Approximately 3% of the patients with FD have the so-called McCune-Albright syndrome, in which bone involvement is followed by skin lesions and endocrine pathologies.  Virtually any bone in the body can be affected. It is a nonhereditary disorder of unknown cause [2]

Of interest to Otorhinolaryngology is the fact that fibrous dysplasia may involve craniofacial bones, causing deformities and dysfunctions, such as hearing loss, retroauricular bulging, and complications (cholesteatoma and facial nerve damage (Papadakis CE et al, Ear Nose Throat J 2000; 79(1):52-7).

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.[3]

Within the temporal bone, involvement of the auditory canal is the most common manifestation of fibrous dysplasia, seen in approximately 8% of patients leading to a conductive hearing loss.  Middle ear involvement is uncommon and results from secondary cholesteatoma from long standing external canal stenosis. Fibrous dysplasia may involve the otic capsule, cochlea and labyrinth. Involvement of the inner ear may also encroach on the intratemporal facial nerve and facial nerve palsy may rarely be seen (Megerian CA et al 2005), and is reportedly seen in 10% of FD of temporal bone.[1]

Malignant transformation has not been reported for temporal bone fibrous dysplasia, but it has already been reported in other disease sites, with 2/3 of these transforming into osteosarcoma.

CT Scan may show the abnormality and Serum chemistry may show deranged Serum calcium, phosphorous and alkaline phosphatase, but these are not pathognomic, and it is often difficult to make a diagnosis of FD of temporal bone on isolated radiological or clinical data. Histology of excision biopsy of bone specimen may be needed.

Important differential diagnoses of temporal bone fibrous dysplasia include Paget’s disease, hyperparathyroidism, local reaction to meningioma, osteoma, eosinophilic granuloma, steochondroma, and sarcomatous neoplasm.

Not all cases of FD of temporal bone requires treatment, largely because there are no effective conservative treatment to control FD. Watchful waiting and follow up is all that is needed in FD with little or no symptoms

Surgery is indicated in FD of Temporal bone with significant symptomatology, especially associated with bone invasion of external auditory canal (enough to produce conductive hearing loss), recurrent infections and secondary cholesteatoma in the external auditory canal. The 3 main goals of surgery include restoration of the function, prevention of complications, and esthetical improvement. Radiotherapy is highly discouraged because of the risk of malignant transformation.

Further Readings

1. Vanier S. Júnior1, Eduardo C. Andrade1, Ana L. S. Didoni1, José C. Jorge2, Nelson S. Filho3, Fabiana R. Yoshimoto3.Fibrous dysplasia of the temporal bone: case report and review of the literature. Rev Bras Otorrinolaringol.V.70, n.6, 828-31, nov./dec. 2004
2. Magu S, Mishra DS, Sood AK, Sharma N. Fibrous dysplasia of the temporal bone. Neurol India [serial online] 2002 [cited 2009 Dec 24];50:374. Available from: http://www.neurologyindia.com/.....3/374/1417
3. Fibrous Dysplasia - eMedicine - http://emedicine.medscape.com/.....4-overview

How To Register for This Event?
Click http://www.lion-web.org/pages/.....ation.html to register now and find your nearest videoconference room .

How to watch the LION event ?
    There are multiple possibilities to watch the life surgery.
Click http://www.lion-web.org/images.....rogram.pdf for more info.

The LION 2009 Broadcast can be viewed on LION Channels 1 or 2.
    
The LION 2009 upgraded configuration will give delegates the chance to choose between two channels of real-time simultaneous live surgery, depending on their otological interests. This choice will also be available for the live-streaming through the LION web site. Swiching between the 2 video channels is easy.
Click http://newsletter.mp6.ch/url-5.....53651.html for more info.

How to ask questions to the surgeons during the event ?
    - If you are watching LION 2009 from a videoconference room you can ask questions when the moderators contact your center (CODECs only) 

- Through the internet you can send your question to the moderators by e-mail lion2009@lion-web.org

http://www.youtube.com/watch?v..... 

Feel free to view and post your comments.

If you have ideas how as to how best s-hear! ORL could be better organized, feel free to email me at biodun@nanootology.org

One option we are working on is getting teaching videos online via s-hear! ORL - something the ENTForum, and ENTinAfrica Blog cannot presently handle. The idea is for you to package your knowhow onto powerpoint, flash, or any other movie type (or simply record it on your cellphone camera) then get it onto YouTube or any online video hosting sites, and let us have the embedded code. You should be able to post it online yourself using s-hear! ORL once we are done with this subweb

s-hear! ORL, what you s-hear! is what you are!
Biodun

a 31 year old female was referred from a secondary care having had initial per nasal biopsy of right nasal mass (histology not specific) 2 weeks previously.
The history was nasal mass of two months duration and associated with bllod stained rhinorrhoea. The picture depicts the appearance at initial examination

What is the diagnosis?

Comments: In the abscence of history of cocaine abuse, the possible differential diagnosis of mid facial necrotizing lesion (http://www.ajnr.org/cgi/conten.....l/21/2/331) as seen in this patient include Wegener's granulomatosis, Stewart's syndrome, Invasive fungal rhinosinusitis, and of course sino-nasal tumors.
This lady had CT- sinuses that revealed soft tissue mass filling up right nasal cavity with extension to the ethmoids and sub-cutaneous space anterior to right maxillary sinus, but no direct orbital, intracranial or maxillary sinus involvement. 
She subsequently had lateral rhinotomy with excision of the mass.

Histology report was poorly differentiated adenocarcinoma. Fungal studies of the speciment was negative. She died while being prepared for post surgical chemo-radiation.
Biodun

• The first report reported the occurence of spontaneous otogenic pneumocephalus in a 54 year old female and could be viewed athttp://content.nejm.org/cgi/co.....?query=TOC.
• The second report by Mason & Nichols from Nashville reported acyclovir-induced crystalluria in an 80-year old HIV positive female. Acyclovir is commonly prescribed in juvenile laryngeal papillomatosis, and it is pertinent clinicians should view this article which is available at http://content.nejm.org/cgi/co.....?query=TOC.

MODERATOR

(Case Presentation by Dr Aminu Bakari, NECC, Kaduna)

Solution: Anthrochoanal polyp.

Diagnosis: Hemangioma involving the auricle and mastoid bone.

Hemangiomas are benign vascular proliferations that arise from capillaries, arterioles, or venules, and in ORL can be found in the external ear, tympanic membrane, middle ear, IAC, and the geniculate ganglion of the facial nerve. 
Unlike the cases reported by Kemink JL et (1983) and Jackson, Levine & McKennan (1987), the EAC and middle ear of this case were essentially free of hemangiomas.
It is remarkable however, that all types of hemangioma have been reported with respect to the auricle - including capillary type (Korotkikh NG et al, 2007), and the cavernous type. 
Management options include microembolization. excision, excision with arterial ligation, radiotherapy, and doing nothing, the latter may be preffered in extensive hemangioma where excision may likely produce more grotesque deformity.

In this index case, we requested for Cranial CT (we suspected some intracranial communication of the mastoid component) and also requested plastic surgeon and neurosurgical review. 
This case is still being followed up

Contrast dacryocystography revealed accumulation of contrast in the lacrimal sac with no visualization of the nasolacrimal duct.

Diagnosis:Nasolacrimal duct obstruction. (NLDO)
Pediatric NLD obstruction could be due to puctal, canalicular, sac, ductal lesions, especially mucocoeles, less commonly canalicilitis or even dacroliths.(Park, Morgestern, Kahwash & Foster, 2004; Barnes, Abou-Rayyah & Rose 2001). It can aslo be due to intranasal lesions, cystic or solids. Congenital nasolacrimal duct obstruction affects more than 6% of newborns (Lala-Gitteau, Majzoub & Pisella, 2007).
In children 6 to <36 months old, probing is a successful primary treatment of NLDO in about three fourths of cases, with no decline in treatment success with increasing age (PEDIG, Tepka, Chandler, et al, 2008). Endoscopic or external DCR is reserved for cases not helped by lacrimal probing.
This patient's epiphora resolved with nasolacrimal probing.

Query: Which are the commonest lesions associated with cartilage erosion of laryngotrachea?

Solution: Common lesions associated with cartilage erosion of laryngotrachea include
1. Inflammatory - Granulomatous infections (TB, Leprosy, Sarcoidosis, Syphilis), Wegener's granulomatosis, Radiation chondronecrosis, relapsing polychondritis, rhemumatoid arthritis, and foreign body reactions.
2. Neoplastic conditions like squamous cell carcinoma of larynx, throid carcinoma, laryngeal chondrosarcomas and lymph node secondaries