. Its pathology, treatment, and effects on the ear.

Twenty-six hemodialysis and renal transplant patients were studied for the effects of age, duration of kidney failure, amounts and types of treatment, disturbed calcium metabolism, and vascular disease on clinical and pathologic inner ear manifestations. Three subgroups of patients were compared with each other: (1) those having hearing loss of unknown etiology, (2) those having strial deposits, and (3) those having neither. Severity of treatment was not necessarily predictive for severity of inner ear disease. Within each subgroup there was wide variability and almost complete overlap for the factors studied when the three groups were compared. Cochlear strial deposits seem to bear little relationship to disturbed calcium meatbolism or to vascular disease, but their size may be related to the presence of hearing loss.

Adolescent↗

Continuing management of conductive hearing loss during language development.

Recently greater attention has been focussed on the possibility that mild fluctuating or intermittent hearing loss, as seen in one of early childhood's most common illnesses, otitis media, may have long-lasting and sometimes devastating effects on language development and learning. Conductive hearing loss in serous otitis media most affects frequencies below 2000 Hz, and the duration of hearing loss after an acute episode of otitis media may last 6-24 months in up to 30% of affected children. The first principle of management of this problem and congenital conductive hearing loss is detection prior to language delay. Special attention should be given to neurologically handicapped, retarded, learning disabled or physically frail children since their deficient speech and language are often attributed to abnormal neurologic or intellectual status. Treatment includes limited use of vasoactive decongestants; for acute suppurative otitis media antibiotics may be chosen empirically with a high percentage of good results. Other measures include speech and language evaluation, home stimulation language programs and temporary or longterm amplification. The surgeon assesses the patient for potential operability. Surgery to correct a congenital conductive lesion should not be attempted in an only hearing ear.

Acoustic Impedance Tests↗

Edge enhancement computed tomography scanning in inflammatory lesions of the middle ear.

The high resolution modification of an EMI 5005 scanner was utilized with an edge enhancement technique to study inflammatory lesions of the temporal bone. Bone erosions caused by cholesteatomas can be accurately delineated because of the circumscribed nature of expansion. The soft tissue masses cannot be appreciated by present CT techniques. Erosions of the horizontal semicircular canal and involvement of the sinus tympani are readily visualized by CT scanning. Computed tomography is limited in demonstrating the stapes superstructure and horizontal portion of the facial canal due to the inability to obtain good coronal scans. The remaining ossicles can be demonstrated with equal clarity with pluridirectional tomography.

Bone Resorption↗

Erythromycin ototoxicity.

Erythromycin is considered one of the safest antibiotics in common use today. In its otolaryngologic use, the authors have found it effective in treating acute suppurative sinusitis and occasionally otitis media, when combined with sulfonamides. There are few complications of erythromycin administration. Probably the least generally acknowledged of these is ototoxicity. There have been three reports of six cases with ototoxic complications from erythromycin, primarily from administration of its intravenous form. The authors present a case study of an 18 year old girl in severe renal failure, who suffered a reversible sensorineural hearing loss from high doses of an oral erythromycin preparation. The clinical manifestations of this case are compared to those previously reported.

Adolescent↗

New patterns in genetic and congenital otonephropathies.

In a series of chronic renal and congenitally deaf patients 24 were identified as having inborn renal and otologic disease. Sixteen patients, representing 14 families, had genetic disorders. Only two had the features of Alport's syndrome. The patients were classified as follows: 1) Probable Alport's--2 patients (1 family); 2) Atypical hereditary nephritis and sensorineural hearing loss--7 patients; 3) Renal and inner ear anomalies--1 patient; 4) Renal, inner ear and multiple anomalies--4 patients. The temporal bone pathology in one case showed primary neural atrophy and a mild Mondini malformation. In another a Scheibe defect and unusual calcific structures were found in the cochlear duct. 5) Renal, external or middle ear and multiple anomalies--6 patients (5 families); 6 Renal, middle and inner ear anomalies and multiple anomalies--2 patients. A temporal bone obtained from one case showed combined middle and inner ear defects. In the other, who had a chromosome defect, predominantly middle ear anomalies were found. 7) Nephrotic syndrome and congenital hearing loss--1 patient; 8) Unclassified--1 patient. Some cases represent entities apparently not previously described. Probably most interesting is the delineation of hereditary nephritis and deafness distinct from Alport's disease.

Abnormalities, Multiple↗

Congenital and acquired deafness in clefting and craniofacial syndromes.

A study of 284 craniofacial defect patients determined factors associated with or predisposing to acquired or congenital hearing loss. Complete otolaryngic and audiologic evaluation was done at the initial assessment of a large group of patients. From there, a smaller number who had had adequate workup and followup was selected. The patients were classified into seven clefting categories, microtia/artresia, facial defects, cranial defects and miscellaneous anomalies of the head and neck. X-ray findings, those present at middle ear surgery and those studied at autopsy, are summarized. The incidence of hearing loss is 88 per cent. The likelihood of congenital hearing loss increases with the number of defects. Certain unifying concepts that may help identify a specific child as being in urgent need of otologic and audiologic testing are presented.

Abnormalities, Multiple↗

Some pathologies of sensory and neural hearing loss.

Recently surgical implantation of devices to stimulate the auditory nerve in man makes it apparent that sensory and neural pathologies of deafness need to be differentiated from each other. In this paper 10 exemplary cases are presented. In addition, an attempt is made to compile the information now available about sensory and neural pathologies in the various diseases that cause deafness. Superficially, it would appear that most such entities are sensory in nature and thus theoretically might be amenable to auditory nerve stimulation. However, loss of supporting cells seems to be associated with cochlear nerve fibre degeneration. Many individuals may, therefore, eventually develop combined pathology. The paucity of knowledge of pathology of the auditory pathways and their radiations in deaf persons is recognized as a limiting factor in attempts to predict which patients might benefit from auditory nerve stimulation. It is not within the scope of this paper to delineate the possible deleterious effects that cochlear implants might have on the auditory nerve.

Adult↗