Mucinoses constitute a diverse group of disorders that have in common the deposition of basophilic, finely granular and stringy material in the connective tissues of the dermis (known as dermal mucinoses); this also can occur in the hair follicles (follicular mucinoses) and finally in the epidermis (epithelial mucinoses). We present the clinical and histological findings of a case of focal mucinosis in a 76-year-old man who was otherwise fit and healthy. A literature review revealed a sparse number of such cases none of which was reported in the ENT literature. Focal cutaneous mucinosis is a benign condition but the authors believe it isof local and systemic interest to the Otolaryngologist as an addition to his or her armentariumof diagnoses.

The first report of a patient with metastatic bronchogenic carcinoma of the skin surrounding the abutment of a bone anchored hearing aid (BAHA) is presented. Complications of bone anchored hearing implantation have been well documented to date. We present a 68-year-old lady who presented with an unusual skin lesion surrounding the abutment of her BAHA. This was the first presentation of her bronchogenic tumour.

We also review the literature regarding cutaneous metastasis and complications of BAHA.

The purpose of this questionnaire study was to evaluate the existing knowledge of binaural hearing and the attitudes and practices of prescribing bilateral hearing aids amongst otolaryngologists in the United Kingdom. Of the 950 questionnaires sent to the current members of the British Association of Otolaryngologists and Head and Neck Surgeons (BAO-HNS), there were 591 respondents (62 per cent). The true response rate with completed questionnaires was 59 per cent. Eighty-one per cent of the respondents were aware of the importance of binaural hearing and had a positive attitude towards binaural fitting. The practice of bilateral hearing aid prescriptions was found to be poor amongst all grades on the NHS (less than 10 per cent of all hearing aid prescriptions). This practice in the private sector was variable, dependent largely on patient preference and affordability. The practice of binaural prescription was higher for patients in the paediatric age group than amongst adults. Two common indications for hearing aid prescriptions for unilateral deafness were otitis media with effusion in children (23 per cent of respondents) and for tinnitus masking in adults (12 per cent of respondents). Many otolaryngologists believed that there was not enough evidence to support bilateral bone-anchored hearing aid implantation and bilateral cochlear implantation. Ninety-four per cent of the respondents believed that binaural hearing was as important as binocular vision.

Over a 12-year period, the Birmingham implantation otology unit has implanted more than 300 patients with bone-anchored hearing aids (BAHA).

The Entific Medical Systems questionnaire was administered to these patients to evaluate the day to day use of the BAHA, professional needs, after-care, wear and tear concerns and service related issues. Data analysis revealed that most patients used their BAHA for more than eight hours a day (90 per cent of BAHA users) and every day of the week (93 per cent of BAHA users). A high degree of satisfaction was expressed as regards sound amplification, listening to radio or television news, listening to music, speech perception in quiet conditions, during conversation with one person in noisy surroundings and conversation with family at home. Some degree of difficulty was expressed with the use of the BAHA during conversation with two or more people in noisy surroundings. A slow process of perceptual acclimatization was noticed with the majority of the patients. The majority of patients were pleased with the service as regards care of the wound, BAHA nursing clinics, device repairs and other service-related issues.

The Birmingham bone-anchored hearing aid (BAHA) programme has fitted more than 300 patients with unilateral bone-anchored hearing aids since 1988. Some of the patients who benefited well with unilateral aids and who had used bilateral conventional aids previously applied for bilateral amplification. To date, 15 patients have been fitted with bilateral BAHAs. The benefits of bilateral amplification have been compared to unilateral amplification in 11 of these patients. Subjective analysis in the form of validated comprehensive questionnaires was undertaken.

The Glasgow benefit inventory (GBI), which is a subjective patient orientated post-interventional questionnaire developed to evaluate any otorhinolaryngological surgery and therapy was administered. The results revealed that the use of bilateral bone-anchored hearing aids significantly enhanced general well being (patient benefit) and improved the patient’s state of health (quality of life). The Chung and Stephens questionnaire which addresses specific issues related to binaural hearing was used. Our preliminary results are encouraging and are comparable to the experience of the Nijmegen BAHA group.

The Birmingham osseointegration programme began in 1988 and during the following 10 years there were a total of 351 bone-anchored hearing aid (BAHA) implantees. In the summer of 2000, a postal questionnaire study was undertaken to establish the impact of the bone-anchored hearing aid on all aspects of patients’ lives.

We used the Glasgow benefit inventory (GBI), which is a subjective patient orientated post-interventional questionnaire especially developed to evaluate any otorhinolaryngological surgery and therapy. It is maximally sensitive to any change in health status brought about by a specific event: in this case the provision of a BAHA.

A total of 312 bone-anchored hearing aid patients, who had used their aids for a minimum period of six months, were sent GBI questionnaires. Two hundred and twenty-seven questionnaires were returned and utilized in the study. The results revealed that the use of a bone-anchored hearing aid significantly enhanced general well being (patient benefit), improved the patient’s state of health (quality of life) and finally was considered a success by patients and their families.

By spring 2000, a total of 351 patients were implanted in the Birmingham bone-anchored hearing aid (BAHA) programme. This group consisted of 242 adults and 109 children. The aim of this retrospective questionnaire study was to directly assess patient satisfaction with their current bone-anchored hearing aid in comparison with their previous conventional air and/or bone-conduction hearing aids.

The Nijmegen group questionnaire was sent by post to 312 patients who used their BAHA for six months or longer. The questionnaire used was first described by Mylanus et al. (Nijmegen group) in 1998. The total response rate was 72 per cent (227 of 312 patients). The bone-anchored hearing aid was found to be significantly superior to prior conventional hearing aids in all respects.

The Birmingham bone-anchored hearing aid programme began in 1988 and by autumn 2000 a total of 351 patients had been fitted with such an aid. The aim of this study was to assess the effectiveness of hearing rehabilitation with the bone-anchored hearing aid. This was a prospective interview-based questionnaire study carried out in the autumn 2000. A total of 84 adult patients were interviewed. Each patient had worn their BAHA for more than one year.

The questionnaire used during these interviews was the Glasgow hearing aid benefit profile (GHABP) and the Glasgow hearing aid difference profile (GHADP). This was first derived and validated by Gatehouse in 1999. The use of bone-anchored hearing aids was found to reduce the level of disability and handicap and provided the most patient benefit and satisfaction.

The Birmingham bone-anchored hearing aid (BAHA) programme, since its inception in 1988, has fitted more than 300 patients with unilateral bone-anchored hearing aids. Recently, some of the patients who benefited extremely well with unilateral aids applied for bilateral amplification. To date, 15 patients have been fitted with bilateral BAHAs. The benefits of bilateral amplification have been compared to unilateral amplification in 11 of these patients who have used their second BAHA for 12 months or longer. Following a subjective analysis in the form of comprehensive questionnaires, objective testing was undertaken to assess specific issues such as ‘speech recognition in quiet’, ‘speech recognition in noise’ and a modified ‘speech-in-simulated-party-noise’ (Plomp) test.

‘Speech in quiet’ testing revealed a 100 per cent score with both unilateral and bilateral BAHAs. With ‘speech in noise’ all 11 patients scored marginally better with bilateral aids compared to best unilateral responses. The modified Plomp test demonstrated that bilateral BAHAs provided maximum flexibility when the origin of noise cannot be controlled as in day-to-day situations. In this small case series the results are positive and are comparable to the experience of the Nijmegen BAHA group.

Maffucci’s syndrome is a rare congenital non-inherited condition, characterized by multiple enchondromata, cutaneous haemangiomata and more recently spindle-cell haemangioma-endotheliomata. It is associated with an increased risk of malignancy including intracranial chondrosarcomas. Early diagnosis is crucial; screening patients with Ollier’s disease is recommended.

The treatment of choice for these intracranial cartilaginous tumours is complete surgical excision but this is often difficult to achieve due to difficult access and relationships with neurovascular structures. An alternative treatment is proton-beam therapy.

We report a case of Maffucci’s syndrome; illustrating the typical clinical and radiological features as well as the known complications of the condition.

Facial nerve haemangioma is a rare benign neoplasm accounting for 0.7 per cent of all tumours involving the temporal bone. The diagnosis of a facial nerve tumour is often missed or delayed. Early diagnosis is imperative as it influences the eventual outcome for facial nerve function. Prognosis is related to the size of the tumour, the severity and the duration of pre-operative paralysis. The definitive diagnosis of a facial nerve tumour rests exclusively with high resolution imaging of the temporal bone using enhanced magnetic resonance imaging (MRI) and thin-sectioned computed tomography (CT). This case emphasizes the crucial role that high quality imaging can play in the diagnosis of facial nerve tumours, and elegantly illustrates the imaging features of facial nerve haemangiomas.