The exact aetiology of congenital cholesteatoma, the less common form of this destructive disease, is still under debate.

A two-year-old boy was referred to paediatric otolaryngology with persistent, bloody, left-sided otorrhoea refractory to oral and ototopical antibiotics. Prior to its onset at age 16 months, all ear examinations on the affected side were normal. Physical examination, imaging with computed tomography and eventual tympanomastoidectomy revealed extensive cholesteatoma. The extent of the disease, age at onset of symptoms and absence of otological disease before initial presentation suggested the diagnosis of congenital cholesteatoma. Review of the family history revealed that the patient's older brother had undergone tympanomastoidectomy for a small, well-encapsulated, mesotympanic congenital cholesteatoma at two years of age.

This case joins a single, previous report describing congenital cholesteatoma in multiple family members, suggesting that in some cases, hereditary factors may play a role in the formation of the disease.

This study aimed (1) to report the long-term effects of infliximab, a murine monoclonal antibody directed against tumour necrosis factor-α, on autoimmune inner ear disease, and (2) to discuss dilemmas surrounding the long-term management of autoimmune inner ear disease.

A 49-year-old man presented with sudden-onset, left-sided, sensorineural hearing loss, tinnitus and vertigo. He was prescribed oral prednisolone, with benefit. Over several subsequent months, he experienced frequent relapses and progressive deterioration of high-frequency hearing bilaterally. Multiple agents failed to stabilise his condition. Following infliximab treatment, there was a documented and sustained improvement in his hearing and tinnitus. He stopped the treatment after 46 weeks, with rapid relapse of his condition. His hearing recovered quickly again after recommencing infliximab.

The benefits of prolonged infliximab use and potential side effects must be balanced against allowing the disease to take its course, with progressive deafness. Randomised controlled trials are required to assess infliximab's optimal duration of use, long-term efficacy and safety in the treatment of autoimmune inner ear disease.

It is rare for isolated sphenoid sinusitis to cause orbital cellulitis. We present a rare case of posterior orbital cellulitis, so caused, together with a review of the relevant literature.

A 29-year-old woman presented with a 6-week history of progressive, unilateral, retro-orbital and periorbital right eye pain. On examination, the only finding was reduced visual acuity in the right eye. A computed tomography scan demonstrated right frontal and sphenoid sinus opacification. Sphenoidotomy and frontal sinus trephination were subsequently performed, following failure to respond to intravenous antibiotics. After surgery, the patient's vision returned to normal.

Isolated sphenoid sinusitis is rare but can cause significant visual disturbance and permanent loss of vision. Vague symptoms unsupported by clinical signs at presentation are a feature of posterior orbital cellulitis. The presented case highlights the problem, and the need for a high index of clinical suspicion even in the absence of firm clinical signs, in order to prevent permanent visual loss.

Craniofacial fibrous dysplasia commonly affects the larger mandible and the maxillary bones. Although involvement of the frontal, temporal and sphenoid bones has been described, fibrous dysplasia of the nasal bone has not been previously described; the two cases reported here have been made rarer by their isolated involvement. Clinical management is dependent on disease activity and patient symptoms.

To present two cases of isolated fibrous dysplasia of the nasal bone: a 46-year-old woman with gradual widening of the nasal bridge and a 47-year-old man with an incidental finding of a nasal bone mass.

Two case reports.

The 46-year-old woman underwent excision of the lesion while the 47-year-old man opted for watchful waiting.

We have presented the first case reports of fibrous dysplasia of the nasal bone. The care of these patients should be customised to their needs and wishes.