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FIBROUS DYSPLASIA OF THE TEMPORAL BONE Assist. Prof. Dr. Mihaela Cristiana Tatu,MD, PhD, ENT Specialist , Dr. Oana Irina Popa, MD, ENT Resident Central University Military Emergency Hospital Bucharest, ENT Clinic Introduction Fibrous dysplasia is a rare disease which remains unobserved for a long time - discovered incidentally when bone fracture or neurological symptoms due to compression appear. Fibrous dysplasia is a benign, localized, clearly demarcated, slowly progressive bone tissue disease, characterized by the replacement of normal bone with an amorphous mass dominated by fusiforme cells and bone trabeculae with variable size and random, disorganized distribution. Clinical examination shows asymmetry, pain and includes neurological examination (compression syndromes), neurosurgical, endocrinological and ENT examination. Investigations: biological (K, Ca, serum alkaline phosphatase levels growth); imaging: Rx, CT, MRI, bone scintigraphy; bone biopsy. Postoperative imaging: On clinical examination: marked hypertrophy of the right temporal bone that determines cranio-facial asymmetry; on the right external auditory meatus - antero-superior, spherical, sessile bone protrusion, completely blocking the EAC, covered with skin of normal appearance; mixed moderate right hearing loss; right purulent, fetid discharge; mastoid tenderness; left ear normal clinically and functional. Surgical intervention: recalibration of right EAC with biopsy from the bone protrusion. mastoidectomy with removal of the secondary cholesteatoma (caused by EAC obstruction). The bone biopsy showed fibrous displasya of the right temporal bone. Head CT scan Case Presentation We present the case of a young female patient, 32 years of age, with right temporal lobe compression. The patient presented craniofacial asymmetry marked by bone hypertrophy on the right side; right mixed moderate hearing loss; presence of a right EAC bone obstruction, purulent fetid otorrhea, right ear pain. Hystory: at age 9 - right acute otitis media, difficult examination - "Clinical onset" of the disease; age 11 - Clinical and radiological diagnosis right EAC osteoma; surgery for otomastoid osteoma- biopsy: osteoma; age 28 – head CT scan "right temporal giant osteoma" (patient refused any surgery). Presumptive diagnosis: fibrous dysplasia of the right temporal bone complicated by induced otomastoid colesteatoma. Differential diagnosis was made with nonosteogenic fibroma; giant cell bone tumor; bone aneurismal cyst; bone adamantinoma; eosinophilic granuloma; plasmacytic myeloma. Investigations: hormone titers in the normal range; profile skull X-ray to "sella turcica" normal size; without major changes of calcemia, magnesemia and alkaline phosphatase; head CT scan; bone scintigraphy; neurological exam: control EEG, complete cranial nerves exam; IQ testing; neurosurgical exam - forming a mixed ENTneurosurgical team. Postoperatory it is important to survey the patient because the disease is evolving and progressing and complications (even life threatening ) may appear: ● restenosis of the cavity after reconstruction, caused by development of concentric bone; ● induced cholesteatoma and its complications; ● progressing hearing loss ; ● stenosis of EAC with its repercussions; ● intracranial extension (ICH and compression threatening events); ● sarcomatous degeneration (0.5% of cases of temporal bone dysplasia) Head MRI Conclusions Surgery is essential for positive diagnosis and for complications treatment. FD is rarely diagnosed. The monosteotic form of the temporal bone is glade. References Gregg J, Reed A. Monostotic fibrous dysplasia in the temporal bone: a late prehistoric occurrence. Am J Phys Anthropol. 1980 Nager G. Fibrous dysplasia. In: Pathology of the Ear and Temporal Bone. Baltimore, Md: Williams & Wilkins; 1993 Lambert PR, Brackmann DE. Fibrous dysplasia of the temporal bone: the use of computerized tomography. Otolaryngol Head Neck Surg. 1984