scholarly journals Histopathology of the Human Inner Ear in the Cogan Syndrome with Cochlear Implantation

2017 ◽  
Vol 22 (2) ◽  
pp. 116-123 ◽  
Author(s):  
Takefumi Kamakura ◽  
Daniel J. Lee ◽  
Barbara S. Herrmann ◽  
Joseph B. Nadol Jr.

The Cogan syndrome is a rare disorder characterized by nonsyphilitic interstitial keratitis and audiovestibular symptoms. Profound sensorineural hearing loss has been reported in approximately half of the patients with the Cogan syndrome resulting in candidacy for cochlear implantation in some patients. The current study is the first histopathologic report on the temporal bones of a patient with the Cogan syndrome who during life underwent bilateral cochlear implantation. Preoperative MRI revealed tissue with high density in the basal turns of both cochleae and both vestibular systems consistent with fibrous tissue due to labyrinthitis. Histopathology demonstrated fibrous tissue and new bone formation within the cochlea and vestibular apparatus, worse on the right. Severe degeneration of the vestibular end organs and new bone formation in the labyrinth were seen more on the right than on the left. Although severe bilateral degeneration of the spiral ganglion neurons was seen, especially on the right, the postoperative word discrimination score was between 50 and 60% bilaterally. Impedance measures were generally higher in the right ear, possibly related to more fibrous tissue and new bone found in the scala tympani on the right side.

1977 ◽  
Vol 86 (4_suppl) ◽  
pp. 1-39 ◽  
Author(s):  
Gary K. Thomas

This animal experimental study (144 cats) was designed to allow observation of the healing processes at the oval window following stapedectomy during the first two postoperative weeks. The first 61 animals (Series I) had no oval window cover except for a blood clot which might form spontaneously. The second 64 animals (Series II) had Gelfoam® as a cover to the oval window following stapedectomy. A third series of 19 animals had temporalis fascia used as the oval window cover, and these ears were studied histologically for up to nine weeks. The unoperated ear acted as a control for the operated ear in all animals and after the animals were killed, both temporal bones were decalcified, imbedded in celloidin, serially sectioned at 20μ, and every tenth section stained and mounted for histologic study. It was observed that oval window closure was effected by endosteal, periosteal, and fibrous tissue proliferation from the traumatized tissues about the oval window fenestra, and that this process was facilitated by the presence of some type of scaffolding material such as a blood clot or Gelfoam®. Temporalis fascia placed in the oval window was observed to be rapidly incorporated into a much thicker oval window neomembrane and to seal the fenestra promptly. Partial removal of the footplate also facilitated more rapid closure of the smaller oval window fenestra, but fibrous tissue and new bone formation was noted to form about bony fragments still attached at the oval window level. Free bony fragments within the vestibule were not observed to stimulate new bone formation. Suppuration was observed most frequently in Series I (no oval window cover), with 67.8% of these animals having some degree of otitis media. In Series II (Gelfoam® cover) 20.6% had otitis media, and in Series III (temporalis fascia) 21% had middle ear infection. Suppuration markedly impaired but did not completely prevent the oval window reparative processes. Animals having some type of oval window cover were noted to have a lesser incidence of suppurative labyrinthine involvement when otitis media was present. Other labyrinthine complications were observed, including endolymphatic hydrops, Wittmaack's hypotonic atrophy, as well as RBC, fibrin, and albuminous precipitates in the labyrinth. Hypotonic atrophy was observed in a high incidence of animals having rupture of one or more endolymphatic structures, suggesting a mechanical mechanism as one possible explanation for this condition. Hypotonic atrophy may represent the end-stage (collapse) in the fluctuant endolymphatic ballooning of hydrops which is seen when saccular or Reissner's membrane ruptures have occurred.


2007 ◽  
Vol 116 (10) ◽  
pp. 731-738 ◽  
Author(s):  
Peter M. M. C. Li ◽  
Mehmet A. Somdas ◽  
Donald K. Eddington ◽  
Joseph B. Nadol

Objectives: In this study we aimed to evaluate new bone and new fibrous tissue formation in the inner ear following cochlear implantation. Methods: Twelve temporal bones from patients who underwent cochlear implantation during life were prepared for histologic study. The specimens were reconstructed by both 2-dimensional and 3-dimensional methods. These reconstructions were used to calculate the total volume and distribution of new bone and new fibrous tissue in the cochlea, the number of spiral ganglion cells, and other histopathologic parameters. Clinical data, including the last-recorded word recognition scores, were obtained from the patients' medical records. Results: New bone and new fibrous tissue were found in all 12 specimens, particularly at the site of cochleostomy. There was a significant correlation between overall damage to the lateral cochlear wall and the total volume of intracochlear new tissue (Spearman rho = .853; p = .0004). The total volume of new tissue did not correlate with word recognition scores or spiral ganglion cell counts. Conclusions: These preliminary results suggest that the degree of damage to the lateral cochlear wall may play an important role in influencing the amount of new tissue formation following cochlear implantation. Intracochlear new tissue does not appear to be an important determinant of performance as measured by word recognition scores or the total number of remaining spiral ganglion cells.


1991 ◽  
Vol 252 ◽  
Author(s):  
N. W. Knuckey ◽  
H. Saskin ◽  
R. Yanagasawa ◽  
M. Flanagan

Spontaneous new bone formation does not occur in the adult calvarium. In humans, cranial defects may require repair for brain protection, headaches or cosmetic. Over the years the search for the ideal cranioplasty material to repair such cranial defects has been extensive. We report an evaluation of alpha plaster of paris as an osteoconductive material in a cat craniectomy defect.Adult cats of either sex were anesthetized with ketamine/halothane and a 15mm biparietal craniectomy defect created. The right side was filled with alpha plaster of paris and the left served as a control. There were 4 animals in each time cohort and animals were sacrificed at 6 weeks, 6 or 12 months. The cranium was removed, fixed, stained, histologically studied and analysised for the percentage of trabecular bone formation. At all durations the control defects were bridged by proliferative fibrous tissue membrane and less than 1% trabecular bone. At six weeks the plaster of paris filled defects were either totally or nearly totally replaced by new bone formation. The plaster was completely resorbed. At 6 wks there was 28% trabecular bone which increased to 93% at 52 wks. Our experiments demonstrate the oeteoconductive potential of alpha plaster of paris in the adult calvarium. This material needs to be evaluated in humans to ascertain its potential as a biocompatible cranioplasty material.


2021 ◽  
pp. 019459982110089
Author(s):  
Rafael da Costa Monsanto ◽  
Renata Malimpensa Knoll ◽  
Norma de Oliveira Penido ◽  
Grace Song ◽  
Felipe Santos ◽  
...  

Objective To perform an otopathologic analysis of temporal bones (TBs) with CHARGE syndrome. Study Design Otopathologic study of human TB specimens. Setting Otopathology laboratories. Methods From the otopathology laboratories at the University of Minnesota and Massachusetts Eye and Ear Infirmary, we selected TBs from donors with CHARGE syndrome. These TBs were serially sectioned at a thickness of 20 µm, and every 10th section was stained with hematoxylin and eosin. We performed otopathologic analyses of the external ear, middle ear (middle ear cleft, mucosal lining, ossicles, mastoid, and facial nerve), and inner ear (cochlea, vestibule, internal auditory canal, and cochlear and vestibular nerves). The gathered data were statistically analyzed. Results Our study included 12 TBs from 6 donors. We found a high prevalence of abnormalities affecting the ears. The most frequent findings were stapes malformation (100%), aberrant course of the facial nerve (100%) with narrow facial recess (50%), sclerotic and hypodeveloped mastoids (50%), cochlear (100%) and vestibular (83.3%) hypoplasia with aplasia of the semicircular canals, hypoplasia and aplasia of the cochlear (66.6%) and vestibular (91.6%) nerves, and narrowing of the bony canal of the cochlear nerve (66.6%). The number of spiral ganglion and Scarpa’s ganglion neurons were decreased in all specimens (versus normative data). Conclusions In our study, CHARGE syndrome was associated with multiple TB abnormalities that may severely affect audiovestibular function and rehabilitation.


2005 ◽  
Vol 114 (7) ◽  
pp. 543-546 ◽  
Author(s):  
Francisco J. Cervera-Paz ◽  
Frederick H. Linthicum

Objectives: Cochlear implantation is a clinically satisfactory procedure, but it is associated with a variable degree of histologic intracochlear trauma. We report a new histologic finding in a cochlear implant specimen from the House Ear Institute collection. Methods: An analysis of 34 temporal bones with single-channel (n = 23) or multichannel (n = 11) cochlear implants was performed. All temporal bones had been fixed for a month in 10% buffered formalin, progressively decalcified in ethylenediaminetetraacetic acid, and embedded in celloidin. After electrode removal, the bones were cut into 20-μm sections and stained. Results: In 1 specimen, the implanted electrode had caused erosion of the bone through the endosteum into the marrow spaces, at the superior-anterior portion of the basal turn. This area showed an intense lymphocytic infiltration surrounded by some new bone formation. Conclusions: Trauma may provoke an inflammatory reaction due to the presence of the foreign body after violation of the endosteum.


2002 ◽  
Vol 13 (2) ◽  
pp. 92-96 ◽  
Author(s):  
Luciana Ibara Yugoshi ◽  
Miguel Angel Sala ◽  
Luiz Guilherme Brentegani ◽  
Teresa Lúcia Lamano Carvalho

The purpose of the present study was to investigate if diclofenac administration interferes with the time course of alveolar wound healing in rats. Forty-two Wistar rats were used, 21 rats received 10 mg/kg/day of diclofenac one day before and 4 days after extraction of the right maxillary incisors and 21 rats received saline. The animals were sacrificed 7, 14 and 21 days after tooth extraction. Progressive new bone formation and a decrease in the volume fraction of blood clot and connective tissue from 1 to 3 weeks after tooth extraction was quantified using the histometric point-counting method. Diclofenac treatment caused a significant delay in new bone formation in association with an impairment of blood clot remission/organization.


2020 ◽  
pp. 019459982093474
Author(s):  
Danielle R. Trakimas ◽  
Renata M. Knoll ◽  
Melissa Castillo-Bustamante ◽  
Elliott D. Kozin ◽  
Aaron K. Remenschneider

Objective Labyrinthitis ossificans (LO) may occur following meningitis and, in cases where cochlear implantation is indicated, complicate electrode insertion. LO is critical to identify for successful cochlear implantation, and histopathology is more sensitive than imaging for identification of LO. Herein we utilize otopathologic techniques to study the timing and location of intracochlear tissue formation following meningitic labyrinthitis (ML). Study Design Retrospective review. Setting Academic institution. Methods Temporal bone specimens with a history of bacterial ML were histologically evaluated. The location and extent of intracochlear tissue formation within the scala tympani (ST) and scala vestibuli (SV) were graded, and spiral ganglion neurons were counted. Results Fifty-one temporal bones were identified: 32 with no intracochlear tissue formation, 9 with fibrosis alone, and 10 with LO. Fibrosis was identified as early as 1.5 weeks after ML, while ossification was found only in specimens that survived multiple years after ML. All LO cases showed ossification of the ST at the round window membrane (RWM) with continuous extension throughout the basal turn. Extent of SV ossification correlated with that in the ST but showed frequent isolated distal involvement of the cochlea. Spiral ganglion neuron counts were lower than those in age-matched controls. Conclusion In this human temporal bone study, we found that postmeningitic LO results in ossification at the RWM with continuous extension into the ST of the basal turn and variable involvement of the SV. Identification of a patent basal turn beyond RWM ossification of the ST should permit full electrode insertion. Level of Evidence Retrospective review.


2011 ◽  
Vol 37 (5) ◽  
pp. 511-518 ◽  
Author(s):  
Flaviana Soares Rocha ◽  
Lara Maria Alencar Ramos ◽  
Jonas Dantas Batista ◽  
Darceny Zanetta-Barbosa ◽  
Eloísa Amália Vieira Ferro ◽  
...  

Autogenous bone tissue has regeneration potential; however, this capacity may not be sufficient in larger bone defects. The aim of this study is to histologically evaluate anorganic bovine bone grafts (GenOx Inorg) with or without platelet-rich plasma (PRP). Two bone lesions were created in calvaria of 12 rabbits. The 24 surgical lesions were separated into 3 groups: coagulous, anorganic, and anorganic with PRP. At the 4-week time point, the animals were euthanized and the grafted area removed, fixed in formalin 10% with phosphate buffered saline, 0.1 M, and embedded in paraffin. The histologic parameters analyzed were new bone filling the defect area, presence of giant cells and particles of the graft, and new bone formation associated with the particles. In the coagulous group, defects were filled with fibrous tissue that attached the periosteum and little bone neoformation in the periphery. In anorganic groups with or without PRP, little new bone formation in the periphery of the defect was observed; however, in the center of some defects there was new bone. Moderate presence of giant cells and little new bone formation was associated with the innumerous graft particles. Histologic results revealed no statistically significant differences among the defects new bone fill between the studied groups (P  =  .64). There was no significant difference in the number of giant cells (P  =  .60), graft particles (P  =  .46), and new bone formation around graft particles (P  =  .26), whether PRP was added or not. Anorganic bone, isolated or mixed with PRP, was biocompatible and osteoconductive, while maintaining bone volume.


2001 ◽  
Vol 110 (9) ◽  
pp. 883-891 ◽  
Author(s):  
Joseph B. Nadol ◽  
Barbara J. Burgess ◽  
Bruce J. Gantz ◽  
Newton J. Coker ◽  
Darlene R. Ketten ◽  
...  

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.


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