A Case Report-Fibrous Dysplasia of Temporal Bone (original) (raw)

Hearing outcome after surgical intervention in fibrous dysplasia of temporal bone

Indian Journal of Otology, 2012

Pure tone audiometry (PTA) revealed a moderately severe conductive type hearing loss in the right ear; the pure-tone average being 63.33 dBHL and air-bone gap (A-B gap) 48.33 dBHL. Hearing sensitivity in the left ear was within normal limit with PTA 15dBHL. Axial High resolution computed tomography (HRCT) of temporal bone revealed replacement of normal bony architecture by ground glass appearance especially involving the squamous and mastoid parts but sparing the otic capsule and the petrous apex. Th ere was loss of normal cellularity of the mastoid air cell system and narrowing of the middle ear space but ossicles, cochlea-vestibular apparatus and Internal auditory canal (IAC) were normal. Computed tomography fi nding of left ear was within normal limit [Figure 1]. Canal wall down mastoidectomy by post-aural route was performed for excision of disease and restoration of hearing mechanism. Whole of mastoid bone including posterior canal wall was found to be replaced by spongy bone which was drilled out with care being taken to save the facial nerve which was eventually found to be in the normal position. Th ere was cholesteatoma in the external auditory canal distal to occlusion.

Fibrous dysplasia of the temporal bone: report of a case and a review of its characteristics

Ear, nose, & throat journal, 2000

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.

Fibrous dysplasia of the temporal bone secondary to ear surgery: a case report

Journal of Medical Case Reports, 2015

Introduction: In this report, we describe the clinical course, diagnostic features and management of a patient with fibrous dysplasia of the temporal bone 7 years after middle ear surgery on the same side. Case presentation: A 16-year-old Caucasian girl presented to our hospital with a growing bone lesion in the roof of the left temporal bone. She had undergone a previous tympanoplasty at 7 years of age because of a cholesteatoma. At the time of that first surgery, no radiological or histological signs indicated a bone disorder. A computed tomographic scan of the temporal bone showed a lesion with rarefaction areas and lytic images inside that affected the roof of the cavity to the tegmen tympani without alterations in the inner ear. A surgical revision of the ear cavity was performed by resecting the lesion and regularizing the cavity. The histopathologic study confirmed fibrous dysplasia. The patient progressed satisfactorily after surgery with no evidence of recurrence. Conclusion: To the best of our knowledge, this is the first report of fibrous dysplasia of the temporal bone secondary to ear surgery.

External auditory canal stenosis due to isolated fibrous dysplasia of temporal bone: a case report

International Journal of Otorhinolaryngology and Head and Neck Surgery, 2019

Benign disease of bone marrow in which marrow is reorganize into fibrous tissue and immature woven bone, is known as fibrous dysplasia. Involvement of temporal bone is rare and can be isolated or in monostotic or polystotic form with various otologic manifestations. We are here reporting a case of fibrous dysplasia of the unilateral temporal bone who presented with hearing loss and stenosis of the external auditory canal. External auditory canal stenosis due to fibrous dysplasia was managed with canalplasty and patient was free of recurrence till last follow up. Fibrous dysplasia of isolated temporal bone is a rare entity which requires high grade of suspicion in a patient who presents with unusual bony swellings in the ear. Its management usually includes resection of the most affected part of temporal bone in order to achieve auditory canal patency and restoration of hearing. After surgery, a close follow up is warranted due to its propensity of recurrence.

Fibrous dysplasia: rare manifestation in the temporal bone

Brazilian Journal of Otorhinolaryngology, 2020

Introduction: Fibrous dysplasia is a benign disorder, in which normal bone is replaced by fibrosis and immature bone trabeculae, showing a similar distribution between the genders, and being more prevalent in the earlier decades of life. Fibrous dysplasia of the temporal bone is a rare condition, and there is no consensus as to whether it is more common in monostotic or polyostotic forms. External auditory meatus stenosis and conductive dysacusis are the most common manifestations, with cholesteatoma being a common complication, whereas the involvement of the otic capsule is an unusual one. Surgical treatment is indicated to control pain or dysacusis, otorrhea, cholesteatoma, and deformity. Objectives: To describe the clinical experience of a tertiary referral hospital with cases of fibrous dysplasia of the temporal bone. Methods: Sampling of patients diagnosed with fibrous dysplasia of the temporal bone, confirmed by tomography, treated at the pediatric otology and otorhinolaryngology outpatient clinics, between 2015 and 2018. The assessed variables were age, gender, laterality, external auditory meatus stenosis, deformity, hearing loss, presence of secondary cholesteatoma of the external auditory meatus, lesion extension and management. Results: Five patients were included, four females and one male, with age ranging from 13 to 34 years. Three had the polyostotic form and two the monostotic form of fibrous dysplasia of the temporal bone. Four patients had local deformity and external auditory meatus stenosis, two of which progressed to cholesteatoma. All patients showed some degree of hearing impairment. All had preserved otic capsule at the tomography. Two patients are currently undergoing clinical observation; two were submitted to tympanomastoidectomy due to secondary cholesteatoma; one was submitted to lesion resection, aiming to control the dysacusis progression.

Different clinical presentation and management of temporal bone fibrous dysplasia in children

World Journal of Surgical Oncology

Background: Fibrous dysplasia is a slowly progressive benign fibro-osseous disorder that involves one or multiple bones with a unilateral distribution in most cases. It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology. Temporal bone involvement is the least frequently reported type, especially in children. We reviewed available articles regarding fibrous dysplasia with temporal bone involvement in children and added four patients aged 7 to 17 years who were diagnosed and treated in our institution from 2006 to 2017. The patients' clinical picture comprised head deformity, external canal stenosis, headache, progressive conductive and/ or sensorineural hearing loss, tinnitus, and sudden deafness. Two patients had experienced severe episodic vertigo with nausea and vomiting. Two were referred to us with external canal obstruction and secondary cholesteatoma formation with broad middle ear destruction. One was diagnosed with acute mastoiditis and intracranial complications. Optimal management of fibrous dysplasia is unclear and can be challenging, especially in children. In our two patients with disease expansion and involvement of important structures, surgical treatment was abandoned and a "wait-and-scan" policy was applied. The other two were qualified for surgical treatment. One patient underwent two surgeries: modified lateral petrosectomy (canal left open) with pathological tissue removal, cavity obliteration, and subsequent tympanoplasty. Another patient with extensive destruction of the left temporal bone underwent canal wall down mastoidectomy with perisinus abscess drainage and revision 12 months later. Tympanoplasty was unsatisfactory in both patients because of slow progression of the middle ear pathology. None of our patients underwent pharmacological treatment. Conclusions: In younger patients, observation and a "wait-and-scan" protocol is relevant until significant function, or cosmetic deficits are obvious. Surgery is not preferred and should be delayed until puberty because fibrous dysplasia has a tendency to stabilize after adolescence. In patients with severe symptoms medical treatment can be implemented, but safety of this treatment in children remain controversial.

Fibrous Dysplasia Involving The Temporal Bone: Report Of Four Cases

The Internet Journal of Otorhinolaryngology, 2003

Fibrous dysplasia of bone is a fibro-osseous tissue disease which has unknown etiology and characterized by replacement of normal bone by a variable amount of fibrous tissue and woven bone. The disease may be unifocal, multifocal or as seen in McCune Albright Syndrome. Fibrous dysplasia rarely involves temporal bone and diagnosis is made with the displacement of neighboring structures. The aim of treatment is cosmetic and functional. We report four cases.

Fibro-Osseous Dysplasia of the Temporal Bone : Review and Case Presentations

2016

Benign fibro-osseous lesions are common in the maxillofacial skeleton. However, their incidence in the temporal bone is rare. They are usually silent but may mimic other temporal bone pathologies. The radiographic features suggest the diagnosis. In some cases, histological confirmation may be needed. Fibro-osseous lesions of the temporal bone should be considered in the differential diagnosis of lesions with atypical otological presentations. In most cases no intervention is planned except when there is a functional deficit. This paper reviews this condition with a presentation of three cases.