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Year : 2002  |  Volume : 50  |  Issue : 3  |  Page : 374-5

Fibrous dysplasia of the temporal bone.






How to cite this article:
Magu S, Mishra D S, Sood A K, Sharma N. Fibrous dysplasia of the temporal bone. Neurol India 2002;50:374


How to cite this URL:
Magu S, Mishra D S, Sood A K, Sharma N. Fibrous dysplasia of the temporal bone. Neurol India [serial online] 2002 [cited 2023 Feb 5];50:374. Available from: https://www.neurologyindia.com/text.asp?2002/50/3/374/1417



We would like to report a case of fibrous dysplasia of the temporal bone in a 15 year female, who presented with occipital headache for four months. On examination there was deviation of the angle of the mouth towards the right side and partial closure of the left eye while laughing. There was no impaired hearing. There was lower motor neurone palsy of the 7th cranial nerve on the left side with downgoing planters. Plain radiographs of the skull showed ground glass appearance of the temporal bone on the left side (Fig. 1,2). Axial CT showed extensive involvement of the left temporal bone and mastoids by pagetoid fibrous dysplasia producing classic ground glass appearance. There is also extension of the process into the occipital bone, sphenoid bone and posterior cranial fossa.
Fibrous dysplasia of bone is characterized by the presence of fibroosseous tissue in the interior of the affected part or parts of the bone and compress 2.5% of all osseous neoplasias and 7% of beningn tumors and occurs more frequent in female patients 2:1.[1] Lichtenostein[2] in 1938 coined the term fibrous dysplasia to describe a disorder characterized by the progressive replacement of normal bone elements by fibrous tissue. The disease can involve any bone in the body. In the head and neck, the skull and facial bones are involved in 10-25% of cases of monostotic fibrous dysplasia and in 50% of the polyostotic variety. Involvement of the temporal bone is relatively rare and only 53 cases have been reported.[3] Fries[4] described three patterns i.e. the pagetoid, sclerotic and cystic. The pagetoid pattern is the commonest and shows both sclerotic and radiolucent areas. Sclerotic lesions are homogenously dense whereas the cystic variety is characterized by a spherical and oval lucency surrounded by a dense bony shell. CT is the best way to display the bony changes while MR is useful in cystic fibrous dysplasia.[3]
Involvement of the auditory canal is the most common manifestation of fibrous dysplasia of the temporal bone seen in approximately 8% of patinets leading to a conductive hearing loss.[5] Middle ear involvement is uncommon and results from secondary cholesteatoma from long standing external canal stenosis.
Fibrous dysplasia may involve the oltic capsule, cochlea and labyrinth.[5] Involvement of the inner ear may also encroach on the intratemporal facial nerve and facial nerve palsy may rarely be seen as seen in our cases. Patients suffering from fibrous dysplasia should be told of their problem and should be seen every three months for an indeterminate period of time, regarless of their age group.[1]


 

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1.Barrionuevo CE, Marcallo FA, Coelho A et al : Fibrous dysplasia and the temporal bone. Arch Otolaryngol 1980; 106: 298-301.   Back to cited text no. 1    
2.Lichthstein L : Polyostotic fibrous dyaplasia. Arch Surg 1938; 36: 874-898.   Back to cited text no. 2    
3.Brown EW, Megerian CA, Mckenna MJ et al : Fibrous dysplasia of the temporal bone : Imaging findings. AJR 1995; 164: 679-682.   Back to cited text no. 3    
4.Fries JW : The roentgen features of fibrous dysplasia of the skull and facial bones : a critical analysis of thirty-nine pathological proven cases. AJR 1957; 77: 71-88.   Back to cited text no. 4    
5.Megerian CA, Sofferman RA, Mckenna MJ et al : Fibrous dysplasia of the temporal bone : ten new cases demonstrating the spectrum of otologic sequelae. Am J Otol 1995; 16: 408-419.   Back to cited text no. 5    

 

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