Facial palsy is a rare presenting feature of cholesteatoma. Prompt treatment usually results in a good outcome, but if treatment is delayed the prognosis can be difficult to predict. We retrospectively reviewed 326 consecutive patients who had undergone temporal bone surgery for cholesteatoma. Eleven patients had presented with facial palsy, of whom eight had petrous apex involvement and three had disease confined to the middle-ear cleft. All patients with middle-ear disease were operated upon within two months of presentation, and all showed some recovery in facial function. The diagnosis had been delayed in all eight cases of apical disease, with four cases having had a long-standing total weakness. One case treated after seven months' partial weakness achieved a full recovery. In three cases of long-standing partial weakness, pre-operative facial function was preserved by maintaining the facial nerve in its normal anatomical location.

Facial palsy associated with cholesteatoma should be treated as early as possible. Recovery can occur even if treatment is delayed for up to seven months. After this time, recovery is increasingly unlikely, but any residual function can be maintained and further deterioration prevented.

We report the case of a 16-year-old boy who presented to us with acute otitis media, facial weakness and retro-orbital pain. Computed tomography and magnetic resonance imaging (MRI) scans of the head and temporal bone revealed otitis media with petrous apicitis. The patient responded to broad-spectrum, parenteral antibiotics, with disappearance of facial weakness and reduction in pain. One month following the completion of treatment, the patient continued to have dull retro-orbital pain and developed ear discharge. A repeat MRI of the temporal bone revealed a persistent inflammatory lesion in the petrous apex, with a nodular, ring-enhancing lesion in the cerebellum, strongly suggestive of tuberculosis. The ear discharge stained positive for acid-fast bacilli and the patient's serum enzyme-linked immunosorbent assay for tuberculosis was reactive. The patient responded well to anti-tubercular treatment and was disease free eight months following the completion of treatment.

A triad of retro-ocular pain, discharging ear and abducens nerve palsy, as described by Gradenigo, has been recognized for 150 years. It has traditionally been treated with surgery, but recent advances in imaging, allied with improved antibiotic treatment, allow conservative management of these cases. We present two cases of Gradenigo’s syndrome: a 6-year-old child and a 70-year-old man, both without cholesteatoma, who were managed without mastoidectomy. They both had full recovery of abducens nerve function, although this took 6 and 12 weeks, respectively. In order to manage patients with Gradenigo’s syndrome safely, accurate diagnostic radiology is essential, and our findings are presented and discussed. With changing medical technology, a review of the diagnostic and treatment options for this rare but serious condition, is timely.

Spontaneous cerebrospinal fluid (CSF) leak via the oval window is uncommon and can result in recurrent bacterial meningitis. Current understanding of spontaneous CSF otorrhoea is reviewed and a diagnostic algorithm is presented.

A seven-year-old boy presented with bilateral congenital deafness and recurrent meningitis. High-resolution computed tomography (HRCT) of the temporal bone showed a labyrinthine deformity and communication between the internal auditory canal (IAC) and the cochlea. Subtotal petrosectomy with closure of the external acoustic meatus and eustachian tube was performed. Post-operatively, the child had no further episodes of meningitis.

This rare and obscure cause of recurrent childhood meningitis requires a high index of suspicion and the use of diagnostic tools, especially HRCT.

We present a case of a papillary tumour of the petrous bone. The established terminology for this rare neoplasm is endolymphatic sac tumour (ELST) but the true origin remains controversial. ELSTs are associated with von Hippel-Lindau disease. They are locally invasive, highly vascular and often require endovascular embolization prior to surgery. Both radiologically and histologically ELSTs are easily mistaken for other more common tumours such as paragangliomas and renal or papillary thyroid carcinoma metastases. This is important because local excision is curative.

Petrosal cholesteatomas are rare lesions, which may be congenital or acquired in nature. We report an exceptional case occurring in a seven-year old girl who presented with a unilateral conductive hearing loss, despite normal tympanic membrane appearance. Early diagnosis was facilitated by computed tomography (CT) scanning. Although this case satisfied the criteria for congenital cholesteatoma, it is likely that the petrous apex was secondarily involved. Complete cholesteatoma removal was accomplished using a transtemporal supralabyrinthine approach, which allowed for hearing preservation, while avoiding the morbidity associated with a craniotomy. The present case constitutes the youngest case of petrosal cholesteatoma reported. We suggest that a lower threshold for the use of CT scanning in unilateral conductive hearing loss may allow for the earlier detection of more cases of petrosal cholesteatomas, as well as facilitating their removal using more limited approaches associated with less morbidity.

Petrous apicitis as a potentially fatal complication of suppurative otitis media presents in a variety of forms. Gradenigo’s triad of abducens paralysis, deep facial pain due to trigeminal involvement and acute suppurative otitis media rarely occurs. The conflicting symptoms reported in the literature usually result in the delayed recognition of the condition with potentially disastrous consequences. The VIth nerve palsy is considered to be the least reliable sign as it is least often present. We present a case in which it was the sole presenting symptom.

This is a case report of a patient with bilateral cholesterol granuloma of the petrous apex, who presented with unilateral symptoms. Initially suspected as having a dermoid cyst, he underwent posterior fossa exploration, drainage and biopsy. Symptoms recurred one year later and subcochlear drainage of the petrous apex cyst was successfully performed. Follow-up for over 18 months shows no clinical nor imaging signs of recurrence.

A MEDLINE literature search was carried out and relevant paper publications reviewed. Case presentation including initial, pre- and post-operative imaging is presented. This is followed by discussion of current concepts on the presentation and management of large cholesterol cysts of the petrous apex.

Congenital cholesteatoma may originate at various sites in the temporal bone. For example, in the petrous apex, the cerebellopontine angle, the middle ear cavity, the mastoid process or the external auditory canal. The least common site being the mastoid process. We present two cases of congenital cholesteatoma of the mastoid process, each presenting with different symptoms and at different ages. Both patients underwent surgical treatment, which confirmed the diagnosis and radiological findings.

A case of chrondrosarcoma of the petrous temporal bone is presented. Chondrosarcomas rarely occur intracranially and typically present as a petrous apex mass. The dilemmas faced in the diagnosis and treatment of petrous apex chondrosarcomas are discussed. This case also gives interesting insight into the natural history of this tumour.

The cemento-ossifying fibroma (COF) is a mesodermal, non-odontogenic tumour of ectopic multipotential periodontal membrane blast cells. It is aggressive, locally destructive, and has a high recurrence rate. A case report of COF of the petromastoid region is presented. This location has not been described until now. Trauma may act as a trigger to sudden growth of the atopic periodontal tissue. Due to the aggressive behaviour of this tumour and its frequent recurrence radical surgery is needed.

Aneuroysm of the intrapectous carotis artery is an extremely rare and potentially serious occurence that presents diagnostic and therapeutic difficulties. Such aneurysms may follow trauma, atherosclerosis, mastoid surgery or most commonly can represent a developmental abnormality. We present the case of an 18-year-old female with a short history of recurrent left-sided otalifa and epistaxis who underwent successful endovascular ballon entrapment of a left intrapetrous carotid aneurysm.

Twenty-three patients with intradural lesions of the petroclival region and prepontine cistern were managed in our centres by the modified transcochlear approach. Total tumour removal was accomplished in 17 patients, while a second stage was planned for the remaining six patients. Two cases died in the immediate postoperative period. All the remaining cases showed a good outcome and returned to work. The basic approach type A is further classified according to its anterior, superior and inferior extension into types B, C and D respectively. The surgical procedure, classification, illustrative case reports and patients' summaries are presented.

Extended subtotal petrosectomy as a treatment for persistent cerebrospinal fluid (CSF) otorhinorrhoea is presented. Four patients were successfully operated on by this technique, all previously having undergone suboccipital removal of vestibular schwannoma: other interventions used had failed to seal the fistulae. The internal auditory canal was the usual pathway for CSF leakage as well as retrosigmoid, retrolabyrinthine, retro- or perifacial cells. Total exenteration of middle ear and mastoid cell tracts, skeletonization of sigmoid sinus, jugular bulb and facial nerve, drilling out of semicircular canals, vestibule, and cochlea, and skeletonization of the internal auditory canal, followed by obliteration, are the main steps of this approach.

This paper offers an account of the contemporary surgical approach to advanced tumours of the external ear based on a series of 11 patients. There were eight squamous, two basal cell carcinomas and one mucoepidermoid tumour. The traditional method of excision was slightly modified by performing microsurgical dissection of the lateral part of the temporal bone rather than chisel osteotomies, and then including it en bloc with the involved soft tissues. The defect was then closed using a scalp or myocutaneous flap and this combination of otological and reconstructive expertise has proved satisfactory. Four patients are alive with no evidence of disease a mean of 4.2 (range 1.0–7.0) years from surgery: two patients who remained free of disease have subsequently died of unrelated conditions 12 and 24 months post-operatively, and in three cases death from recurrent disease occurred a mean of 1.4 (range 0.9–2.1) years after our surgery. There were two postoperative deaths. Based on the actuarial survival of 36 per cent and a successful disease clearance rate of 54 per cent, our conclusion is that the outlook of this condition has not dramatically improved since the original descriptions of the management of this problem first appeared, although intervention remains justifiable because of the potential curability and relief of symptoms.