Experience of the 'Ear Glove' in children with microtia.

INTRODUCTION: Microtia is a congenital condition which can be found in isolation or as part of a syndrome. The key factors to consider when treating a child with microtia are hearing, speech and language development, cosmesis, and the psychological impact on the patient as well as the family. As children age and become more self-aware, the anxiety about transition from primary to secondary school can often be a trigger for carers and child to want a cosmetic solution at a younger age. Any form of cosmetic surgery ideally requires a child with an understanding of what is involved, as well as sufficient growth and anatomy to provide soft tissue resources for surgery. An additional issue for some children with microtia is the concern about adding to their already 'different' appearance by using a bone conduction solution/hearing implant. We present the outcomes of a novel non-surgical prosthesis 'Ear Glove' offered to pediatric patients with microtia. METHODS: Children with microtia are seen in the multidisciplinary outpatient clinic and reviewed by the team which includes an Otolaryngologist, Audiologist, Plastic surgeon and Maxillofacial prosthetist. When discussing cosmesis, all reconstruction options are explored. These include a 'no treatment' option, both adhesive and implant-retained prosthetic ears, and autologous and/or MedporⓇ ear reconstruction (age appropriate). All children who chose to undergo the adhesive non-surgical prosthetic option 'Ear Glove' for microtia were identified by our prosthetic department (n = 9), and their outcomes reviewed. RESULTS: Nine children have been fitted with the 'Ear Glove', with all 9 achieving excellent symmetry and aesthetics. Seven patients continue to successfully use their prostheses either daily or for special occasions. Two of these patients also decided to undergo bone anchored hearing implant surgery. One patient opted to change his treatment plans and chose 'no treatment' after feeling he preferred his 'little' ears. Finally, one patient reported the daily use of adhesive to be a deterrent. No skin reactions to the adhesive were reported in any patient. CONCLUSIONS: The 'Ear Glove' is increasingly being used by microtia patients in our department to good effect. This non-surgical alternative allows young patients to appreciate the cosmetic results of the surgical options before committing to an invasive procedure.

Clinical Evaluation of a Novel Laser-Ablated Titanium Implant System for Bone Anchored Hearing Systems in a Pediatric Population and the Relationship of Resonance Frequency Analysis With Implant Survival.

OBJECTIVE: To evaluate the clinical outcomes of pediatric patients implanted a novel 4.5 mm wide laser ablated titanium bone anchored implant system and to evaluate the implant stability over the first 12-month period. STUDY DESIGN: A prospective, single-subject, repeated measure, cohort study. Participants served as their own controls. SETTING: Community and tertiary referral hospital pediatric assessment center. PATIENTS: A total of 115 consecutive pediatric patients aged 4 to 15 years were implanted with 176 laser ablated titanium bone anchored implants from January 2016 to January 2019. MAIN OUTCOME MEASURE: Clinical outcomes, implant failure rates, and post implantation implant stability quotient (ISQ) scores were studied over the first 12-month period. Data were analyzed for statistical significance through mixed effect modeling, with the significance level p = 0.01. RESULTS: A median 12-month survival of 96.6% was observed. Six implants (3.5%) were lost in total, one of these (0.6%) was lost due to trauma. Adverse skin reactions (Holgers grade 2-4) were observed in 4.4% of all postoperative visits, occurring in 22 individuals (19.1%). Neither the ISQ high (ISQH) nor ISQ low (ISQL) values increased significantly between the stage 1 and 2 surgeries. In contrast, the ISQ results, irrespective of abutment size, demonstrated an increasing trend from 49.1 to 57 over the 12 months review period. A statistically significant change was only demonstrated from the 3 months follow up onwards. CONCLUSION: The use of 4.5 mm wide laser-ablated titanium bone anchored hearing implants resulted in superior survival rates and excellent clinical outcomes compared with previous implant systems.

Clinical evaluation and resonance frequency analysis of laser-ablated titanium bone-anchored hearing implant system in children with Down Syndrome.

OBJECTIVES: To evaluate complication rates and resonance frequency analysis (RFA) of the stability of a new laser-ablated titanium Bone Anchored hearing Implant system in children with Down syndrome. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Five year clinical outcomes and evaluation following implantation of the Oticon™ wide bone anchored hearing system in 47 children.

OBJECTIVES: 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. METHODS: 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. RESULTS: 47 children (27 M:20F) were implanted with 70 Oticon wide implants 23 bilateral, 27 unilateral. Mean age at the time of implantation was 9y 6 m. The follow up was for a mean of 5.4 years. Significant soft tissue complications requiring treatment was found in 11% (n = 8) of loaded fixtures, abutment tightening on two patients, abutment exchange 6% (n = 4) and a 10% (n = 7) fixture failure. CONCLUSION: The Oticon™ wide implant system produces favourable results with regards to peri-abutment skin complications, fixture stability and revision surgery rates when compared to similar cohorts of children studied at Birmingham Children's Hospital.

Longitudinal study of use of the pressure free, adhesive bone conducting hearing system in children at a tertiary centre.

OBJECTIVES: To assess the long-term compliance and usability of the non-implantable, adhesive bone conduction hearing aid system in children. Review of patient demographics, compliance and continued use. Identification of factors that impact on future patient selection. METHODS: Retrospective case series review of all children aged 5 and above fitted with the adhesive bone conduction hearing aid at a paediatric tertiary centre in the UK between 2015 and 2019. Data collected from medical and audiological records. Patient demographics, skin complications, patient feedback and changes in hearing aid provision were recorded. RESULTS: 82 children (40 female, 42 male) were provided with 89 adhesive hearing devices. To date 72 (87.8%) of the fitted patients, continue to use the device daily with minimal reported skin complications. Of the 10 patients that no longer use the adhesive aid, 5 no longer use any hearing device at all and the remaining 5 patients use an alternative hearing system. These include spectacle aids (n = 2) and bone anchored hearing implant (n = 3). CONCLUSION: Adhesive aid products are reported to provide comparable audiological results to the commercial softband hearing aids. They provide an excellent alternative in the treatment of conductive hearing loss without the costs and possible complications of a surgical intervention. A compliance rate of 87.8% of all patients fitted with the adhesive system demonstrates a high level of patient satisfaction. The device may also provide an appropriate stepping stone to implanted device once a child reaches the age in which an autonomous decision can be made. Limitations of the device have been the variability in the longevity of the adhesive adaptor and interference with headscarves, hats and glasses with a low frequency of transient minor skin reactions reported.

First Pediatric Experience With a Novel, Adhesive Adapter Retained, Bone Conduction Hearing Aid System.

OBJECTIVES: To assess the audiological outcomes, practicalities, and impact on quality of life of a new, nonimplantable, adhesive retained bone conduction hearing aid in children. STUDY DESIGN: A prospective, single-subject repeat measure, cohort study. SETTING: Community and in pediatric assessment center. PATIENTS: Twenty-one children aged between 5 and 15 years with a conductive hearing loss of >/=25 dB HL in the better hearing ear. INTERVENTION: Audiological comparisons were made using pure-tone thresholds; unaided, with a softband aid, and with the new adhesive retained bone conducting system. MAIN OUTCOME MEASURES: Comparison of hearing threshold levels. Data analysis via paired t-testing, significance set at p value <0.01. Quality of life was assessed via the Glasgow Children's Benefit Inventory and a 10 cm linear analogue scale. A hearing aid review questionnaire provided insight into practical use. RESULTS: Statistically significant improvement in thresholds of 7.3 dB HL (p=0.0001) was demonstrated with the adhesive system as compared with softband aids. After 4 weeks of usage, the mean hearing thresholds for the adhesive hearing system improved from 55 dB HL ±â€Š2.4 to 31 dB HL± 7.9 in unaided and aided conditions.Improvements in QOL were demonstrated with LAS and GCBI. Four children reported mild skin reactions. Eighty-six percent reported improved self-confidence. CONCLUSION: The adhesive aid produces comparable audiological results to the commercial softband hearing aids. It provides an excellent alternative in the treatment of conductive hearing loss without the possible complications and costs of a surgical intervention. Furthermore, it preserves skin envelope over the mastoid for those who wish to proceed with an autologous pinna reconstruction in the future.

Clinical outcomes following Cochlear™ BIA300 bone anchored hearing aid implantation in children.

OBJECTIVE: Bone anchored hearing implants (BAHI) have been in use for over 30 years, and are commonly implanted in children for a range of indications. The Cochlear™ BIA300 system was launched in 2010 and used at The Birmingham Children's Hospital from 2011. Here we report the long-term outcomes of children implanted with the Cochlear™ BIA300 BAHI system in our centre. METHODS: A retrospective case note analysis was performed to identify outcomes in all children who underwent BIA300 implantation between 2011 and 2013. RESULTS: 52 children with a total of 78 implants were included. Mean age at implantation was 8.7 years. Mean follow-up was 43.5 months. Overall, 60 (77%) implants developed soft tissue complications requiring treatment. Forty-eight (62%) required topical treatment; 27 (35%) required systemic treatment; and 27 (35%) required surgical soft tissue revision under general anaesthesia. CONCLUSIONS: The Cochlear™ BIA300 system appears to be associated with higher than expected rates of soft tissue reaction in children, with late as well as early soft tissue complications requiring both medical and surgical treatment.

Microtia and Social Media: Patient Versus Physician Perspective of Quality of Information.

INTRODUCTION: Previous research demonstrates that patients seek high-quality information on the World Wide Web, especially in rare conditions such as microtia. Social media has overtaken other sources of patient information but quality remains untested. This study quantifies the quality of information for patients with Microtia on social media compared with nonsocial media websites and compares physician and patient scoring on quality using the DISCERN tool. METHODS: In phase 1, quality of the top 100 websites featuring information "Microtia" was ranked according to quality score and position on Google showing the position of social media websites among other nonsocial media websites. Phase 2 involved independent scoring of websites on microtia compared with a patient group with microtia to test whether physicians score differently to patients with t test comparison. RESULTS: Social media websites account for 2% of the scored websites with health providers linking to social media. Social media websites were among the highest ranked on Google. No correlation was found between the quality of information and Google rank. Social media scored higher than nonsocial media websites regarding quality of information on microtia. No significant difference existed between physician and patient quality of information scores on social media and nonsocial media websites (p 1.033). CONCLUSION: Physicians and patients objectively score microtia websites alike. Social media websites have higher use despite being few in number compared with nonsocial media websites. Physicians providing links to social media on information websites on rare conditions such as microtia are engaging in current information-seeking trends.

Adjunctive Treatment in Juvenile Nasopharyngeal Angiofibroma: How Should We Approach Recurrence?

A recent case of advanced, recurrent juvenile nasopharyngeal angiofibroma (JNA) at our institution has highlighted the limited evidence regarding adjunctive treatment. We present the case of a 10-year-old boy who is the first to undergo multiple-staged surgical resections alongside vincristine treatment. We performed a review of the literature analyzing the roles of radiation therapy, cytotoxic drugs, and novel targeted agents in JNA relapse. Small cohort studies suggest radiotherapy and flutamide are the most rational treatment options for residual and recurrent JNA. Our review highlights the need for further research into the management of primary and recurrent JNA.

Clinical results of Cochlear™ BIA300 in children: Experience in two tertiary referral centers.

OBJECTIVES: To evaluate clinical outcomes of the Cochlear™ BIA300 in the pediatric population. STUDY DESIGN: Historical cohort study. SETTING: Two tertiary referral centers. PATIENTS: All patients implanted with the BIA300 from November 2011 to January 2014 and 17 years or younger during surgery were included in this cohort study. MAIN OUTCOME MEASURES: Number of soft tissue reaction scores according to Holgers, local and systemic treatment of soft tissue reactions, revision surgeries and implant loss. RESULTS: Since introduction of the BIA300, 79 children have been implanted in both centers. During the mean follow-up of 11.7 months per implant, 15.7% of 115 implants received at least two local treatments for peri-abutment soft tissue reactions. Moreover, in 32 implants an adverse soft tissue reaction (Holgers 2 or higher) was noted at least once. In 28.7% of implants one or multiple revision surgeries were required. Implant loss occurred in 4 patients (3.5% of all implants), additionally, in five children the abutment had to be removed because of persistent soft tissue problems. CONCLUSIONS: The current study confirms good implant survival for these implants in pediatric patients. The number of adverse soft tissue reactions found in the current study resembles numbers reported on previous generation implants and abutments in children. However, for revision surgery (soft tissue revision and/or abutment change), an increase in frequency is noticed compared to reported results on previous generation implants and abutments, whether this is the result of the new implant or other factors cannot be concluded on the current series. The total aspect of the presented data are of importance in the decision making for a specific type of percutaneous bone anchored hearing implant.

Bone anchored hearing aids in children.

Bone-anchored hearing devices have evolved over recent years. This article provides an overview of the device history, indications, evolution of surgical technique, evidence for benefit and focuses on the challenges that are faced in the pediatric population.

Magnetic resonance imaging and bone anchored hearing implants: pediatric considerations.

OBJECTIVE: Recent developments in bone conduction hearing systems have seen the introduction of transcutaneous devices comprising of magnetic components. Our aim was to identify the number of children implanted with a traditional, non-magnetic percutaneous bone anchored hearing implant (BAHI) who would not have been eligible for a transcutaneous implant based on magnetic resonance imaging (MRI) need. METHODS: A retrospective case review of 206 children who had a percutaneous BAHI at the Birmingham Children's Hospital (January 2009-October 2012) for auditory rehabilitation. RESULTS: Twenty-eight percent (56/206) of children required at least one MRI scan after receiving a BAHI and 10 percent of patients (20/206) required two or more MRI scans. The main indication for MRI scanning was for neurological co-morbidities; a MRI brain was the most common scan performed. CONCLUSION: Although transcutaneous hearing devices/middle ear implants have their clear benefits, it may be argued that these relatively more invasive surgical procedures may not be the best option for the child who will require MRI scanning at some point in the future. Clinicians should be mindful of any need for MRI scanning when considering implant choices in the pediatric 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.

Are contrast swallows a relevant investigation for paediatric stridor?

The contrast swallow has traditionally been used in the investigation of paediatric stridor with the aim of identifying pathology such as vascular rings and confirming the presence of gastro-oesophageal reflux. Currently, a laryngotracheobronchoscopy (LTB) is the gold standard investigation for stridor and frequently, a child is commenced on antireflux medications based on clinical history. We seek to identify whether a contrast swallow has a valuable and cost effective role in the investigation of paediatric stridor. This was a retrospective audit study. All the children who underwent a contrast swallow as part of the investigation for stridor were identified from 2008 to 2011. All requests were made by clinicians from the ENT department at Birmingham Children's Hospital. All patient demographics and radiological reports were reviewed. The final patient list was cross referenced with theatre records to identify those that underwent a LTB The cost of the contrast swallow was identified by the contracting department at Birmingham Children's Hospital. A total of 117 contrast swallows were requested during the study period. The age range was 1 week to 50 months with an average patient age of 6 months. 45/117 (38 %) of contrast swallows identified pathology. 33/45 (73 %) demonstrated reflux. Only 12/117 (10 %) of all cases suggested pathology other than reflux. A total of 51 patients underwent LTB. 22/51 (43 %) had a subsequent contrast swallow. Since the average cost per contrast swallow in the outpatient setting was £ 77, and only 9 cases demonstrated significant unexpected pathology, 108 children had a contrast swallow that did not significantly alter the management of their stridor. 9 cases were identified in which the contrast study results altered the management of the child. An estimated £ 8,000 were spent on investigations that made no impact on the subsequent management of the child. Contrast swallows requested for the investigation of paediatric stridor identified 33 cases of gastro-oesophageal reflux and 12 cases of alternative pathology. Among those children with radiological evidence of reflux, 70 % (23/33) had already been commenced on antireflux medication based on their clinical symptoms. Only 8 % of findings from a contrast swallow investigation resulted in a change to the management of the stridulous child. The authors suggest that it is more cost effective to reserve requests for a contrast swallow until the diagnostic LTB is performed. This strategy will not only reduce exposure to ionising radiation in a significant number of children, but also identifies airway pathology more effectively and ensures that contrast swallows are used as an appropriate adjunctive diagnostic investigation.

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 aids in children: an update.

OBJECTIVE: Over recent years, there have been a significant number of publications reporting evolving bone anchored hearing device (BAHD) indications and modifications in surgical techniques. We aim to present a review of recent trends in paediatric BAHD surgery and also discuss alternative treatment options available. METHODS: All papers referring to paediatric BAHD surgery (English language) were identified from Medline, Pubmed, Cochrane library and Embase search in May 2011. Abstracts were read and relevant papers were obtained. RESULTS: BAHDs have evolved over recent years as technology has advanced. New bone conduction hearing devices have recently been launched such as the Ponto system [Oticon™], Alpha 1 (M) hearing system [Sophono], Soundbite system [Sonitus] and the Vibrant Soundbridge system [Medel]. Modifications to existing implant systems have significantly altered BAHD practice with earlier loading of the sound processor now a positive step forward. CONCLUSIONS: The latest generation of percutanous devices have been designed to reduce skin complications, promote better osseointegration and earlier loading of the sound processor. Alternative devices without a skin-penetrating abutment are now available and have shown promising results in the paediatric population.

Paediatric Baha.

Bone-anchored hearing aids (Baha(®)) are a well-established and readily available option for aural rehabilitation for children. Audiological outcomes and paediatric quality of life results have added to the evidence of benefit of Baha. Recent years have seen an increase in the number of centres worldwide providing a Baha service for children. Indications for Baha have evolved. The paediatric population present a variety of challenges for clinicians who undertake Baha surgery. Complex medical conditions, social and educational issues, and trauma are such examples. The surgical techniques are now varied and the timing of surgery varies between surgeons and Baha centres. Fortunately, serious complications are very rare, and to date there are no more than six significant such case reports in the literature. Careful patient selection and a multidisciplinary team approach with an understanding of the problems that present in children should result in a successful outcome for the child.

Bone-anchored hearing aids versus conventional hearing aids.

Hearing amplification technology has been evolving since the 19th century. Currently in most audiology departments, the mainstay of hearing rehabilitation is performed with conventional air and bone conduction aids. These are cost-effective, non-invasive hearing aids but are not without their drawbacks. This chapter explores the advantages and disadvantages of conventional hearing aids compared with the bone-anchored hearing aids. Although the bone-anchored hearing aids are a more expensive invasive option, there is increasing evidence that the benefits outweigh the disadvantages. Users report improved quality of life, health status and audiological rehabilitation.

Pediatric myringoplasty: definition of "success" and factors affecting outcome.

INTRODUCTION: Recent studies have reported a high success rate of pediatric myringoplasty, but their definition of success and length of follow-up vary widely. Authors debate whether the success of a pediatric myringoplasty should be defined purely by an intact tympanic membrane or should analysis of middle ear health and audiometric data also be included. For the purposes of this study, success was defined as an intact tympanic membrane at 12 months postsurgery without evidence of effusion and preservation of hearing. OBJECTIVES: To determine the true success rate of myringoplasty and identify any independent factors that significantly influence outcome. MATERIALS AND METHODS: This was a retrospective analysis of 132 myringoplasties performed at Birmingham Children's Hospital. Cases were grouped into those children aged younger than 8 years and those aged 8 years and older. RESULTS: An intact tympanic membrane was present in 80.0% of cases at the first postoperative visit. However, at 12 months, the previously defined success rate, cases decreased to 67.3%. Younger age (p < 0.0047) and anterior perforations (p <0.0038) were the only factors found to be associated with a poorer outcome. CONCLUSION: The true rate of success of a pediatric myringoplasty would seem to be lower than rates commonly quoted to parents, and this finding is in keeping with other otologists using a similar definition of success. Younger age at the time of surgery and anterior perforations have a less successful outcome. These findings are important when counseling/consenting carers of younger children about to undergo a myringoplasty.