Mucopolysaccharidosis I-H (Hurler's Syndrome) and Human Temporal Bone Histopathology

1984 ◽  
Vol 93 (1) ◽  
pp. 65-69 ◽  
Author(s):  
P. A. Schachern ◽  
D. A. Shea ◽  
M. M. Paparella
Keyword(s):  
Otitis Media ◽  
Stria Vascularis ◽  
Round Window ◽  
Enzyme Analysis ◽  
Mucopolysaccharidosis I ◽  
Human Temporal Bone ◽  
Hurler’S Syndrome ◽  
Round Window Niche ◽  
Temporal Bones ◽  
Histopathologic Findings

Six temporal bones from three patients with mucopolysaccharidosis I-H are described. All three patients were diagnosed as having mucopolysaccharidosis I-H by enzyme analysis, and all fit the phenotypic criteria of this disease. Family histories of the three cases described were negative for mycopolysaccharide-storage diseases. All three of the patients suffered chronic recurrent otitis media from infancy through death. Common histopathologic findings include otitis media, residual mesenchyme in the round window niche, partial occlusion of the middle ear cavity, and basophilic concretions within the stria vascularis. The common severe histopathologic changes observed in this study and similar findings in the temporal bones described in other studies lead the authors to believe that ear involvement in this disease may be more common than was previously suspected.

Locating the scala media in the fixed human temporal bone for therapeutic access: a preliminary study

2006 ◽  
Vol 120 (11) ◽  
pp. 914-915 ◽  
Author(s):  
H Pau ◽  
P Fagan ◽  
S Oleskevich
Keyword(s):  
Temporal Bone ◽  
Round Window ◽  
Oval Window ◽  
Horizontal Line ◽  
Point Of Entry ◽  
Anterior Edge ◽  
Human Temporal Bone ◽  
Round Window Niche ◽  
Temporal Bones ◽  
A Site

Objective: To investigate the location of the scala media in relation to the round window niche in human temporal bones.Design: Ten human temporal bones were investigated by radical mastoidectomy and promontory drill-out.Setting: Temporal bone laboratory.Outcome measures: The distance from the scala media to the anterior edge of the round window niche, measured by Fisch's stapedectomy measuring cylinders.Results: The scala media was identified at the transection point of a vertical line 1.6 to 2.2 mm (mean=1.8 mm; standard deviation=0.2) anterior to the anterior edge of the round window niche and a horizontal line 0.2 mm inferior to the lower border of the oval window.Conclusion: This report demonstrates the point of entry into the scala media via the promontory in fixed temporal bone models, which may provide a site of entry for stem cells and gene therapy insertion.

Microfissure between the round Window Niche and Posterior Canal Ampulla

1977 ◽  
Vol 86 (1) ◽  
pp. 49-57 ◽  
Author(s):  
Yasumasa Okano ◽  
Eugene N. Myers ◽  
David R. Dickson
Keyword(s):  
Round Window ◽  
Pathological Finding ◽  
Anatomical Structure ◽  
Fetal Life ◽  
Human Fetuses ◽  
Posterior Canal ◽  
Mesenchymal Tissue ◽  
One Year ◽  
Round Window Niche ◽  
Temporal Bones

The microfissure between the round window niche and the posterior canal ampulla was examined in fetal, child, and adult human temporal bones. The communication which was filled with mesenchymal tissue and blood vessels extending from the middle ear, was observed between the round window niche and the posterior canal ampulla in 10 to 15-week human fetuses. The mesenchymal tissue in the communication was replaced by cartilage with advancing age of the fetus. No communication was observed at birth. From newborn to 12 months of age, no microfissure was observed. After one year of age, the microflssure was first observed and the frequency of the observation increased with increasing age of the patient. After six years of age, the microfissure was observed bilaterally in 100% of cases studied. The microflssure has its origin from the communication between the round window niche and the posterior canal ampulla in fetal life, and is a normal developmental and anatomical structure, and not a pathological finding.

Middle-ear endoscopy and trans-tympanic drug delivery using an interventional sialendoscope: feasibility study in human cadaveric temporal bones

2010 ◽  
Vol 124 (12) ◽  
pp. 1263-1267 ◽  
Author(s):  
G Peters ◽  
J Lin ◽  
M A Arriaga ◽  
D W Nuss ◽  
B Schaitkin ◽  
...  
Keyword(s):  
Drug Delivery ◽  
Eustachian Tube ◽  
Middle Ear ◽  
Guide Wire ◽  
Round Window ◽  
Human Cadavers ◽  
Incudostapedial Joint ◽  
Round Window Niche ◽  
Temporal Bones ◽  
Inferior Quadrant

AbstractObjective:This study aimed to assess the feasibility of using a 1.3 mm, semi-rigid, interventional salivary endoscope for middle-ear endoscopy, and as a trans-tympanic route for delivery of medication, in human cadaveric temporal bones.Study design:Human cadaveric study.Methods:Five temporal bones harvested from human cadavers were examined. A 1.3 mm, interventional sialendoscope was used to make endoscopy-assisted myringotomy incisions in the postero-inferior quadrant (n = 5) and the antero-inferior quadrant (n = 3).Results:Middle-ear examination was successful in all specimens (n = 5). Access to the round window niche and adequate visualisation of the round window were achieved in all five temporal bones (100 per cent). A guide wire could be navigated to the round window niche without difficulty. Other structures identified in all specimens included the incudostapedial joint, stapedius tendon, pyramidal eminence and facial nerve via an extended myringotomy incision. The anterior middle-ear space was also successfully examined through an endoscope-guided anterior myringotomy. The opening to the eustachian tube was visualised and cannulated with a guide wire in all preparations in which this was attempted (n = 3).Conclusions:The 1.3 mm, interventional sialendoscope allowed adequate visualisation of the eustachian tube, middle-ear space and round window niche, with interventional capabilities, in a cadaveric model. Our result validates the feasibility of its use for trans-tympanic drug delivery.

S239 – Labyrinthine Changes in Otitis Media

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P155-P155
Author(s):  
Shruti Siddharth Joglekar ◽  
Armin Farajzadeh Deroee ◽  
Norimasa Morita ◽  
Sebahattin Cureoglu ◽  
Schachern Patricia ◽  
...  
Keyword(s):  
Inner Ear ◽  
Otitis Media ◽  
Stria Vascularis ◽  
Chronic Otitis Media ◽  
Inflammatory Cells ◽  
Spiral Ligament ◽  
Histopathologic Study ◽  
Significant Difference ◽  
Temporal Bones ◽  
Inflammatory Changes

Objectives Otitis media causes labyrinthine changes and subsequent sensorineural hearing loss. The aim of this histopathologic study was to evaluate the extension of inflammation to the inner ear and its effects. Methods Out of 614 temporal bones with otitis media, 47 (30 cases) with chronic and 35 (21 cases) with purulent were selected for histopathologic study. Subjects with a history of acoustic trauma, head trauma, ototoxic drugs and other otologic and systemic diseases affecting the inner ear were excluded. The pattern of labyrinthine inflammation was classified as localized purulent, localized serous, generalized seropurulent and generalized serous. Inner ear findings were compared to age-matched controls. Results 19% of temporal bones with chronic and 9% of temporal bones with purulent otitis media showed labyrinthine inflammatory changes. In chronic otitis media, inflammatory changes were: 56% localized purulent; 22% localized serous; 11% generalized seropurulent; and 11% generalized serous. Inflammatory changes in temporal bones with purulent otitis media included: 67% localized purulent; and 33% generalized seropurulent. Pathological findings included: serofibrinous precipitates and inflammatory cells in the scala tympani of basal turn and cochlear aqueduct; significant decrease in area of stria vascularis (p = 0.033); and loss of hair cells in the organs of Corti. No significant difference was found in area of spiral ligament area or number of fibrocytes in diseased and control bones. Conclusions Middle ear/inner ear interaction in otitis media can result in labyrinthine inflammation and cochlear damage. Early diagnosis and treatment of otitis media is important in preventing inner ear damage.

The Crista Fenestra and Its Impact on the Surgical Approach to the Scala Tympani during Cochlear Implantation

2017 ◽  
Vol 22 (1) ◽  
pp. 50-55 ◽  
Author(s):  
Roberto D. Angeli ◽  
Joel Lavinsky ◽  
Enio T. Setogutti ◽  
Luiz Lavinsky
Keyword(s):  
High Resolution ◽  
Cochlear Implantation ◽  
Round Window ◽  
Ct Scanning ◽  
Round Window Membrane ◽  
Scala Tympani ◽  
High Resolution Computed Tomography ◽  
High Resolution Ct ◽  
Round Window Niche ◽  
Temporal Bones

Objective: The aim of this work was to describe the dimensions of the crista fenestra and determine its presence by means of high-resolution computed tomography (CT) for the purpose of cochlear implantation via the round window approach. Methods: A series of 10 adult human temporal bones underwent high-resolution CT scanning and were further dissected for microscopic study of the round window niche. Results: In all of the specimens, the round window membrane was fully visualized after the complete removal of bony overhangs. The crista fenestra was identified as a sharp bony crest located in the anterior and inferior borders of the niche; its area ranged from 0.28 to 0.80 mm2 (mean 0.51 ± 0.18). The proportion of the area occupied by the crista fenestra in the whole circumference of the round window ranged from 23 to 50% (mean 36%). We found a moderate positive correlation between the area of the niche and the dimensions of the crista fenestra (Spearman rho: 0.491). In every case, high-resolution CT scanning was unable to determine the presence of the crista fenestra. Conclusion: The crista fenestra occupies a variable but expressive area within the bony round window niche. Narrower round window niches tended to house smaller crests. The presence of the crista fenestra is an important obstacle to adequate access to the scala tympani. Nevertheless, a high-resolution CT scan provides no additional preoperative information with regard to its presence for the purpose of surgical access to the scala tympani via the round window niche.

Microfissure in the Oval Window Area

1981 ◽  
Vol 90 (2) ◽  
pp. 174-180 ◽  
Author(s):  
Takehiko Harada ◽  
Isamu Sando ◽  
Eugene N. Myers
Keyword(s):  
Temporal Bone ◽  
Round Window ◽  
Vertical Plane ◽  
Careful Analysis ◽  
Oval Window ◽  
Annular Ligament ◽  
Round Window Niche ◽  
Window Area ◽  
Most Probable Explanation ◽  
Temporal Bones

Examination of 331 human temporal bones revealed the presence of a microfissure in the oval window area in 25%. The youngest temporal bone in which the microfissure was observed was from a child eight years old and the oldest temporal bone was from an individual 102 years old. It appears that the microfissure in this area occurs extremely rarely before 10 years of age, that it begins to be observed after the age of 10, and that the incidence of this fissure increases sharply at about age 40. In 61% of the bones in which the microfissure was observed, it was present both superior and inferior to the oval window in almost the same vertical plane. In none of the bones, however, did it extend beyond the annular ligament to the footplate. The histological appearance of the microfissure in this area was quite similar to that of the microfissure between the posterior canal ampulla and round window niche, although the latter appeared to be wider in general. The histogenesis of the microfissure is still unknown, but upon careful analysis of the results of this study and literature review, the authors consider nontraumatic spontaneous fracture by mechanical stress to be the most probable explanation for its occurrence. Possible clinical significance of the microfissure is also discussed.

Chronic Intracochlear Electrode Implantation: Cochlear Pathology and Acoustic Nerve Survival

1974 ◽  
Vol 83 (2) ◽  
pp. 202-215 ◽  
Author(s):  
Robert A. Schindler ◽  
Michael M. Merzenich
Keyword(s):  
Hair Cells ◽  
Bone Growth ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Round Window ◽  
Spiral Ganglion ◽  
Nerve Fibers ◽  
Spiral Ligament ◽  
Scala Tympani ◽  
Temporal Bones

The temporal bones of ten cats implanted with intracochlear electrodes for three to 117 weeks were stained with hematoxylin and eosin and examined with light microscopy. The electrodes were embedded in Silastic® which was molded to fill the most basal 9 mm of the scala tympani. They were inserted directly into the scala through the round window. Among our observations were the following: 1) All or nearly all hair cells were lost in the basal coil during the first several weeks after implantation. Some, but not all, supporting cells were also lost. There was extensive hair cell loss in the middle and apical turns, although some hair cells were seen there in all examined cats. 2) There was evidence of degeneration of spiral ganglion cells in the basal cochlea in several animals, but most primary auditory neurons including (with two exceptions) most of those in the region directly over the electrode, survived implantation in every cat. The radial nerve fibers of the spiral ganglion cells also survived long-term implantation. The functional viability of remaining spiral ganglion cells was confirmed in acute neurophysiological experiments conducted just before the animals were sacrificed. 3) More severe degeneration was seen in two cats in which the electrode perforated the basilar partition. In these animals, there was loss of many spiral ganglion cells, and evidence of new bone growth in the region of the perforation. 4) The appearance of the stria vascularis and spiral ligament in some implanted animals paralleled their descriptions following occlusion of the cochlear vein. 5) Connective tissue formed around the electrode surfaces, apparently displacing perilymph and sealing the electrode into the scala tympani. There was no evidence of perilymph fistula in any animal. 6) There was little evidence of progressive degeneration of the organ of Corti or spiral ganglion from three to 34 weeks after implantation. Some of the implications and limitations of these findings are discussed.

Mesenchyme Remaining in Human Temporal Bones

1986 ◽  
Vol 95 (3_part_1) ◽  
pp. 349-357 ◽  
Author(s):  
Tetsuo Takahara ◽  
Isamu Sando ◽  
Yoshie Hashida ◽  
Yoshihiro Shibahara
Keyword(s):  
Middle Ear ◽  
Round Window ◽  
Oval Window ◽  
The Body ◽  
First Year ◽  
Sinus Tympani ◽  
Hematoxylin And Eosin ◽  
Round Window Niche ◽  
Temporal Bones ◽  
Middle Ear Cleft

This study was conducted in order to gain basic information about mesenchyme remaining in the middle ear cleft in normal infants. Because no previous report has objectively described the quantity or quality of such postnatal remnants in normal infants, it has not been possible to accurately evaluate the significance of mesenchyme remaining in the middle ears of infants with pathologic conditions, such as otitis media and congenital anomalies. Thus, 53 temporal bones obtained from 41 individuals reported upon here—from 26 weeks' gestation to 8 years of age—will be controls for future studies of pathologic conditions. The children from whom these temporal bones were obtained had no anomalies of the ear (or any other known part of the body); nor did they have any pathologic conditions in the ear. The temporal bones were prepared for histologic study with hematoxylin and eosin staining and were examined under the light microscope. After areas of the mesenchyme in the histologic sections were projected and illustrated for each of 19 portions in the middle ear cleft under the microprojector, those areas were measured by compensating polar planimetry. The findings obtained in this study were as follows: (1) The amount of mesenchyme remaining in the days after birth seemed to gradually decrease in volume with increased maturity and had nearly disappeared within the first year in normal infants. Where mesenchyme was noted in the temporal bones of infants over 1 year of age, it was usually present only in small niches. (2) Most mesenchyme found in temporal bones of normal infants was seen in the mastoid antrum; it was also found in the aditus ad antrum, the epitympanum, the hypotympanum, the sinus tympani, the round window niche, the oval window niche, and the mesotympanum.

IMMUNE STATUS OF EPSTEIN - BARR VIRUS-INFECTED CHILDREN WITH SECRETORY MIDDLE OTITIS

2020 ◽  
pp. 60-64
Author(s):  
Alina Volodymyrivna Chumakova ◽  
Yuliia Viktorivna Lozova
Keyword(s):  
Otitis Media ◽  
Epstein Barr Virus ◽  
Immune Status ◽  
Enzyme Analysis ◽  
Comprehensive Treatment ◽  
Upper Respiratory Tract ◽  
Adequate Treatment ◽  
Barr Virus ◽  
Cell Immunity ◽  
Epstein Barr

Recently the role of herpes viruses in an aggravation of inflammatory diseases of the upper respiratory tract, in particular, herpes simplex virus and Epstein − Barr virus, has become increasingly evident in otorhynolaryngology practice. To determine the extent of infection with Epstein − Barr virus and to study the immunogram of the first level for the children with secretory otitis media, 48 patients aged 3−9 years were examined for the purpose of an adequate treatment. Infection was revealed by serological diagnosis (enzyme immunoassay) with the determination of IgM to capsid complex (VCA) and IgG to early antigen (EA). Level 1 immunograms were also determined by immune enzyme analysis. Children with secretive middle otitis (22.9 %) were infected with Epstein − Barr virus, corresponding to an acute phase of the disease, as well as they had a reduce cell immunity. All children received comprehensive treatment for secretory middle otitis. It was concluded about the need for children with middle otitis to be screened for an infection with the Epstein−Barr virus and treated conservatively by an immunologist. Key words: secretory middle otitis media, etiology of Epstein − Barr virus, immune status of children, treatment.

Sign in / Sign up

Export Citation Format

Share Document