Quantitative Study of Scarpa's Ganglion and Vestibular Sense Organs in Endolymphatic Hydrops

1981 ◽  
Vol 90 (2) ◽  
pp. 121-125 ◽  
Author(s):  
Ernst Richter
Keyword(s):  
Hearing Loss ◽  
Ganglion Cell ◽  
Endolymphatic Hydrops ◽  
Sense Organs ◽  
Episodic Vertigo ◽  
Cell Counts ◽  
Scarpa's Ganglion ◽  
Caloric Response ◽  
Temporal Bones ◽  
Fluctuating Hearing Loss

The density of vestibular hair cells and the number of neurons in Scarpa's ganglion were estimated for 11 temporal bones with endolymphatic hydrops. The ten subjects from which these bones were taken all exhibited decreased caloric response (when tested), fluctuating hearing loss, and episodic vertigo. The degeneration of vestibular sense organs was found within the “normal” range for all except one case, which showed total degeneration of the posterior crista ampullaris. Ganglion cell counts in all cases were low. In three of the ten subjects, counts fell below the lowest values seen in a sample of “normal” ears. These three subjects exhibited fluctuating hearing loss and episodic vertigo for more than six years prior to death. In cases of unilateral endolymphatic hydrops there was no significant difference between counts in the affected and unaffected ear. Thus, the apparent ganglion cell degeneration may be due to ear disease other than endolymphatic hydrops.

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.

Delayed Endolymphatic Hydrops

1978 ◽  
Vol 87 (6) ◽  
pp. 743-748 ◽  
Author(s):  
Harold F. Schuknecht
Keyword(s):  
Hearing Loss ◽  
Endolymphatic Hydrops ◽  
Meniere’S Disease ◽  
Ménière’S Disease ◽  
Disease Entity ◽  
Profound Hearing Loss ◽  
Episodic Vertigo ◽  
Delayed Endolymphatic Hydrops ◽  
Ménière's Disease ◽  
Fluctuating Hearing Loss

Delayed endolymphatic hydrops is a disease entity that can be differentiated from Meniere's disease. Typically it occurs in patients who have sustained a profound hearing loss in one ear, usually from infection or trauma, and then after a prolonged period of time develop either episodic vertigo from the same ear (ipsilateral delayed endolymphatic hydrops) or fluctuating hearing loss, also sometimes with episodic vertigo, in the opposite ear (contralateral delayed endolymphatic hydrops). The ipsilateral form of the disease may be treated by labyrinthectomy but no satisfactory therapy is available for the contralateral form of the disease.

Temporal Bone Studies of the Human Peripheral Vestibular System

2000 ◽  
Vol 109 (5_suppl) ◽  
pp. 14-19 ◽  
Author(s):  
Luis Velázquez-Villaseñor ◽  
Kojiro Tsuji ◽  
Conrad Wall ◽  
Saumil N. Merchant ◽  
Robert J. Glynn ◽  
...  
Keyword(s):  
Ganglion Cell ◽  
Inner Ear ◽  
Average Rate ◽  
Cell Counts ◽  
Age Related ◽  
Inner Ear Disease ◽  
Scarpa's Ganglion ◽  
Normal Human ◽  
Interaural Difference ◽  
Temporal Bones

Scarpa's ganglion cell counts were performed in 106 serially sectioned, normal human temporal bones from 75 individuals. Of these 106 bones, 15 were from neonates less than 30 days old, 14 were from infants between the ages of 1 and 12 months, and the remainder were distributed throughout each decade of life, with sample sizes ranging from 4 to 10 per decade. All temporal bones had to meet 2 criteria: no symptoms or signs of inner ear disease except for presbycusis in the medical case history and no abnormality in the inner ear on light microscopy. The total ganglion cell counts declined significantly with age at an average rate of 57 cells per year. The age-related decline was significantly greater in the superior division than in the inferior division. There was also a significant sex effect, independent of age: for any age, the count in men averaged 1,526 cells higher than in women. There was no significant interaural difference. Mathematical models were developed to compute the mean and 95% prediction intervals for Scarpa's ganglion cell counts in terms of age and sex parameters. The counts and models will serve as a normative database against which to compare counts made in temporal bones from subjects with known vestibular disorders.

Delayed Endolymphatic Hydrops: A Case Study

2008 ◽  
Vol 19 (03) ◽  
pp. 204-209 ◽  
Author(s):  
Rachel Lazaro ◽  
Larry Lundy ◽  
David Zapala
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Endolymphatic Hydrops ◽  
Sensorineural Hearing ◽  
Ménière’S Disease ◽  
Episodic Vertigo ◽  
Delayed Endolymphatic Hydrops ◽  
Patient’S History ◽  
Estudio De Caso

Delayed endolymphatic hydrops (DEH) is an unusual variation of Ménière's disease characterized by episodic vertigo that develops some time after the onset of a profound, typically unilateral sensorineural hearing loss. This case study describes a 48-year-old male who presented with complaints of episodic vertigo and disequilibrium 15 years following the onset of unilateral sensorineural hearing loss. The patient's history, audiologic findings, and vestibular evaluation led to the diagnosis of DEH. The case highlights the diagnostic and treatment challenges associated with this condition and focuses attention on principles that guide the audiologist in collecting evidence that aids in solving these challenges. El hidrops endolinfático retardado (DEH) es una variante inusual de la Enfermedad de Ménière, caracterizada por vértigo episódico que se desarrolla en el tiempo luego del inicio de una hipoacusia sensorineural unilateral típica. Este estudio de caso describe una varón de 48 años que presentó quejas de vértigo episódico y desequilibrio, 15 años después del inicio de una hipoacusia sensorineural unilateral. La historia del paciente, los hallazgos audiológicos y la evaluación vestibular llevaron al diagnóstico de DEH. El caso destaca los retos diagnósticos y terapéuticos asociados con esta condición y concentra su atención en los principios que guían al audiólogo en la recolección de evidencia que ayude a resolver estos retos.

Histopathology of Cochlear Implants in Humans

2001 ◽  
Vol 110 (9) ◽  
pp. 883-891 ◽  
Author(s):  
Joseph B. Nadol ◽  
Barbara J. Burgess ◽  
Bruce J. Gantz ◽  
Newton J. Coker ◽  
Darlene R. Ketten ◽  
...  
Keyword(s):  
Ganglion Cell ◽  
Cochlear Implantation ◽  
Single Channel ◽  
Spiral Ganglion ◽  
Electrode Array ◽  
Speech Comprehension ◽  
Spiral Ganglion Cell ◽  
Cell Counts ◽  
Significant Difference ◽  
Temporal Bones

The insertion of an intrascalar electrode array during cochlear implantation causes immediate damage to the inner ear and may result in delayed onset of additional damage that may interfere with neuronal stimulation. To date, there have been reports on fewer than 50 temporal bone specimens from patients who had undergone implantation during life. The majority of these were single-channel implants, whereas the majority of implants inserted today are multichannel systems. This report presents the histopathologic findings in temporal bones from 8 individuals who in life had undergone multichannel cochlear implantation, with particular attention to the type and location of trauma and to long-term changes within the cochlea. The effect of these changes on spiral ganglion cell counts and the correlation between speech comprehension and spiral ganglion cell counts were calculated. In 4 of the 8 cases, the opposite, unimplanted ear was available for comparison. In 3 of the 4 cases, there was no significant difference between the spiral ganglion cell counts on the implanted and unimplanted sides. In addition, in this series of 8 cases, there was an apparent negative correlation between residual spiral ganglion cell count and hearing performance during life as measured by single-syllable word recognition. This finding suggests that abnormalities in the central auditory pathways are at least as important as spiral ganglion cell loss in limiting the performance of implant users.

S240 – Human Cochlear Implant Histopathology

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P155-P155
Author(s):  
Helen Xu ◽  
Natasha Pollak ◽  
Sebahattin Cureoglu ◽  
Michael M Paparella
Keyword(s):  
Cochlear Implant ◽  
Ganglion Cell ◽  
Cell Population ◽  
Ganglion Cells ◽  
Clinical Performance ◽  
Spiral Ganglion ◽  
Spiral Ganglion Cell ◽  
Cell Counts ◽  
Temporal Bones ◽  
Normal Spiral

Objectives 1) To exam the histopathology of multichannel cochlear implant temporal bones. 2) To evaluate the relationship of residual spiral ganglion cell counts to clinical hearing performance. Methods 8 temporal bones from 4 cochlear implant patients were examined histologically. Paired comparisons were made between implanted and nonimplanted temporal bones. Clinical performance data was obtained from patient charts. Results There were varying amounts of inflammation (fibrosis and ossification) in the basal turn of the cochlear in all implanted temporal bones. Trauma to the facial nerve at facial recess site was noticed in 1 case. Compared with nonimplanted ears, 2 implanted bones with less than 10-year duration of implantation had no significant changes of spiral ganglion cell population. One case with prolong implant duration (15 years) showed about 36% decrease of spiral ganglion cells at the implanted site. The case with best speech recognition (89% with CID sentence) had the highest residual spiral ganglion cells (30% of normal spiral ganglion cell population). 2 cases with poor clinical performance (< 10% with CID sentence) had the residual spiral ganglion cells at 11% and 22%. The case with moderate clinical performance (30% with CID sentence) had 14% of normal spiral ganglion cell population. Surviving dendrites varied from 5% to 30% among 4 cases with no relationship to clinical performance. Conclusions Our findings suggest prolonged implantation may affect spiral ganglion cell population. There is no reverse relationship between residual spiral ganglion cells in implanted temporal bones to clinical speech performance observed from our limited cases.

Endolymphatic Hydrops: An Overview and Classification

1983 ◽  
Vol 92 (5_suppl) ◽  
pp. 1-20 ◽  
Author(s):  
Harold F. Schuknecht ◽  
Aina J. Gulya
Keyword(s):  
Clinical Case ◽  
Endolymphatic Hydrops ◽  
Traumatic Event ◽  
Pathological Condition ◽  
Case Histories ◽  
Episodic Vertigo ◽  
Common Manifestation ◽  
Symptomatic Form ◽  
Fluctuating Hearing Loss ◽  
Over Time

Endolymphatic hydrops (EH) is a pathological condition which is the final common manifestation of a variety of otologic insults. In this paper we develop a classification which, on the basis of clinical and pathological data, distinguishes symptomatic and asymptomatic forms. Clinical case histories and temporal bone studies are presented to illustrate and substantiate this classification. The symptomatic form becomes evident by the hallmark symptoms of episodic vertigo and fluctuating hearing loss. The asymptomatic form is silent. Inter-conversion from one form to another may occur over time. Each of the forms can be subdivided, according to etiology, into embryopathic, acquired, and idiopathic types. The embryopathic type comprises those cases in which some noxious influence interferes with prenatal labyrinthine development. The acquired type includes those cases in which a documented insult, be it inflammatory or traumatic, is suffered by a previously normal labyrinth. The inflammation may be viral, bacterial, or spirochetal in nature, while the traumatic event may be either accidentally or surgically sustained. The idiopathic type includes cases in which the instigating event precipitating the EH is unknown. Menière's disease represents merely one example of the EH group of disorders, namely symptomatic idiopathic EH.

Survival of Scarpa's Ganglion in the Profoundly Deaf Human

1993 ◽  
Vol 102 (6) ◽  
pp. 425-428 ◽  
Author(s):  
Charlotte M. Chiong ◽  
Robert J. Glynn ◽  
Wen-Zhuang Xu ◽  
Joseph B. Nadol
Keyword(s):  
Ganglion Cell ◽  
Ganglion Cells ◽  
Linear Regression Analysis ◽  
Spiral Ganglion ◽  
Normal Hearing ◽  
Auditory Brain Stem Response ◽  
Spiral Ganglion Cell ◽  
Cell Counts ◽  
Profound Deafness ◽  
Scarpa's Ganglion

The electrically evoked auditory brain stem response in some cochlear implant patients may be confounded by evoked potentials generated by vestibular neurons. The magnitude of this contribution to the response from the vestibular system is unknown, in part because the survival of cells within Scarpa's ganglion in profoundly deaf humans is unknown. Therefore, we undertook a quantitative study of Scarpa's ganglion in 48 deaf subjects who in life would have been candidates for cochlear implantation and in 5 subjects with normal hearing. The numbers of residual cells in both Scarpa's ganglion and the spiral ganglion in deaf subjects were significantly less than in individuals with normal hearing. Bivariate analysis demonstrated a highly significant positive correlation between cell counts of Scarpa's ganglion and the spiral ganglion. The durations of hearing loss and of profound deafness were negatively correlated with Scarpa's ganglion cell counts. However, in contrast to spiral ganglion cell survival, the cause of profound deafness did not predict the number of Scarpa's ganglion cells. Multiple linear regression analysis using a variety of clinical parameters demonstrated that the best predictor of the number of Scarpa's ganglion cells in profoundly deaf humans was the number of remaining spiral ganglion cells.

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