Temporal Bone Histopathology in Charge Association

2002 ◽  
Vol 111 (5) ◽  
pp. 397-401 ◽  
Author(s):  
Shin-Ichi Haginomori ◽  
Makoto Miura ◽  
Isamu Sando ◽  
Margaretha L. Casselbrant
Keyword(s):  
Inner Ear ◽  
Temporal Bone ◽  
Middle Ear ◽  
Congenital Heart ◽  
The Other ◽  
Otologic Surgery ◽  
Charge Association ◽  
Ear Anomalies ◽  
Temporal Bones ◽  
Central Nervous System Anomalies

Three temporal bones obtained at autopsy from 2 patients with CHARGE association (Coloboma, congenital Heart disease, Atresia of choanae, mental Retardation and/or central nervous system anomalies, Genital hypoplasia, and Ear anomalies) were examined histopathologically. Both temporal bones from 1 patient showed multiple anomalies in the middle ear, inner ear, and facial nerve. However, the temporal bone obtained from the other patient showed almost normal structures in the inner ear, middle ear, and eustachian tube. These results are different from those of 2 previous reports of temporal bone histopathology regarding CHARGE association. This difference suggests that CHARGE association may arise not from one etiopathogenetic factor, but from complex factors. Special attention to dehiscent facial canal and perilymphatic gusher during otologic surgery in patients with CHARGE association is discussed.

Temporal Bone Anomalies Associated with Congenital Heart Disease

1979 ◽  
Vol 88 (1) ◽  
pp. 72-78 ◽  
Author(s):  
Tetsuya Egami ◽  
Isamu Sando ◽  
Eugene N. Myers
Keyword(s):  
Inner Ear ◽  
Temporal Bone ◽  
Middle Ear ◽  
Congenital Heart ◽  
Jugular Bulb ◽  
Facial Canal ◽  
Large Defect ◽  
High Jugular Bulb ◽  
Ear Anomalies ◽  
Bone Anomalies

A temporal bone histopathological study was conducted to detect temporal bone anomalies in 20 ears (10 cases) of individuals with congenital heart anomalies. We restricted our study to patients more than one year of age, and to heart anomalies of unknown etiology. The temporal bones were obtained from refrigerated cadavers, fixed in formalin, embedded in celloidin, cut in a horizontal plane, stained with H & E, and mounted on glass slides for light microscopic study. Anomalies observed in the middle ear were: remnants of mesenchymal tissue (8 ears), wide angle of the facial genu (6 ears), persistence of the stapedial artery (5 ears), large defect of the facial canal (4 ears), high jugular bulb (4 ears), and bulky incus (2 ears). Inner ear anomalies consisted of a shortened cochlea (5 ears), anomaly of the horizontal canal (3 ears), anomaly of the posterior canal (2 ears), obliteration of the cochlear aqueduct (2 ears), and patent utriculoendolymphatic valve (1 ear). Most of the anomalies observed appeared to be due to arrested development, and resembled features which may be found at various stages of fetal life. Structural anomalies were more commonly found in the mesoderm than in the ectoderm, and middle ear anomalies were more frequently encountered than anomalies of the inner ear. No definite relationship between these anomalies observed in the temporal bone and hearing problems which had been recorded clinically for these patients could be detected. However, the large defect (more than one third of the circumference) of the facial canal, the high jugular bulb, and the stapedial artery persistence should be recognized as problems since they may be encountered during middle ear surgery.

Temporal Bone Findings in Cases of Salt Water Drowning

1983 ◽  
Vol 92 (2) ◽  
pp. 134-136 ◽  
Author(s):  
George Kelemen
Keyword(s):  
Inner Ear ◽  
Temporal Bone ◽  
Middle Ear ◽  
Salt Water ◽  
Round Window ◽  
Scuba Diving ◽  
Serial Sections ◽  
Histopathological Findings ◽  
Histopathological Features ◽  
Temporal Bones

The otologic literature contains no description of the histopathological features of temporal bones of persons who perished in drowning accidents. Three temporal bones from two fatal cases of drowning were studied in serial sections. Two temporal bones were from a man aged 31 years who collapsed after scuba diving and died three days later. One temporal bone was from a 32-year-old man, serving in the navy, who drove his car, probably accidentally, into the sea and drowned in it. Histopathological findings appeared to be similar in the two cases. Diffuse hemorrhage was evident throughout the middle ear and inner ear. The tympanic and round window membranes appeared to be intact although hemorrhage had occurred within the tissue layers of the membranes. This finding contrasts with observations of ruptured membranes by other investigators.

scholarly journals CT Imaging Categorization and Biomarker Study of Anomalous Tympanic Segment of the Facial Nerves in Patients With Hearing Loss in the Absence of Microtia

2019 ◽  
Vol 98 (6) ◽  
pp. 340-345
Author(s):  
Nisa Oren ◽  
Daniel Thomas Ginat
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Temporal Bone ◽  
Charge Syndrome ◽  
Oval Window ◽  
High Resolution Computed Tomography ◽  
Facial Nerves ◽  
Ear Anomalies ◽  
Temporal Bones ◽  
Congenital Syndrome

The purpose of this study is to categorize anomalous tympanic facial nerve (FN) on high-resolution computed tomography (HRCT) and to determinate the significance of associated temporal bone anomalies and congenital syndromes without microtia in patients with hearing loss. A retrospective analysis of HRCT findings in 30 temporal bones in 18 patients with anomalous FN was performed. Abnormalities of the tympanic FN were categorized as follows: category 1: FN medially positioned, but above the oval window; category 2: FN in the oval window niche; and category 3: FN below the oval window. Potential associated findings that were assessed included stapes abnormalities, oval window atresia, and inner ear anomalies, as well as the presence of a known congenital syndrome with hearing loss. The most common type of anomalous tympanic FN was category 1 (67%, n = 20), following by group 2 (20%, n = 6) and group 3 (13%, n = 4). Stapes anomalies were detected in 77% of temporal bones (n = 23), oval window atresia was detected in 43% of temporal bones (n = 13), and inner ear anomalies were detected in 70% of temporal bones (n = 21). Anomalous tympanic facial nerves in temporal bone with conductive hearing loss were often (60%) not associated with oval window atresia. The combination of aberrant tympanic FN and inner ear anomalies was significantly ( P = .038) associated with a known congenital syndrome (6 patients), including CHARGE syndrome, oculo-auriculo-vertebral spectrum, Pierre-Robin sequences, and Down syndrome. Therefore, an anomalous tympanic FN in conjunction with inner ear anomalies appears to be a biomarker for certain congenital syndromes with hearing loss in the absence of microtia.

Spectrum of Middle and Inner Ear Abnormalities in Infants With Congenital Heart Defects

2005 ◽  
Vol 133 (2) ◽  
pp. 260-268 ◽  
Author(s):  
Seckin O. Ulualp ◽  
Charles G. Wright ◽  
Peter S. Roland
Keyword(s):  
Inner Ear ◽  
Temporal Bone ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Outer Hair Cell ◽  
Heart Defects ◽  
Round Window ◽  
Cell Loss ◽  
Spiral Ligament ◽  
Temporal Bones

Objective: To evaluate temporal bone histopathology in infants with congenital heart defects (CHD). Study Design and Setting: A retrospective review of our temporal bone collection was conducted to identify temporal bones acquired from infants with CHD. Subjects were divided into nonsyndromic and syndromic CHD groups. The presence of temporal bone abnormalities and the incidence of abnormalities that may result in hearing impairment were determined. Results: Thirty-eight temporal bones obtained from 16 infants with nonsyndromic CHD and 4 with syndromic CHD were evaluated. Nonsyndromic CHD cases had abnormalities such as a mesenchymal remnant, malformed stapes, persistent stapedial artery, shallow round window, dehiscent facial nerve canal, short cochlea, strial basophilic deposits, deformity of the spiral ligament, bulging Reissner's membrane, hypoplastic lateral semicircular canal, and cupular deposits. Syndromic CHD cases had abnormalities including narrow round window niche, facial canal dehiscence, strial basophilic deposits and cysts, and outer hair cell loss. Middle and inner ear abnormalities that may impair hearing were observed in 6 subjects with nonsyndromic CHD and in 1 subject with syndromic CHD. Conclusions: A wide variety of temporal bone defects were documented in infants with CHD. Congenital middle and inner ear abnormalities should be anticipated in the hearing assessment and otologic surgery of infants with CHD.

scholarly journals The Effect of Pulling Out Cochlear Implant Electrodes on Inner Ear Microstructures: A Temporal Bone Study

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Ingo Todt ◽  
Rainer O. Seidl ◽  
Arne Ernst
Keyword(s):  
Cochlear Implant ◽  
Inner Ear ◽  
Temporal Bone ◽  
Basilar Membrane ◽  
Temporal Sequence ◽  
Pull Out ◽  
Temporal Bones

The exchange of an cochlear implant or the re-positioning of an electrode have become more frequently required than a decade ago. The consequences of such procedures at a microstructural level within the cochlea are not known. It was the aim of the present study to further investigate the effects of an CI electrode pull-out. Therefore 10 freshly harvested temporal bones (TB) were histologically evaluated after a cochlear implant electrode pull-out of a perimodiolar electrode. In additional 9 TB the intrascalar movements of the CI electrode while being pulled-out were digitally analysed by video- capturing. Histologically, a disruption of the modiolar wall or the spiral osseous lamina were not observed. In one TB, a basilar membrane lifting up was found, but it could not be undoubtedly attributed to the pull-out of the electrode. When analyzing the temporal sequence of the electrode movement during the pull-out, the electrode turned in one case so that the tip elevates the basilar membrane. The pull- out of perimodiolarly placed CI electrodes does not damage the modiolar wall at a microstructural level and should be guided (e.g., forceps) to prevent a 90 o turning of the electrode tip into the direction of the basilar membrane.

Effect of Increased Inner Ear Pressure on Middle Ear Mechanics

1998 ◽  
Vol 118 (5) ◽  
pp. 703-708 ◽  
Author(s):  
Eugene N. Myers ◽  
Shingo Murakami ◽  
Kiyofumi Gyo ◽  
Richard L. Goode
Keyword(s):  
Inner Ear ◽  
Phase Angle ◽  
Middle Ear ◽  
Major Effect ◽  
Measuring System ◽  
Annular Ligament ◽  
Middle Ear Mechanics ◽  
Stapes Footplate ◽  
Temporal Bones ◽  
Inner Ear Pressure

Velocity of malleus, umbo, and stapes footplate in response to stepwise increases up to +400 mm H2O in hydrostatic pressure of the inner ear was investigated in 10 fresh human temporal bones by using a laser Doppler interferometer. The sound-pressure input was 114 dB SPL, and the frequency range was 0.4 to 5.0 kHz. Static displacement of these sites was also measured by a video measuring system. When the inner ear pressure was increased, the malleus and stapes moved outward. Amplitude of umbo velocity decreased below 1.0 kHz with a slight increase around 2.0 kHz, whereas stapes velocity decreased at all frequencies with the major effect below 1.0 kHz. The phase angle of malleus umbo velocity advanced markedly in response to the increased inner ear pressure between 1.0 and 1.4 kHz. Change in the vibration of the umbo was thought to be primarily caused by an increased stiffness of the middle ear conduction system, and that of the stapes was caused by distention of the annular ligament and increased cochlear impedance produced by the increased inner ear pressure. These changes in TM vibration and its phase angle may help detect indirectly an elevation of inner ear pressure. (Otolaryngol Head Neck Surg 1998;118:703–8.)

Congenital Perilymphatic Fistula: Computed Tomography Appearance of Middle Ear and Inner Ear Anomalies

1994 ◽  
Vol 111 (3P1) ◽  
pp. 243-249 ◽  
Author(s):  
Jane L. Weissman ◽  
Peter C. Weber ◽  
Charles D. Bluestone
Keyword(s):  
Computed Tomography ◽  
Retrospective Study ◽  
Inner Ear ◽  
Middle Ear ◽  
Round Window ◽  
Clinical Suspicion ◽  
Perilymphatic Fistula ◽  
Computed Tomography Scans ◽  
Ear Anomalies ◽  
Tomography Appearance

Congenital perilymphatic fistula is an abnormal communication between the inner ear and middle ear. Inner ear anomalies have been described on computed tomography scans. Middle ear anomalies have been found at surgery; the most frequent are anomalies of the stapes and round window. This retrospective study describes the appearance of the inner and middle ear on computed tomography scans, and of the middle ear at surgery, in 10 patients (15 ears) in whom perilymphatic fistula was found at surgery. Twelve of 15 stapes were abnormal at surgery; 4 of these 12 (33%) could be seen on computed tomography scans. Two stapes normal at surgery were normal on computed tomography. Three round windows were abnormal at surgery; none of these was seen on computed tomography scans. There were also four dysplastic cochleas, four dysplastic vestibules, and three dilated vestibular aqueducts. Computed tomography scans identified an abnormal inner ear, middle ear, or both in 8 (53%) of the 15 ears with perilymphatic fistula. An inner ear or middle ear anomaly on computed tomography may heighten clinical suspicion of congenital perilymphatic fistula.

Inner ear pathology in osteogenesis imperfecta congenita

1980 ◽  
Vol 94 (7) ◽  
pp. 697-705 ◽  
Author(s):  
Makoto Igarashi ◽  
Austin I. King ◽  
C. Willy Schwenzfeier ◽  
Tsuneo Watanabe ◽  
Bobby R. Alford
Keyword(s):  
Osteogenesis Imperfecta ◽  
Inner Ear ◽  
Temporal Bone ◽  
Middle Ear ◽  
Stria Vascularis ◽  
Bony Labyrinth ◽  
Congenital Form ◽  
Basal Zone ◽  
Ear Ossicles ◽  
Osteogenesis Imperfecta Congenita

AbstractThis temporal bone report describes the inner ear deformities which were found in addition to the bony pathology in a case of osteogenesis imperfecta congenita. The labyrinthine pathology includes anomalously positioned and enlarged vestibular spaces, the existence of a scala communis (on one side) and the existence of hematoxylin dark-stained material in the basal zone of the stria vascularis.The appearance of temporal bone reports of osteogenesis imperfecta congenita is sporadic. Friedmann (1974)described changes in the bony capsule in one case of osteogenesis imperfecta congenita Zajtchul and Lindsay(1975)reported three cases of the congenital form of osteogenesis imperfecta with their temporal bone findings. Within recent years, Altmann reported three cases in 1962, Bretlau and Jorgensen reported one case in 1969, Bergstrom and others reported one case in 1972, and Bergstrom described the temporal bone findings in four infants in 1977. The pathologic description of the temporal bone in osteogenesis imperfecta congenita has been focused more or less on the structures of the bony labyrinth and the middle ear ossicles, with a brief description of the inner ear. In this report, we describe anomalous inner ear structures in osteogenesis imperfecta congenita.

THE VALUE OF NAVIGATION SYSTEM IN COCHLEAR IMPLANTATION IN PATIENTS WITH ABNORMALITIES OF THE INTERNAL AND MIDDLE EAR

2019 ◽  
Vol 25 (3) ◽  
pp. 12-20
Author(s):  
V.I. Fedoseev ◽  
◽  
N.A. Mileshina ◽  
E.V. Kurbatova ◽  
S.S. Osipenkov ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Inner Ear ◽  
Temporal Bone ◽  
Congenital Malformation ◽  
Middle Ear ◽  
Navigation System ◽  
Cochlear Implantation ◽  
Internal Auditory Canal ◽  
Mondini Dysplasia ◽  
Ct And Mri

The use of navigation system during the coclear implantation (CI) in patients with congenital and acquired malformation of the inner ear is necessary because of surgical difficulties. The purpose of our work is to evaluate the effectiveness of the navigation system in congenital and acquired anomalies of the middle and inner ear. Materials and methods The authors followed-up 27 children with bilateral congenital malformation of the inner ear and 58 patients with acquired cochlea obliteration (12 adults and 46 children with the acquired obliteration were included in the group). 55 patients have the acquired obliteration of the inner ear, 3 patients – the total obliteration of the inner ear. Audiological, CT and MRI were performed. All our patients the CI was performed. Conclusion.The use of navigation system during CI in patients with a combination of malformation of the inner and middle ear to increase the efficiency of surgical treatment. The duration of anesthesia and surgery in children with congenital malformation increases when navigation is applied All patients after meningitis or with the fracture of the temporal bone should be performed MRI The patients with an extension of the internal auditory canal, anomaly of Mondini dysplasia of the cochlear, the partial obliteration of the cochlea have good rehabilitation results after CI. The patients with the total obliteration of the inner ear have unsatisfactory results of rehabilitation.

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