Temporal Bone Findings in Hemifacial Microsomia

1978 ◽  
Vol 87 (3) ◽  
pp. 399-403 ◽  
Author(s):  
H. K. Chandra Sekhar ◽  
Nobuhtro Toktta ◽  
S. Alexic ◽  
M. Sachs ◽  
John F. Daly
Keyword(s):  
Temporal Bone ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Organ Of Corti ◽  
Petrous Bone ◽  
Nerve Fibers ◽  
Structural Abnormality ◽  
Hemifacial Microsomia ◽  
Partial Deficiency ◽  
The Right

— The temporal bone findings in a case of hemifacial microsomia are described with photographs. The right facial hypoplasia was associated with anophthalmia and microtia on the same side. The right petrous bone was hypoplastic and showed total superior dehiscence of the internal acoustic meatus. The otic capsule was deformed with an underdeveloped cochlear modiolus grossly deficient in spiral ganglion population. The spiralling cochlear shell showed partial deficiency of the interscalar septum between the middle and apical coils. The cochlear duct was shorter than that on the normal side; the organ of Corti however was normal. The vestibular system did not show any structural abnormality except for the degeneration and reduction of the Scarpa's ganglion cells and nerve fibers. An additional interesting fact was that the facial nerve was totally absent in the temporal bone except for its nervus intermedius component.

Temporal Bone Histopathology in Deafness Due to Cryptococcal Meningitis

1979 ◽  
Vol 88 (5) ◽  
pp. 630-636 ◽  
Author(s):  
Takehiko Harada ◽  
Isamu Sando ◽  
Eugene N. Myers
Keyword(s):  
Temporal Bone ◽  
Cryptococcal Meningitis ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Organ Of Corti ◽  
Nerve Fibers ◽  
Vestibular Nerve ◽  
Cochlear Nerve ◽  
Internal Auditory Meatus ◽  
Temporal Bones

This paper reports on a patient who survived an attack of cryptococcal meningitis eight years prior to his death. A bilateral sensorineural hearing loss had been noted a short time before the patient was admitted to the hospital, and was the only complication after he recovered from the disease. Histopathologic study of the temporal bones showed a similar pattern of pathology in both ears, the most striking finding being a severe loss of spiral ganglion cells in Rosenthal's canal, and of cochlear nerve fibers in the osseous spiral lamina and internal auditory meatus. The vestibular nerve was mostly free from pathology. The organ of Corti was atrophic but the hair cell population appeared to be almost normal. A slight number of cryp-tococci were observed in limited areas of the cochlear and the saccular nerves in the internal auditory meatus. The severe pathology of the cochlear nerve was compatible with audiologic evaluations, which pointed to a retrocochlear lesion. Thus, this case demonstrates some characteristic aspects of cryptococcal infection of the temporal bone: The primary site of invasion was the cochlear nerve in the internal auditory meatus and the modiolus, leading to the loss of ganglion cells and nerve fibers, while the vestibular nerve appears to have been resistant to infection.

Deafness in Cryoglobulinemia

1982 ◽  
Vol 91 (3) ◽  
pp. 250-255 ◽  
Author(s):  
Yasuya Nomura ◽  
Shigeo Mori ◽  
Mineko Tsuchida ◽  
Teruhisa Sakurai
Keyword(s):  
Temporal Bone ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Spiral Ganglion ◽  
Organ Of Corti ◽  
Semicircular Canals ◽  
Histopathological Study ◽  
Tectorial Membrane ◽  
Circulatory Disturbance ◽  
Total Deafness

We present a histopathological study of a 44-year-old female with essential cryoglobulinemia. She had suffered from purpura and ulcer in winter, bilateral tinnitus and progressive hearing loss. An audiogram taken a week before her death showed bilateral total deafness. In the cochlea of the left temporal bone, the organ of Corti was either missing or present as a mound. The stria vascularis was atrophic throughout the cochlea. The tectorial membrane showed drooping and encapsulation. Reissner membrane was in the normal position. Eosinophilic precipitate was noted in the scala media at the site where Reissner membrane bulged. The spiral ganglion cells were well preserved. In the lower basal turn, there was fibrosis and ossification intermingled in the scala tympani. Ossification was most marked near the basal end. The semicircular canals and vestibule were almost totally ossified and fused with surrounding bone. There was a small, cyst-like structure in the vestibule containing eosinophilic fluid. Our findings indicate that the deafness was the result of circulatory disturbance due to cryoglobulinemia. To our knowledge, this is the first cryoglobulinemia case in which temporal bone findings are reported.

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.

scholarly journals Induction of Redox-Active Gene Expression by CoCl2 Ameliorates Oxidative Stress-Mediated Injury of Murine Auditory Cells

Antioxidants ◽  
2019 ◽  
Vol 8 (9) ◽  
pp. 399 ◽  
Author(s):  
Jhang Ho Pak ◽  
Junyeong Yi ◽  
Sujin Ryu ◽  
In Ki Kim ◽  
Jung-Woong Kim ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Hypoxia Inducible Factor ◽  
Spiral Ganglion ◽  
Organ Of Corti ◽  
Luciferase Reporter ◽  
Related Factor ◽  
Redox Active

Free radicals formed in the inner ear in response to high-intensity noise, are regarded as detrimental factors for noise-induced hearing loss (NIHL). We reported previously that intraperitoneal injection of cobalt chloride attenuated the loss of sensory hair cells and NIHL in mice. The present study was designed to understand the preconditioning effect of CoCl2 on oxidative stress-mediated cytotoxicity. Treatment of auditory cells with CoCl2 promoted cell proliferation, with increases in the expressions of two redox-active transcription factors (hypoxia-inducible factor 1α, HIF-1α, nuclear factor erythroid 2-related factor 2; Nrf-2) and an antioxidant enzyme (peroxiredoxin 6, Prdx6). Hydrogen peroxide treatment resulted in the induction of cell death and reduction of these protein expressions, reversed by pretreatment with CoCl2. Knockdown of HIF-1α or Nrf-2 attenuated the preconditioning effect of CoCl2. Luciferase reporter analysis with a Prdx6 promoter revealed transactivation of Prdx6 expression by HIF-1α and Nrf-2. The intense immunoreactivities of HIF-1α, Nrf-2, and Prdx6 in the organ of Corti (OC), spiral ganglion cells (SGC), and stria vascularis (SV) of the cochlea in CoCl2-injected mice suggested CoCl2-induced activation of HIF-1α, Nrf-2, and Prdx6 in vivo. Therefore, we revealed that the protective effect of CoCl2 is achieved through distinctive signaling mechanisms involving HIF-1α, Nrf-2, and Prdx6.

Cochlear Neuronal Loss as Determined by Use of a Neurofilament Protein Antibody in Cases of Bilateral Profound Deafness

1998 ◽  
Vol 77 (8) ◽  
pp. 662-668
Author(s):  
Yukiko Iino ◽  
Taku Komiya ◽  
Tomoko Fujii ◽  
Yoshinori Nakamoto ◽  
Satoru Koyama ◽  
...  
Keyword(s):  
Measles Virus ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Neuronal Loss ◽  
Organ Of Corti ◽  
Afferent Neurons ◽  
Neurofilament Protein ◽  
Efferent Neurons ◽  
Temporal Bones ◽  
Bilateral Deafness

The temporal bones of two patients with profound bilateral deafness from infancy were studied immunohistochemically, using a neurofilament protein antibody to detect the cochlear neuronal elements. One patient exhibited Mondini dysplasia of the inner ear, with the organ of Corti almost completely deteriorated. The other patient is the first reported case involving complete aplasia of the organ of Corti in all turns. In both cases, the immunohistochemical staining clearly revealed a severe reduction in the number of afferent neurons, such as dendrites, spiral ganglion cells and cochlear axons. The number of efferent spiral bundles in the osseous spiral lamina and intraganglionic portion also decreased in parallel with the reduction in the number of cochlear afferent neurons. Our results are inconsistent with previously reported cases of presbycusis and acquired deafness induced by the measles virus, in which efferent neurons were preserved while afferent neurons degenerated. The loss of both the efferent and afferent neurons might be characteristics of congenital deafness.

Nerve Fiber Synapses on Spiral Ganglion Cells in the Human Cochlea

1979 ◽  
Vol 88 (6_suppl2) ◽  
pp. 1-17 ◽  
Author(s):  
Robert S. Kimura ◽  
Carol Y. Ota ◽  
Tadahiko Takahashi
Keyword(s):  
Nerve Fiber ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Nerve Fibers ◽  
Parasympathetic Nerve ◽  
Human Cochlea ◽  
Cell Processes ◽  
Unmyelinated Nerve ◽  
Axodendritic Synapses ◽  
Axosomatic Synapses

Nerve fiber synapses were observed on the small myelinated and unmyelinated spiral ganglion cells of the human cochlea. The nerve fibers penetrated the junctions of the ensheathing satellite cells and myelin lamellae, and partly invaginated into the perikarya to establish axosomatic synapses. One small myelinated neuron with a nerve fiber synapse demonstrated multipolar cell processes. However, most small neurons with identical ultrastructural characteristics did not show these synapses. Axodendritic synapses were also seen on the dendritic processes of the small neurons and between varicose nerves and unmyelinated nerve fibers some distance from the small neurons. While these observations conform in some aspects with the concept of parasympathetic nerve fibers and neurons in the cochlea, they are also compatible with the idea that the small neurons may have an auditory function influenced by the synaptic contacts of efferent fibers from the olivocochlear bundle.

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.

Ultrastructural Cochlear Changes following Acoustic Hyperstimulation and Ototoxicity

1976 ◽  
Vol 85 (6) ◽  
pp. 740-751 ◽  
Author(s):  
David J. Lim
Keyword(s):  
Hair Cells ◽  
Sensory Cell ◽  
Ganglion Cells ◽  
Organ Of Corti ◽  
Acoustic Trauma ◽  
Nerve Fibers ◽  
Outer Hair Cells ◽  
Type I ◽  
Cell Degeneration ◽  
Inner Hair Cells

Using guinea pigs and chinchillas as experimental animals, modes and patterns of sensory cell damage by acoustic hyperstimulation and kanamycin intoxication were compared. In general, outer hair cells were more vulnerable to both acoustic trauma and ototoxicity (particularly in the basal turn) than inner hair cells. However, in kanamycin ototoxicity, the inner hair cells were more vulnerable in the apical coil. Nerve endings and nerve fibers generally were resistant to both acoustic trauma and kanamycin intoxication, and their degeneration appears to be secondary to the sensory cell degeneration. A large number of unmyelinated nerve fibers were seen in both the organ of Corti and the osseous spiral lamina even three months after the organ of Corti had been completely degenerated by ototoxicity. The total number of unmyelinated and myelinated nerve fibers in the osseous spiral lamina far exceeded the scanty surviving ganglion cells in Rosenthal's canal, indicating the possibility of regeneration of these fibers following kanamycin intoxication. The remaining few ganglion cells were mainly type II or type III cells, and a majority of the type I ganglion cells appeared to be degenerated. Signs of strial damage were observed in both acoustic trauma and ototoxicity, but their pattern did not correlate well with that of sensory cell degeneration.

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