The role of bone anchored hearing aids in children with Down syndrome

doi:10.1016/j.ijporl.2008.01.035

International Journal of Pediatric Otorhinolaryngology

Volume 72, Issue 6, June 2008, Pages 751-757

https://doi.org/10.1016/j.ijporl.2008.01.035 Get rights and content

Summary

Objectives

To evaluate complication rates and outcomes of children with Down syndrome fitted with a Bone Anchored Hearing Aid (Baha^®). To evaluate whether the Bone Anchored Hearing Aid is a successful form of aural rehabilitation in children with Down syndrome from a patients’ perspective.

Study design

Retrospective case analysis and postal questionnaire study.

Setting

The Birmingham Children's Hospital, UK.

Methods

A total of 15 children were fitted with a Baha^® between February 1992 and February 2007. The age range was 2–15 years. A postal questionnaire was sent to each family. The Glasgow Children's Benefit Inventory (GCBI) was used in this study.

Outcome measures

Implantation results, skin reactions and other complications were recorded. Quality of life after receiving a Baha^® was assessed with the GCBI.

Results

All 15 patients are using their Baha^® 7 days a week for more than 8 h a day after a follow-up of 14 months with continuing audiological benefit. No fixtures were lost, and skin problems were encountered in 3 (20%). Regarding quality of life, all 15 patients had improved social and physical functioning as a result of better hearing.

Conclusions

Baha^® has an important role in the overall management of individuals with Down syndrome after conventional hearing aids and/or ventilation tubes have been considered or already failed. This study has shown a 20% rate of soft tissue reaction and there were no fixture losses in this group. No significant increase in complication rates was identified in children with Down syndrome. Finally, there was a significantly improved quality of life in children with Down syndrome after receiving their Baha^®. There was a high patient/carer satisfaction with Baha^®. Two of our series had bilateral two stage fixture procedures without any complications. More consideration should be given to bilateral bone anchored hearing aids in this group.

Introduction

The Bone Anchored Hearing Aid (Baha^®) has been successfully used as a method of aural rehabilitation in adults with primarily chronic suppurative otitis media or difficulties with conventional aids. In the paediatric population, in addition to the above indications, the Baha^® has been successful with the management of hearing loss as a result of congenital abnormalities.

Pictures from 1505 have been found showing children with Down syndrome. It was formally recognised as a specific entity by an English physician, Dr. John Down. The syndrome now bears his name [1].

Down syndrome is the most common congenital condition affecting around 1 in 600 to 1 in 900 live births worldwide. The rate varies according to contraception and termination attitudes in different communities and countries [1]. It is currently estimated that there are 2 million people with Down syndrome worldwide and 50,000 of them are in the United Kingdom (UK) [2].

Hearing loss is a recognised problem in this group of patients, and this is most commonly otitis media with effusion (OME). If untreated, this may affect language and behaviour and so treatment should be instigated early for them to achieve their best potential [3].

Section snippets

Aim

The aim of this study was firstly, to evaluate complication rates and outcomes of children with Down syndrome fitted with a Baha^® and secondly, to evaluate whether the bone anchored hearing aid is a successful form of aural rehabilitation in these children from a patient/carer perspective.

Methods

A retrospective case analysis of all children with Down syndrome fitted with a Baha^® on the Birmingham Paediatric bone anchored hearing aid programme was undertaken. Records were available from February 1992 to February 2007. The outcome data included medical history, surgery, complications and post-operative follow-up.

A postal questionnaire was sent to each patient/family. The Glasgow Children's Benefit Inventory (GCBI) was the tool used in this study. A linear analogue scale was added to the

Outcomes

A total of 15 children with Down syndrome were fitted with a Baha^® between 1992 and 2007.

Age: The age range was 2–15 years. The mean age at implantation was 7 years and 4 months. There were 8 female and 7 males.

Indication: The most common indication for Baha^® provision was chronic suppurative otitis media (CSOM) (Fig. 1).

Previous interventions: Eleven children had had previous ventilation tubes. Seven of this group had more than one set. The remaining 4 children had extremely narrow ear canals.

Discussion

There is now considerable experience with the use of the Baha^® and there is no doubt it is an extremely valuable means of aural rehabilitation. Until recently, very little was reported about the use of the Baha^® in this group of children.

In children with Down syndrome hearing loss is a recognised problem. The incidence of hearing loss in this group has been reported to be as high as 78% [4]. It is predominantly OME. This is thought to result from a combination of a short, narrow and easily

Conclusions

Bone anchored hearing aids have been successfully used in the UK for more than 20 years, yet it is only in recent years that the role of the Baha^® in Down syndrome has been evaluated [1], [3], [4], [5], [6].

Their

Acknowledgements

Dr. Paul Davies, Senior Lecturer in Statistics, Birmingham University, for the statistical analysis of the data.

Miss Kate Rhodes and Miss Naomi Parish, for all their help with retrieval of patient records.

References (11)

P.Z. Sheehan et al.
UK and Ireland experience of bone anchored hearing aids (Baha) in individuals with Down syndrome
Int. J. Pediatr. Otorhinolaryngol.
(2006)
L. Tietze et al.
Utilization of bone-anchored hearing aids in children
Int. J. Pediatr. Otorhinolaryngol.
(2001)
The Downs Syndrome Association....
R.A. Joseph et al.
Hearing loss in children with Down syndrome
Int. J. Pediatr. Otorhinolaryngol.
(2001)
T.J. Balkany et al.
Hearing loss in Down's syndrome. A treatable handicap more common than generally recognized
Clin. Pediatr.
(1979)

There are more references available in the full text version of this article.

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Management outcomes of otitis media with effusion in children with down syndrome: A systematic review
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Three of the included studies assessed BAHAs as a treatment option in trisomy 21 subjects. In these studies, BAHAs were considered as an alternative method of hearing amplification and aural rehabilitation, after unsuccessful attempts with conventional hearing aids and PET tubes has failed to manage the hearing loss secondary to OME [4,24,25]. The total number of included patients was 18, with the mean age of implantation of 8.4 years [10,15].
To assesses the current state of uncertainty concerning the management options used for otitis media with effusion (OME) in children with trisomy 21.
A systematic review of adhering to the PRISMA statement of studies evaluating the management of OME in trisomy 21 children prior to September 2021 was conducted. Studies were identified using the following medical databases: PubMed, Google Scholar, CINAHL, Scopus and Medline. Data extraction was performed by screening of titles and abstracts based on eligibility criteria, followed by full-article analysis of selected records.
Twenty articles were included in this review. Studies showed conflicting outcomes regarding the different interventions used for OME in children with trisomy 21. Of those evaluating pressure equalizing tubes (PET), some studies report pronounced complication rates and recommend using a conservative approach unless complications arise and/or hearing loss is severe. In contrast, other studies reported significantly reduced complication rates and improved hearing with earlier intervention and adaptations to PETs. Hearing aids may be provided after multiple failed PETs.
Clinical equipoise still persists regarding the best method to manage children with trisomy 21 who have OME. Although PETs exhibited the lowest complication rates and highest improvement rates, further prospective trials are warranted to assess the various treatment modalities and determine which of them would provide the best outcome while reducing complications as well as the age of treatment.
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Implant survival and soft tissue outcomes can also be directly compared to a previous paediatric DS cohort from this same institution. In 2008 McDermott et al. followed up 15 patients over a 14-month period who were implanted using the narrower Brannemark flanged fixture system and demonstrated a no fixture failures, a 20% adverse skin response rate and a 6.7% revision surgery rate [17]. This previous work allows for a unique comparison being matched with an identical geographical catchment area, audiological and support team.
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A prospective, single-subject, repeat measure, cohort study in which each participant served as their own control. Consecutive paediatric patients 4yrs- 15 years old, with a primary diagnosis of Down syndrome (trisomy 21) were implanted between January 2015–January 2020 with BHX Oticon wide implants. Evaluation of soft tissue reactions, fixture failure rates and post implantation Implant stability Quotient (ISQ) at both fixtures and abutment levels were studied over a 12-month period. Data was analysed for statistical significance through mixed effect modelling with significance set at p = 0.01.
31 consecutive paediatric patients with a diagnosis of Down syndrome were implanted with 43 Ponto BHX Oticon™ implant system. Twelve children had bilateral implants and nineteen were unilateral. Over the 12 month follow up 2 fixtures (4.6%) were lost, and adverse skin reactions (Holgers >2) were recorded in 3.2% of all clinical reviews. Implant level stability quotient showed no statically significant change between first and second stage 71.1–71.7. Abutment level ISQ increased from 46.2 to 56.7 p = 0.0001 at the 12-month review point as compared to that recorded at loading.
Implant survival and adverse skin reactions were found to be in keeping with those in published literature and much improved compared to previous implant systems placed at this centre. Although abutment level ISQ showed an increase over the review period no correlation between this and implant loss can be concluded.
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The Oticon™ wide implant system was launched in 2009 and used at Birmingham Children's Hospital from 2014. To evaluate clinical outcomes of the Oticon™ wide implant (Oticon Medical), with a focus on skin complication rates and fixture loss over a 5-year period in a tertiary paediatric hospital in the UK.
Retrospective 5-year longitudinal case record review of 47 children who were implanted with the Oticon™ wide implant system at Birmingham Children's Hospital (BCH) between January 2014 and January 2016.
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The role of bone anchored hearing aids in children with Down syndrome

Summary

Objectives

Study design

Setting

Methods

Outcome measures

Results

Conclusions

Introduction

Section snippets

Aim

Methods

Outcomes

Discussion

Conclusions

Acknowledgements

Int. J. Pediatr. Otorhinolaryngol.

Int. J. Pediatr. Otorhinolaryngol.

Hearing loss in children with Down syndrome

Int. J. Pediatr. Otorhinolaryngol.

Hearing loss in Down's syndrome. A treatable handicap more common than generally recognized

Clin. Pediatr.