Introduction: Temporal bone dissection is essential training for otological surgery. Organ retention scandals have made it difficult to obtain consent for cadaveric temporal bone removal. The current literature does not address the means of acquiring a steady supply of cadaveric temporal bones for medical education and training. The US national temporal bone registry has established a temporal bone donor bank for medical education and research. Could a similar programme in the UK be an answer to the paucity of temporal bones?

Objectives: (1) To ascertain the degree of interest amongst our regional patient population in a ‘living will’ pledge for temporal bone donation for medical education and research. (2) To delineate a demographic profile of potential temporal bone donors.

Design, setting and participants: One thousand questionnaires were distributed to patients and relatives attending out-patient clinics; 920 people responded.

Results: Seventy per cent of respondents supported temporal bone donation for medical education and research. Potential temporal bone donors tended to be ENT clinic attenders, to suffer from hearing difficulties or to have had previous ear surgery (p<0.001). Strong support also came from non-ENT clinic attenders.

Conclusions: There was strong support amongst our regional patient population for a ‘living will’ pledge for temporal bone donation for medical education and research. Based on our donor profile, we propose a temporal bone donor programme, starting on a regional basis with possible expansion nationwide. This programme would recruit donors from amongst patients attending ENT out-patient clinics, as a long term solution to improve the supply of temporal bones.

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.

Communication disorders represent a major and growing problem worldwide. In Europe, the specialty of phoniatrics has developed partly in response to this important issue. This article reviews training and workforce issues in phoniatrics and raises key questions and issues that need resolution in the future.

Background: We present the results of a retrospective review of children undergoing implantation with bone-anchored hearing aids (BAHAs) at the Great Ormond Street Hospital for Children.

Methods: The case notes of 71 children undergoing BAHA placement at the Great Ormond Street Hospital for Children between December 1990 and August 2002 were reviewed. Outcome measures included hearing thresholds, incidence of fixture loss, skin reaction and need for revision. Quality of life outcomes were also measured.

Results: Eighty-five ears had been implanted. Fifty-four per cent of children had experienced no complications, 42 per cent had required revision surgery and 26 per cent had experienced fixture loss at some point. Young age at implantation was associated with an adverse outcome. Trauma and failure of osseointegration had been the commonest reasons for failure. A skin reaction around the abutment had occurred at some point in 37 per cent of children but had persisted for longer than six months in only 9 per cent; this had been associated with fixture loss. The use of fixture site split skin grafts had reduced problems with skin hypertrophy and hair overgrowth. Hearing thresholds when using BAHAs had been comparable to those when using bone conduction hearing aids. However, BAHAs had significant additional benefits in terms of sound quality, ease of use and overall quality of life.

Conclusion: Bone-anchored hearing aids provide significant benefits over other types of hearing aid, both audiologically and in terms of quality of life. Careful selection of candidates and meticulous follow up are required in order to minimize complications.

Objective: To conduct a study of patients presenting with perichondritis of the auricle and to analyse the possible aetiological factors responsible, the bacteriological agents involved, the treatment modalities and the complications of such infections.

Setting: Academic department of otolaryngology.

Design: A retrospective clinical study of patients treated over a five-year period.

Participants: Sixty-one patients with clinically proven perichondritis of the auricle, with or without diabetes mellitus (i.e. malignant otitis externa).

Results: Based on the severity of the disease, otherwise uncomplicated patients were assigned to group A and divided into three cohorts. Patients with perichondritis secondary to malignant otitis externa were analysed separately as group B. Men formed the majority of the patients and most were young (16–35 years). Trauma was the main cause (46 per cent) and Pseudomonas aeruginosa the most common micro-organism isolated. The condition was managed conservatively with antibiotics alone in 19 patients (31 per cent) and these cases had no residual deformity at follow up (group A, stage one). Incision and drainage was performed in a further 19 patients (31 per cent), resulting in minor residual deformity in one half (group A, stage two). Debridement was performed in 17 patients, and these patients had either gross (29 per cent) or minor residual deformity (71 per cent; group A, stage three). Six patients with perichondritis secondary to malignant otitis externa were managed by wound debridement via a post-auricular approach; all had minor residual deformities.

Conclusions: Perichondritis can be divided into two groups, depending on cartilage loss and on the presence or absence of malignant otitis externa. The treatment used and the residual deformity that will ensue are entirely dependent on the stage of disease.

Objectives: To evaluate the predictive role of the audiometric Carhart's notch for the assessment of middle-ear pathology prior to surgical intervention.

Method: In this retrospective analysis, a total of 315 operated ears of 305 patients were evaluated regarding their pre-operative pure tone audiograms and peri-operative findings. The probable relationship between the middle-ear pathologies found and the Carhart's notch found on pre-operative pure tone audiometry was investigated. Patients with conductive hearing loss who obtained at least a 10 dB improvement (at 1 and 2 kHz frequencies) in their bone conduction threshold post-operatively were included in the Carhart's notch group. The pathologies underlying Carhart's notch were compared.

Results: Three hundred and fifteen ears of 305 consecutive patients with conductive hearing loss were operated on due to middle-ear pathology. In patients with otosclerosis and tympanosclerosis, a Carhart's notch was seen at 2 kHz in 28 (93 per cent) patients but at 1 kHz in only two (7 per cent). However, in patients with chronic otitis media, a Carhart's notch was seen at 1 kHz in 10 (55 per cent) patients and at 2 kHz in eight (45 per cent) patients.

Conclusions: Otitis media with effusion, tympanosclerosis and congenital malformations should be considered in the differential diagnosis of a patient with a Carhart's notch seen on pure tone audiometry. A Carhart's notch at 2 kHz indicates stapes footplate fixation, whereas one at 1 kHz indicates a mobile stapes footplate; the footplate mobility can thus be predicted pre-operatively.

For fifteen years oral ciprofloxacin has been the standard treatment for malignant otitis externa, a sometimes fatal osteomyelitis of the skull base usually caused by Pseudomonas aeruginosa. Resistance to ciprofloxacin is developing. Over a 16-month period, we saw five cases where malignant otitis externa progressed, with the development of cranial nerve palsies in four cases, despite oral ciprofloxacin. Prolonged intravenous antibiotic therapy became necessary. One case was managed largely as an out-patient, but four patients spent many weeks in hospital. Only two cases had diabetes and this was monitored and controlled. Pseudomonas aeruginosa was isolated in four of the five cases, but antibiotic sensitivity to ciprofloxacin was not determined. In one case a later isolate was tested and found to be ciprofloxacin resistant. Progress was monitored by serial C-reactive protein (CRP) and white cell count. For diagnosis and assessing response to treatment we considered serial magnetic resonance imaging or computed tomography more useful than isotope bone scan. There must be a readiness to use intravenous antibiotics, as a response to ciprofloxacin can no longer be assumed. Bacterial isolates must be tested for sensitivity to antibiotics including ciprofloxacin, and further biopsy and culture are essential if treatment fails.

Introduction: Epistaxis is the most common nasal emergency and if nasal packing is required this commonly results in admission.

Methods: A literature search could find no published (UK) protocols for the management of this common condition in accident and emergency (A&E) departments. This paper presents a retrospective review of 116 patients with epistaxis, following implementation of the new peer reviewed protocol in June 2004.

Results: Apart from cautery, 62 had nasal packing inserted. Only 17 required admission. Forty-six patients were discharged with nasal packing in situ and only seven (16 per cent) returned due to bleeding. The overall return rate was 11 per cent.

Discussion: We feel this is a safe and logical protocol. Compared to mandatory admission after nasal pack insertion, we saved 39 admissions in five months. There were also the added benefits to patients of being able to recuperate at home rather than in hospital and avoidance of the risk of hospital acquired infection.

Background: Submucosal resection is accompanied by significant post-operative pain and discomfort. The aim of this randomized, double-blinded clinical trial was to study the efficacy of a local block anaesthetic, delivered after induction of general anaesthesia, in reducing post-operative pain.

Methods: Patients aged 16 years and over who were scheduled for elective submucosal resection were randomly assigned to receive either standardized general anaesthesia, general anaesthesia with local anaesthetic infiltration or general anaesthesia with placebo infiltration. Haemodynamic stability, intra-operative blood loss, post-operative pain (over a seven day follow-up period), analgesics consumption, hospital stay, and the patient's and surgeon's levels of satisfaction were assessed.

Results: We found significantly lower results for pack removal pain score, volume of intra-operative blood loss, number of patients suffering from headache, altered dental sensation or nasal pain, number of patients who consumed analgesics, and length of hospital stay, comparing the infiltration group with the general anaesthesia and placebo groups (p<0.05).

Conclusion: This clinical trial showed that infiltration with the local anaesthetics fentanyl and clonidine substantially reduced post-operative pain and shortened patients' hospital stay.

We present an unusual case of bilateral aural symptoms in a 34-weeks pregnant woman with Hughes syndrome. This report underscores the unpredictable nature of this syndrome and its ENT complications. No previous case of fluctuating hearing loss and otorrhoea in Hughes syndrome has been described.

It is well known that cholesteatoma is three-dimensional; hence, we feel that its surgical management requires a three-dimensional approach in order to achieve the best curative and functional results. Retraction pockets are undeniably caused by chronic and recurrent eustachian tube obstruction. However, we found that the presence of a large mastoid antrum was an important, additional aetiological factor in the formation of a retraction pocket and its progression to cholesteatoma formation, with bone destruction and subsequent complications.

Canal wall down tympanomastoidectomy – the ‘on-disease’ approach – is an innovative, three-dimensional technique based on universally accepted surgical principles. We modified the technique to ensure complete exposure and thereby eradication of the disease, with a resultant small cavity. Working in a three-dimensional field, we began drilling at the posterior meatal wall, lowering it while simultaneously widening the cavity as the mastoid was drilled to reach the antrum and the aditus. The bridge was lowered and the incus removed to completely expose the entire disease. The facial ridge was debulked and the temporalis fascia graft placed so as to simplify the middle-ear cleft.

We present a comprehensive report of this technique, based upon 600 patients studied retrospectively over a five-year period. After one-year follow up, 546 patients had a dry, healed cavity.

Canal wall down tympanomastoidectomy performed by the on-disease approach ensures complete eradication of the disease, with excellent curative as well as functional results.

Acoustic shock is a recently recognised clinical entity: following an abrupt, intense and unanticipated acoustic stimulus, usually delivered by a telephone handset or headset, some individuals report a symptom cluster that includes otalgia, altered hearing, aural fullness, imbalance, tinnitus, dislike or even fear of loud noises, and anxiety and/or depression. Symptoms start shortly after the triggering acoustic incident and can be short-lived or can last for a considerable time. If persistent, the condition can lead to significant disability. Proposed mechanisms include involvement of the tensor tympani muscle, hyperexcitability of central auditory pathways, and a precursive state of raised anxiety or arousal. A formal treatment programme has not yet been proposed, but the potential utility of modern therapeutic techniques for tinnitus and hyperacusis are considered. Given the large number of UK residents working in telephone call centres, this condition is of considerable clinical importance.

Objectives: We aimed to identify the frequency with which the following conditions were present as a second disability in cochlear-implanted, prelingually deaf persons: mild and moderate mental retardation; learning disability; attention deficit/hyperactivity disorder; cerebral palsy; congenital blindness; and autism. We also aimed to document the development of auditory perception in patients having one of these additional disabilities.

Study design: A retrospective study was designed to pursue the above aims.

Methods: We examined the records of 398 cochlear-implanted, prelingually deaf patients who had received a cochlear implant at least one year previously. Patients were selected who showed a delay in motor, cognitive or emotional development. The selected cases were referred for psychological evaluation in order to identify patients with additional disabilities. We then compared these patients' auditory perception prior to and one year following cochlear implantation.

Results: A total of 60 (15 per cent) cochlear-implanted, prelingually deaf patients were diagnosed with additional disabilities. These were classified as: mild mental retardation in eight cases (13.33 per cent); moderate mental retardation in five (8.33 per cent); learning disability in 20 (33.33 per cent); attention deficit/hyperactivity disorder in 15 (25 per cent); cerebral palsy in five (8.33); congenital blindness in three (5 per cent); and autism in four (6.66 per cent). All patients showed significant development in speech perception, except for autistic and congenitally deaf-blind patients.

Conclusion: Although cochlear implantation is not contraindicated in prelingually deaf persons with additional disabilities, congenitally deaf-blind and autistic patients showed limited development in auditory perception as a main outcome of cochlear implantation. These patients require unique rehabilitation in order to achieve more auditory development.

‘User-friendliness’ is an important factor in the choice of ossicular prosthesis. The current titanium prostheses have a flat, open head plate and are designed to sit under the tympanic membrane. Previously, the author had designed titanium prostheses with a malleus notch extension at the head plate. The present study aimed to assess whether these customised prostheses were user-friendly, compared with conventional prostheses.

Fourteen surgeons were recruited to examine the user-friendliness of several ossicular prostheses. They performed ossiculoplasties on temporal bones and rated the user-friendliness of the malleus notch prosthesis against that of some of the more popular conventional ossicular prostheses.

For malleus-stapes assembly, eight out of 13 surgeons preferred the malleus notch prosthesis to the Düsseldorf and Goldenberg designs. For malleus-footplate assembly, six out of 10 surgeons preferred the malleus notch prosthesis to the Düsseldorf and Richards designs. Most of the surgeons stated that the reconstruction was more stable using the malleus notch prosthesis.

The XIth cranial nerve or accessory nerve provides the motor supply to the sternocleidomastoid and trapezius muscles. It is frequently encountered during neck surgery, and as such is at risk of iatrogenic injury, resulting in the ‘shoulder syndrome’. Historically, the nerve was sacrificed on oncological grounds during radical neck dissection. However, the basis for sacrifice is unfounded in the majority of cases, and accessory nerve sparing selective neck dissection has equal oncological efficacy. The path of the nerve in the neck is very variable, and there is not a wholly reliable landmark for its identification. However, there are a number of methods described in the literature to guide the surgeon in its identification. This paper provides a systematic review of all the methods available for identification of the accessory nerve, and comments on the reliability of each. In doing so, the detailed anatomy of the accessory nerve is also described.

We aimed to investigate whether auditory brainstem response (ABR), assessed immediately after grommet insertion, was an accurate measure of hearing threshold.

Ten children were recruited (20 ears). Patients underwent transient evoked otoacoustic emission (TEOAE) and ABR tests before and after grommet insertion under general anaesthetic. A third assessment was conducted at a six-week review, without anaesthetic or sedation. The hearing threshold in dB and the presence of TEOAE were recorded by senior audiologists. The results of each investigation were compared. At review, only 15 ABR and 17 TEOAE tests, of a possible 20, were recorded.

Transient evoked otoacoustic emissions were recorded in 30 per cent of ears pre-operatively, 20 per cent of ears post-operatively and 82 per cent of ears at six-week review. The average ABR thresholds were 23.75 dB (range 10–45 dB) pre-operatively, 27.25 (range 15–40 dB) post-operatively and 15 dB (range 5–25 dB) at six-week review. Comparing pre- and post-operative assessment, decreased thresholds were seen in six ears and elevated thresholds in 11. Comparing the six-week review ABR with the post-operative ABR, 13 ears had decreased thresholds, by as much as 20 dB. Thresholds of 35 dB or greater were measured in six ears immediately after grommet insertion (maximum 40 dB). Threshold shifts of 15 dB or greater was observed in seven ears. The intra-subject variation was shown to be significant (p<0.001) when repeated measures analysis of variance was applied.

We conclude that there is evidence that ABR can be inaccurate after grommet insertion, overestimating hearing thresholds. We believe that the mechanism by which the error is realised is temporary threshold shift.