Osteogenesis Imperfecta of the Temporal Bone and its Relation to Otosclerosis

1988 ◽  
Vol 97 (6) ◽  
pp. 585-593 ◽  
Author(s):  
George T. Nager
Keyword(s):  
Hearing Loss ◽  
Connective Tissue ◽  
Bone Formation ◽  
Osteogenesis Imperfecta ◽  
Temporal Bone ◽  
Bone Dysplasia ◽  
Lamellar Bone ◽  
Current Classification ◽  
Sound Conduction ◽  
Loss Of Hearing

Osteogenesis imperfecta (OI) designates a heterogeneous group of heritable disorders of connective tissue that in addition to bone may affect tendons, ligaments, fascia, skin, sclerae, blood vessels, teeth, and hearing. The current classification identifies at least four major syndrome groups or types. It also recognizes a considerable number of additional syndromes that may represent supplementary types or subgroups. Loss of hearing is the least constant of the prominent features of OI. Its incidence varies between 26% and 60%. In OI, formation and remodelling of bone are variously affected. In the temporal bone the development of the inner ear capsule may be involved severely. In the stapes the disturbance in lamellar bone formation can lead to extreme thinness, dehiscence, and nonunion of the stapedial superstructure with the footplate. Osteogenesis imperfecta can be associated with otosclerosis, another bone dysplasia with a different morphology. Otosclerosis, in turn, may interfere with sound conduction and perception. Thus, the hearing loss encountered in OI may be the result of OI, otosclerosis, or a combination of both.

scholarly journals Temporal bone imaging in osteogenesis imperfecta patients with hearing loss

2013 ◽  
Vol 123 (8) ◽  
pp. 1988-1995 ◽  
Author(s):  
Freya K. R. Swinnen ◽  
Jan W. Casselman ◽  
Els M. R. De Leenheer ◽  
Cor W. R. J. Cremers ◽  
Ingeborg J. M. Dhooge
Keyword(s):  
Hearing Loss ◽  
Osteogenesis Imperfecta ◽  
Temporal Bone ◽  
Bone Imaging ◽  
Temporal Bone Imaging

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.

Temporal Bone Fractures in Children

1979 ◽  
Vol 87 (3) ◽  
pp. 323-329 ◽  
Author(s):  
Robert S. Shapiro
Keyword(s):  
Hearing Loss ◽  
Facial Nerve ◽  
Temporal Bone ◽  
Bone Fractures ◽  
Age Distribution ◽  
Loss Of Consciousness ◽  
Tympanic Membrane Perforations ◽  
Fractures In Children ◽  
Loss Of Hearing ◽  
Transverse Fractures

Fifty children with temporal bone fractures were treated during a 40-month period. The diagnosis and management of temporal bone fractures in children is reviewed. The author's cases are classified as to longitudinal and transverse fractures, and the figures are presented for age distribution, sex, cause, loss of consciousness, CSF otorrhea, hearing loss, permanent tympanic membrane perforations, and facial nerve Involvement. The author has followed a conservative method of management. Permanent impairment was not frequent, although all the patients with transverse fractures suffered total loss of hearing in the involved ear.

scholarly journals Osteogenesis imperfecta as a cause of death

2018 ◽  
Vol 21 (1) ◽  
pp. 23-27
Author(s):  
Anastasia A. Malygina ◽  
Tatiana A. Grebennikova ◽  
Anatoly N. Tiulpakov ◽  
Zhanna E. Belaya
Keyword(s):  
Connective Tissue ◽  
Osteogenesis Imperfecta ◽  
Classification System ◽  
Clinical Case ◽  
Clinical Symptoms ◽  
Type Ii ◽  
Multiple Fractures ◽  
Current Classification ◽  
Tubular Bones ◽  
Current Classification System

Osteogenesis imperfecta (OI) is a rare heterozygous connective tissue disordercaused by mutations in genes that affect collagen components (in most cases mutations in COL1A1 и COL1A2 genes). The current classification system includes 15 types of OI, one of which (type II) is characterized by 100% intrauterine or perinatal mortality. The structure of mortality in other OI types is poorly understood because of the heterogeneity of clinical symptoms and the severity of connective tissue damage. W present a clinical case of type III osteogenesis imperfecta, complicated by generalized osteoporosis with multiple fractures of vertebrae and tubular bones and progressive kyphoscoliosis. Late-initiated treatment led to progression of the disease and led to cardiopulmonary insufficiency and death of the patient. Our clinical case highlights the importance of timely diagnosis, treatment and regular observation in patients with OI.

Differential Osteoclast Activation in Endochondral and Intramembranous Bone

1993 ◽  
Vol 102 (8) ◽  
pp. 616-619 ◽  
Author(s):  
Richard A. Chole
Keyword(s):  
Hearing Loss ◽  
Bone Resorption ◽  
Osteogenesis Imperfecta ◽  
Temporal Bone ◽  
Otitis Media ◽  
Middle Ear ◽  
Chronic Otitis Media ◽  
Intramembranous Bone ◽  
Inner Surface ◽  
Osteoclast Activation

Osteoclast activation within the temporal bone leads to hearing loss in a variety of pathologic conditions, including otosclerosis, osteogenesis imperfecta, Paget's disease of bone, and chronic otitis media. Although endochondral (EC) bone is remodeled in these diseases, it is relatively more resistant to remodeling when compared to intramembranous (IM) bone. In this study, localized bone resorption and remodeling were induced on the inner surface of the middle ear by pressurization with air to 10 mm Hg above atmospheric. Osteoclast surface increased in IM bone, but not in EC bone. This study supports the concept that there may be fundamental differences between EC and IM bone and that EC is more resistant to pressure-induced localized resorption than IM bone.

scholarly journals Cochlear Implantation Following Explorative Tympanotomy in Patients With Sudden Sensorineural Hearing Loss: Surgical Features and Audiological Outcomes

2021 ◽  
pp. 014556132110091
Author(s):  
Robin Rupp ◽  
Joachim Hornung ◽  
Matthias Balk ◽  
Matti Sievert ◽  
Sarina Müller ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Connective Tissue ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Round Window ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Round Window Membrane ◽  
Hearing Results ◽  
Round Window Niche

Objective: To investigate the anatomical status of the round window niche and hearing outcome of cochlear implantation (CI) after explorative tympanotomy (ExT) with sealing of the round window membrane in patients with sudden sensorineural hearing loss at a tertiary referral medical center. Methods: Between January 1, 2007, and July 30, 2020, 1602 patients underwent CI at our department. Out of these, all patients previously treated by ExT with sealing of the round window membrane because of unilateral sudden hearing loss were included in the study. A retrospective chart review was conducted concerning method of round window membrane sealing, intraoperative findings during CI, postoperative imaging, and hearing results. Results: Twenty one patients (9 females; 8 right ears; 54.3 years [± 12.9 years]) underwent ExT with sealing of the round window membrane with subsequent CI after 26.6 months (± 32.9 mo) on average. During CI, in 76% of cases (n = 16), the round window niche was blocked by connective tissue due to the previous intervention but could be removed completely in all cases. The connective tissue itself and its removal had no detrimental effects on the round window membrane. Postoperative computed tomography scan showed no electrode dislocation. Mean postoperative word recognition score after 3 months was 57.4% (± 17.2%) and improved significantly to 73.1% (± 16.4%, P = .005) after 2 years. Conclusion: Performing CI after preceding ExT, connective tissue has to be expected blocking the round window niche. Remaining tissue can be removed safely and does not alter the round window membrane allowing for a proper electrode insertion. Short- and long-term hearing results are satisfactory. Consequently, ExT with sealing of the round window membrane in patients with sudden sensorineural hearing loss does not impede subsequent CI that can still be performed safely.

scholarly journals EXPERIMENTAL STUDIES ON HETEROPLASTIC BONE FORMATION

1920 ◽  
Vol 32 (6) ◽  
pp. 745-766 ◽  
Author(s):  
Goichi Asami ◽  
William Dock
Keyword(s):  
Connective Tissue ◽  
Bone Formation ◽  
Direct Contact ◽  
Endochondral Ossification ◽  
Experimental Studies ◽  
Rabbit Kidney ◽  
Transitional Epithelium ◽  
Ear Cartilage ◽  
Calcium Deposits ◽  
New Formation

1. Bone formation in the rabbit kidney with ligated vessels takes place (a) through the activity of young fibroblasts which accumulate to form a membrane-like structure; (b) subsequently by direct ossification of hyaline connective tissue in continuity with preformed bone; and (c) through erosion of lime placques by granulating tissue and laying down of lamellar bone by cells derived from fibroblasts. 2. Bone formation in the rabbit kidney begins not in direct contact with calcium deposits, but in the loose vascular connective tissue close under the transitional epithelium of the calices. 3. With autotransplanted ear cartilage of the rabbit there is an active new formation of cartilage in the connective tissue which surrounds the transplants, and the bone is formed by the fibroblasts from the perichondrium which erode and invade the calcified areas in this new cartilage. 4. The process of bone formation in the kidney is similar to that found in normal membranous ossification, while with the transplanted ear cartilage the process is identical with endochondral ossification.

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