This review of ectodermal dyplasias (ED) presents the particular syndromes that might present to the otolaryngologist for management and discusses the ear, nose and throat manifestations of the condition.

Specialist training at the registrar grade in the United Kingdom has undergone significant change in the recent past. One of the effects has been a reduction in the length of time spent in training. This and the application of the European working time directive have the potential to reduce trainees’ surgical exposure. The proportion of tympanic membrane procedures performed by reconstructive otolaryngology registrars in the Grampian University hospitals was audited to monitor the impact of these changes.

Case notes of all patients who underwent myringoplasty or tympanoplasty between July 1998 and June 1999 were analysed retrospectively. Details of the surgeons’ grade were recorded. The proportion of myringoplasties performed by registrars as determined by the Royal College of Surgeons of England National Comparative Audit survey, carried out in 1995 before the widespread implementation of recommended changes in otolaryngology registrar training, was set as the gold standard.

In the period July 1998–1999 registrars had performed fewer myringoplasties than the standard, 17 per cent versus 34.2 per cent respectively (p = 0.035). A strategy to increase registrar exposure to myringoplasty surgery was then adopted by the department and the proportion of myringoplasties performed by registrars re-audited prospectively. The proportion of myringoplasties undertaken by registrars increased in the period January 2001 to July 2001 compared to July 1998–1999, 53 per cent versus 17 per cent respectively (p < 0.0007).

Changes in working practice can address shortfalls in registrars' exposure to surgical procedures.

Noise exposure is one of the major causes of permanent hearing loss in society. Exposure of health service staff to intense levels of noise in the workplace is a potential risk for the development of temporary and permanent hearing loss.

In this prospective study, 18 members of the orthopaedic staff underwent hearing assessment by pure tone audiometry and speech discrimination prior to noise exposure at the workplace and immediately following cessation of work. The number of hours of exposure and noise levels in the workplace was also analysed.

Only minimal temporary sensorineural threshold shifts were detected post-noise exposure. There was no change in speech discrimination scores and no individuals complained of tinnitus. The number of hours of exposure ranged from 1.5 to 8.5 hours (mean 5.2 hours). Recorded sound levels for instruments ranged from 119.6 dB at source to 73.1 decibels at 3 metres.

Although high sound levels are recorded in the orthopaedic operating theatre, the intermittent nature exposure to the intense noise may protect staff against hearing loss, speech discrimination difficulties and tinnitus.

Cochlear implantation is regarded as a safe and effective treatment for the profoundly deaf. However, a proportion of patients suffer complications after implant surgery. This paper examines the complications encountered in 240 adult cochlear implant operations performed in Manchester between June 1988 and June 2002. Minor complications were defined as those that either settled spontaneously or with conservative management. The total number of minor complications was 61 (25.4 per cent of cases). Non-auditory stimulation, which resolved with implant reprogramming, was present in 53 cases (22.1 per cent). Major complications were defined as those requiring further surgery, explantation or causing a significant medical problem, and occurred in 15 patients (6.25 per cent). These included implant extrusion, implant sepsis, electrode migration, flap-related problems, and persistent non-auditory stimulation. Nine of the 15 patients suffering a major complication required explantation. There were no post-operative deaths, cases of meningitis, nor persistent facial palsies in the series.

Electroneuronography (ENoG) has become a useful test for estimating the degree of facial nerve degeneration and predicting the prognosis in patients with facial nerve palsy. Test results may be influenced by several factors, including the electrode positions, skin resistance, stimulus magnitude, and possible artifacts. Regarding recording electrode positions, different groups have used two different locations, the nasolabial fold and nasal ala. The authors compared the waveforms recorded from these two locations in ENoG recordings to obtain the optimal waveform. Twenty healthy volunteers and 25 patients with unilateral facial nerve palsy were included in this study. Recordings were carried out with the recording electrode placed on the nasolabial fold, followed by placement on the nasal ala after 10 minutes. The following parameters were assessed: (1) the supramaximal threshold, (2) amplitude and shape of the waveform, (3) interside difference, and (4) test-retest variability. There was no significant difference in the amplitude of the waveform, interside difference, and test-retest variability between the two groups. However, when the electrode was placed on the nasal ala, the threshold was significantly lower, an ideal biphasic configuration was present in almost all cases (97.5 per cent) of normal volunteers and it was easier to identify the waveform. Placement of the recording electrode on the nasal ala would be the preferred method.

Atrophic rhinitis is a chronic inflammatory disease of the nose, which is more common in India. Chronic dacryocystitis is its rare complication. The authors found four cases of chronic dacryocystitis from March 2002 to October 2003 due to atrophic rhinitis. It was diagnosed clinically by the regurgitation test and lacrimal syringing. These cases were treated conservatively for a period of six weeks to make the nasal mucosa healthier and were then subjected to endoscopic dacryocystorhinostomy (end-DCR) under local anaesthesia. The procedure was found to be more difficult due to bleeding and the healing time was prolonged as compared to other cases of end-DCR. After one to one and half years of follow-up the primary success rate was 75 per cent but after revision surgery in one case, all cases were successful. Hence it was concluded that atrophic rhinitis is no more a contraindication for end-DCR. However, meticulous initial preparation and post-operative follow-up is necessary to improve the result.

The purpose of this study was to assess the practicality and validity of laryngeal ultrasound to establish vocal fold movement in children with suspected vocal fold palsy. Fifty-five consecutive patients (age range three days to 12 years) with suspected vocal fold palsy underwent both laryngoscopy and laryngeal ultrasound. Ultrasonographic findings correlated with endoscopic findings in 81.2 per cent of cases. This, however, rose to a concordance rate of 89.5 per cent in patients aged over 12 months. Laryngeal ultrasound is well-tolerated, safe and non-invasive and the authors feel that it is a useful adjunct to endoscopy in the diagnosis of vocal fold palsy.

Reports of the impact of larynx cancer treatment modality on quality of life are conflicting, in part due to varying study methodology. The aims of this study were to (1) provide preliminary comparisons of quality of life following radiotherapy or combination therapy; (2) evaluate a number of measures of quality of life and thereby (3) inform future prospective studies. Thirty-six laryngeal cancer patients, 24 following radiotherapy, 12 following radiotherapy and laryngectomy completed the Functional Assessment of Cancer Therapy (FACT) - General/Head and Neck subscale; Nottingham Health Profile (NHP); and the Hospital Anxiety and Depression scale (HAD), three to 12 months post-treatment. Results showed trends towards a less good quality of life in the combined therapy group over a wide range of outcomes, significant for the disease specific FACT head and neck subscale, NHP emotion (p = 0.04) and isolation (p = 0.027). To the authors' knowledge, however, this is the first demonstration of greater impact of laryngeal cancer on quality of life in younger subjects, who had lower scores among others on emotional wellbeing (p = 0.015) and anxiety (p = 0.035). Younger patients thus appear more likely to need more intensive support through treatment. Many of the physical and psychosocial domains derived from the three tools used were highly correlated. In other words, given the known high morbidity of the disease and its treatment, the selection of tools for head and neck quality of life assessment may be much less important than their universal application.

Peritonsillar abscess is the most common deep infection of the head and neck that occurs in adults; the treatment of the disease remains controversial. A prospective study using a single high dose steroid treatment for peritonsillar abscess, was undertaken in 62 patients to determine the treatment's effectiveness in relieving symptoms such as fever, throat pain, dysphagia and trismus. All patients were randomly assigned to two groups: 28 patients received intravenous antibiotic therapy and a single dose placebo and 34 patients were treated with single use of high dose steroid in addition to intravenous antibiotic. Patients were hospitalized after needle aspiration and therefore their clinical courses and responses to therapy could be rigorously assessed. Comparison of clinical outcomes with respect to hours hospitalized, throat pain, fever, trismus were assessed between the two groups. Clinical outcomes revealed a statistically significant difference between the two groups (p < 0.01), indicating that single use of high dose steroid prior to antibiotic therapy is more effective than the use of an antibiotic alone. These results suggest that single intravenous use of steroid in addition to antibiotic therapy is an excellent choice for the management of peritonsillar abscess.

Patients with squamous cell carcinoma of the hypopharynx (HPSCC) are often seen in advanced stages and have a poor prognosis. The authors analysed 104 patients who had HPSCC and underwent surgery as the primary treatment between 1986 and 1995 in their institute. Of the 104 patients, 83 patients (80 per cent) had advanced T3 or T4 staged and 64 patients (62 per cent) had cervical metastasis. Thirteen patients (13 per cent) had conservation surgery with laryngeal preservation and 69 patients (66 per cent) received post-operative radiotherapy. The five-year overall and disease-specific survival was 47 per cent and 62 per cent, respectively. Recurrence occurred in 38 patients (37 per cent), including 12 (12 per cent) with local, 22 (21 per cent) with regional, and 12 (12 per cent) with distant recurrence. Sixteen patients (15 per cent) had recurrence at multiple sites. The site and size of the primary tumour, neck biopsy before surgery, early post-operative complications, and pathological nodal stage were significant prognostic factors of disease-specific survival (DSS) in univariate analysis. Neck biopsy before surgery and site of primary tumour were significant factors in multivariate analysis. In conclusion, surgical treatment for the HPSCC patients has achieved good local-regional control and survival. Bilateral neck dissection for the tumour across the midline and avoiding neck biopsy before surgery may reduce regional recurrence.

This paper reports an unusual case in which aseptic meningitis presented with sudden sensorineural hearing loss (SSNHL) associated with intralabyrinthine haemorrhage (ILH). A seven-year-old girl presented with sudden right-sided hearing loss with dizziness. She did not have a previous history of bleeding disorders. This child was assessed using audiograms and magnetic resonance imaging (MRI). The patient's hearing loss was irreversible. Steroid therapy was not effective. SSNHL associated with ILH can be one of the negative prognostic factors in children.

Antrochoanal polyps were first documented in the 18th century. They represent one of the most common types of nasal polyps in children without cystic fibrosis. Only a few reports on children who had a history of snoring due to an antrochoanal polyp and only two cases where the antrochoanal polyp caused documented obstructive sleep apnoea (OSA) have been published so far. This report adds a third case of paediatric OSA induced by an antrochoanal polyp in a 12-year-old boy. After endonasal endoscopically-controlled polypectomy and a recurrence, transoral osteoplastic antrotomy in combination with endoscopic endonasal polypectomy eliminated the antrochoanal polyp and OSA was resolved. The authors have reviewed essential historical aspects about children suffering from snoring and/or OSA caused by an antrochoanal polyp.

Peritonsillar abscess formation is an uncommon complication of infectious mononucleosis (IM). Early case reports implicated corticosteroids in the development of such abscesses, however, subsequent studies suggested that these drugs do not promote the formation of abscesses at several sites outside the central nervous system. It has recently been demonstrated that zwitterionic polysaccharides, in bacterial capsules, form complexes with CD4+ T lymphocytes leading to abscess formation. A patient is presented who developed peritonsillar abscess a few days after initiation of corticosteroid therapy for IM; the medical literature was reviewed in respect of this subject. It appears that the occurrence of these abscesses in IM is not strongly linked to corticosteroid treatment. The authors, therefore, recommend that steroids should not be withheld from patients with severe IM on the basis that they may precipitate the development of peritonsillar abscess.

Tracheostomy remains the primary method of treatment of acute airway obstruction due to malignant invasion and compression of the trachea. However, the development of tracheal stents has provided an alternative effective treatment modality. This case report and literature review highlights the benefits of intra-luminal stenting, including resolution of distressing obstructive symptoms, and subsequent improvement in quality of life.

Retropharyngeal space swelling is a rare occurrence following minor head and neck trauma. Upper airway obstruction is a potentially life-threatening sequela. The authors present a case of retropharyngeal space haematoma following minor blunt head and neck trauma. Management was conservative with gradual spontaneous resolution of the haematoma. The literature is reviewed and the management and treatment principles presented.

Two cases of subcutaneous emphysema of the neck as a result of abuse in infancy are presented to add to the variety of findings associated with the maltreatment of infants. Pharyngeal perforations as a result of abuse are rare. These cases are presented as a reminder to the attending clinician that although most cases of subcutaneous emphysema may resolve without any complications a detailed examination for the cause should be performed and the history carefully verified.

Spontaneous cervical haematomas are rare occurrences for which a wide variety of aetiologies have been reported. Although the pathogenesis of this condition can be quite diverse, all known cases have emanated from a cervical source. The authors report the first case of a spontaneous cervical haematoma arising from an intrathoracic source. A case review and Medline search from 1962–2003 was carried out.

No prior experience with intrathoracic sources for cervical haematomas has previously been reported. Transcervical haematoma evacuation confirmed the intrathoracic origin and a median sternotomy was required to gain control of this haemorrhage.

Thoracic sources should be considered in the differential diagnosis of a spontaneous cervical haematoma. Preparations for a combined procedure with a thoracic surgeon can be predicted pre-operatively based on imaging studies.

In this paper two cases are reported in which spontaneous entry of air into the head appears to have occurred through a hypercellular mastoid air cell system. In both these cases forceful sneezing and nose blowing were considered contributory factors. They underwent surgical repair of the bony defects which, combined with less vigorous nose blowing, has affected a successful repair. The aetiology of pneumocephalus is discussed and a review of the pertinent literature is also presented.