Different clinical presentation and management of temporal bone fibrous dysplasia in children (original) (raw)

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.

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: 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.

Fibrous dysplasia originating from the middle ear

Journal of Clinical and Experimental Investigations, 2013

Fibrous Dysplasia is rarely seen in adults. The anterior craniofacial bones are more commonly involved than more lateral or posterior regions. Loss of vestibular function, tinnitus and hearing loss may be seen associated with sphenoid and temporal bone involvement. We aimed to report the case of a thirty-year-old female patient with fibrous dysplasia located in the middle ear.

A Case Report-Fibrous Dysplasia of Temporal Bone

Research in Medical & Engineering Sciences

We present a case of 35 year old female with monotonic fibrous dysplasia which presented to us with blocked sensation, impaired hearing, and Discharge right ear with mass in the right mastoid region for the last one year. Main complaint of the patient was decreased hearing and found to be conductive hearing loss. Computed tomography showed diffusely expanded right temporal bone/mastoid, petro us parts and EAC with increased bone density. Patient underwent surgery for contouring of bone and canalplasty which improved hearing status, blocking sensation and discharge completely. Although there was cosmetic deformity but patient was not concerned regarding that, which also improved. Patient is under regular follow-up with very good functional and cosmetic results. From his case report, we learn the importance of conservative surgery and regular follow up in few selective cases of temporal bone fibrous dysplasia.

Fibrous dysplasia originating from the middle ear Orta kulak orijinli fibröz displazi

2013

Fibrous Dysplasia is rarely seen in adults. The anterior craniofacial bones are more commonly involved than more lateral or posterior regions. Loss of vestibular function, tinnitus and hearing loss may be seen associated with sphenoid and temporal bone involvement. We aimed to report the case of a thirty-year-old female patient with fibrous dysplasia located in the middle ear.

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 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.