Ann-Louise McDermott

The birmingham pediatric bone-anchored hearing aid program: a 15-year experience.

Objective: To evaluate the complication rates and outcomes of children who were fitted with a bone-anchored hearing aid (BAHA) on the Birmingham BAHA program. Study Design: Retrospective case analysis of clinical records of all children implanted at Birmingham Childrens Hospital since the beginning of the program in 1992 until February 2007. Patients: A total of 182 children younger than 16 years old fitted with a BAHA. Of these children, 107 had a significant medical history. Results: Surgery was performed as a 2-stage procedure in 174 children. The healing time was between 3 and 4 months in 112 (64%) cases. Single-stage surgery was performed in 8 cases. Implant failures were 14% of 230 loaded fixtures (32 fixtures lost in total). Multiple-fixture failures (18 fixture failures) occurred in 7 patients. Adverse skin reactions appeared in 34 (17%) patients during a 15-year follow-up period. Revision surgery was undertaken in 14 (8%) cases because of skin overgrowth around the abutment. Five of these cases required multiple surgical skin reductions. Conclusion: The Birmingham Program has a high proportion of syndromic patients with complex medical problems. The fixture failure rate was found to be 14%. This included the multiple-fixture failures in children younger than 3 years old. There was 1 serious complication. The BAHA is a reliable and effective treatment for selected patients. Our program currently has 97% of its children wearing their BAHA on a daily basis with continuing audiologic benefit.

Maffucci's syndrome : clinical and radiological features of a rare condition

Maffuccis 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 Olliers 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 Maffuccis syndrome; illustrating the typical clinical and radiological features as well as the known complications of the condition.

Quality of life in children fitted with a bone-anchored hearing aid.

Objectives: To evaluate the self-rated quality of life and benefits associated with the use of a Bone-Anchored Hearing Aid (BAHA). To assess any change in health status after a BAHA. Study Design: This was a retrospective postal questionnaire study. The Glasgow Childrens Benefit Inventory was the validated tool used. Children with a BAHA on the Birmingham pediatric Bone-Anchored Hearing Aid Programme from February 1992 to February 2007 were included. Patients: One hundred fifteen children were sent a postal questionnaire. Eighty-four were returned, giving a response rate of 73%. All children had worn their BAHA for more than 6 months. Children with an implant-retained auricular prosthesis were excluded from the study, as were children who were older than 16 years at the time of the questionnaire. Patients with bilateral BAHA were advised to answer the questionnaire with reference to their first BAHA. Results: The BAHA was a success in the pediatric population. All 84 children reported a positive benefit with their BAHA. The median benefit score was +54. No child demonstrated deterioration in health status after their BAHA. Conclusion: The use of a BAHA significantly enhanced general well-being, improved patient state of health (quality of life), and finally, was considered a success by patients and their families. This study demonstrates a significant benefit from BAHA as measured by the Glasgow Childrens Benefit Inventory.

An intra-individual comparison of the previous conventional hearing aid with the bone-anchored hearing aid: the Nijmegen group questionnaire

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.

Patient satisfaction with bilateral bone-anchored hearing aids: the Birmingham experience.

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 patients 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.

Quality-of-life outcomes after bone-anchored hearing device surgery in children with single-sided sensorineural deafness.

Objective To report our experience in a series of children with single-sided sensorineural deafness where a bone-anchored hearing device (BAHD) was used for auditory rehabilitation. Study Design Retrospective case review. Setting Tertiary referral centre. Patients Eight children (4 boys and 4 girls) who had BAHD surgery for single-sided sensorineural deafness between 2007 and 2010. Intervention(s) Bone-anchored hearing device was used for auditory rehabilitation. Main Outcome Measure(s) Glasgow Children’s Benefit Inventory (GCBI), Single-sided Deafness (SSD) Questionnaire and change in health benefit scores (visual analogue scale). Results All but one of the children showed a positive GCBI score; the child that reported a negative score was because of low self confidence and self-esteem issues secondary to bullying at school. The results of the SSD questionnaire were generally positive with a mean satisfaction score of the BAHD as 9/10. All the children had an improvement in heath benefit. Conclusion Our findings add further evidence to support patient perceived benefit of a BAHD in single sided sensorineural deafness in the paediatric population.

Bone-anchored hearing devices in children with unilateral conductive hearing loss: a patient-carer perspective.

Objectives: We sought to determine the outcome of implantation of a bone-anchored hearing device in children with unilateral conductive hearing loss. Methods: A retrospective case note analysis was used in a tertiary referral pediatric hospital to study 17 consecutive cases of pediatric patients with unilateral conductive hearing loss who were fitted with a bone-anchored hearing device between 2005 and 2010. Results: The average age of the patients at the time of bone-anchored hearing device fitting was 10 years 6 months (range, 6 years 3 months to 16 years). Qualitative subjective outcome measures demonstrated benefit. The vast majority of patients reported improved social and physical functioning and improved quality of life. All 17 patients are currently using their bone-anchored hearing device on a daily basis after a follow-up of 6 months. Conclusions: This study has shown improved quality of life in children with unilateral hearing loss after implantation of their bone-anchored hearing device. There was a high degree of patient satisfaction and improvement in health status reported by children and/or carers. Bone-anchored hearing devices have an important role in the management of children with symptomatic unilateral hearing loss. Perhaps earlier consideration of a bone-anchored hearing device would be appropriate in selected cases.

Day to day use and service-related issues with the bone-anchored hearing aid: the Entific Medical Systems questionnaire.

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 crucial role of imaging in detection of facial nerve haemangiomas.

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.

De novo laryngeal carcinoma in childhood

Numerous factors contribute towards a late diagnosis of laryngeal malignancy in childhood. These include its rarity, the similarity of its early symptoms to those of other benign, common childhood conditions as well as the relative difficulty encountered during paediatric laryngeal examination. We believe that these cases are of sufficient interest when they occur to warrant reporting since the consequences of late diagnosis in these young patients can be serious. We present a case of an 11-year-old boy with advanced squamous cell carcinoma of the larynx (T3N0M0), who was successfully treated with primary total laryngectomy and bilateral selective neck dissections to avoid the potential additional morbidity of radical radiotherapy.