The first reported treatment of Nager syndrome associated hearing loss with bone-anchored hearing aids: case report.

OBJECTIVE: To report the first case of treatment of Nager syndrome associated conductive hearing loss with bone-anchored hearing aids, in a three-year-old boy. METHOD: Clinical case report and current literature review regarding the use of bone-anchored hearing aids in the treatment of conductive hearing loss in children. RESULTS: A three year eight month old boy with Nager syndrome was successfully treated for conductive hearing loss using bilateral bone-anchored hearing aids. CONCLUSION: This is the first case report of the use of bone-anchored hearing aids to treat Nager syndrome associated conductive hearing loss. Treatment was safe and successful in this case.

Bone-anchored hearing aids in children and young adults: the Freeman Hospital experience.

OBJECTIVE: To investigate the utilisation of bone-anchored hearing aids and Softband, as well as the effects on quality of life, amongst the paediatric and young adult population of Freeman Hospital, Newcastle Upon Tyne, UK. METHOD: Retrospective, anonymised, cross-sectional survey using the Glasgow Benefit Inventory and Listening Situation Questionnaire (parent version), administered at least three months following the start of bone-anchored hearing aid or Softband use. RESULTS: One hundred and nine patients were included, of whom syndromic children made up a significant proportion (22 of 109). Patients using bone-anchored hearing aids obtained significant educational and social benefit from their aids. The mean Listening Situation Questionnaire difficulty score was 17 (15 patients), which is below the trigger score of 22+ at which further reassessment and rehabilitation is required. 87% (of 15 patients) did not require further intervention. The overall mean GBI score for the 22 patients (syndromic and non-syndromic) was +29. CONCLUSION: The use of bone-anchored hearing aids and Softband results in significant improvements in quality of life for children and young adults with hearing impairment. There is significant under-utilisation of bone-anchored hearing aids in children with skull and congenital abnormalities, and we would advocate bone-anchored hearing aid implantation for these patients.

Bone-anchored hearing aid modified with directional microphone: do patients benefit?

BACKGROUND: Bone-anchored hearing aids are well established in the treatment of patients with a conductive or mixed hearing loss. However, one of the main problems is that of sound localisation. This can be improved with a directional microphone. This study compared the quality of life of bone-anchored hearing aid wearers before and after the use of a directional microphone. METHOD: Eleven patients were included. They were required to wear the directional microphone for 12 weeks. Quality of life was measured using the Glasgow benefit inventory questionnaire, before and after the study period. RESULTS: The response rate was 82 per cent. The total benefit from the directional microphone was +49.7. The three components of the Glasgow benefit inventory were analysed separately, as follows: general subscale +57.4; physical health +42.6; and social scale +25.9. CONCLUSION: This is the first study to demonstrate a significant improvement in quality of life from a directional microphone fitted to a bone-anchored hearing aid.

Magnetic resonance imaging: is a single scan ever enough for the diagnosis of acoustic neuroma?

A patient presented with unilateral, right-sided hearing loss and tinnitus and underwent gadolinium-enhanced magnetic resonance imaging (MRI). A pure tone audiogram showed a right-sided sensorineural hearing loss. The MRI scan was initially negative but when repeated seven years later, following a further deterioration of symptoms, it showed a 2 mm, right-sided acoustic neuroma. This case has great potential significance for the diagnosis of acoustic neuroma, and it may raise medico-legal issues regarding the exclusion of this diagnosis. The case illustrates that a single negative scan may not be adequate if pure tone audiograms show deterioration in hearing loss.

Intratympanic gentamicin for Ménière's disease: effect on quality of life as assessed by Glasgow benefit inventory.

OBJECTIVES: To evaluate patients' quality of life after receiving intratympanic gentamicin as treatment for Ménière's disease. DESIGN: Retrospective study using the Glasgow benefit inventory scale (GBI) questionnaire. PARTICIPANTS: Patients diagnosed with Ménière's disease, according to the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) diagnostic criteria (refractory to medical management), who were treated with intratympanic gentamicin injection. MATERIALS AND METHODS: This retrospective study included all patients diagnosed with Ménière's disease (according to AAO-HNS criteria; refractory to medical management) undergoing gentamicin labyrinthectomy in our unit over the preceding three years. Other causes of hearing loss were ruled out as all patients underwent magnetic resonance imaging, electronystagmography and sway magnetometry. Patients underwent gentamicin infiltration at a concentration of 2 ml of 30 mg/ml and were reviewed six weeks later and received a repeat injection if no benefit was evident. Six months after their last follow up, they were sent a GBI questionnaire. Twenty-one questionnaires were posted, and the response rate was 81 per cent. RESULTS: As per the responses received, the total benefit of intratympanic gentamicin injection was found to be +30.3. The three components of GBI were analysed separately; it was found that general benefit was greatest (+33.3), followed by physical benefit (+28.1) and social benefit (+21.6). CONCLUSION: This study suggests that gentamicin definitely improves quality of life in patients with Ménière's disease and should be the first line of treatment if medical management fails.

The effect of otitis media with effusions on balance in children.

There is increasing interest in the effect, otitis media with effusions (OME) has on the balance in children. The aim of our investigation was to determine whether a universal effect on balance could be demonstrated in children with OME by using sway posturography. Assessment was made in 20 children with proven OME before and after the insertion of bilateral ventilation tubes. Sway posturography was performed on each occasion in each of the four recording conditions in the presence and absence of both reduced optic fixation and reduced proprioception. The pathlength traversed during the recording interval was measured and analysed by a split-unit anova. The results demonstrate an overall improvement in mean pathlength of 20% following treatment of the effusions (P < 0.001) (95% CI 14-25%). The effect of reduced optic fixation and of reduced proprioception were similar when increasing the mean pathlength by 22% (P < 0.001). These results demonstrate that OME has a universal effect on balance in all recording conditions.