An evidence-based approach to the 12-min consultation for a child with Down's syndrome.

BACKGROUND: Down's syndrome is the commonest genetic condition. ENT problems are common in these children and can affect their development and quality of life. METHOD: English literature review and experience of the senior author in managing ENT problems in children and adults with Down's syndrome. SEARCH STRATEGY: We carried out a Medline search on 31/03/2012 of English language publications using the following keywords: Down/Down's syndrome, hearing loss, ear infections, sleep apnoea, thyroid. RESULTS: The prevalence, presenting features and management of many common ENT conditions are significantly different in children who have Down's syndrome. This is particularly true of otitis media with effusion and sleep-disordered breathing. CONCLUSION: The outpatient consultation for a child with Down's syndrome should assess a range of specific ENT problems affecting this patient group including hearing issues, sleep-disordered breathing, recurrent upper respiratory tract infections, as well as other health conditions like hypothyroidism, atlantoaxial instability and cardiac issues, which can potentially affect the management of the patient.

Bone-anchored hearing aids for people with bilateral hearing impairment: a systematic review.

BACKGROUND: Bone-anchored hearing aids (BAHAs) are indicated for people with conductive or mixed hearing loss who can benefit from amplification of sound. In resource limited health care systems, it is important that evidence regarding the benefit of BAHAs is critically appraised to aid decision-making. OBJECTIVE OF REVIEW: To assess the clinical effectiveness of BAHAs for people with bilateral hearing impairment. TYPE OF REVIEW: Systematic review. SEARCH STRATEGY: Nineteen electronic resources were searched from inception to November 2009. Additional studies were sought from reference lists, clinical experts and BAHA manufacturers. EVALUATION METHOD: Inclusion criteria were applied by two reviewers independently. Data extraction and quality assessment of full papers were undertaken by one reviewer and checked by a second. Studies were synthesised through narrative review with tabulation of results. RESULTS: Twelve studies were included. Studies suggested audiological benefits of BAHAs when compared with bone-conduction hearing aids or no aiding. A mixed pattern of results was seen when BAHAs were compared to air-conduction hearing aids. Improvements in quality of life with BAHAs were found by a hearing-specific instrument but not generic quality of life measures. Issues such as improvement of discharging ears and length of time the aid can be worn were not adequately addressed by the studies. Studies demonstrated some benefits of bilateral BAHAs. Adverse events data were limited. The quality of the studies was low. CONCLUSIONS: The available evidence is weak. As such, caution is indicated in the interpretation of presently available data. However, based on the available evidence, BAHAs appear to be a reasonable treatment option for people with bilateral conductive or mixed hearing loss. Further research into the benefits of BAHAs, including quality of life, is required to reduce the uncertainty.

Bone-anchored hearing aids (BAHAs) for people who are bilaterally deaf: a systematic review and economic evaluation.

BACKGROUND: A bone-anchored hearing aid (BAHA) consists of a permanent titanium fixture, which is surgically implanted into the skull bone behind the ear, and a small detachable sound processor that clips onto the fixture. BAHAs are suitable for people with conductive or mixed hearing loss who cannot benefit fully from conventional hearing aids. OBJECTIVES: To assess the clinical effectiveness and cost-effectiveness of BAHAs for people who are bilaterally deaf. DATA SOURCES: Nineteen electronic resources, including MEDLINE, EMBASE and The Cochrane Library (inception to November 2009). Additional studies were sought from reference lists and clinical experts. REVIEW METHODS: Inclusion criteria were applied by two reviewers independently. Data extraction and quality assessment were undertaken by one reviewer and checked by a second. Prospective studies of adults or children with bilateral hearing loss were eligible. Comparisons were BAHAs versus conventional hearing aids [air conduction hearing aid (ACHA) or bone conduction hearing aid (BCHA)], unaided hearing and ear surgery; and unilateral versus bilateral BAHAs. Outcomes included hearing measures, validated measures of quality of life (QoL), adverse events and measures of cost-effectiveness. For the review of cost-effectiveness, full economic evaluations were eligible. RESULTS: Twelve studies were included (seven cohort pre-post studies and five cross-sectional 'audiological comparison' studies). No prospective studies comparing BAHAs with ear surgery were identified. Overall quality was rated as weak for all included studies and meta-analysis was not possible due to differences in outcome measures and patient populations. There appeared to be some audiological benefits of BAHAs compared with BCHAs and improvements in speech understanding in noise compared with ACHAs; however, ACHAs may produce better audiological results for other outcomes. The limited evidence reduces certainty. Hearing is improved with BAHAs compared with unaided hearing. Improvements in QoL with BAHAs were identified by a hearing-specific instrument but not generic QoL measures. Studies comparing unilateral with bilateral BAHAs suggested benefits of bilateral BAHAs in many, but not all, situations. Prospective case series reported between 6.1% and 19.4% loss of implants. Most participants experienced no or minor skin reactions. A decision analytic model was developed. Costs and benefits of unilateral BAHAs were estimated over a 10-year time horizon, applying discount rates of 3.5%. The incremental cost per user receiving BAHA, compared with BCHA, was £ 16,409 for children and £ 13,449 for adults. In an exploratory analysis the incremental cost per quality-adjusted life-year (QALY) gained was between £ 55,642 and £ 119,367 for children and between £ 46,628 and £ 100,029 for adults for BAHAs compared with BCHA, depending on the assumed QoL gain and proportion of each modelled cohort using their hearing aid for ≥ 8 or more hours per day. Deterministic sensitivity analysis suggested that the results were highly sensitive to the assumed proportion of people using BCHA for ≥ 8 hours per day, with very high incremental cost-effectiveness ratio values (£ 500,000-1,200,000 per QALY gained) associated with a high proportion of people using BCHA. More acceptable values (£ 15,000-37,000 per QALY gained) were associated with a low proportion of people using BCHA for ≥ 8 hours per day (compared with BAHA). LIMITATIONS: The economic evaluation presented in this report is severely limited by a lack of robust evidence on the outcome of hearing aid provision. This has lead to a more restricted analysis than was originally anticipated (limited to a comparison of BAHA and BCHA). In the absence of useable QoL data, the cost-effectiveness analysis is based on potential utility gains from hearing, that been inferred using a QoL instrument rather than measures reported by hearing aid users themselves. As a result the analysis is regarded as exploratory and the reported results should be interpreted with caution. CONCLUSIONS: Exploratory cost-effectiveness analysis suggests that BAHAs are unlikely to be a cost-effective option where the benefits (in terms of hearing gain and probability of using of alternative aids) are similar for BAHAs and their comparators. The greater the benefit from aided hearing and the greater the difference in the proportion of people using the hearing aid for ≥ 8 hours per day, the more likely BAHAs are to be a cost-effective option. The inclusion of other dimensions of QoL may also increase the likelihood of BAHAs being a cost-effective option. A national audit of BAHAs is needed to provide clarity on the many areas of uncertainty surrounding BAHAs. Further research into the non-audiological benefits of BAHAs, including QoL, is required.

UK and Ireland experience of cochlear implants in children with Down syndrome.

OBJECTIVES: Down Syndrome (DS) is associated with a high incidence of hearing loss. The majority of hearing loss is conductive, but between 4 and 20% is sensorineural, which in the main is mild or moderate and is managed with conventional behind-the-ear hearing aids. Cochlear implantation is an elective invasive procedure, performed to provide some form of hearing rehabilitation in individuals with severe to profound sensorineural hearing loss, and initially candidacy criteria were strict--excluding patients with additional disabilities. With good results and expanding experience, more candidates with additional disabilities are now being implanted. A survey of UK and Ireland Cochlear Implant Programmes sought to identify the number of individuals with DS who have been implanted with a cochlear implant (CI) and to provide relevant information on outcomes of implantation in these individuals. METHODS: E-mail survey of all programmes within the British Cochlear Implant Group (BCIG). Postal questionnaire to programmes identified to have implanted a child with Down Syndrome, with data collection on pre-operative assessment, surgical and post-operative outcomes. Case series review. RESULTS: Three of 23 BCIG programmes have implanted a child with Down Syndrome. Four children have received implants. No intraoperative or post-operative surgical complications were encountered. All children had middle ear disease, but no problems with implantation were associated with their middle ear condition. All children remain implant users, 12 months to 4 years post-implantation. CONCLUSION: Cochlear implantation is an option for a child with Down Syndrome and associated severe to profound sensorineural hearing loss. Clinicians caring for these children and their families should consider referral for assessment by a Cochlear Implant Programme.

An approach to bilateral bone-anchored hearing aid surgery in children: contralateral placement of sleeper fixture.

OBJECTIVE: Bone-anchored hearing aid surgery in younger children is a two-stage procedure, with a titanium fixture being allowed to osseointegrate for several months before an abutment is fitted through a skin graft. In the first procedure, it has been usual to place a reserve or sleeper fixture approximately 5 mm from the primary fixture as a backup in case the primary fixture fails to osseointegrate. This ipsilateral sleeper fixture is expensive, is often not used, and is placed in thinner calvarial bone where it is less likely to osseointegrate successfully. The authors have implanted the sleeper fixture on the contralateral side, with the additional objective of reducing the number of procedures for bilateral bone-anchored hearing aid implantation, providing a cost-effective use for the sleeper. METHODS: The authors implanted the bone-anchored hearing aid sleeper fixture in the contralateral temporal bone instead of on the ipsilateral side in seven successive paediatric cases with bilateral conductive hearing loss requiring two-stage bone-anchored hearing aids, treated at the Royal Manchester Children's Hospital, UK. RESULTS: The seven patients ranged in age from five to 15 years, with a mean age of 10 years; in addition, a 20-year-old with learning disability was also treated. In each case, the contralateral sleeper fixture was not needed as a backup fixture, but was used in four patients (57 per cent) as the basis for a second-side bone-anchored hearing aid. CONCLUSIONS: In children with bilateral conductive hearing loss, in whom a bilateral bone-anchored hearing aid is being considered and the second side is to be operated upon at a later date, we recommend placing the sleeper fixture on the contralateral side at the time of primary first-side surgery. Our technique provides a sleeper fixture located in an optimal position, where it also offers the option of use for a second-side bone-anchored hearing aid and reduces the number of procedures needed.

Bone anchored hearing aids: a preliminary assessment of the impact on outpatients and cost when rehabilitating hearing in chronic suppurative otitis media.

OBJECTIVES: To compare the difference in ENT and Audiology visits, treatments dispensed and potential savings pre- and post-bone anchored hearing aid (BAHA) insertion in patients with chronic suppurative otitis media exacerbated by behind the ear hearing aids. DESIGN: A retrospective pilot study. SETTING: District General Hospital. PARTICIPANTS: All patients who had BAHA inserted from January 2001 to January 2006. PARAMETERS: Age, gender, number of visits per month, treatments per month dispensed from the ENT and Audiology Departments and direct and limited indirect medical costs pre- and post-BAHA insertion. RESULTS: Twelve of 26 (46%) adult patients had BAHA inserted over the 5-year period for CSOM. The male to female ratio was 1 : 3 with a median age of 61 (range 29-81). The number of visits and treatments dispensed per month in the ENT Department fell from a mean of 0.42-0.33 (P < 0.08) and 0.22-0.14 (P < 0.02) respectfully. When the difference in medical cost was taken into account BAHA offered a potential saving of pound 627.80 per patient. CONCLUSION: Although the initial acquisition of surgical equipment and BAHA sound processors is expensive, there is a reduction in the number of treatments and visits required for patients with chronic suppurative otitis media after BAHA is inserted leading to a reduction in average costs.

Parental satisfaction with health services provided to children with Down syndrome in north-west England: an ENT perspective.

AIMS: To evaluate parents' satisfaction with medical and allied health services provided to children with Down syndrome in north-west England, comparing ENT and its allied services with other areas of health service provision. METHODS: A questionnaire survey of parents attending a north-west England Down syndrome association conference. Demographic data, departments visited, satisfaction with each service (scored one to five), waiting times for each service (scored one to five), service need (scored one to three) and accessibility (scored one to three) were recorded. RESULTS: Otolaryngology had been used by 50 per cent of children, with a satisfaction of 2.63 (the second worst score). Speech and language therapy was used by 90 per cent of the children, with a satisfaction of 3.26 (the worst score). The service felt to be most needed and also most difficult to access was speech and language therapy. CONCLUSION: Otorhinolaryngology departments should assess how they can improve their service to this population with specific ENT needs. Speech and language services for children with Down syndrome should be expanded.