Chronic Intracochlear Electrode Implantation: Cochlear Pathology and Acoustic Nerve Survival

1974 ◽  
Vol 83 (2) ◽  
pp. 202-215 ◽  
Author(s):  
Robert A. Schindler ◽  
Michael M. Merzenich
Keyword(s):  
Hair Cells ◽  
Bone Growth ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Round Window ◽  
Spiral Ganglion ◽  
Nerve Fibers ◽  
Spiral Ligament ◽  
Scala Tympani ◽  
Temporal Bones

The temporal bones of ten cats implanted with intracochlear electrodes for three to 117 weeks were stained with hematoxylin and eosin and examined with light microscopy. The electrodes were embedded in Silastic® which was molded to fill the most basal 9 mm of the scala tympani. They were inserted directly into the scala through the round window. Among our observations were the following: 1) All or nearly all hair cells were lost in the basal coil during the first several weeks after implantation. Some, but not all, supporting cells were also lost. There was extensive hair cell loss in the middle and apical turns, although some hair cells were seen there in all examined cats. 2) There was evidence of degeneration of spiral ganglion cells in the basal cochlea in several animals, but most primary auditory neurons including (with two exceptions) most of those in the region directly over the electrode, survived implantation in every cat. The radial nerve fibers of the spiral ganglion cells also survived long-term implantation. The functional viability of remaining spiral ganglion cells was confirmed in acute neurophysiological experiments conducted just before the animals were sacrificed. 3) More severe degeneration was seen in two cats in which the electrode perforated the basilar partition. In these animals, there was loss of many spiral ganglion cells, and evidence of new bone growth in the region of the perforation. 4) The appearance of the stria vascularis and spiral ligament in some implanted animals paralleled their descriptions following occlusion of the cochlear vein. 5) Connective tissue formed around the electrode surfaces, apparently displacing perilymph and sealing the electrode into the scala tympani. There was no evidence of perilymph fistula in any animal. 6) There was little evidence of progressive degeneration of the organ of Corti or spiral ganglion from three to 34 weeks after implantation. Some of the implications and limitations of these findings are discussed.

Cochlear Changes in Patients with Type 1 Diabetes Mellitus

2005 ◽  
Vol 133 (1) ◽  
pp. 100-106 ◽  
Author(s):  
Hisaki Fukushima ◽  
Sebahattin Cureoglu ◽  
Patricia A. Schachern ◽  
Takeshi Kusunoki ◽  
Mehmet F. Oktay ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Type 1 Diabetes ◽  
Type 1 Diabetes Mellitus ◽  
Hair Cells ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Spiral Ganglion ◽  
Temporal Bones ◽  
Lateral Walls

OBJECTIVE: To evaluate the effects of diabetes on cochlear elements in human beings. STUDY DESIGN AND SETTING: Twenty-six temporal bones (mean age, 37.5 years) with type 1 diabetes and 30 age-matched controls were examined by light microscopy. We compared the findings of cochlear vessels, hair cells, spiral ganglion cells, and cochlear lateral walls. RESULTS: In diabetics, the walls of vessels of the basilar membrane ( P < 0.001) and vessels of the stria vascularis were ( P < 0.01) significantly thicker in all turns and loss of outer hair cells (OHCs) was significantly greater in the lower basal turn ( P < 0.01). Atrophy of the stria vascularis in all turns ( P < 0.0001) and loss of spiral ligament cells in upper turns ( P < 0.01) were significantly higher than controls. No significant difference was obtained in the number of spiral ganglion cells between groups. CONCLUSION: This study suggests that type 1 diabetes mellitus can cause cochlear microangiopathy and subsequently degeneration of cochlear lateral walls and OHCs.

The Temporal Bone in the Preauricular Pit, Cervical Fistula, Hearing Loss Syndrome

1976 ◽  
Vol 85 (2) ◽  
pp. 268-275 ◽  
Author(s):  
Naomi Fitch ◽  
John R. Lindsay ◽  
Herbert Srolovitz
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Histological Study ◽  
Spiral Ganglion ◽  
Middle Cranial Fossa ◽  
Nerve Fibers ◽  
Horizontal Canal ◽  
Temporal Bones

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.

Electron Microscopic Findings in Presbycusic Degeneration of the Basal Turn of the Human Cochlea

1979 ◽  
Vol 87 (6) ◽  
pp. 818-836 ◽  
Author(s):  
Joseph B. Nadol
Keyword(s):  
Light Microscopy ◽  
Hair Cells ◽  
Basilar Membrane ◽  
Ganglion Cells ◽  
Nerve Fibers ◽  
Degenerative Changes ◽  
Supporting Cells ◽  
Neural Degeneration ◽  
Basal Turn ◽  
Temporal Bones

Three human temporal bones with presbycusis affecting the basal turn of the cochlea were studied by light and electron microscopy. Conditions in two ears examined by light microscopy were typical of primary neural degeneration, with a descending audiometric pattern, loss of cochlear neurons in the basal turn, and preservation of the organ of Corti. Ultrastructural analysis revealed normal hair cells and marked degenerative changes of the remaining neural fibers, especially in the basal turn. These changes included a decrease in the number of synapses at the base of hair cells, accumulation of cellular debris in the spiral bundles, abnormalities of the dendritic fibers and their sheaths in the osseous spiral lamina, and degenerative changes in the spiral ganglion cells and axons. These changes were interpreted as an intermediate stage of degeneration prior to total loss of nerve fibers and ganglion cells as visualized by light microscopy. In the third ear the changes observed were typical of primary degeneration of hair and supporting cells in the basal turn with secondary neural degeneration. Additional observations at an ultrastructural level included maintenance of the tight junctions of the scala media despite loss of both hair and supporting cells, suggesting a capacity for cellular “healing” in the inner ear. Degenerative changes were found in the remaining neural fibers in the osseous spiral lamina. In addition, there was marked thickening of the basilar membrane in the basal turn, which consisted of an increased number of fibrils and an accumulation of amorphous osmiophilic material in the basilar membrane. This finding supports the concept that mechanical alterations may occur in presbycusis of the basal turn.

Otologic Histopathology of Fabry's Disease

1989 ◽  
Vol 98 (5) ◽  
pp. 359-363 ◽  
Author(s):  
Patricia A. Schachern ◽  
Michael M. Paparella ◽  
Donald A. Shea ◽  
Tae H. Yoon
Keyword(s):  
Temporal Bone ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Cell Loss ◽  
The Body ◽  
Spiral Ligament ◽  
Fabry’S Disease ◽  
Fabry's Disease ◽  
Temporal Bones ◽  
Spiral Ganglia

Fabry's disease is a rare progressive X-linked recessive disorder of glycosphingolipid metabolism. The accumulation of glycosphingolipids occurs in virtually all areas of the body, including the endothelial, perithelial, and smooth-muscle cells of blood vessels, the ganglion cells of the autonomic nervous system, and the glomeruli and tubules of the kidney. Although otologic symptoms have been described in these patients, to our knowledge there have been no temporal bone histopathologic reports. We describe the clinical histories, audiometric results, and temporal bone findings of two patients with this rare disorder. Both patients demonstrated a bilateral sloping sensorineural hearing loss audiometrically. Middle ear findings of seropurulent effusions and hyperplastic mucosa were seen in all four temporal bones. Strial and spiral ligament atrophy in all turns, and hair cell loss mainly in the basal turns, were also common findings. The number of spiral ganglion cells was reduced in all temporal bones; however, evidence of glycosphingolipid accumulation was not observed in the spiral ganglia.

Fine Structure Histopathology of Labyrinthitis Ossificans in the Gerbil Model

2005 ◽  
Vol 114 (2) ◽  
pp. 161-166 ◽  
Author(s):  
Steven P. Tinling ◽  
Vishad Nabili ◽  
Hilary A. Brodie
Keyword(s):  
Connective Tissue ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Spiral Ganglion ◽  
Organ Of Corti ◽  
Spiral Ligament ◽  
Otic Capsule ◽  
Dense Connective Tissue ◽  
Labyrinthitis Ossificans ◽  
End Stage

Labyrinthitis ossificans (LO) is the pathological deposition of new bone within the lumen of the cochlea and labyrinth. This process occurs most commonly as a result of infection or inflammation affecting the otic capsule. Trauma and vascular compromise can also lead to neo-ossification within the otic capsule. The mechanism that regulates this process remains unestablished. This study details the end-stage histopathology in high-resolution plastic thin sections. Twenty Mongolian gerbils were infected by intrathecal injection of Streptococcus pneumoniae type 3 followed by subcutaneous penicillin G procaine (8 days) and were painlessly sacrificed 3 months later. The cochleas were serially divided and sectioned for light and electron microscopy. Sixteen of 20 animals (27 of 40 cochleas) demonstrated LO. Cochlear damage was most extensive in the vestibule and basal turn and decreased toward the apex, which often appeared normal. The histopathologic findings consisted of 1) new bone, calcospherites, osteoid, and fibrosis without dense connective tissue or osteoblasts extending from the endosteal wall into the lumen of the vestibule and scala tympani; 2) areas of dense connective tissue and osteoid enclosed by epithelial cells conjoined with the organ of Corti, stria vascularis, spiral ligament, and vestibular (Reissner's) membrane; and 3) partial to complete loss of the organ of Corti, spiral ligament cell bodies, stria vascularis, and spiral ganglion cells. Osteoblastic activity was not demonstrated in end-stage ossification in LO in the gerbil model. Neoossification appears to occur by calcospherite deposition along collagen-like fibrils within osteoid. The destruction of the organ of Corti, spiral ganglion cells, stria vascularis, and cells of Reissner's membrane and the spiral ligament occurs even in the absence of ossification of the cochlear duct.

scholarly journals NTPDase1 and NTPDase2 Immunolocalization in Mouse Cochlea: Implications for Regulation of P2 Receptor Signaling

2002 ◽  
Vol 50 (11) ◽  
pp. 1435-1441 ◽  
Author(s):  
Srdjan M. Vlajkovic ◽  
Peter R. Thorne ◽  
Jean Sévigny ◽  
Simon C. Robson ◽  
Gary D. Housley
Keyword(s):  
Hair Cells ◽  
Spiral Ganglion ◽  
Organ Of Corti ◽  
Nerve Fibers ◽  
Outer Hair Cells ◽  
Spiral Ligament ◽  
Receptor Signaling ◽  
P2 Receptor ◽  
Efferent Nerve ◽  
Root Region

Cellular, molecular, and physiological studies have demonstrated an important signaling role for ATP and related nucleotides acting via P2 receptors in the cochlea of the inner ear. Signal modulation is facilitated by ectonucleotidases, a heterologous family of surface-located enzymes involved in extracellular nucleotide hydrolysis. Our previous studies have implicated CD39/NTPDase1 and CD39L1/NTPDase2, members of the ectonucleoside triphosphate diphosphohydrolase (E-NTPDase) family, as major ATP-hydrolyzing enzymes in the tissues lining the cochlear endolymphatic and perilymphatic compartments. NTPDase1 hydrolyzes both nucleoside triphosphates and diphosphates. In contrast, NTPDase2 is a preferential nucleoside triphosphatase. This study characterizes expression of these E-NTPDases in the mouse cochlea by immunohistochemistry. NTPDase1 can be immunolocalized to the cochlear vasculature and neural tissues (primary auditory neurons in the spiral ganglion). In contrast, NTPDase2 immunolabeling was principally localized to synaptic regions of the sensory inner and outer hair cells, stereocilia and cuticular plates of the outer hair cells, supporting cells of the organ of Corti (Deiters’ cells and inner border cells), efferent nerve fibers located in the intraganglionic spiral bundle, and in the outer sulcus and root region of the spiral ligament. This differential expression of NTPDase1 and 2 in the cochlea suggests spatial regulation of P2 receptor signaling, potentially involving different nucleotide species and hydrolysis kinetics.

Inner Ear Degeneration in Acoustic Neurinoma

1976 ◽  
Vol 85 (3) ◽  
pp. 343-358 ◽  
Author(s):  
Fumiro Suga ◽  
John R. Lindsay
Keyword(s):  
Inner Ear ◽  
Blood Circulation ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Nerve Fibers ◽  
Sensory Cells ◽  
Tectorial Membrane ◽  
Acoustic Neurinoma ◽  
Pathological Findings ◽  
Temporal Bones

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.

Comparison of Cochlear Histopathology following Two Implant Designs for Use in Scala Tympani

1980 ◽  
Vol 89 (2_suppl) ◽  
pp. 11-14 ◽  
Author(s):  
Dwight Sutton ◽  
Josef M. Miller ◽  
Bryan E. Pfingst
Keyword(s):  
Basilar Membrane ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Spiral Ganglion ◽  
Mechanical Damage ◽  
Scala Tympani ◽  
Basal Turn ◽  
Induced Changes

Two designs of intracochlear implants were evaluated for their histopathologic effects in the monkey ear. A molded electrode designed to fit the contour of the basal turn of the scala tympani tended to create basilar membrane fistulas and osseous spiral lamina fracture, along with relatively extensive loss of spiral ganglion cells in sites adjacent to the implant. A delicate, free-fit electrode induced local encapsulation but little or no mechanical damage and limited degeneration of spiral ganglion cells. Both electrode types occasionally induced changes in stria vascularis.

Histopathology of Human Temporal Bone After Cis-Platinum, Radiation, or Both

1998 ◽  
Vol 118 (6) ◽  
pp. 825-832 ◽  
Author(s):  
DICK L. HOISTAD ◽  
FRANK G. ONDREY ◽  
CEMIL MUTLU ◽  
PATRICIA A. SCHACHERN ◽  
MICHAEL M. PAPARELLA ◽  
...  
Keyword(s):  
Temporal Bone ◽  
Hair Cells ◽  
Stria Vascularis ◽  
Combined Treatment ◽  
Spiral Ganglion ◽  
Outer Hair Cells ◽  
Vascular Walls ◽  
Facial Nerves ◽  
Temporal Bones ◽  
Normal Controls

Preserving organs by use of multiple modalities has become protocol in treating squamous cell carcinomas of the head and neck, but cis-platinum and radiation can impair hearing. To determine the effect of cis-platinum, radiation, or a combination of these treatments on the temporal bone, we studied histopathologic slides of 15 human temporal bones: four after cis-platinum, five after radiation, two after combined treatment, and four from normal controls. Hair cells and cells in spiral ganglia were counted in reconstructed organs of Corti. Lumen-to-diameter indexes in arterioles near facial nerves were quantified for four normal controls and seven irradiated patients. Available audiograms were compared. Decreased spiral ganglion cells, loss of inner and outer hair cells, and atrophy of stria vascularis were demonstrated in groups receiving cis-platinum, radiation, and combinations, compared with age-matched controls. Arterioles around facial nerves demonstrated fibrinous clots within the intima, endothelial proliferation, and hypertrophy and fibrosis of vascular walls in smooth muscle. Fibrosis in connective tissue was clearly progressive after radiation. Cis-platinum and radiation can contribute to otologic sequelae, including sensorineural hearing losses, vascular changes, serous effusion, or fibrosis. Prophylactic treatments and techniques to deliver them should be considered for protection of temporal bones and preservation of hearing after oncologic modalities. (Otolaryngol Head Neck Surg 1998;118:825–32.)

Cochlear and Vestibular Lesions in Capsular Otosclerosis as Seen in Microdissection

1978 ◽  
Vol 87 (2_suppl2) ◽  
pp. 1-40 ◽  
Author(s):  
Lars-Göran Johnsson ◽  
Joseph E. Hawkins ◽  
Fred H. Linthicum
Keyword(s):  
Hair Cells ◽  
Round Window ◽  
Organ Of Corti ◽  
Otic Capsule ◽  
Scala Tympani ◽  
Toxic Factor ◽  
Basal Turn ◽  
Vestibular Pathology ◽  
Temporal Bones ◽  
State Of Activity

In 24 temporal bones from patients with otosclerosis prepared by the method of microdissection and surface preparations, otosclerotic foci could be clearly seen during removal of the otic capsule. The state of activity of each focus was estimated on the basis of its consistency and vascularity. Small anterior foci constituted the most common form of involvement of the otic capsule. All were judged to be inactive, and none of them appeared to have caused obvious sensorineural degeneration. No cases of “pure cochlear otosclerosis” were seen. Sensorineural degeneration was associated with large anterior foci which reached the upper basal turn. One specimen displayed a circumscribed sensorineural degeneration in the upper basal turn, with an almost exact correspondence between the location and extent of the cochlear lesion and the site of invasion by the otosclerotic process in the bone and endosteum bordering on scala media and scala tympani. It is postulated that a toxic factor had diffused from the focus and acted directly on the organ of Corti. When multiple foci were present they were usually poorly defined. The otosclerotic process involved the round window, with new lamellar bone formation in the scala tympani of the lower half of the basal turn. The most extensive sensorineural degeneration in the entire material was seen in this group. One specimen also had severe cochlear hydrops. In three specimens large shunts were observed to connect the otosclerotic foci with the cochlear vasculature, which was severely dilated. Where otosclerosis involved the endosteum of the scala tympani, loss of vessels was observed. One specimen with extensive active capsular otosclerosis had severe sensorineural degeneration of the vestibular system. Vestibular pathology in fenestrated ears is also described. In a specimen from a patient with no caloric reaction, numerous hair cells were present in the macular organs.

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