Fibrous Dysplasia of the Temporal Bone: Report of a Case and a Review of its Characteristics

2000 ◽  
Vol 79 (1) ◽  
pp. 52-57 ◽  
Author(s):  
Chariton E. Papadakis ◽  
Charalambos E. Skoulakis ◽  
Emmanuel P. Prokopakis ◽  
Antonios A. Nikolidakis ◽  
John G. Bizakis ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Fibrous Dysplasia ◽  
Temporal Bone ◽  
Bone Development ◽  
Osteoblastic Differentiation ◽  
Unknown Etiology ◽  
Normal Bone ◽  
Mixed Hearing Loss ◽  
Characteristic Features ◽  
Mesenchymal Precursor

Fibrous dysplasia is an uncommon benign disorder of unknown etiology. It represents a disturbance of normal bone development—specifically a defect in osteoblastic differentiation and maturation that originates in the mesenchymal precursor of the bone. Because fibrous dysplasia shows a predilection for the facial and cranial bones, where it causes deformity and dysfunction, the disease is of particular interest to the otolaryngologist. In this paper, we report a case of fibrous dysplasia of the temporal bone, the first symptom of which was a mixed hearing loss. We discuss the characteristic features of this specific location of the disease, the differential diagnosis, and the treatment policy. We also address the issue of secondary sensorineural hearing loss.

Rare proliferative and inflammatory pathologies localized in the temporal bone - a review of the literature

2019 ◽  
Vol 8 (2) ◽  
pp. 1-5
Author(s):  
Adam Roszkowski Roszkowski ◽  
Alicja Witkowska ◽  
Piotr Baranek ◽  
Anna Rzepakowska ◽  
Emilia Wnuk ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Fibrous Dysplasia ◽  
Temporal Bone ◽  
Inflammatory Pseudotumor ◽  
Soft Tissues ◽  
Bone Structure ◽  
Histopathological Examination ◽  
Fibrous Tissue ◽  
Diagnostic Methods ◽  
Unknown Etiology

Proliferative-inflammatory pathologies may occupy the temporal bone, resulting in: hearing loss, vestibular dysfunction, and neuropathies from cranial nerve compression. Although their occurrence is episodic, the appropriate diagnostic procedure is extremely important to achieve expected therapeutic effect. The aim of study was characterization of selected proliferative-inflammatory pathologies that may occupy the temporal bone: fibrous dysplasia, inflammatory pseudotumor, osteoradionecrosis, and presentation of diagnostic methods for the differentiation of these diseases as well as discussion on appropriate therapeutic options. Fibrous dysplasia (fibrous dysplasia) is a slowly progressive, benign bone disorder of unknown etiology characterized by abnormal proliferation of fibrous tissue. IPT (inflammatory pseudotumor) is a rare, non-malignant inflammatory process of unknown etiology, characterized by connective tissue proliferation and infiltration of inflammatory cells. Osteoradionecrosis of the temporal bone (TB-ORN) is a rare but potentially fatal complication of radiotherapy for head and neck cancer. Due to the similarity of symptoms with typical inflammatory conditions of middle ear (pain, otorrhea, hearing loss), selected disorders may be a dilemma regarding the diagnosis and proper further treatment. The clinical examination is mandatory, however radiological imaging may demonstrate the existence of specific changes and direct the diagnosis. The computed tomography of fibrous dysplasia shows the abnormal organization of the bone structure. Magnetic resonance, as the most sensitive for inflammatory pseudotumors, visualizes inflammatory infiltrates in soft tissues. The spiral tomography of temporal identifies the erosion in the course of osteoradionecrosis. However the final diagnosis may be establish post the histopathological examination and exclusion of the neoplastic process.

Management of Temporal Bone Fractures

Neurotrauma ◽  
2019 ◽  
pp. 103-112
Author(s):  
Hongzhao Ji ◽  
Brandon Isaacson
Keyword(s):  
Hearing Loss ◽  
Temporal Bone ◽  
Bone Fractures ◽  
Surgical Decompression ◽  
Vascular Injuries ◽  
Surgical Exploration ◽  
Mixed Hearing Loss ◽  
Csf Leakage ◽  
Spontaneous Improvement ◽  
Fluid Leak

Temporal bone fractures can present with a variety of symptoms and physical exam findings. Facial paralysis, hearing loss, spinal fluid leak, vestibular dysfunction, and vascular injuries are all potential issues that may occur in the setting of skull base trauma. The indications and interpretation of facial nerve electrophysiology studies with respect to the need for surgical decompression or repair are reviewed. Injuries to the middle and inner ear may result in conductive, mixed, or pure sensorineural hearing loss depending on the location of the fracture. Surgical repair of conductive or mixed hearing loss should be delayed because spontaneous improvement often occurs. CSF leakage presents with clear or serosanguineous aural discharge and typically resolves with conservative measures. Occasionally spinal fluids leaks require lumbar subarachnoid drainage or surgical exploration and repair. Evaluation and management of other issues arising as a result of temporal bone trauma will also be reviewed.

scholarly journals Management of the Temporal Bone Fibrous Dysplasia With External Auditory Canal Stenosis and Secondary Cholesteatoma in an Asian Population: A 11-Case Series

2020 ◽  
pp. 014556132092792
Author(s):  
Kun Zhang ◽  
Peng Qu ◽  
Bing Wang ◽  
Endong Zhang ◽  
Bing Chen
Keyword(s):  
Hearing Loss ◽  
Fibrous Dysplasia ◽  
Temporal Bone ◽  
External Auditory Canal ◽  
Perioperative Complications ◽  
Case Series ◽  
Asian Population ◽  
Canal Stenosis

Objective: This article summarizes the experience of diagnosis and treatment of temporal bone fibrous dysplasia (FD) with external auditory canal (EAC) stenosis and secondary cholesteatoma in the Chinese population, in order to improve the quality of life of patients in the future. Methods: Eleven patients with FD of the temporal bone who underwent surgery were retrospectively reviewed. Results: All lesions originated from the temporal bone, and all involved of the EAC. There were 11 cases of cholesteatoma in the EAC, 4 cases of cholesteatoma in the middle ear. The most common symptoms were hearing loss (100%), tinnitus (36.4%), and otorrhea (36.4%). Two patients were severe-profound sensorineural hearing loss, and one patient was complicated with subperiosteal abscesses. All 11 patients underwent surgery. There were no perioperative complications in this series and median follow-up time was 4.2 years. Conclusion: Temporal bone FD remains a rare diagnosis, especially in the Asian population. The lesions mainly lead to stenosis of the EAC, especially at the osteochondral junction. Cholesteatoma is the main complication of this disease, which is secondary to occlusion of the EAC with the growth of the lesion. Canaloplasty of EAC combined with wide meatoplasty can provide excellent prognosis in most cases.

Monostotic fibrous dysplasia of the temporal bone

1999 ◽  
Vol 113 (8) ◽  
pp. 772-774 ◽  
Author(s):  
J. Xenellis ◽  
A. Bibas ◽  
L. Savy ◽  
P. Maragoudakis ◽  
P. Nomicos
Keyword(s):  
Computed Tomography ◽  
Fibrous Dysplasia ◽  
Temporal Bone ◽  
Symptomatic Disease ◽  
Normal Bone ◽  
Limited Surgery

AbstractFibrous dysplasia is a slowly progressive bony disorder where normal bone is replaced by abnormal fibroosseous tissue. Its monostotic variety in the temporal bone is very rare and such a case is presented here. Computed tomography (CT) may be adequate for the diagnosis and follow-up of these patients. Limited surgery should only be considered in cases of symptomatic disease.

Monostotic Fibrous Dysplasia of the Temporal Bone with Associated Lymphadenopathy

1994 ◽  
Vol 73 (5) ◽  
pp. 328-330 ◽  
Author(s):  
Martin J. Donnelly ◽  
Donald P. McShane ◽  
Hugh Burns
Keyword(s):  
Fibrous Dysplasia ◽  
Temporal Bone ◽  
Endocrine Disorders ◽  
Normal Bone ◽  
Mucous Membranes ◽  
Long Term Follow Up ◽  
Fibrous Dysplasia Of Bone ◽  
Abnormal Pigmentation

Fibrous dysplasia of bone is a benign idiopathic disorder where abnormal fibro-osseous tissue replaces normal bone. The bony abnormalities may be associated with endocrine disorders and abnormal pigmentation of the skin and mucous membranes. Involvement of the temporal bone is a very rare occurrence. We report a case of monostotic fibrous dysplasia of the temporal bone with associated lymphadenopathy. To our knowledge this association has not been previously described in the literature. We also emphasise the need for long term follow up of patients with this condition as cholesteatoma may develop insidiously.

Polyostotic fibrous dysplasia: A case report

2020 ◽  
Vol 12 (2) ◽  
pp. 43-45
Keyword(s):  
Case Report ◽  
Fibrous Dysplasia ◽  
Unknown Etiology ◽  
Pathological Features ◽  
Polyostotic Fibrous Dysplasia ◽  
Fibrous Stroma ◽  
Normal Bone ◽  
Skeletal Frame ◽  
Gnas1 Gene

Fibrous dysplasia (FD) is a fibro-osseous anomaly, where in normal bone is substituted with fibrous stroma. It is of unknown etiology but recently reported to be associated with mutation in GNAS1 gene (20q13.2) and consists of three subtypes monostotic, polyostotic and craniofacial. Craniofacial FD (CFD) mainly affects the bones of the craniofacial skeletal frame. This article reports a case of a 10 year old boy diagnosed with polyostotic fibrous dysplasia with clinical, radiographical and tissue pathological features.

scholarly journals Cochlear Otosclerosis: An Undiagnosed Cause of Progressive Sensorineural Hearing Loss

2020 ◽  
Vol 28 (2) ◽  
pp. 127-137
Author(s):  
Asish Kumar Lahiri ◽  
Anandita Gupta ◽  
Rakesh Vohra ◽  
Shalabh Sharma ◽  
Satinder Singh
Keyword(s):  
Hearing Loss ◽  
High Resolution ◽  
Ct Scan ◽  
Sensorineural Hearing Loss ◽  
Temporal Bone ◽  
Young Patients ◽  
Oval Window ◽  
Sensorineural Hearing ◽  
Unknown Etiology ◽  
High Resolution Ct

Introduction Otosclerosis presents as conductive or mixed hearing loss depending upon the stage of the disease. Isolated sensorineural hearing loss though known to occur has been rarely reported. Pure cochlear otosclerosis presenting as sensorineural hearing loss should be considered as a differential diagnosis in young patients presenting with progressive hearing loss. The aim of this article is to note the prevalence of cochlear otosclerosis in young patients presenting with progressive sensorineural hearing loss.  Materials and Methods Retrospective chart analysis of 19 patients who presented with progressive SNHL with unknown etiology was done. Results Otosclerosis involving the pericochlear region was diagnosed in 27 ears which was identified by high resolution CT scan of temporal bone. In four ears, in addition, there was demineralization just anterior to oval window without clinical evidence of stapes fixation. Conclusion In young patients with progressive SNHL, high resolution CT scan of temporal bone should be done to identify progressive cochlear otosclerosis. Medical management should be initiated in these patients to halt the progression of disease.

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