Adult presentation of the Dandy-Walker syndrome is extremely rare. We report a case where the patient was first diagnosed at 75 years of age because of sudden onset of unilateral senorineural deafness and episodic vertigo. He remained well for a further three years until developing the more classical adult presenting features of cognitive impairment, disordered gait and ataxia. The radiological and otological investigative findings are discussed, together with their implications.

Bilateral simultaneous facial nerve palsy is an extremely rare clinical entity with Bell’s palsy responsible for a mere 20 per cent of cases. It is, therefore, important that clinicians are aware of the differential diagnosis when evaluating a case.

An anatomical study of 50 fresh adult human cadavers was performed in order to verify prevalence of Galen’s anastomosis (GA) and to evaluate whether factors such as gender, ethnicity, side of the neck, and individual stature may interfere with GA prevalence. The results were analysed using the Chi-square test, Student t-test, and F-Snedecor test. GA was observed in 87 of 100 dissections. There was no statistically significant difference regarding GA prevalence between groups separated by ethnicity (p = 0.853), gender (p = 0.198), side of the neck (p = 0.766), or individual height (p = 0.199). Therefore, the GA was a frequent anatomical finding, and this result was not influenced by any studied factor. Comparing our data with previous studies, we also concluded that the GA seems to play an important role in the innervation of the larynx, even though its function remains unclear. Also, it is reasonable to consider GA a constant anatomical constituent.

Group A streptococcal meningitis is rarely encountered today, although group A streptococcal severe infections are on the increase. We present here a case of an adult male with bacterial meningitis as a complication of otitis media induced by Group A Streptococcus. The approach to diagnosis and treatment considerations are discussed.

Pathological lesions confined to the internal auditory canal (IAC) commonly present with cochleovestibular symptoms; sensorineural hearing loss, tinnitus and balance disturbance. The commonest lesion of the IAC is vestibular schwannoma. Other lesions include meningioma, facial neuroma, cavernous haemangioma, lipoma and arachnoid cyst. Presentation with facial palsy and an intracanalicular lesion is suggestive of pathology other than acoustic neuroma. Magnetic resonance imaging (MRI) cannot reliably distinguish intracanalicular vestibular schwannomas from meningiomas. Particular care is required for surgery of these lesions: the facial nerve typically does not lie in a protected anterior position within the IAC.

A case of a female child of six years of age with a plexiform neurofibroma of the larynx caused by von Recklinghausen’s disease is presented. Laryngeal involvement in neurofibromatosis type 1 (NF1) is rare and only 19 paediatric cases have been reported. The tumour was biopsied and lateral pharyngotomy with supraglottic hemilaryngectomy was performed in order to relieve obstructive symptoms. Problems related to this unusual tumour localization are discussed and a review of the literature is presented.

We present the first case of a middle cerebral artery aneurysm presenting as isolated hyperacusis. The patient had a Guglielmi detachable coil (GDC) embolization of his aneurysm with complete resolution of his symptoms. The pathophysiological mechanism is discussed. We suggest further radiological investigation in young patients presenting with this symptom.

Necrotizing sialometaplasia is an uncommon, benign, self-limiting condition which can stimulate malignancy. The commonest site of occurrence is the hard palate. We report the first case with full thickness palatal involvement. The clino-pathological features of this condition are discussed.

The results of long-term follow-up after surgical treatment of two patients with intractable benign paroxysmal positional vertigo are reported. Argon laser irradiation of the blue-lined posterior and lateral semicircular canals in one patient, and of only the posterior canal in the other was performed seven and six years ago, respectively. Argon laser irradiation was carried out 10 times in succession three mm along the canal to occlude it. The power applied each time was 1.5.W on the dial of the laser device for 0.5.sec. Relief of vertigo was noted on the second post-irradiation day. There has been no recurrence of vertigo in these patients.

Pharyngo-laryngo-oesophagectomy and gastric pull-up (PLOGP) is a complex and relatively uncommon procedure. The aim of this study is to analyse the results of PLOGP in patients with post-cricoid and cervical oesophageal squamous cell carcinomas.

This study was a retrospective review of 26 patients (11 males + 15 females, mean age 63.5 years) who underwent PLOGP from 1988 to 1997. Eighteen (69 per cent) patients were staged as T3 and eight (31 per cent) T4. Eighteen (69 per cent) patients had N0, seven (27 per cent) N1 and one (four per cent) N2 disease. Multiple primary tumours were recorded in three (11.5 per cent) patients. Four (15 per cent) patients had pre-operative radiotherapy with poor response and two (eight per cent) required emergency tracheotomy prior to surgery. Feeding jejunostomy was performed on 19 (73 per cent) and neck lymph node dissection in eight (31 per cent) patients.

The mean duration of surgery was five hours (range 3.5 to 7.5) with a mean blood loss of 840 ml (range 160 to 1800), a mean stay in ICU of 4.2 days and hospital stay ranged from nine to 84 days (mean 34). Three (11.5 per cent) patients died (pneumonia – one, congestive heart failure – one pulmonary embolus – one) in the early post-operative period. Eight (31 per cent) patients remain alive from 30 to 136 months (mean 58 months). Two (eight per cent) patients died with no evidence of disease. Thirteen (50 per cent) patients died of their disease between two to 51 months (mean 17.3 months) post-operatively. Kaplan-Meier survival estimates for one year was 65 per cent, for three years 35 per cent and for five years 26 per cent (see Figure 1). Median survival in the whole series was 18 months.

Post-operative speech was with an electrolarynx in 16 (62 per cent). One patient (four per cent) used gastric speech and one patient (four per cent) used a Blom-Singer valve effectively. Five (19 per cent) patients had no speech post-operatively. All patients maintained oral feeding. Gastric transposition constitutes a safe and reliable method of restoring the continuity of the upper digestive tract following pharyngo-laryngo-oesophagectomy.

Abstract Traumatic fracture of the stapes occurs rarely following head injury. Ossiculardislocation is more commonly encountered. When present, stapes fractures are usually associated with an underlying temporal bone fracture. A higher incidence has been reported in childhood, possibly because of the greater flexibility of the skull in this age group. This report highlights the fact that these fractures can be associated with a relatively minor head injury. This possibility should be kept in mind when evaluating patients, especially children, who have a persistent conductive deafness of more than 30 dB with an intact tympanic membrane following any form of head injury. An exploratory tympanotomy with appropriate ossicular reconstruction, as described in this case, can yield excellent results.

A 71-year-old lady underwent successful excision of a haemangioma of the frontal sinus, via an osteoplastic flap approach. Haemangioma of the paranasal sinuses is an extreme rarity. A case is presented and the literature reviewed.

It is common to see cholesterol granuloma in the mastoid air cells, less common in the orbit, and uncommon in the paranasal sinuses. Cholesterol granuloma is thought to be due to an interruption to normal aeration with impaired lymphatic drainage, resulting in a closed cavity where it may form. These expanding cysts cause bone destruction and compression of the surrounding structures that lead to clinical symptoms. Diagnosis and management of cholesterol granuloma cysts can be challenging. Magnetic resonance imaging (MRI) and computed tomographic (CT) scans are usually diagnostic. We present a rare case of cholesterol granuloma in the frontal sinus, few cases have been reported in the literature.

Familiarity with four types of free tissues transfers allows appropriate reconstruction of most defects in the head and neck region functionally and aesthetically. These include jejunal graft, radial forearm, rectus abdominus myocutaneous and fibula osteocutaneous flaps. Free colonic and gastric patches were used occasionally.

We report our experience of 215 free tissue transfers for reconstruction of defects in the head and neck region after tumour extirpation.

The overall success rate was 94 per cent. The commonest cause of failure was related to arterial inflow (70 per cent). Three patients died in hospital, but their mortality was not related to the tissue transfers. Complications due to free tissue transfer at both the donor and recipient sites were few and manageable.

The co-operation between the two surgical teams, together with the timely application of suitable salvage procedures, contribute to an optimal outcome.

Nasal septal abscess complicating acute sinusitis is rare. There have been very few reports in the literature of this condition. We present a 12-year old-male with a nasal septal abscess complicating acute pansinusitis.

We present two subjects with previously undiagnosed acoustic neuromas who complained of vertigo whenever they ran. One had normal hearing while the other already had a unilateral sensorineural deafness. Hyperventilation for 30 seconds provoked an ipsilateral beating nystagmus and reproduced the vertiginous sensation in both subjects. Hyperventilation is a simple bedside test that should be performed when assessing a subject with vertigo or when there is a clinical suspicion of an acoustic neuroma.

This is a case of a 35-year-old who had complained of noisy breathing for 15 years, and had been on treatment for ‘chronic asthma’. She presented to the Accident and Emergency department with an acute episode of difficulty with breathing, and on admission was found to have stridor and bilateral abductor vocal fold palsy. Further workup revealed a syringomyelia with an associated Chiari type 1 malformation. It is important to consider the above diagnosis in the differential of young adults with breathing difficulties. A discussion of syringomyelia, Chiai malformations and bilateral vocal fold palsy follows.

Co-existent comorbidity is a major determinant of treatment outcome in head and neck cancer. Most of the work pertaining to this topic has been done in the United States, where the standard practice is for trained cancer registrars to grade comorbidity using validated indexes by retrospective notes review. The adult comorbidity evaluation – 27 index (ACE-27) is a validated instrument that has been widely used in head and neck cancer. Although the required clinical data may be available in the notes, a significant amount of historical information is required to grade comorbidity. The aim of this study was to assess the accuracy and inter-rater reliability of the retrospective notes review process, in a typical setting in the United Kingdom (UK), by comparing the information obtained on notes review alone by a physician to that available after a structured patient interview. The study concludes that the retrospective notes review is an accurate and reliable technique for grading comorbidity whose completeness can be improved by the use of patient questionnaire as part of a structured interview.

Second primary tumours occur frequently in patients with a history of head and neck malignancies. Delays in making an early and correct diagnosis can seriously affect the therapy management and survival.

This was a retrospective study of 120 patients with a history of head and neck cancer, presenting with a second primary tumour. Current follow-up strategies and the use of routine sonographic imaging of the head and neck regions were evaluated, and the impact that tumour chronology, the tumour site and the various treatment modalities have on the survival were assessed. Forty-two per cent of patients developed a metachronous second malignancy more than five years after diagnosis of the index tumour. The accuracy of colour-duplex sonography in detection of second primaries in the head and neck was 82.3 per cent. First and second primary tumours located in the larynx were observed to have the highest five-year survival rate. Patients who developed metachronous tumours had a five-year survival rate of 68.9 per cent for the index tumours, and a 26 per cent five-year survival rate with the occurrence of a second neoplasm. With synchronous tumours a mean survival time of 18 months and a five-year survival rate of 11.9 per cent was found (p < 0.0001). Where clinically appropriate an aggressive treatment strategy was employed and yielded the most favourable results with a five-year survival rate of 66.8 per cent and 35.9 per cent for index tumours and second primary malignancies, respectively.

Since more than 40 per cent of the metachronous second primaries in patients with a history of head and neck malignancy occur beyond the five-year follow-up period, an extended protocol with individually adjusted close monitoring of high-risk patients seems appropriate. Colour-duplex sonography is a valuable screening investigation for the early detection of second primary tumours. The treatment of a second primary is often less successful than for the same malignancy occurring primarily. The prognosis of synchronous tumours is significantly lower when compared to malignancies of a metachronous nature, despite some encouraging individual results. Only the early implementation of aggressive treatment methods for second primaries is successful in terms of survival.

Most of the malignancies arising from the upper respiratory system are squamous cell carcinomas (SCC). Basaloid squamous cell carcinoma (BSCC) is a rarely seen high grade variant of SCC that carries a poor prognosis. In this article, we present a case of BSCC of the larynx. In addition, we provide a review of the relevant literature along with a discussion of the differential diagnosis.