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.

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.

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.

Otopathology in a Case of Type I Waardenburg's Syndrome

2001 ◽  
Vol 110 (9) ◽  
pp. 875-882 ◽  
Author(s):  
Saumil N. Merchant ◽  
Michael J. McKenna ◽  
Aubrey Milunsky ◽  
Clinton T. Baldwin ◽  
Joseph B. Nadol
Keyword(s):  
Neural Crest ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Spiral Ganglion ◽  
Low Frequency ◽  
Tectorial Membrane ◽  
Type I ◽  
Dark Cells ◽  
Pathological Alteration ◽  
Microscopic Studies

We report a case of type I Waardenburg's syndrome that provides insight into the etiopathogenesis of sensorineural hearing loss (SNHL) in this syndrome. The subject, a 76-year-old woman with type I Waardenburg's syndrome (dystopia canthorum, heterochromia irides, and white hair), had congenital low-frequency SNHL in her right ear only, which had remained relatively stable throughout her life. Blood leukocyte DNA studies revealed a PAX-3 mutation with a 1 base pair C-to-A substitution in exon 5 at base 602. Light microscopic studies of the right cochlea showed intact neurosensory structures in only the lower basal turn, with the remainder of the cochlea showing absence of melanocytes, absence of stria vascularis, missing hair cells, dysmorphogenesis of the tectorial membrane, and lack of peripheral processes of the spiral ganglion cells. There was pathological alteration of the vestibular dark cells with marked reduction of melanocytes associated with these dark cells. The left inner ear was normal, with a full complement of neurosensory structures, including melanocytes. Because the PAX-3 gene is involved in neural crest development and melanocytes migrate from the neural crest to the ear, the findings in this case are consistent with the hypothesis that defective melanocyte migration or defective melanocyte function results in defective development of the stria vascularis (and perhaps other structures of the ear). leading to SNHL.

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.

Blebs in inner and outer hair cells: a pathophysiological hypothesis

2008 ◽  
Vol 122 (11) ◽  
pp. 1151-1155 ◽  
Author(s):  
R Ramírez-Camacho ◽  
J R García-Berrocal ◽  
A Trinidad ◽  
J M Verdaguer ◽  
J Nevado
Keyword(s):  
Hair Cells ◽  
Outer Hair Cell ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Spiral Ganglion ◽  
Organ Of Corti ◽  
Outer Hair Cells ◽  
Inner Hair Cells ◽  
Hearing Thresholds

AbstractIntroduction:The ototoxic effects of cisplatin include loss of outer hair cells, degeneration of the stria vascularis and a decrease in the number of spiral ganglion cells. Scanning microscopy has shown balloon-like protrusions (blebs) of the plasma membrane of inner hair cells following cisplatin administration. The present study was undertaken to identify the possible role of inner and outer hair cell blebs in the pathogenesis of cisplatin-induced ototoxicity.Materials and methods:Twenty-five guinea pigs were injected with cisplatin and their hearing tested at different time-points, before sacrifice and examination with scanning electron microscopy.Results and analysis:Seven animals showed blebs in the inner hair cells at different stages. Hearing thresholds were lower in animals showing blebs.Discussion:Cisplatin seems to be able to induce changes in inner hair cells as well as in other structures in the organ of Corti. Blebbing observed in animals following cisplatin administration could play a specific role in the regulation of intracellular pressure.

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.

Histopathological Observations of Presbycusis

1976 ◽  
Vol 85 (2) ◽  
pp. 169-184 ◽  
Author(s):  
Fumiro Suga ◽  
John R. Lindsay
Keyword(s):  
Hearing Loss ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Spiral Ganglion ◽  
Organ Of Corti ◽  
Histopathological Change ◽  
High Tone ◽  
Present Material ◽  
Aged Patients ◽  
Vascular Elements

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 arteriosclerosis 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.

Sudden Deafness

1977 ◽  
Vol 86 (3) ◽  
pp. 269-279 ◽  
Author(s):  
Isamo Sando ◽  
Anthony Loehr ◽  
Takehiko Harada ◽  
John H. Sobel
Keyword(s):  
Viral Infection ◽  
Endolymphatic Hydrops ◽  
Stria Vascularis ◽  
Organ Of Corti ◽  
Sudden Deafness ◽  
Histopathological Study ◽  
Tectorial Membrane ◽  
Viral Origin ◽  
Cochlear Aqueduct ◽  
Temporal Bones

The histopathological study of two cases of sudden deafness is presented. The temporal bones showed cochleosaccular abnormality. The most striking pathological changes were collapse of the organ of Corti, atrophy of the tectorial membrane, atrophy of the stria vascularis, decrease in the number of the cochlear nerves, collapse of the saccular membrane and partial absence of the sensory epithelial layer in the saccular macula. These changes are quite similar in type to those occurring in labyrinthitis of known viral etiology and to those in previously reported cases of sudden deafness which were assumed to be of viral origin. This evidence suggests that a viral infection was the most probable etiology of sudden deafness in these ears. In addition, unusual findings of endolymphatic hydrops limited to the extreme basal end of the cochlear duct were found in Case 1. A patent cochlear aqueduct and circumscribed perilymphatic labyrinthine ossification in the superior semicircular canal were also observed. With these histopathological findings, the possibility of viral infection via the meninges as well as via the hematogenous route into the inner ear is proposed.

scholarly journals Histopathology of the Human Inner Ear in Alström's Syndrome

2015 ◽  
Vol 20 (4) ◽  
pp. 267-272 ◽  
Author(s):  
Joseph B. Nadol Jr ◽  
Jan D. Marshall ◽  
Roderick T. Bronson
Keyword(s):  
Inner Ear ◽  
Autosomal Recessive ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Genetic Disorder ◽  
Spiral Ganglion ◽  
Organ Of Corti ◽  
Outer Hair Cells ◽  
Spiral Ligament ◽  
Human Inner Ear

Alström's syndrome is an autosomal recessive syndromic genetic disorder caused by mutations in the ALMS1 gene. Sensorineural hearing loss occurs in greater than 85% of patients. Histopathology of the inner ear abnormalities in the human has not previously been fully described. Histopathology of the inner ear in Alström's syndrome is presented in 2 genetically confirmed cases. The predominant histopathologic correlates of the sensorineural loss were degeneration of the organ of Corti, both inner and outer hair cells, degeneration of spiral ganglion cells, and atrophy of the stria vascularis and spiral ligament.

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.

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