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Biomedical subjects

J R Lindsay

Publications and source records attributed to J R Lindsay.

16 recordsLinked to original sources

Inner ear degeneration in Reye's syndrome.

A 9-year-old girl developed Reye's syndrome. On admission to the hospital on the fourth day of illness, responses for verbal stimuli had been absent, but were present for painful stimuli. Coma developed on the fourth day and was present until death ten days later. The fundus of both internal meatus showed vascular congestion of nerves with evidence of repeated hemorrhages. All structures within the endolymphatic system showed advanced degeneration, with exception of the tectorial membrane and cupulae. One ear showed vascular congestion, formation of thrombi, and free hemorrhage within the modiolus and spiral ganglion. In the other ear, congested vessels in nerves in the meatus were obstructed by swelling at the cribriform area. The degenerative changes corresponded closely to those produced experimentally in guinea pigs by interruption of arterial circulation to the inner ear.

Child

Sensorineural deafness due to osteitis fibrosa.

A 91-year-old woman with deafness died from renal failure. Autopsy revealed osteitis fibrosa cystica generalisata, chronic myeloid leukemia, renal atrophy, and hyperplastic parathyroid glands. The temporal bones showed senile osteoporosis, osteitis fibrosa, and chronic myeloid leukemia. The inner ears showed extensive degeneration of cochlear sensorineural elements. The perilymph showed a general increased staining reaction with hematoxylin-eosin that was most evident in localized areas, where marrow spaces of osteitis fibrosa communicated directly with perilymph. It appears that the active diseased marrow exerted a toxic effect on the sensorineural elements of the cochlea by diffusion through the perilymph.

Aged

Histopathologic features of the inner ear associated with Kearns-Sayre syndrome.

We describe the histopathologic features of the inner ear in a 19-year-old girl with bilateral total deafness associated with Kearns-Sayre syndrome. The inner ear shows advanced degree of cochleo-saccular degeneration, with almost complete absence of the organ of Corti in all turns. The spiral ganglion shows a reduction of about 60% to 70% of cells, with almost complete degeneration of nerve fibers in the bony spiral lamina. PAS-positive material was found accumulated in globules between the collapsed Reissner membrane and remains of marginal cells of the stria and in the degenerated sensory cell area of the saccular macula.

Adult

Transfer of sulphur to the digestive tract of sheep.

The transfer of sulphate from plasma to digestive tract and from digestive tract to plasma in crossbred sheep was estimated by the use of isotope dilution techniques with Na235SO4. The passage of 35S along the digestive tract was simultaneously measured by reference to two inert radioactive markers infused intraruminally. In the first experiment, three sheep given a roughage-based diet containing 174 +/- 7 mg S/day received an intravenous infusion of Na235SO4 for 7 days before collections were made of plasma and of digesta from the rumen, abomasum and terminal ileum. Similar collections were made in the second experiment in which four sheep received intraruminal infusions of Na235SO4. From estimates of infusion rate of 35S, specific radioactivity of 35S in plasma and digesta and rate of flow of sulphur in the digestive tract the following calculations were made: The transfer of sulphate from the plasma to the rumen was calculated as 29 mg S/day. Of this only 12 mg S/day passed as organic sulphur in digesta from the stomach. As the net gain of sulphur in the stomach in this experiment was 153 mg/day, sulphate transferred from the plasma contributed only a small amount of sulphur derived from endogenous sources in the stomach. In contrast, the substantial passage of 35S into the intestinal lumen during intravenous infusion of 35SO4 suggested that 38 and 41 mg S/day of the 236 and 145 mg organic S/day flowing from the small and large intestine respectively was derived from plasma sulphate, corresponding to about 26% of the dose.

Animals

Recurrent meningitis and labyrinthine gusher, related to congenital defects of the labyrinthine capsule and stapes footplate.

A congenital defect in the bony footplate of the stapes is now known to be a point of lowered resistance to extension of bacterial middle ear suppuration to the vestibule and thence to the meninges. Tomographic demonstration of the congenital pathologic condition of the ear permits the surgeon preoperatively to chart his surgical course. Despite antibiotic and chemotherapy, recurrent meningitis remains a serious disease as manifested by the death of two of the patients reported. The capacity for surgical correction of the congenital defect is manifested by one of our patients who has been free of meningitis for more than two years after surgery. If a congenital defect is considered in each case of recurrent meningitis, it is believed the mortality of this serious disease can be reversed.

Adult

Paget's disease and sensori-neural deafness: temporal bone histopathology of Paget's disease.

Four cases with Paget's disease of the temporal bone are presented to illustrate the pathogenesis of the associated deafness. One case illustrates the combination of severe deafness due to bilateral otosclerosis with probably asymptomatic bilateral Paget's disease. One case with advanced Paget's disease presents features to explain early stages of sensori-neural deafness before actual cellular invasion of the inner ear. One case of profound deafness due to Paget's disease presents a different stage of cellular invasion of the inner ear by the disease on each side. One case illustrates invasion of the internal auditory meatus by Paget's disease with infiltration of the acoustic division of the nerve and profound deafness.

Adult

Labyrinthitis ossificans due to chronic otitis media.

Histopathology of two cases of unilateral labyrinthitis ossificans in the presence of bilateral chronic suppurative otitis media is presented. In both cases, ossification and fibrosis were limited to the scala tympani of the lower basal turn, apparently due to inflammation entering through the round window membrane. The round window was occluded by new bone and fibrous tissue presumably resulting in a conductive hearing loss in addition to the conductive loss due to middle ear lesions. Degeneration of the hair cells of the organ of Corti was limited to the lower basal turn where ossification and fibrosis occurred.

Adult

Acrocephalosyndactly (Apert's syndrome): Temporal bone findings.

The middle ear and ossicular chain were not complete in the specimen, but the remnants present appeared normally developed except for the stapediovestibular joint region. The annular ligament was incompletely developed in two areas where fixation by undedifferentiated cartilage occurred. The subarcuate fossa, normally filled with fibrous tissue and usually narrowed by a rim of newly deposited periosteal bone at birth, was unusually large. Included in this fibrous tissue were remnants of the primary cartilaginous capusule undergoing absorption, as well as islands of partially removed endochondral bone. The abnormalities in both instances indicated a disturbance in maturation or resorption of the embryonal cartilaginous capsule.

Abnormalities, Multiple

Osteogenesis imperfecta congenita and tarda: a temporal bone report.

The temporal bone report of an operated case of osteogenesis imperfecta tarda is presented. Histological examination confirmed the presence of bilateral fixation of the footplate by otosclerosis as the cause of the conductive hearing loss. Fragility of bony septae in the mastoid and of the stapedial crura were observed. Sensorineural impairment in later years with a reduction in neural elements in the cochlea appear related to the extent and activity of the otosclerotic foci. Additional temporal bone reports of three cases of osteogenesis imperfecta congenita show lack of deposition of the skein-like bone in the endochondral layer, sparse bony septae in marrow spaces and deficiency of the perosteal layer. The stapedial crura were thin and in two cases both were deformed and fractured.

Aged

Histopathological observations of presbycusis.

Temporal bone histopathology of 17 aged patients who had spontaneous and gradually progressive bilateral sensorineural hearing losses associated with aging was studied. Six cases in the present material showed the gradually sloping audiometric curve; nine cases, abrupt high tone hearing loss; and two cases, the flat audiometric curve. The most prominent histopathological change in the inner ear was a decrease in the population of the spiral ganglion cells. However, diffuse senile atrophy was also often seen in the organ of Corti and the stria vascularis. A positive correlation between the degree of artheriosclerosis and the degree of sensorineural degeneration in the cochlea was not obtained in the present cases. Also, the correlation was not found to be consistent between the type of the audiometric curve and the localization of lesions in the sensory, the neural or the vascular elements in the cochlea. Our observations show that a certain type of audiometric curve does not necessarily indicate a lesion in a specific cochlear element.

Aged

The temporal bone in the preauricular pit, cervical fistula, hearing loss syndrome.

Histological study of the temporal bones of an infant with the preauricular pit, cervical fistula, hearing loss syndrome revealed abnormalities in the middle ear, the vestibular system, and the cochlea. There is a gross bilateral abnormality in the form and relationship of the middle ear spaces, the middle cranial fossa and the inner ear. The horizontal canal lacks ampulla and crista; the posterior canal terminates a short distance from the ampulla. The cochlear cavity is approximately four fifths of normal size. The modiolus of the cochlea shows minor abnormalities. Spiral ganglion cells and peripheral nerve fibers are present in all coils, but are below normal in population in the basal and apical coils. The stria vascularis is slightly deformed and partly atrophic in the upper apical coil. An occasional concretion is present in the base of the stria vascularis in the middle and apical coils.

Adult

Inner ear degeneration in acoustic neurinoma.

The temporal bones of three cases of acoustic neurinoma are described to illustrate histopathological features of inner ear lesions due to chronic partial obstruction of blood circulation by the tumor in the internal auditory meatus. Degenerative changes in the inner ear due to acoustic neurinoma were evaluated and compared with changes in the opposite ear. The main pathological findings in the inner ear which were attributed to the tumor were degeneration of nerve fibers and of ganglion cells, degeneration of the stria vascularis, degeneration of the tectorial membrane, fibrosis and ossification of a semicircular canal. Fairly good preservation of sensory cells was observed in the presence of total degeneration of nerve fibers and ganglion cells and subtotal degeneration of the stria vascularis.

Aged

Labyrinthitis ossificans.

Three cases with postinflammatory inner ear sequelae are presented to illustrate unusual histopathologic changes. Endolymphatic hydrops without changes in the perilymphatic system was present in one ear following "influenza" meningitis and labyrinthitis ossificans in the contralateral ear. The characteristic histopathological changes of the temporal bones with hematogenic bacterial infection were an extensive labyrinthine ossification associated with a generalized sclerotic change of the whole periotic bone. Bony fixation of the stapedial footplate occurred with the generalized inflammatory process of the otic capsule. Severe and diffuse labyrinthitis ossificans occurred in one case due to tympanogenic inflammation spreading through the round window membrane in the course of suppurative otitis media. A general immunosuppression leading to fatal termination was the apparent factor predisposing to the inner ear complication.

Adult

Ear anomalies associated with renal dysplasia and immunodeficiency disease. A histopathological study.

The histopathologic study of the temporal bones of a case with low-set rudimentary auricles, without preauricular pits or cervical fistula is presented. Abnormalities of the middle and inner ear, fusion of the kidneys, hydrocephalus, short-limbed dwarfism and immunodeficiency are described. An abnormally low position of the middle cranial fossa in relation to the petrous pyramid was observed. The cochlea was of normal length. The modiolus was poorly developed with apparently normal population of ganglion cells and moderate diffuse hydrops of the cochlear duct and saccule. The stria vascularis was partially degenerated in the upper apical coil. Vestibular abnormalities included bilateral absence of common crus of the vertical canals and unusually high origin of endolymphatic aqueduct with no medial dilation present, the convoluted portion of the sac located beneath the dura.

Abnormalities, Multiple

Necrotizing ("malignant") external otitis histopathologic processes.

The histologic findings in a serially sectioned temporal bone, from a patient who succumbed to brain abscess secondary to necrotizing ("malignant") external otitis, are described. The mechanism of invasion of the ear canal appears to be due to local bone necrosis. This in turn extends to the submucosal vasculature of the pneumatic spaces. The infective process extends submucosally, establishing one or several sites of bone destruction. The lumen of the pneumatic space is not involved. In this process, the periphery of the fibrous inflammatory tissue formation is the site of active bone destruction. In pneumococcal petrositis, the peripheral fibrous elements are protective. The process in malignant external otitis may extend directly to adjacent central nervous system structures inoculating the structure with Pseudomonas. Development of Pseudomonas brain abscesses can be slow, allowing for new bone closure of the site from which the infection spreads as demonstrated in this specimen. Therefore, apparent local control of the disease can be established while a central infective process progresses.

Aged