Larsen's syndrome is primarily known as an orthopaedic disease. However, it has been found to be associated with conductive hearing loss caused by ossicular malformation. The possibility of a sensorineural hearing impairment as an additional part of this syndrome has been rather neglected in the past.

Case report and literature review.

We present a teenage boy suffering from typical Larsen's syndrome. Despite no history of recurrent middle-ear infections, he showed a bilaterally symmetrical, mixed hearing loss dominated by an extensive sensorineural component. In order to review the corresponding literature, a Medline search was performed using the criteria ‘Larsen's syndrome’ and ‘hearing loss/deficit’.

The literature review showed the presented case to be the first sufficiently documented report describing a predominant sensorineural hearing loss in a Larsen's syndrome patient. Although a specific association of Larsen's syndrome with sensorineural hearing loss is still speculative, this case might give grounds for further examinations of this rare finding.

Ménière's disease is a diagnosis requiring expert clinical judgment. There are several differences between the American Academy of Otolaryngology Head and Neck Surgery diagnostic guidelines and Prosper Ménière's original description of the disease.

Six hundred and fifty patients attending a specialist balance clinic had their clinical profiles fully analysed according to each set of diagnostic criteria.

Application of the American Academy of Otolaryngology Head and Neck Surgery guidelines resulted in the diagnosis of three times more patients than did use of Ménière's diagnostic criteria. Treatment options for patients in both groups were similar. It is unlikely that the application of different diagnostic criteria would have an effect on the treatment of individual patients.

The American Academy of Otolaryngology Head and Neck Surgery guidelines are more sensitive and less specific in diagnosing Ménière's disease than Prosper Ménière's original description. Standardised criteria should be applied across published series, in order to make results accurate, comparable and useful in the long term. We would recommend the introduction of a stricter diagnostic category for definite Ménière's disease.

We postulated that epistaxis patients frequently have their nose packed in the accident and emergency department without any reasonable prior attempt at nasal assessment and nasal cautery.

Telephone survey of 104 accident and emergency departments.

A 100 per cent response rate was achieved. In all but one of the departments surveyed, first line management was carried out by accident and emergency doctors. Seventy-four per cent of accident and emergency doctors perform nasal cautery as first line management. Within accident and emergency departments, the availability and usage of head illumination, nasal speculums, topical anaesthesia and suction was grossly suboptimal. Only one-sixth of the accident and emergency doctors surveyed had received training in the management of epistaxis, whilst 81 per cent of accident and emergency departments would admit nasally packed patients under the ENT team.

Despite the majority of accident and emergency doctors claiming to attempt nasal cautery before packing, this is unlikely to be effective if performed without the correct equipment. Better training and equipment need to be provided to accident and emergency doctors in order to optimise the management of epistaxis patients. This could potentially reduce inappropriate admissions.

The influence of congenital cytomegalovirus infection on cochlear function has been well recognised; however, its impact on the vestibular system in infants has not been examined. The purpose of the present study was to evaluate vestibular function in a group of infants, using caloric stimulation tests and vestibular-evoked myogenic potential measurements.

Vestibular-evoked myogenic potentials and auditory brainstem responses were recorded and caloric stimulation was performed in 66 infants aged three months, comprising 40 healthy controls and 26 infants with congenital cytomegalovirus infection.

No reaction to caloric stimulation was elicited from 16 examined ears, no vestibular-evoked myogenic potentials were recorded from 12 ears, and profound sensorineural hearing loss was diagnosed in eight ears. Pathological results were observed predominantly in infants with symptoms of intrauterine congenital cytomegalovirus infection present at birth.

In infants with clinical symptoms of congenital CMV infection present at birth, abnormal vestibular test results occurred more frequently than abnormal auditory brainstem response results. Vestibular organs should be routinely examined in individuals with congenital cytomegalovirus infection.

The aim of the present study was to investigate the dose-related effectiveness of corticosteroids in the management of otitis media with effusion, by using the objective assessment techniques of tympanometry and cytokine measurement.

The eustachian tubes of 42 male rats were obstructed. Thirty-six subjects with effusion were randomly divided into three equal groups: controls; 0.5 mg/kg/day corticosteroid; and 1 mg/kg/day corticosteroid. Concentrations of interleukin-1β and tumour necrosis factor α in the middle-ear effusions were analysed using enzyme-linked immunosorbent assay.

An effusion occurred on the 14th post-operative day and was present on the 30th post-operative day in the control group. Otomicroscopic examination and tympanometric measurement showed an improvement in the intervention groups. There was a significant difference between both intervention groups and the control group for both interleukin-1β and tumour necrosis factor α concentrations (p < 0.01). However, there was no significant difference between the 0.5 mg/kg and the 1 mg/kg corticosteroid groups (p > 0.05).

The study confirmed a short term beneficial effect of systemic steroids on resolution of otitis media with effusion in a rat model. Our results support the theory that corticosteroids significantly decrease concentrations of both interleukin-1β and tumour necrosis factor α. In our model, the 0.5 mg/kg corticosteroid dose was as effective as the 1 mg/kg corticosteroid dose. Further studies should be carried out to determine the optimal dose and duration of corticosteroids in the treatment of otitis media with effusion.

The aim of this study was to investigate the frequency of posterior semicircular canal benign paroxysmal positional vertigo in each ear, and to assess the association between the ear affected by benign paroxysmal positional vertigo and the head-lying side during sleep onset. Based on a previous study which used objective methods to prove the preference of the elderly for the right head-lying side during sleep, we hypothesised that a predominance of the same head-lying side in benign paroxysmal positional vertigo patients may affect the pathophysiology of otoconia displacement.

We conducted a prospective study of out-patients with posterior semicircular canal benign paroxysmal positional vertigo, confirmed by a positive Dix–Hallpike test.

One hundred and forty-two patients with posterior semicircular canal benign paroxysmal positional vertigo were interviewed about their past medical history, focusing on factors predisposing to benign paroxysmal positional vertigo. All patients included in the study were able to define a predominant, favourite head-lying side, right or left, during sleep onset.

The Dix–Hallpike test was found to be positive on the right side in 82 patients and positive on the left side in 54; six patients were found to be positive bilaterally. During sleep onset, 97 patients habitually laid their head on the right side and the remaining 45 laid their head on the left. The association between the affected ear and the head-lying side during sleep onset was statistically significant (p < 0.001).

Our study found a predominance of right-sided benign paroxysmal positional vertigo, a subjective preference amongst patients for a right head-lying position during sleep onset, and an association between the ear affected by benign paroxysmal positional vertigo and the preferred head-lying side during sleep onset. The clinical and therapeutical implications of this observation are discussed.

To compare hearing results in patients undergoing myringostapediopexy or myringolenticulopexy in canal wall down mastoidectomy.

Case series of one surgeon. A retrospective review of 83 consecutive mastoid cavity operations for primary cholesteatoma. Only those patients who had undergone either myringostapediopexy or myringolenticulopexy were included.

District general hospital.

Forty-two procedures were performed in 40 patients. The mean age was 42 years old. The average follow up was 5.9 years.

Seventeen patients underwent myringolenticulopexy (the incus head was excised, leaving the lenticular process attached to the stapes prior to graft placement) and 25 underwent myringostapediopexy (type III tympanoplasty).

Audiometry three to six months after surgery, and status of mastoid cavity after a minimum follow up of one year.

Comparison of post-operative hearing results for the two groups showed a statistically significant hearing advantage for myringolenticulopexy (p = 0.029). In the myringolenticulopexy group, 92 per cent achieved a post-operative air–bone gap of less than 30 dB, compared with 62 per cent in the myringostapediopexy group. The mean post-operative air–bone gaps in the two groups were 17.5 and 24.7 dB, respectively.

When surgically feasible, the technique of myringolenticulopexy is a useful method of preserving serviceable hearing in single-stage mastoid cavity surgery.

To report a series of 53 cases of facial schwannoma, to review the current literature, addressing contentious issues, and to present a management algorithm.

Retrospective case review combined with review of current literature.

A review of the case notes of 53 patients with intracranial and intratemporal facial schwannoma, from two tertiary referral centres, was undertaken. This represents the largest series of facial schwannomas with clinical correlations in the literature. Data relating to epidemiological, clinical and management details were tabulated and compared with other large series. A review of the current literature was performed, and a management algorithm presented.

There were 23 (43 per cent) female and 30 (57 per cent) male patients. Patients' ages at presentation ranged from five to 84 years, with a mean of 49 years. Twenty-five (47 per cent) of the tumours were present on the left side and 28 (53 per cent) on the right side. Hearing loss was the most common presenting symptom, being present in 31/53 (58 per cent) patients, followed by facial weakness in 27/53 (51 per cent). Two patients (4 per cent) were completely asymptomatic, and their facial neuromas were diagnosed incidentally. The schwannoma extended along more than one segment of the facial nerve in 39 patients (74 per cent), with the mean number of segments involved being 2.5. A conservative approach of clinical observation was undertaken in 20 patients (38 per cent). Thirty-three patients (62 per cent) underwent surgery, with a total of 36 procedures. The translabyrinthine approach was most common, being utilised in 17 of the 36 procedures. Two patients underwent revision surgery for residual or recurrent disease on three occasions. There was total removal of tumour in 21 cases; the remainder had subtotal or no removal with drainage or decompression of the tumours. Twenty-one nerve reconstructions were performed, and 18 facial rehabilitation procedures were performed on 14 patients.

The results of this case series are similar to those of other reported series. The diagnosis of facial schwannoma is now generally made pre-operatively, due to improved imaging techniques and heightened awareness. Clinical assessment of facial function and imaging form the mainstays of surveillance for these tumours. These tumours are managed via clinical observation or surgical intervention; the latter can range from simple procedures (such as drainage of cystic components) to aggressive tumour removal and facial nerve reconstruction. Facial rehabilitation procedures may also be applied. The timing of intervention is contentious; surgical intervention is indicated when facial function deteriorates to a House–Brackmann grade IV level.

Facial schwannomas are rare lesions, and reported series are generally small. Due to the complex management issues involved, these tumours are best managed in a tertiary referral setting. Observation is preferred until facial function deteriorates to a House–Brackmann grade III level, at which time surgery is considered. When facial function deteriorates to House–Brackmann grade IV, surgical intervention is indicated. We advocate surgical management based on the treatment algorithm described.

Rhinosporidiosis is a granulomatous infection usually affecting the nasal mucosa and conjunctiva. The disease is widely prevalent in India and Sri Lanka. Tracheo-bronchial involvement is extremely rare and is potentially life threatening. Diagnosis of tracheal involvement is a challenge due to the risk of bleeding during attempted bronchoscopic biopsy.

A 73-year-old man was admitted with severe respiratory distress, for which emergency tracheostomy was performed. At tracheostomy, a fleshy mass was seen emerging from the wound. Pathological examination of the mass confirmed rhinosporidiosis involving the trachea. Complete excision of the mass was performed after initial stabilisation of the patient.

Tracheo-bronchial rhinosporidiosis, a rare complication of nasopharyngeal infection, should be considered in a known case presenting with severe respiratory distress.

The aim of this retrospective analysis was to evaluate the diagnosis of vertigo, dizziness and imbalance in elderly patients in two tertiary neurotology clinics.

Six hundred and seventy-seven patients aged over 65 years were assessed by the same senior author, and 601 patients were enrolled in the study.

The most frequent diagnoses were benign paroxysmal positional vertigo (42.43 per cent), idiopathic vestibulopathy (20.29 per cent), migraine vestibulopathy (13.15 per cent), Ménière's disease (12.47 per cent) and acute vestibular attack (6.49 per cent).

This retrospective study showed that 93.5 per cent of the elderly patients studied had been diagnosed with peripheral vertigo. The majority of patients had been classified as having benign paroxysmal positional vertigo, idiopathic vestibulopathy or migraine vestibulopathy.

The characteristics of otoacoustic emissions that make them ideally suited for universal newborn hearing loss screening could potentially be useful for the screening of older children. This study was performed in order to assess the role of otoacoustic emissions in a screening programme for middle-ear disorders and hearing loss in school-age children.

Cross-sectional, preliminary screening study.

Primary schools of Argolida municipality, south-east Greece, between December 2004 and March 2005.

All the primary school students of Argolida were invited, by press releases and individually by their teachers, to attend a session of otological and audiological screening.

One hundred and ninety-six children were evaluated using transient evoked otoacoustic emissions. Twenty per cent failed in both ears, while in 32 per cent otoacoustic emissions could not be produced in at least one ear. Younger children had higher rates of absent transient evoked otoacoustic emissions. The absence of otoacoustic emissions was highly correlated with tympanic membrane changes seen on otoscopy and the presence of a type B tympanogram. As a single screening modality, otoacoustic emissions had a 100 per cent sensitivity in diagnosing hearing loss worse than 30 dB, and a 90 per cent sensitivity and 64 per cent specificity in diagnosing hearing loss worse than 25 dB, which did not improve by adding tympanometry to the screening protocol.

These results strongly suggest the potential usefulness of otoacoustic emission testing in screening school-age children for hearing loss. Further studies, taking into account cost-effectiveness issues, are indicated.

Herbal remedies and other natural supplements have become popular alternative medical therapy. Patients using these products may present to ENT surgeons with epistaxis or with unexpected, excessive peri-operative bleeding.

The purpose of this review was to consolidate the available data regarding herb–drug interactions and the direct effects of herbal medicines and nutritional supplements in isolation, regarding disturbance in haemostasis, so as to emphasise their individual importance.

A literature review was performed using the Medline (PubMed) and UKMi databases.

Searches of these databases revealed 24 herbal products with documented interactions with anticoagulants and 98 herbal products with theoretical interactions with the coagulation system. Herbal products with effects on coagulation when given alone were also documented.

When encountering patients presenting with epistaxis, or in the elective surgical setting, a thorough enquiry about the use of herbal medicines is of great importance.

Dizziness is a common presenting complaint in primary healthcare. One differential diagnosis is Ménière's disease. The UK National Health Service ‘Prodigy’ guidelines have been produced to help healthcare professionals to diagnose and manage Ménière's disease. The aim of this audit was to consider how Ménière's disease is managed in general practice, and to assess how well general practitioners adhere to the Prodigy guidelines.

We identified general practices near the Torbay region of south Devon which had a ‘TQ’ postcode. There were 41 practices, staffed by a total of 203 general practitioners. We wrote to each general practitioner, asking them to fill in a questionnaire regarding Ménière's disease. Our practice response rate was 68 per cent and our individual rate 43 per cent.

The mean correct answer rate was >50 per cent. The respondents achieved this despite few being aware of any formal guidelines, and none being aware of the Prodigy guidelines.

Detailed knowledge of Ménière's disease was uncommon, and formal guidelines were not frequently consulted.

The purpose of this study was to explore the pathogenic role of bone in sinus surgery, and to determine whether mucosal disease after sinus surgery may be induced by underlying disease within the bone.

Twenty-five rabbits were divided into five groups. In group one, wide surgical removal of the maxillary sinus mucosa and creation of a nasoantral window were undertaken. In group two, only nasoantral window creation was undertaken. In group three, the mucosa of the anterior maxillary sinus was removed. In group four, a strip of mucosa around the ostium was removed. Group five was used as a control.

After three months, in the groups which had undergone wide surgical removal, the medial sinus walls were observed to be retracted and the inner curtain of the bone disturbed, with alteration of the haversian canal system and osteoclastic bone resorption.

After radical sinus surgery, electron microscopic changes were observed in the bony walls, similar to those changes seen in osteomyelitis.

The ototoxic effects of cisplatin include loss of outer hair cells, degeneration of the stria vascularis and a decrease in the number of spiral ganglion cells. Scanning microscopy has shown balloon-like protrusions (blebs) of the plasma membrane of inner hair cells following cisplatin administration. The present study was undertaken to identify the possible role of inner and outer hair cell blebs in the pathogenesis of cisplatin-induced ototoxicity.

Twenty-five guinea pigs were injected with cisplatin and their hearing tested at different time-points, before sacrifice and examination with scanning electron microscopy.

Seven animals showed blebs in the inner hair cells at different stages. Hearing thresholds were lower in animals showing blebs.

Cisplatin seems to be able to induce changes in inner hair cells as well as in other structures in the organ of Corti. Blebbing observed in animals following cisplatin administration could play a specific role in the regulation of intracellular pressure.

There is a paucity of evidence to guide the post-operative follow up of patients undergoing middle-ear ventilation tube insertion for the first time. This study was conceived to identify current practice at our institution (Ninewells Hospital, Dundee) and to inform subsequent change in our follow-up procedure.

Two cycles of data collection and analysis were performed. All paediatric patients undergoing ventilation tube insertion for the first time were identified. Patients who had previously undergone ventilation tube insertion or additional procedures such as adenoidectomy or tonsillectomy were excluded. The first data collection period comprised all of the year 2000, and the second 18 months over 2003–2004. A minimum of 20 months' follow up was allowed for. Data regarding clinical findings and audiometry were recorded at each follow-up appointment.

We identified a total of 50 patients meeting our criteria for inclusion in the first cohort. There were a total of 156 appointments between surgery and data collection (a mean of 3.12 per child). A total of 113 (72 per cent) appointments lead to no medical intervention. The only statistically significant difference between patients requiring further ventilation tube insertion (n = 10) and those not requiring further treatment during the study period (n = 40) was the average hearing threshold (p < 0.01). These findings prompted a change in the post-operative regime; all patients undergoing ventilation tube insertion were subsequently seen at three months for a pure tone audiogram, and further review depended on clinical and audiometric findings. Records for 84 children were identified and collected for the second cohort, there were a total of 154 appointments (a mean of 1.83 per child). In only 18 appointments (12 per cent) were normal findings and hearing recorded and children given a further review appointment. Sixteen of 29 (55 per cent) children with abnormal clinical findings (otorrhoea, tube blockage or extrusion) required some form of intervention (p < 0.05). Twenty-six had a mean hearing threshold worse than 20 dB at first review. Nineteen (73 per cent) required further intervention of some sort (p < 0.01).

Our study demonstrated that the vast majority of review appointments resulted in no clinical intervention. We therefore question the need for regular follow up in this patient group. Twenty per cent (10 of 50 and 18 of 84) of our patients required further ventilation tube insertion within the study periods. This is consistent with rates reported in the literature. Children with abnormal clinical findings or a mean hearing threshold greater than 20 dB were significantly more likely to require further intervention. We would recommend one post-operative review with audiometry, three months after surgery. At this initial appointment, further review should be offered to those children with poor hearing, early extrusion, blockage or infection, as they are more likely to require further ventilation tube insertion. This strategy is dependent on good links with community primary care providers and easy access to secondary care for further management, should this be required.