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

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 8h 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.     

Bone anchored hearing aids (BAHA) used for treatment of conductive hypoacusis--current problems in Poland

Basic principles, indications and limitations of BAHA implantation as well as different kinds of operation techniques are presented. The beginning and the development of BAHA use in Poland are mentioned together with some difficulties in broadening of this kind of implantation.     

The BAHA HC200/300 in comparison with conventional bone conduction hearing aids

A retrospective study was performed on 89 patients from a consecutive series who received a BAHA HC200/300 after having previously used conventional bone conduction hearing aids. The patients’ performance with the BAHA HC 200/300 was compared to their performance with conventional bone conduction hearing aids. The patients were divided into two groups, depending on the time of implantation (before or after May 1992). The patients in group 1 (long-term users) were asked to fill in a questionnaire, the same one as they had filled in at the initial BAHA fitting more than 5 years previously. The answers were compared to their original opinions and difference scores were calculated. The long-term clinical results from group 1 are also presented. Although they are encouraging, the patients’ opinion about the BAHA deteriorated somewhat over time. The audiometric results of group 2 were highly comparable with those of group 1. This confirms the positive results with the BAHA found in previous studies.     

The BAHA HC200/300 in comparison with conventional bone conduction hearing aids.

A retrospective study was performed on 89 patients from a consecutive series who received a BAHA HC200/300 after having previously used conventional bone conduction hearing aids. The patients' performance with the BAHA HC 200/300 was compared to their performance with conventional bone conduction hearing aids. The patients were divided into two groups, depending on the time of implantation (before or after May 1992). The patients in group 1 (long-term users) were asked to fill in a questionnaire, the same one as they had filled in at the initial BAHA fitting more than 5 years previously. The answers were compared to their original opinions and difference scores were calculated. The long-term clinical results from group 1 are also presented. Although they are encouraging, the patients' opinion about the BAHA deteriorated somewhat over time. The audiometric results of group 2 were highly comparable with those of group 1. This confirms the positive results with the BAHA found in previous studies.     

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.     

Functional and patient-reported outcomes of bone-anchored hearing aids (BAHA): A prospective case series study

Objective: The purpose of this study was to evaluate the functional and patient-reported outcomes, and their correlation, after percutaneous bone-anchored hearing aid(BAHA) implantation.Methods: A prospective study was conducted between January 2018 and December 2020 in a tertiary care center. All adult patients who were implanted with a percutaneous BAHA device during this evaluation period were included in the study. Complete auditory function and patients reported outcome measures(PROMs) were...     

Benefit from binaural hearing aids in individuals with a severe hearing impairment

The relative benefits of binaural as opposed to monaural hearing aids were assessed by the use of a diotically presented, audiovisual speech-in-noise task in a group of bilateral, severely hearing-impaired individuals. Significantly greater benefit was gained from binaural amplification, irrespective of hearing level and degree of asymmetry. The benefit was considered to be due to central summation. It is concluded that binaural amplification should be attempted in all bilateral, severely hearing-impaired subjects. About 80% will accept this and be able to demonstrate benefit in an audiovisual task.     

The evidence base for the application of contralateral bone anchored hearing aids in acquired unilateral sensorineural hearing loss in adults

? Acquired unilateral sensorineural hearing loss reduces the ability to localize sounds and to discriminate in background noise. ? Four controlled trials attempt to determine the benefit of contralateral bone anchored hearing aids over contralateral routing of signal (CROS) hearing aids and over the unaided condition. All found no significant improvement in auditory localization with either aid. Speech discrimination in noise and subjective questionnaire measures of auditory abilities showed an advantage for bone anchored hearing aid (BAHA) > CROS > unaided conditions. ? All four studies have material shortfalls: (i) the BAHA was always trialled after the CROS aid; (ii) CROS aids were only trialled for 4 weeks; (iii) none used any measure of hearing handicap when selecting subjects; (iv) two studies have a bias in terms of patient selection; (v) all studies were underpowered (vi) double reporting of patients occurred. ? There is a paucity of evidence to support the efficacy of BAHA in the treatment of acquired unilateral sensorineural hearing loss. Clinicians should proceed with caution and perhaps await a larger randomized trial. ? It is perhaps only appropriate to insert a BAHA peg at the time of vestibular schwanoma tumour excision in patients with good preoperative hearing, as their hearing handicap increases most.     

Bone-anchored hearing aids (Baha) in congenital aural atresia: personal experience

Aims

Aural atresia is a congenital disease constituted by partial or complete lack of development of the external auditory canal, which is generally associated with malformations of the auricle and middle ear.Reconstruction of the auditory canal and correction of any deformities of the middle ear have yielded unpredictable results and variable functional outcomes, and there is a high rate of complications. Therefore, the use of bone-conduction hearing aids, such as the Baha, may represent a valid alternative for subjects who have conductive hearing loss with cochlear reserve that, as a rule, is fully conserved.The aim of this work is to reexamine our experience with the management of conductive and mixed hearing loss using the Baha system in children with bilateral aural atresia.

Methods

We examined 31 patients with bilateral congenital aural atresia in whom a Baha system had been implanted. The patients, 16 males and 15 females, were between 5 and 14 years of age (mean 8.7).The following parameters were assessed for each patient: mean preoperative air and bone conduction for frequencies between 0.5 and 4 kHz; mean preoperative threshold with conventional bone-conduction hearing aids; mean postoperative threshold with the Baha system; improvement in quality of life evaluated with the Glasgow Children's Benefit Inventory; rate and type of surgical complications.

Results

The mean preoperative air- and bone-conduction thresholds were 51.2 ± 12.5 and 14.1 ± 6.3 dB HL, respectively. The mean preoperative threshold with a conventional bone-conduction hearing aid was 29.3 ± 7.2 dB HL, and the mean postoperative threshold with the Baha system was 18.1 ± 7.5 dB HL. Quality of life improved for all operated patients.

Conclusions

The results of our study of the Baha system to treat patients with bilateral aural atresia were extremely satisfactory compared both with those of surgical reconstruction of the auditory canal and those of traditional bone-conduction hearing aids. Furthermore, great improvement was noted in quality of life, while the rate of complications was very low.Therefore, we are convinced that the Baha system is the treatment of choice for hearing loss due to bilateral congenital aural atresia.     

Bone-anchored hearing aid (BAHA): report of 2 cases

In this first case report in Japan, we described 2 patients treated using a bone conductive hearing aid with skin-penetrating implant, or bone-anchored hearing aid (BAHA). Both suffered from chronic otitis media and had received bilateral canal down tympanomastoidectomy. Case 1 was a 61-year-old woman with a conventional air conductive hearing aid in her right ear. There was no aural discharge in either ear. A pure-tone average showed 82.5 dBHL (air), 44.3 dBHL (bone) in the right ear and 93.8 dBHL (air), 48.8 dBHL (bone) in the left ear. Case 2 was a 38-year-old woman with a conventional hearing aid in her left ear, from which there was persistent aural discharge. A pure-tone average showed 48.8 dBHL(air), 15.0 dBHL (bone) in the right ear, and 60.0 dBHL(air), 20.0 dBHL(bone) in the left ear. Both underwent BAHA implant in the right ear without adverse reaction during 9 months of postoperative follow-up after surgery. No difference was seen in the aided hearing level or speech discrimination score between BAHA and air conduction hearing aids but both patients preferred to BAHA because of its greater comfort and audibility.     

A double blind cross-over study of high-frequency emphasis hearing aids in individuals with noise-induced hearing loss

This study compared the benefits of a high-frequency emphasis hearing aid and a more conventional, flatter response aid. Subjects with moderate noise-induced hearing loss were fitted binaurally within a double blind procedure. Each aid type was worn for 1 month, during which the subjects completed diaries concerning their problems in various listening situations. At the end of each month, a satisfaction questionnaire was completed and free field assessment, consisting of speech in noise discrimination measurement and warble tone threshold determinations, was performed. Results indicated that although there were few significant differences found between the aids using any of the assessment techniques, the subjects generally preferred the aid with the high-frequency emphasis.     

Application and usage of the bone anchored hearing aid

Performance of two cases of direct utilization of osseous leadership was aim of work through anchorage in temple' s bone of apparatus of aural type BAHA. In first case this method was applied from bilateral at seven aged boy lack of external ear and under development of central. Ear and co-existent cholesteatoma. Authors introduce technique of introducing of titanic implant to temple's bone, they talk over rule of working of at anchor apparatus--leaning's onto phenomenon osseointegration. In both cases positive functional results were got. At one child prolapsus of implant followed in result of injury. On basis of own observation advantage aural of method were introduced. Difficulties in her and survival were shown also.     

Wound related complications following full thickness skin graft versus split thickness skin graft on patients with bone anchored hearing aids

Objectives: The aim of this study was to review complications occurring in bone anchored hearing aid (BAHA) patients in relation to wound healing following full thickness skin graft versus split thickness skin graft. Design: Retrospective study. The medical notes of 22 patients who underwent insertion of BAHA over 24 months were reviewed. Setting: ENT Department at a District General Hospital in the UK. Participants: All patients were adults and underwent one stage procedure following the standard Branemark technique. In 11 cases the skin abutment interface was established by use of full thickness skin graft inset around the implant, and in the other 11 by use of split thickness skin graft. Main outcomes measures: The incidence of delayed wound healing resulting in an increase of number of visits for change of wound dressings. In addition the degree of soft tissue reactions around the interface was examined. Results: There was a clear difference between the split and full thickness skin graft groups in relation to the severity of adverse skin reactions and number of visits required for change of dressings. The split thickness group required from three minimum to 13 maximum (median 4) visits in outpatients during the initial observation period until healing was complete. The full thickness group demonstrated one minimum and three maximum (median 2) visits. Conclusions: In our hands the full thickness skin graft is superior to a split thickness graft.     

Ten years’ experience with bone conduction hearing aids in the Western Cape,South Africa

Untreated conductive and mixed hearing losses as a result of middle ear pathology or congenital ear malformations can lead to poor speech, language and academic outcomes in children. Lack of access to centralised hearing healthcare in resource-constrained environments limits opportunities for children with hearing loss. Red Cross War Memorial Children’s Hospital (RCWMCH) is one of only two dedicated paediatric hospitals in sub-Saharan Africa. Between 2016 and 2021, 29 children received implanted bone conduction hearing devices, and 104 children were fitted with bone conduction devices on softbands. The authors’ experience at RCWMCH suggests that bone-anchored hearing devices, either fitted on softbands or on implanted abutments, can provide solutions in settings where patients have limited access to hearing healthcare and optimal classroom environments. Hearing healthcare should be accessible and delivered at the appropriate level of care to mitigate the adverse effects of hearing loss in children.ContributionThis article describes strategies employed at RCWMCH such as fitting bone conduction hearing devices on a softband immediately after hearing loss diagnosis and conducting follow-up via remote technology to make hearing healthcare more accessible to vulnerable populations.     

Expanding the indications for the bone anchored hearing system (BAHS) in patients with single sided deafness

PurposeProvide data to support expansion of FDA indications for the Bone anchored hearing system (BAHS).Materials and methodsThis retrospective study in a tertiary otologic referral center included106 consecutive subjects who were implanted with a Bone Anchored Hearing System (BAHS) between January 2009 and January 2015 for single sided deafness. Subjects were divided into three groups by bone conduction pure tone average (PTA) of the better hearing ear: 0–20 dB (group 1), 21–40 dB (group 2) and 41–55 dB (group 3). All patients underwent BAHS implantation. Speech perception data (Hearing In Noise Test and Consonant-Nucleus-Consonant testing) was collected before and after surgical intervention. Patient-reported quality of life measures were obtained at least 6 months after activation. These included the Abbreviated Profile of Hearing Aid Benefit and Glasgow Benefit Inventory.ResultsAll three groups of subjects demonstrated statistically significant improvement in outcome measures following BAHS. Subject reported quality of life outcome measures demonstrated significant improvement in disability from hearing loss and in quality of life.ConclusionsPatients with single sided deafness who have bone conduction thresholds worse than 20 dB in their contralateral ear are still able to benefit significantly from BAHS.     

Hearing with the bone-anchored hearing aid (BAHA,HC 200) compared to a conventional bone-conduction hearing aid

Sixteen patients have been fitted with a standard bone-anchored hearing aid (HC 200), to replace their conventional bone-conduction aid. The average pure tone threshold at 0.5, 1 and 2 kHz varied from 35 to 75 dB HL, with a sensorineural component varying from 0 to 30 dB HL. The patients' performance with the bone-anchored aid was compared to that with the conventional bone-conduction aid in an acoustic-free field. The maximum phoneme score in quiet was 100% in most patients; in 6 patients, the score with the bone-anchored aid was better (range from 5 to 10%). The speech-in-noise ratio was significantly better in 11 patients (range from—1.4 to—8 dB). None of the patients had poorer results on either test with the bone-anchored aid. The improved speech recognition was ascribed to better performance of the hearing aid in the higher frequency range (above 2 kHz) and to relatively less distortion.     

Tolerable delay for speech production and perception: effects of hearing ability and experience with hearing aids

Objective: Processing delay is one of the important factors that limit the development of novel algorithms for hearing devices. In this study, both normal-hearing listeners and listeners with hearing loss were tested for their tolerance of processing delay up to 50?ms using a real-time setup for own-voice and external-voice conditions based on linear processing to avoid confounding effects of time-dependent gain. Design: Participants rated their perceived subjective annoyance for each condition on a 7-point Likert scale. Study sample: Twenty normal-hearing participants and twenty participants with a range of mild to moderate hearing losses. Results: Delay tolerance was significantly greater for the participants with hearing loss in two out of three voice conditions. The average slopes of annoyance ratings were negatively correlated with the degree of hearing loss across participants. A small trend of higher tolerance of delay by experienced users of hearing aids in comparison to new users was not significant. Conclusion: The increased tolerance of processing delay for speech production and perception with hearing loss and reduced sensitivity to changes in delay with stronger hearing loss may be beneficial for novel algorithms for hearing devices but the setup used in this study differed from commercial hearing aids.