Viable spiral ganglion cells in congenital and acquired profound hearing loss

1980 ◽  
Vol 94 (4) ◽  
pp. 367-376 ◽  
Author(s):  
B. Ghorayer ◽  
A. Sarwat ◽  
Fred H. Linthicum
Keyword(s):  
Hearing Loss ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Profound Hearing Loss

scholarly journals Histopathology of the Human Inner Ear in the p.L114P COCH Mutation (DFNA9)

2016 ◽  
Vol 21 (2) ◽  
pp. 88-97 ◽  
Author(s):  
Barbara J. Burgess ◽  
Jennifer T. O''Malley ◽  
Takefumi Kamakura ◽  
Kris Kristiansen ◽  
Nahid G. Robertson ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Clinical Findings ◽  
Spiral Ligament ◽  
Vestibular Disorder ◽  
Profound Hearing Loss ◽  
The Usa ◽  
Human Inner Ear

The histopathology of the inner ear in a patient with hearing loss caused by the p.L114P COCH mutation and its correlation with the clinical phenotype are presented. To date, 23 COCH mutations causative of DFNA9 autosomal dominant sensorineural hearing loss and vestibular disorder have been reported, and the histopathology of the human inner ear has been described in 4 of these. The p.L114P COCH mutation was first described in a Korean family. We have identified the same mutation in a family of non-Asian ancestry in the USA, and the temporal bone histopathology and clinical findings are presented herein. The histopathology found in the inner ear was similar to that shown in the 4 other COCH mutations and included degeneration of the spiral ligament with deposition of an eosinophilic acellular material, which was also found in the distal osseous spiral lamina, at the base of the spiral limbus, and in mesenchymal tissue at the base of the vestibular neuroepithelium. This is the first description of human otopathology of the COCH p.L114P mutation. In addition, it is the only case with otopathology characterization in an individual with any COCH mutation and residual hearing, thus allowing assessment of primary histopathological events in DFNA9, before progression to more profound hearing loss. A quantitative cytologic analysis of atrophy in this specimen and immunostaining using anti-neurofilament and anti-myelin protein zero antibodies confirmed that the principal histopathologic correlate of hearing loss was degeneration of the dendritic fibers of spiral ganglion cells in the osseous spiral lamina. The implications for cochlear implantation in this disorder are discussed.

scholarly journals Intrinsically Self-renewing Neuroprogenitors From the A/J Mouse Spiral Ganglion as Virtually Unlimited Source of Mature Auditory Neurons

2020 ◽  
Vol 14 ◽  
Author(s):  
Francis Rousset ◽  
Vivianne B. C. Kokje ◽  
Rebecca Sipione ◽  
Dominik Schmidbauer ◽  
German Nacher-Soler ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Progenitor Cells ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Specific Cell ◽  
Auditory Neurons ◽  
Self Renewal ◽  
Starting Point ◽  
Ganglion Neurons

Nearly 460 million individuals are affected by sensorineural hearing loss (SNHL), one of the most common human sensory disorders. In mammals, hearing loss is permanent due to the lack of efficient regenerative capacity of the sensory epithelia and spiral ganglion neurons (SGN). Sphere-forming progenitor cells can be isolated from the mammalian inner ear and give rise to inner ear specific cell types in vitro. However, the self-renewing capacities of auditory progenitor cells from the sensory and neuronal compartment are limited to few passages, even after adding powerful growth factor cocktails. Here, we provide phenotypical and functional characterization of a new pool of auditory progenitors as sustainable source for sphere-derived auditory neurons. The so-called phoenix auditory neuroprogenitors, isolated from the A/J mouse spiral ganglion, exhibit robust intrinsic self-renewal properties beyond 40 passages. At any passage or freezing–thawing cycle, phoenix spheres can be efficiently differentiated into mature spiral ganglion cells by withdrawing growth factors. The differentiated cells express both neuronal and glial cell phenotypic markers and exhibit similar functional properties as mouse spiral ganglion primary explants and human sphere-derived spiral ganglion cells. In contrast to other rodent models aiming at sustained production of auditory neurons, no genetic transformation of the progenitors is needed. Phoenix spheres therefore represent an interesting starting point to further investigate self-renewal in the mammalian inner ear, which is still far from any clinical application. In the meantime, phoenix spheres already offer an unlimited source of mammalian auditory neurons for high-throughput screens while substantially reducing the numbers of animals needed.

Survival of Spiral Ganglion Cells in Profound Sensorineural Hearing Loss: Implications for Cochlear Implantation

1989 ◽  
Vol 98 (6) ◽  
pp. 411-416 ◽  
Author(s):  
Joseph B. Nadol ◽  
Yi-Shyang Young ◽  
Robert J. Glynn
Keyword(s):  
Hearing Loss ◽  
Ganglion Cell ◽  
Cell Count ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Bivariate Analysis ◽  
Spiral Ganglion Cell ◽  
Cell Counts ◽  
Profound Deafness ◽  
Temporal Bones

Ninety-three temporal bones from 66 patients who were profoundly deaf during life were reconstructed by analysis of serial light microscopic sections. The correlations of total and segmental spiral ganglion cell counts with age, duration of hearing loss and profound deafness, and cause of hearing loss were evaluated. Bivariate analysis demonstrated that total spiral ganglion cell count tended to be lower in older than in younger deaf individuals and lower with longer duration of hearing loss and total deafness. However, multiple regression analysis demonstrated that the cause of hearing loss was the single most significant determinant of total spiral ganglion cell count. Patients with deafness due to aminoglycoside toxicity or sudden idiopathic deafness had the highest residual spiral ganglion cell count and patients with deafness due to presumptive postnatal viral labyrinthitis, bacterial labyrinthitis, and congenital or genetic causes had the lowest numbers of residual spiral ganglion cells.

scholarly journals Cochlear Implantation: An Overview

2018 ◽  
Vol 80 (02) ◽  
pp. 169-177 ◽  
Author(s):  
Nicholas Deep ◽  
Eric Dowling ◽  
Daniel Jethanamest ◽  
Matthew Carlson
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Auditory Brainstem ◽  
Electrical Signal ◽  
Sensorineural Hearing ◽  
Acoustic Energy ◽  
Lateral Skull Base ◽  
Single Sided Deafness

AbstractA cochlear implant (CI) is a surgically implanted device for the treatment of severe to profound sensorineural hearing loss in children and adults. It works by transducing acoustic energy into an electrical signal, which is used to stimulate surviving spiral ganglion cells of the auditory nerve. The past 2 decades have witnessed an exponential rise in the number of CI surgeries performed. Continual developments in programming strategies, device design, and minimally traumatic surgical technique have demonstrated the safety and efficacy of CI surgery. As a result, candidacy guidelines have expanded to include both pre and postlingually deaf children as young as 1 year of age, and those with greater degrees of residual hearing. A growing proportion of patients are undergoing CI for off-label or nontraditional indications including single-sided deafness, retrocochlear hearing loss, asymmetrical sensorineural hearing loss (SNHL) in adults and children with at least 1 ear that is better than performance cut-off for age, and children less than 12 months of age. Herein, we review CI design, clinical evaluation, indications, operative technique, and outcomes. We also discuss the expanding indications for CI surgery as it relates to lateral skull base pathology, comparing CI to auditory brainstem implants, and address the concerns with obtaining magnetic resonance imaging (MRI) in CI recipients.

scholarly journals PIN1 Protects Hair Cells and Auditory HEI-OC1 Cells against Senescence by Inhibiting the PI3K/Akt/mTOR Pathway

2021 ◽  
Vol 2021 ◽  
pp. 1-17
Author(s):  
Yanzhuo Zhang ◽  
Zhe Lv ◽  
Yudong Liu ◽  
Huan Cao ◽  
Jianwang Yang ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hair Cells ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Cell Senescence ◽  
Prolyl Isomerase ◽  
Age Related ◽  
Pin1 Protein

A growing amount of evidence has confirmed the crucial role of the prolyl isomerase PIN1 in aging and age-related diseases. However, the mechanism of PIN1 in age-related hearing loss (ARHL) remains unclear. Pathologically, ARHL is primarily due to the loss and dysfunction of hair cells (HCs) and spiral ganglion cells (SGCs) in the cochlea. Therefore, in this study, we aimed to investigate the role of PIN1 in protecting hair cells and auditory HEI-OC1 cells from senescence. Enzyme-linked immunosorbent assays, immunohistochemistry, and immunofluorescence were used to detect the PIN1 protein level in the serum of ARHL patients and C57BL/6 mice in different groups, and in the SGCs and HCs of young and aged C57BL/6 mice. In addition, a model of HEI-OC1 cell senescence induced by H2O2 was used. Adult C57BL/6 mice were treated with juglone, or juglone and NAC, for 4 weeks. Interestingly, we found that the PIN1 protein expression decreased in the serum of patients with ARHL, in senescent HEI-OC1 cells, and in the cochlea of aged mice. Moreover, under H2O2 and juglone treatment, a large amount of ROS was produced, and phosphorylation of p53 was induced. Importantly, PIN1 expression was significantly increased by treatment with the p53 inhibitor pifithrin-α. Overexpression of PIN1 reversed the increased level of p-p53 and rescued HEI-OC1 cells from senescence. Furthermore, PIN1 mediated cellular senescence by the PI3K/Akt/mTOR signaling pathway. In vivo data from C57BL/6 mice showed that treatment with juglone led to hearing loss. Taken together, these findings demonstrated that PIN1 may act as a vital modulator in hair cell and HEI-OC1 cell senescence.

Scala Vestibuli Partition with Deafness and Renal Disease

1973 ◽  
Vol 82 (6) ◽  
pp. 871-875 ◽  
Author(s):  
Ruth Gussen
Keyword(s):  
Hearing Loss ◽  
Renal Disease ◽  
Immunosuppressive Therapy ◽  
Ganglion Cells ◽  
Chronic Renal Disease ◽  
Spiral Ganglion ◽  
Spiral Ligament ◽  
Cochlear Hearing Loss ◽  
The Brain ◽  
Scala Vestibuli

Spiral ligament projections, partitioning the beginning of the scala vestibuli, bilaterally, were demonstrated in a patient with chronic renal disease, who had a bilateral midfrequency cochlear hearing loss. There were also spongy spiral ligament changes, strial atrophy and decreased spiral ganglion cells in the basal and middle cochlear turns. The patient was maintained on immunosuppressive therapy for four years after receiving a renal transplant. He died of a primary malignant lymphoma of the brain and cryptococcal meningitis, both probably related to immunosuppressive therapy.

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.

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.

R450 – Manganese Superoxide Dismutase in Spiral Ganglion Cells

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P195-P196
Author(s):  
Yu-Lan M. Ying ◽  
Carey D Balaban
Keyword(s):  
Superoxide Dismutase ◽  
Hearing Loss ◽  
Temporal Bone ◽  
Manganese Superoxide Dismutase ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Manganese Superoxide ◽  
Human Temporal Bone ◽  
Temporal Bones

Problem Manganese superoxide dismutase (Mn SOD2) is a key metabolic anti-oxidant enzyme of the superoxide dismutase family for detoxifying the free radical cascade inside the mitochondria of the cochlea via activation of downstream uncoupling proteins. Copper/zinc superoxide dismutase (Cu/Zn SOD1) is localized in the cytoplasm. This study examined whether the pattern of expression of these SODs in the cochlea is correlated with the differential cellular vulnerability found in basal versus apical turn of the cochlea. Methods Immunohistochemical methods were used to identify the distribution of Mn SOD2 and Cu/Zn SOD1 in paraffin embedded sections of paraformaldehyde fixed formic acid decalcified temporal bones from mice, rats, and macaques; and special archival celloidin-embedded human temporal bone sections. Results In mice, rats and macaques, both the proportion of Mn SOD2 immunopositive type 1 spiral ganglion cells and the intensity of immunoreactivity were elevated near the cochlear apex. Strongly stained Mn SOD2 type 1 spiral ganglion cells were also observed in archival human temporal bone sections. In contrast, the Cu/Zn SOD1 immunopositive type 1 spiral ganglion cells were distributed identically across cochlear turns in rats and macaques. Conclusion These findings suggest that spiral ganglion cellular responses to ROS exposure may vary along the cochlear spiral, with a lower response capacity in the basal turn. Significance Hair cells and spiral ganglion cells appear to be more vulnerable to ototoxins at the base of the cochlea than at the apex. Our data raises the general hypothesis that a lower Mn SOD2 anti-oxidative capacity at the cochlear base could contribute to the high frequency hearing loss seen in presbycusis and ototoxin-induced hearing loss. The conservative pattern of Mn SOD2 immunostaining across species further suggests that it may be a fundamental mechanism in ROS metabolism and signaling. Support PA Lions Hearing Research Foundation, American Otologic Society Research Fellowship.

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