Hearing impairment has long been recognized as a common feature in osteogenesis imperfecta. The figures in some publications could be taken to imply that, with increasing age, the proportion of osteogenesis imperfecta patients with hearing impairment approaches 100 per cent.

The incidence of hearing loss in a large survey of 1394 patients with osteogenesis imperfecta was examined. It was found that the most common age of onset was in the second, third and fourth decades of life. At the age of 50 approximately 50 per cent of the patients had symptoms of hearing impairment; over the next 20 years there was little further increase.

Differences were shown between patients with different clinical types of osteogenesis imperfecta as delineated in the Sillence classification; hearing loss was significantly less common in the type IV disease than in the type I disorder. Among the 29 families with osteogenesis imperfecta type IA there were distinct differences in the likelihood of hearing loss. These findings provide insights which will be valuable in giving patients advice on the likelihood of developing hearing loss in the future.

Alar (external valve) collapse may be iatrogenic or can occur as a consequence of ageing or trauma. We have utilized an autogenous cartilage graft inserted as an underlay graft to the alar rim to treat 17 patients who have presented with nasal obstruction due to alar collapse. We report the short- and medium-term functional results and the cosmetic sequelae of our approach and conclude that this procedure offers an improvement in nasal airway performance in patients with external valve collapse without producing serious adverse cosmetic sequelae.

The management of chronic sino-nasal disease has changed significantly in the last 15 years with the advent of topical nasal steroids and minimally invasive endoscopic surgery, but survey data from district hospitals in the UK have not been published to date. We examined the current management of 383 consecutively scanned patients with sino-nasal symptoms in three district hospitals. Survey standards were set, and were attained in 56 per cent for symptom recording, 60 per cent for pre-scan diagnosis, 62 per cent for endoscopic examination in out-patients, and 73 per cent for topical steroid use before scanning. The variability of sinus disease makes it difficult to be dogmatic about proper indications for surgery, but subsequent surgical management, as ascertained from the notes, showed large differences between consultants in the three hospitals. The rate of minor complications was 2.2 per cent, there were no major complications, and the vast majority of patients stated that their nasal symptoms were improved by surgery. The survey showed that endoscopic sinus surgery is safe and effective when practised in a district hospital setting.

Recent investigations have suggested human papillomavirus (HPV) to be involved in the development of sinonasal papillomas (SNP). Forty-three patients operated for SNP were studied to determine the prevalence of HPV-DNA sequences in these tumours and to evalulate their value as a prognostic parameter. The original sections of all cases were reviewed and reclassified according to the WHO. Paraffin blocks available from 37 patients were subjected to in situ hybridization (ISH) and polymerase chain reaction (PCR). Histology revealed 34 cases of inverted papilloma (IP) (79 per cent), five cases of exophytic papilloma (EP) (12 per cent) and four cases of columnar cell papilloma (CCP) (nine per cent). Recurrences developed in seven of 41 patients (17 per cent), and malignancy occurred in four of 43 patients (nine per cent). HPV was detected in four of 37 specimens (11 per cent) both by ISH and PCR. In particular, HPV-11 was found in three lesions (two EP, one IP) (eight per cent), and HPV-6b was detected in one lesion (one EP) (three per cent). Our findings suggest a possible role for HPV in the pathogenesis of exophytic papillomas. As no correlation was found to malignancy and recurrence of disease, screening for HPV seems not to be useful as a prognostic parameter.

Diagnosis of allergic fungal sinusitis (AFS) in patients who present with rhinosinusitis and polyposis is based upon certain clinical, histopathological and mycological histopathological criteria are the demonstration of fungal hyphae in allergic mucin and absence of tissue invasion in the excised polyps. Previous reports have indicated difficulty in demonstrating fungal hyphae on histological examination in up to 75 per cent of cases. Analysis of a series of 25 patients with AFS, suggested methods to ensure demonstration of fungal hyphae and thus increase diagnostic yield in cases with suspected AFS. criteria. Specific diagnostic

Epithelial cells and bacteria were sampled from the tonsillar surfaces of seven patients (six males, one female; median age 16 years, range 10 to 21 years) suffering from acute infectious mononucleosis with concomitant pharyngotonsillitis. By using gold-labelled antiserum to human IgG and secretory IgA (sIgA), micro-organisms on the tonsillar surfaces coated with these immunoglobulins could be identified by tracing the gold particles in the transmission electron microscope. The patients harboured significantly fewer bacteria coated with immunoglobulins than did healthy controls. More bacteria were coated with IgG immunoglobulins than with sIgA. Reduced immunoglobulin-coating of the bacteria on the tonsillar surfaces during infectious mononucleosis can explain their tendency to immense local colonization and proneness to penetrate into the epithelial cells.

The surgical closure of septal perforations remains a distinctive challenge to the otorhinolaryngologist. This is demonstrated by the modest success in most techniques. An alternative method, involving surgical enlargement of the perforation with posterior edge repair, is described and the outcome is investigated. Thirteen patients with perforations of up to 50.mm in size underwent this technique. A questionnaire interview was conducted and symptom scores were obtained. The length of hospitalization, follow-up period and post-operative complications were evaluated as were measures of morbidity.

The results showed a significant improvement in the symptom scores for nasal crusting, epistaxis and overall discomfort. This technique is straightforward and is especially suitable for larger perforations. The successful improvement in symptoms and an associated low morbidity makes it a complement to alternative surgical closure techniques.

Mucocutaneous exposure is an important route of transmission of hepatitis viruses and HIV in healthcare workers. Few data exist in the literature on the risk of transconjunctival exposure during many surgical procedures. We investigated the use of eye protection during tonsillectomy and measured the incidence of potential eye splash. No otolaryngologist surveyed routinely used eye protection during tonsillectomy. Splash events occurred in 23 out of 103 tonsillectomies. In all cases the surgeon was unaware of the splash. Splash events were significantly more likely to occur during adult tonsillectomy (p < 0.05). We strongly advise the routine use of eye protection during tonsillectomy.

This is a retrospective review of 74 consecutive patients who presented with a nasal septal perforation or ulcer. The aim of this study was to evaluate the contribution of the investigations used to identify the pathology underlying their septal perforation or ulcer. In the majority of cases there was an anterior (85 per cent) smooth, well-circumscribed septal lesion with an occasional bleeding point. Seventy-seven per cent were less than two cm in diameter. The primary presenting symptom was intermittent epistaxis, with the exception of patients with granulomatous disease, who presented more frequently with nasal obstruction. Thirty-five patients (47 per cent) were idiopathic, 29 (39 per cent) traumatic, eight (11 per cent) inflammatory and two (three per cent) infectious. The nasal septum was biopsied in 71 cases, and histology showed non-specific or non-diagnostic findings in 39 (55 per cent), chronic inflammation in 12 (17 per cent), acute inflammation in six (eight per cent), acute to chronic inflammation in four (six per cent) and histological changes consistent with trauma in nine (13 per cent). The anti-neutrophil cytoplasmic antibody test (ANCA) successfully identified six patients with Wegener's granulomatosis, and the angiotensin converting enzyme test (ACE) supported a clinical diagnosis of sarcoidosis in one patient. The remaining investigation methods (CRP, ESR, chest radiograph, biochemistry and coagulation screen) did not contribute to the diagnosis. In this study, the appearance of a nasal septal perforation or ulcer or its location did not predict the clinical diagnosis. A nasal septal bopsy did not significantly contribute toward the clinical diagnosis. On the basis of these findings, a biopsy of a septal perforation or ulcer is unlikely to contribute to the management of patients unless the supportive tests of ANCA or ACE are abnormal or unless malignancy is suspected. This conclusion is restricted to the management of septal perforations as we have found nasal biopsies of polyps of other lesions to be of value.

The clinical features of 58 consecutive patients presenting with a new case of chronic otitis media were prospectively collected over a 15-month period. Twenty-three ears had a keratin filled marginal or attic defect (14 with cholesteatoma), 20 had a self-clearing marginal or attic defect, and 21 had a central tympanic membrane perforation (including one cholesteatoma). Twenty patients (35 per cent) had an abnormal finding in the opposite ear. The patients’ ages were dispersed over a wide range of age groups with a mean age of 34 years. Hearing loss was the most common presenting symptom (78 per cent), followed by otorrhoea (64 per cent). A significant proportion of patients denied any history of otorrhoea. Our findings should alert the clinician to suspecting a new case of COM in patients with hearing loss of any age, with, or without, a history of otorrhoea, regardless of their background ear history or the duration of their symptoms.

Nasal obstruction is common in children, and is often attributed to adenoid enlargement. This prospective study was performed to determine whether routine nasal endoscopy is of value for children undergoing surgery for nasal obstruction. Forty-eight children aged two to nine years undergoing adenoidectomy, and six normal controls, were examined under general anaesthesia with a 4 mm rigid endoscope. A video of the endoscopy was subsequently assessed independently by an observer blinded to the original findings and the presence of nasal symptoms.

The endoscopist and independent assessor were in agreement regarding 86 per cent of the findings. Three quarters of the children had abnormalities on endoscopy in addition to enlarged adenoids, and in 23 per cent these were potentially of major clinical significance (unsuspected foreign body, gross septal deviation, gross hypertrophy of the turbinates). Endoscopy produced no post-operative complications and was possible in children as young as two years of age, without decongestants.

Nasal endoscopy is a safe, objective and useful means of identifying potentially significant abnormalities in children with nasal obstruction.

We present our experience using conchal composite grafts in major and minor nasal reconstruction. We believe that composite grafts have an important and under-utilized role as an adjunct to the paramedian forehead flap on total and subtotal nasal reconstruction, as well as in providing an internal lining and support in stenosis of the nasal vestibule. The use of a composite conchal graft as an inner lining and support is often preferential to a septal composite or hinge flap that often results in a septal perforation with its associated morbidity.

We recognize that a composite conchal graft used for reconstruction of the alar margin provides a suboptimal result after several months. This is because it is not always possible to replace a whole aesthetic subunit and whilst the site the graft is harvested from often allows the correct contour to be chosen, the quality of skin is often different and this can be seen after several weeks when any post-operative oedema has settled.

We reviewed 80 patients admitted to our hospital who were diagnosed with acute epiglottitis between January 1995 and March 1999, and their clinical features, evolution and treatments were analysed. No sexual predominance was found, and there was no patient younger than 16 years of age. The patient fatality rate was 1.3 per cent, and the hospitalization period was markedly longer than those of other reports.

The biological activity of acinic cell carcinoma is uncertain. Histological dedifferentiation is one possible reason for recurrent disease, and this study was undertaken to assess its importance in acinic cell carcinoma. The initial and recurrent specimens from five patients with acinic cell carcinoma were assessed histologically and using flow cytometry, AgNOR estimation and morphometric analysis for evidence of dedifferentiation. No objective evidence of a change in biological aggressiveness in recurrent acinic cell carcinoma was identified. In this limited series of a rare salivary gland tumour, it would appear that factors other than dedifferentiation, such as close/involved margins, histological type and stage have a more meaningful effect on the likelihood of recurrence and prognosis.

A randomized clinical trial was conducted to determine the efficacy of mastoid obliteration in controlling persistent ear discharge, wax accumulation, fungal infection and granulation tissue formation in patients with cavity problems following modified radical mastoidectomy (MRM). Thirty patients underwent revision mastoidectomy with mastoid obliteration using a temporoparietal fascial flap. They were then followed up over a one-year period and the stated parameters observed. They were compared with 30 patients with similar complaints who were treated conservatively and kept under observation for 12 months. On follow-up, the number of patients in both the groups suffering from various cavity problems was compared. The chi-squared test was applied to the results and it was determined that there was a significantly lower incidence of discharging ear and formation of granulations in the operative group. However, audiological status, development of otomycosis and wax accumulation did not reveal any significant variation between the two groups.

The conventional technique for cochlear implantation is via a mastoidectomy and posterior tympanotomy. An alternative approach for cochlear implantation is hereto described.

The middle ear is entered through a suprameatal approach (SMA) bypassing the mastoid cavity. This surgical approach shortens the duration of the procedure to approximately one hour. The introduction of the cochlear implant electrode array involves drilling in the suprameatal region and the osseous portion of the external auditory canal at a safe distance from the anatomical position of the facial nerve. This prevents possible injury by direct trauma or drill overheating of the chorda tympani or facial nerves.

We report 15 consecutive patients who were operated on using the SMA technique. No complications were encountered as a result of this surgical technique but further experience may be necessary.

To date there exists no sensible way of classifying the intensity of haemorrhage following tonsillectomy, though this is a prerequisite when comparing the results presented in literature. We evaluated the incidence of post-operative haemorrhage according to our classification in 602 patients who underwent tonsillectomy at our department in 1999. In 21 patients a grade 1 (spontaneous cessation) bleeding occurred. One patient had a grade 2 (infiltration anaesthesia), 14 patients had a grade 3 bleeding (treatment under general anaesthesia) with one patient receiving a blood transfusion. Two patients had a grade 4 bleeding (ligature of the external carotid artery). There was no patient with grade 5 bleeding (lethal outcome). Grades 1 and 3 had the same incidence rates. Primary haemorrhage (<24 hours) can be expected in the majority of patients undergoing tonsillectomy. Secondary haemorrhage is rare and can be life-threatening.

The aim of our research is to develop a laryngeal tissue augmentation technique that will be useful in treating glottal and neoglottal insufficiency.

In a previous study the feasibility of insertion of tubular expanded polytetrafluoroethylene (e-PTFE) tissue implants into the larynx of a porcine model was demonstrated. In this paper we report the clinical, endoscopic, computed tomography (CT) and magnetic resonance image (MRI) and histopathological follow-up findings. These results demonstrate host tolerance of the implant over the period of study (six months) with little tissue reactivity to tubular e-PTFE implants in the porcine model. The tubular shape of the implant enhances stabilization by the rapid and complete host tissue penetration of the implant’s interstices, making tubular e-PTFE a potentially useful soft tissue augmentation material for larygneal incompetence.

Encephaloceles have been traditionally treated by a transcranial or external approach. The advent of endoscopic sinus surgery has allowed a new intranasal approach for the treatment of basal encephaloceles, minimising patient morbidity. We discuss the classification and presentation of encephaloceles, and present our experience in the repair of six cases. We believe transnasal endoscopic repair is the method of choice in dealing with selected basal encephaloceles.

Frequently, the clinical picture in the oropharynx alone does not lend itself to a reliable differentiation between acute pharyngotonsillitis in infectious mononucleosis and a streptococcal inflammation. Such a differentiation, however, is essential for the indication of antibiotic therapy. Therefore, it was the aim of the present study to investigate whether or not endoscopic verification of larger-than-normal lymphatic tissue with fibrinous membranes in the nasopharynx would enhance the reliability of diagnosis.

Fifty hospitalized patients exhibiting the clinical picture of acute pharyngotonsillitis were examined for the following parameters: nasopharyngeal endoscopy, determination of glutamine-oxaloacetic transaminase (GOT), glutamine-pyruvic transaminase (GPT), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), leucocytes and haemogram, antibodies to viral capsid antigen (VCA)-IgM, Epstein-Barr virus nuclear antigen (EBNA), and cytomegalovirus (CMV).

In 24 patients with acute tonsillitis/peritonsillar abscess, and in 26 patients with infectious mononucleosis, the total leucocyte count in the blood and the blood/erythrocyte sedimentation rate were comparable. Atypical lymphocytes or absolute lymphocytosis were not seen in acute tonsillitis, but were found in 88.5 per cent of patients with infectious mononucleosis. Elevated transaminase levels were noted in 77 (GOT) and 88.5 per cent (GPT) of patients with infectious mononucleosis, and in acute tonsillitis in 4.2 (GOT) and 12.5 per cent (GPT) of patients. The CRP was higher than normal in 91.7 per cent of patients with acute tonsillitis, and in 57.7 of patients with infectious mononucleosis. In none of the patients with acute tonsillitis was lymphatic tissue with fibrinous membranes seen in the nasopharyngeal cavity. In contrast, nasopharyngeal endoscopy in infectious mononucleosis was positive in 24 out of 26 patients. Hence, the sensitivity was 92.3 per cent, the specificity 100 per cent, and the prediction value for a positive test turned out to be 1.

Endoscopy of the nasopharngeal cavity employed as a simple and supplementary procedure adds to the reliability of diagnosis of infectious mononucleosis.