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Biomedical subjects

R Gussen

Publications and source records attributed to R Gussen.

At least 19 recordsLinked to original sources

Surgical trauma to the lateral semicircular canal with preservation of hearing.

Surgical trauma to the lateral semicircular canal (LSC) is a rare complication which does not always result in cochlear damage. The authors present six such cases, discuss the histopathological findings of one case, and review 12 previous reports. Transient cochlear depression with normalization of function within 6 weeks of trauma occurred in most instances. Vestibular symptoms were pronounced and, although compensation was not delayed, positional dizziness and instability usually persisted for several months and occasionally for a year or more. Histopathological findings (two specimens) were significant for obliterative scarring of the LSC at the site of injury and for the structural integrity of the labyrinth and cochlea. The mechanism of cochlear protection in these cases remains obscure; however, clean injuries away from the ampullary end of the canal occurring in patients without preexisting inner ear disease appear to have a better prognosis. Intraoperative closure of the defect, postoperative bed rest, and antibiotics are the basic elements of management.

Adult

The endolymphatic sac in the Mondini disorder.

The endolymphatic sacs are described in temporal bone specimens from a 31-year-old man with bilateral Mondini disorder. The ducts and sacs are thin-walled, cyst-like structures with complete absence of loose vascular perisac tissue, and are directly apposed to the bone of the vestibular aqueduct. Histological evidence of severe bone erosion is present in these specimens and is most marked in the intermediate and distal portions of the vestibular aqueduct. It is also present in the foveal region of the posterior temporal bone surface underlying the sac. Erosion of the bony wall of the paravestibular canaliculus (PVC) is demonstrable, with incorporation of the vein of the PVC inside the margin of the widened vestibular aqueduct. These findings suggest a causal relationship between pressure within the endolymphatic duct and sac and erosion of the surrounding bone. The absence of endolymphatic hydrops of the cochlea and vestibular organs in the Mondini disorder contrast significantly with the endolymphatic hydrops seen in Meniere's disease.

Adult

The fate of an ossicular allograft in tympanoplasty.

Correction of ossicular defects in tympanoplasty most commonly involves the use of commercially available prostheses or preserved allograft ossicles. Incus autografts and tragal cartilage autografts are also used by many surgeons. Presculptured preserved allograft ossicles have not been used widely, but are used almost exclusively by our clinic. The fate of ossicular grafts has been reported by a number of in investigators with evidence obtained at revision surgery. In this paper we will be able to trace the fate of a presculptured preserved autograft ossicle clinically and pathologically. The unique aspect of this study is the demonstration of the ossicular status in post mortem temporal bone dissection followed by histopathological serial section studies.

Aged

Vascular mechanisms in Meniere's disease.

Normal venous drainage of the vestibular organs through the vein of the paravestibular canaliculus (PVC) may be crucial to inner ear fluid mechanics. It is proposed that increased venous pressure, with resultant venous insufficiency of the vestibular organs, may result in endolymphatic hydrops unless collateral venous circulation develops. Certain variations in pattern of venous drainage where the vestibular organs drain predominantly through the PVC vein may be a predisposing factor. In patients with Meniere's disease, different mechanisms can cause venous insufficiency. One suggested mechanism is morphologic change in the microcirculation of the intermediate portion of the endolymphatic sac. Microcirculation changes may be associated with fibrosis of the perisac tissues or shortening of the intermediate sac region or might be physiologically determined. Venous insufficiency may also result from anomalies of the PVC vein.

Ear, Inner

Vascular mechanisms in Meniere's disease. Theoretical considerations.

Unimpeded venous drainage of the vestibular organs via the paravestibular canaliculus (PVC) vein is crucial to inner ear fluid mechanisms. With increased venous pressure, insufficient drainage may result in endolymphatic hydrops, unless collateral veins develop. Different mechanisms cause venous insufficiency in different cases of Meniere's. One suggested mechanism is alteration of the intermediate endolymphatic sac (IES) microcirculation with decreased regulation of blood held within subepithelial capillaries of the sac. Increased blood may thus drain into the veins, with increased venous pressure and inadequate vestibular drainage. Altered microcirculation may be morphological as in fibrosis or shortening of the IES, or physiological where the microcirculation receives abnormal stimuli or reacts abnormally to normal stimuli. Venous insufficiency may result from PVC vein anomalies. Variations in venous pattern whereby vestibular organs preponderantly drain through the PVC vein may predispose to Meniere's disease.

Collateral Circulation

Sudden hearing loss associated with cochlear membrane rupture. Two human temporal bone reports.

Cochlear membrane ruptures occurred in the left temporal bones of two patients--one was a result of barotrauma caused by flying and was associated with sudden deafness, tinnitus, and some vertigo and the second occurred in a patient with profound deafness in a previously normal-hearing ear. Both occurred as ruptures of Reissner's membrane at the junction of the ductus reuniens with the cecum vestibulare portion of the cochlear duct. With healing, a balloon-like structure formed from the rupture site into the adjacent vestibule, resulting in a secondarily ruptured saccule duct in one case and in collapse of the saccule in the second case. Left-sided preponderance of such ruptures and the vulnerability of the ductus reuniens junction with the cochlea are described.

Aged

Middle and inner ear changes in congenital rubella.

Temporal bone changes in a 14-month-old female infant with congenital rubella are described. The inner ears showed cochleo-saccular degeneration, with more severe involvement of the right side. Perivascular round cell infiltration was demonstrated in the base of the cochlea, stria vascularis, and superior ampullary cribriform region, consistent with viral infection. Whether the latter was related to the rubella or the the patient's terminal viral infection is undetermined. Predominant involvement of the cochlea saccule may have been related o their blood supply from the vestibulo-cochlear artery, which is a more direct continuation of the labyrinthine artery than are the other branches. Middle ear changes consisted of a fetal type of stapes, a small area of malleus head fixation on one side, and absence of the medial component of the posterior incudal ligament, with a joint-like attachment between the short process of the incus and the medial wall of the fossa incudis. This may be an abnormality, or it might represent a variation of normal development.

Ductus Arteriosus, Patent

Saccule otoconia displacement into cochlea in cochleosaccular degeneration.

Eight human temporal bones from five patients demonstrated varying degrees of cochleosaccular degeneration. Otoconial debris, with tissue reaction, was demonstrated within the ductus reuniens and cochlear duct, as far distal as the middle of the ascending basal limb. Saccule degeneration could be the primary lesion in cochleosaccular degeneration related to heredity or aging, with cochlear changes secondary to the presence of displaced otoconia. In cochleosaccular degeneration of viral cause, if saccule otoconial displacement occurs, it may possibly produce additional damage in a cochlea involved by viral infection. Cochlear damage might be related to the severity of otoconial displacement and/or to impaired clearing mechanisms of the cochlea. Retrograde cochlear hydrops, secondary to collapse of the saccule, occurs first in the cecum vestibulare and may extend distally.

Adult

Temporal bone findings in rhabdomyosarcoma with predominantly petrous involvement.

Temporal bone rhabdomyosarcomas are rare tumors that occur almost exclusively in children. They arise most commonly in the middle ear, from which they extend through the mastoid and petrous portions of the temporal bone, eventually invading the cranium. We report the histopathologic findings of a rhabdomyosarcoma with anterior and medial displacement, predominantly involving the petrous bone. Recognition and separate study of this type of tumor is clinically important. Predominantly petrosal rhabdomyosarcomas are less prevalent than similar lesions with lateral expansion and tend to develop significant ear symptoms late in the course of the disease. When first diagnosed, these lesions usually exhibit signs of intracranial extension, and consequently are less amenable to treatment and are more rapidly fatal.

Child

Histological evidence of specialized microcirculation of the endolymphatic sac.

A specialized type of blood vessel is demonstrated within the dense soft tissue areas of the vestibular aqueduct and vascular channels of the surrounding bone, including the paravestibular canaliculus (PVC). The vessel wall is formed by the collagen-smooth muscle bundles of these areas. The lumen of these vessels is irregular, and segments appear to be closed by apposition of the bundles. The vessels are continuous with the capillaries of the endolymphatic sac. It is not clear whether they are on the venous side of the capillary system, or on the arterial side and might possibly be part of an arteriovenous anastomoses system. Their possible role in some cases of Meniere's disease must be considered.

Adult

Round window niche melanocytes and webby tissue. Possible collateral route of vascular and perilymph circulation.

Two hundred thirteen human temporal bones were studied for characteristics of the round window niche. In 163 specimens melanocytes were found within the attachments of the round window membrane and in the niche, most prominently at the level of the cochlear aqueduct opening. Sixty-two specimens demonstrated discrete webby foci within the lining of the niche, 16 of which completely or partially covered the tympanic surface of the round window membrane, unassociated with fibrous tags or adhesions, and in some cases contained congested vascular channels and melanocytes. The webby foci were sometimes associate; with melanocytes, while melanocytes were present without demonstrable webby foci. These findings may represent a collateral route of vascular or perilymph-related drainage from the inner ear.

Adolescent

Nonotosclerotic ankylosis of the stapedial footplate.

A woman with a long history of chronic otitis media had a stapedectomy after a surgical diagnosis of otosclerosis. In the histologic study of the temporal bones, done after her death four years later, the main portion of the stapes footplate was present but fractured and no otosclerosis was present. New celluluar bone which simulated otosclerosis filled the anterior oval window niche and was continuous with the middle ear surface of the footplate fragments. The new bone extended anteriorly to overlie a tympanosclerotic focus and inferiorly was continuous with the periosteum of the middle ear. At least part of the new bone formation occurred after stapedectomy. This may explain the poor long-term results frequently encountered when a stapedectomy is done on patients with tympanosclerotic fixation of the stapes footplate.

Diagnosis, Differential

Atypical ossicle joint lesions in rheumatoid arthritis with sicca syndrome (Sjögren syndrome).

Atypical incudomalleal and incudostapedial joint changes were found in a 55-year-old woman with long-standing rheumatoid arthritis and sicca syndrome (Sjögren syndrome). Available audiograms taken at 37 and 42 years of age demonstrated slight bilateral high-frequency loss of hearing. The ossicular joint changes involved dissolution of disk material together with proliferation of synovial-type elements of the disk and articular surfaces, with formation of pannus-like tissue. There was extensive destruction of cartilage, with cellular collagenous tissue extending along the exposed bone surfaces. Despite strong similarities to rheumatoid arthritis, a definite diagnosis cannot be made in the absence of the inflammatory, lymphocyteplasma cell component.

Arthritis, Rheumatoid

Inner ear pathologic features following mumps infection. Report of a case in an adult.

Temporal bone studies in an adult with a moderately severe, bilateral sensorineural hearing loss revealed bilateral cochlear changes 13 years after mumps infection. The organ of Corti was completely absent in the greater part of the superior horizontal basal limbs, with occasional hair cell loss throughout the rest of the cochlea. The outer sulcus cell area was degenerated. The stria vascularis was normal, as was the tectorial membrane, except for small hyaline droplets. The number of nerve fibers was extremely decreased in the spiral bony lamina of the basal turns. Basophilic material, possibily representing degeneration of otoliths, was present in the saccule and utricle, bilaterally, with small amounts in all of the ampullae. This was considered to be either a possible result of cytotoxic cancerocidal therapy, or an incidental nonspecific change.

Adult

Labyrinthine otosclerosis and sensorineural deafness. Pathologic findings of the spiral ligament.

In severe labyrinthine otosclerosis, spiral ligament changes occur predominantly adjacent to the stria vascularis, and consist of a decrease or loss of the capillary-perilymph system. It is suggested that this results in increased concentrations of metabolic products in the cochlear fluids that can no longer be absorbed effectively. If vascular shunts form they might represent collateral channels, with the flow of blood from the cochlea into the otosclerotic focus, bypassing the deficient capillary network in the spiral ligament. The zone of dense collagenization is described as a widening of the soft tissues endosteum of the cochlear capsule due to erosion of the bone, with the bone surface now set further back. This may result in greater mobility or loosening of the suspension mechanism of the basilar membrane.

Aged