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.

HISTOPATHOLOGY OF THE TEMPORAL BONE IN OSTEOGENESIS IMPERFECTA CONGENITA

1985 ◽  
Vol 95 (2) ◽  
pp. 193???199 ◽  
Author(s):  
GILEAD BERGER ◽  
MICHAEL HAWKE ◽  
ALAN JOHNSON ◽  
DAVID PROOPS
Keyword(s):  
Osteogenesis Imperfecta ◽  
Temporal Bone ◽  
Osteogenesis Imperfecta Congenita

Osteogenesis Imperfecta Congenita and Tarda: A Temporal Bone Report

1975 ◽  
Vol 84 (3) ◽  
pp. 350-358 ◽  
Author(s):  
Joan T. Zajtchuk ◽  
John R. Lindsay
Keyword(s):  
Hearing Loss ◽  
Osteogenesis Imperfecta ◽  
Temporal Bone ◽  
Histological Examination ◽  
Conductive Hearing Loss ◽  
Conductive Hearing ◽  
Osteogenesis Imperfecta Congenita ◽  
Endochondral Layer

The temporal bone report of an operated case of osteogenesis imperfecta tarda is presented. Histological examination confirmed the presence of bilateral fixation of the footplate by otosclerosis as the cause of the conductive hearing loss. Fragility of bony septae in the mastoid and of the stapedial crura were observed. Sensorineural impairment in later years with a reduction in neural elements in the cochlea appear related to the extent and activity of the otosclerotic foci. Additional temporal bone reports of three cases of osteogenesis imperfecta congenita show lack of deposition of the skein-like bone in the endochondral layer, sparse bony septae in marrow spaces and deficiency of the periosteal layer. The stapedial crura were thin and in two cases both were deformed and fractured.

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.

The ear

2021 ◽  
pp. 497-518
Author(s):  
Daniel R. van Gijn ◽  
Jonathan Dunne
Keyword(s):  
Inner Ear ◽  
Temporal Bone ◽  
Middle Ear ◽  
Semicircular Canals ◽  
Lateral Wall ◽  
Oval Window ◽  
External Ear ◽  
Membranous Labyrinth ◽  
Stapes Footplate ◽  
Petrous Temporal Bone

The delicate yet definitive deflections of the pinna (wing/fin) of the external ear contribute to the collection of sound. The external acoustic meatus is responsible for the transmission of sounds to the tympanic membrane, which in turn separates the external ear from the middle ear. The middle ear is an air filled (from the nasopharynx via the eustachian tube), mucous membrane lined space in the petrous temporal bone. It is separated from the inner ear by the medial wall of the tympanic cavity – bridged by the trio of ossicles. The inner ear refers to the bony and membranous labyrinth and their respective contents. The osseus labyrinth lies within the petrous temporal bone. It consists of the cochlea anteriorly, semicircular canals posterosuperiorly and intervening vestibule – the entrance hall to the inner ear whose lateral wall bears the oval window occupied by the stapes footplate.

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.

Labyrinthine sequestration secondary to auditory implantation: report of two cases and review of the literature

2015 ◽  
Vol 129 (12) ◽  
pp. 1238-1242 ◽  
Author(s):  
E Warner ◽  
N Eze ◽  
S Connor ◽  
D Jiang
Keyword(s):  
Conservative Treatment ◽  
Inner Ear ◽  
Middle Ear ◽  
Surgical Intervention ◽  
Case Reports ◽  
Treatment Options ◽  
Review Of The Literature ◽  
Bony Labyrinth ◽  
Radiological Changes ◽  
Infective Complications

AbstractBackground:Auditory implantation into the inner ear is increasingly performed for a variety of indications. Infective complications are rare, but when they occur they can have devastating consequences.Case reports:This paper reports two cases where vestibular sequestration of the bony labyrinth developed following implantation into the middle ear.Conclusion:To the authors' knowledge, these are the first reported cases where vestibular sequestration has resulted from auditory implant surgery. This paper outlines the radiological changes characteristic of this pathology. It also describes the surgical and conservative treatment options for this condition, challenging the previously accepted belief that affected patients always require aggressive surgical intervention.

Assembling a functional tympanic membrane: signals from the external acoustic meatus coordinate development of the malleal manubrium

Development ◽  
2000 ◽  
Vol 127 (19) ◽  
pp. 4127-4136 ◽  
Author(s):  
M. Mallo ◽  
H. Schrewe ◽  
J.F. Martin ◽  
E.N. Olson ◽  
S. Ohnemus
Keyword(s):  
Inner Ear ◽  
Tympanic Membrane ◽  
Middle Ear ◽  
Essential Role ◽  
Acoustic Pressure ◽  
Developmental Process ◽  
Terrestrial Mammals ◽  
Ear Ossicles ◽  
Membrane Vibrations

In terrestrial mammals, hearing starts with the perception of acoustic pressure by the tympanic membrane. Vibrations in this membrane are then transduced into the inner ear by the ossicle chain of the middle ear, composed of the malleus, incus and stapes. The proper connection of the ossicle chain with the tympanic membrane, provided by the insertion of the manubrium of the malleus into the eardrum, is essential for the functionality of the hearing apparatus. We describe here the mechanisms regulating the development of the manubrium and its integration into the tympanic membrane. We show that the external acoustic meatus (EAM), which eventually forms the outer epithelium of the tympanic membrane, plays an essential role in this developmental process. Histological and expression analyses indicate that the manubrium develops close to the EAM with a similar temporal sequence. In addition, when the middle ear ossicles are allowed to develop in vitro under conditions that do not support further EAM development, the manubrium develops only up to the stage of its induction at the time of explantation. Moreover, genetically or teratogenically derived alterations in the EAM also have an effect on manubrial development. Finally, we show that the EAM is the source of two quite opposite activities, one that induces chondrogenesis and another that represses it. The combination of these two activities results in the proper positioning of the manubrium.

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.

scholarly journals Morphology and function of Neandertal and modern human ear ossicles

2016 ◽  
Vol 113 (41) ◽  
pp. 11489-11494 ◽  
Author(s):  
Alexander Stoessel ◽  
Romain David ◽  
Philipp Gunz ◽  
Tobias Schmidt ◽  
Fred Spoor ◽  
...  
Keyword(s):  
Temporal Bone ◽  
Functional Properties ◽  
Middle Ear ◽  
Spatial Configuration ◽  
Brain Size ◽  
Modern Human ◽  
Small Sample ◽  
Last Common Ancestor ◽  
Tympanic Cavity ◽  
Ear Ossicles

The diminutive middle ear ossicles (malleus, incus, stapes) housed in the tympanic cavity of the temporal bone play an important role in audition. The few known ossicles of Neandertals are distinctly different from those of anatomically modern humans (AMHs), despite the close relationship between both human species. Although not mutually exclusive, these differences may affect hearing capacity or could reflect covariation with the surrounding temporal bone. Until now, detailed comparisons were hampered by the small sample of Neandertal ossicles and the unavailability of methods combining analyses of ossicles with surrounding structures. Here, we present an analysis of the largest sample of Neandertal ossicles to date, including many previously unknown specimens, covering a wide geographic and temporal range. Microcomputed tomography scans and 3D geometric morphometrics were used to quantify shape and functional properties of the ossicles and the tympanic cavity and make comparisons with recent and extinct AMHs as well as African apes. We find striking morphological differences between ossicles of AMHs and Neandertals. Ossicles of both Neandertals and AMHs appear derived compared with the inferred ancestral morphology, albeit in different ways. Brain size increase evolved separately in AMHs and Neandertals, leading to differences in the tympanic cavity and, consequently, the shape and spatial configuration of the ossicles. Despite these different evolutionary trajectories, functional properties of the middle ear of AMHs and Neandertals are largely similar. The relevance of these functionally equivalent solutions is likely to conserve a similar auditory sensitivity level inherited from their last common ancestor.

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