Bone-conduction Aids Research Articles

Treacher Collins syndrome: current and emerging treatment options

Treacher Collins syndrome is a rare congenital disorder characterized by bilateral craniofacial malformations, including mandibular and zygomatic hypoplasia, cleft palate, and eye anomalies, resulting in significant variability in symptom severity. The syndrome, with an incidence of approximately 1 in 50,000 births, is primarily caused by mutations in the TCOF1 gene and less frequently by mutations in POLR1C and POLR1D genes, disrupting ribosome biogenesis crucial for craniofacial development. Clinical manifestations encompass diverse craniofacial, auditory, and skeletal abnormalities, often leading to airway complications and hearing loss. Management involves a multidisciplinary approach, combining surgical interventions such as mandibular distraction osteogenesis and orthognathic surgeries with advanced imaging techniques for precise planning. Hearing loss is managed with bone conduction aids and cochlear implants, although outcomes may vary due to associated anomalies. Genetic testing aids in diagnosis and family planning decisions, while emerging therapies like genome editing and regenerative medicine show promise for future treatments. Despite challenges in long-term management and relapse rates in surgical corrections, ongoing research aims to enhance therapeutic efficacy and patient outcomes, emphasizing the need for tailored treatment strategies addressing both functional and aesthetic aspects.

Bone-Anchored Hearing Aid Effects on Vestibular Function: A Preliminary Report.

Cochlear receptors are sensitive to vibratory stimuli. Based on this sensibility, bone-anchored hearing aids have been introduced to correct unilateral or bilateral conductive or mixed hearing loss and unilateral deafness. The vestibular system is also sensitive to the vibratory stimulus and this type of response is used in clinics to test its functionality. Being aware of this double separated sensibility, we wondered whether bone vibration, which activates the acoustic receptors of patients with bone conduction aids, can also influence the functionality of the vestibular system. To this end, we recruited 12 patients with a bone-anchored hearing aid and evaluated their vestibular function with and without an activated vibratory acoustic device. Our results show that the vibratory stimulus delivered by the bone conduction aid also reaches and stimulates the vestibular receptors; this stimulation is evidenced by the appearance or modification of some nystagmus findings during bedside vestibular testing. Despite this, none of these patients complained of dizziness or vertigo during prosthesis use. Nystagmus that appeared or changed during acoustic vibratory stimulation through the prosthesis was almost all predominantly horizontal, unidirectional with respect to gaze or body position, inhibited by fixation, and most often consistent with vestibular function tests indicating peripheral vestibular damage. The findings of sound-evoked nystagmus seem to indicate peripheral rather than central vestibular activation. The occurrence of some predominantly horizontal and high-frequency induced nystagmus seems to attribute the response mainly to the utricle and lateral semicircular canal.

Results of a Novel, Nonsurgical Bone-Conduction Hearing Aid for the Treatment of Conductive Hearing Loss in Australian Children.

To determine and compare the benefits a novel adhesive bone-conduction system and a conventional bone-conduction hearing aid (BCHA) on a softband for children with conductive hearing loss. Prospective, single-subject randomized, crossover trial. Tertiary referral center in Australia. Eight children aged from 4 to 17 years with conductive hearing loss. Rehabilitative with participants using the novel adhesive bone-conduction aid and a BCHA. Aided thresholds, as well as speech perception in quiet, unaided and aided with the novel adhesive bone-conduction aid and BCHA on a softband. For the six older children, speech in noise testing was also conducted. The mean unaided four frequency average hearing levels was 48 dB HL for air conduction, 10.5 dB HL for bone conduction, with a mean air-bone gap in the aided ear of 37.5 dB HL.Four-frequency average hearing level aided thresholds were 20.2 dB for the novel device and 19.8 dB for the BCHA, with no significant difference between the devices. Aided monosyllabic word scores improved from an average of 45% in the unaided condition to 81.6 and 85% for the novel adhesive and BCHA devices, respectively. Aided speech in noise performance improved, on average, by 1.6 dB SNR when wearing the BCHA and the novel adhesive device, with no significant difference in performance between the two devices. The novel device provided equivalent performance to the BCHA on all measures and can be considered as an alternative device for pediatric patients with conductive hearing loss.

Barriers to Pediatric Osseointegrated Bone-Conduction Hearing Devices.

To identify social, demographic, and clinical barriers for implantation with Osseointegrated Bone Conduction Devices (OBCD) in pediatric candidates. Retrospective cohort study of 94 children who met standard OBCD implantation criteria. Tertiary stand-alone children's hospital. Retrospective chart review comparing demographic (age, race, state of residence, and insurance) and clinical (severity and etiology of hearing loss, medical comorbidities, and early intervention) factors impacting implantation. Members of the existing cohort were then contacted to obtain a better understanding of qualitative factors impacting surgical decision. Of the identified 94 surgical candidates, 47 (50%) underwent OBCD implantation. State of residence significantly impacted implantation rates, with children from the District of Columbia and Virginia being less likely to receive an implant than those from Maryland. Private insurance, race, and ethnicity did not impact rate of implantation (OR 2.8 [95% CI 0.78-10]; 1.34 [95% CI 0.44-3.68]; and 1.0 [95% CI 0.42-2.43], respectively). Children with anotia or microtia and children younger than 10 years old were less likely to have an implant (OR 10.6 (95% CI 1.74-65). Thirty-nine children participated in the qualitative portion. Themes that emerged as reasons to forgo implantation included a child's young age, planned reconstruction for microtia or atresia, and overall device functionality and usage. Thirtyseven children (39%) of the cohort declined surgery and currently wear a nonsurgical bone conduction aid regularly. Despite known benefits of implantation, only one-half of children who were candidates underwent OBCD. Unlike cochlear implantation, where insurance status is a major risk factor for implantation delay and underperformance, for OBCD, implantation barriers appear to be more multifactorial and include medical, demographic, and social factors.

The Use of a Novel, Nonsurgical Bone Conduction Hearing Aid System for the Treatment of Conductive Hearing Loss

Conventional bone conduction hearing aid solutions often require a cumbersome retention system such as a headband or cap. This study aims to determine if a novel, nonsurgical bone conduction aid utilizing an adhesive attachment over the mastoid is equivalent to the conventional bone conduction hearing aid (BCHA) for the management of conductive hearing loss. Prospective, single-subject randomized, crossover trial. Tertiary referral center. Eleven adults and 1 child between 11 and 70 years of age with unilateral conductive hearing loss were enrolled. Patients had their baseline hearing assessed and were randomized to receive either the novel device or BCHA headband system. Hearing loss etiologies were varied and included cholesteatoma, otosclerosis, chronic otitis media, and previous head and neck surgery. Patients had their baseline hearing assessed and trialled both the novel device or the BCHA headband system. Patients were randomly assigned one device which was worn for 2 weeks followed by a 2-week trial with the alternate device. Pure-tone thresholds and speech discrimination in quiet and noise were tested and patients also completed the short form of the Speech Spatial and Quality of Hearing (SSQ) questionnaire. The mean unaided 4 frequency pure-tone average (PTA) air conduction threshold was 53.9 dB, bone conduction was 11.9 dB, and the mean air bone gap was 42 dB in the target ear. One patient with an adhesive skin reaction could not complete the protocol. Aided PTA and threshold testing between 250 Hz to 8000 Hz showed statistically equivalent results between both devices. The aided CNC word score, signal-to-noise ratio, and SSQ scores were also equivalent between both devices. The novel bone conduction aid demonstrates equivalent performance to the conventional BCHA headband solution. The novel device should be considered an alternative option where the cosmetic and comfort issues of a headband worn device are a concern.

Hearing in Noise With Unilateral Versus Bilateral Bone Conduction Hearing Aids in Adults With Pseudo-conductive Hearing Loss.

The role of bilateral bone conduction amplification in patients with bilateral conductive hearing loss is unclear because cranial attenuation is usually considered negligible, and both cochleae can be stimulated with similar efficacy by each device. The aim of the study was to determine if bilateral bone-conduction hearing aids can improve hearing in noise in a homogeneous group of normal-hearing subjects with bilateral pseudo-conductive hearing loss. Prospective, comparative. Department of Communication Sciences and Disorders in University of Haifa, Israel. Department of Communication Sciences and Disorders in University of Haifa, Israel. Induction of bilateral pseudo-conductive hearing loss of more than 35 dB using earplugs and earmuffs. Hearing quality on blinded comparison of unilateral versus bilateral amplification with bone-conduction hearing aids under different locations of noise. Unilateral and bilateral amplification had similar efficacy when both signal and noise were presented from the front. However, bilateral amplification was significantly better when signals were presented from the front and noise was presented from both sides (SNR -10: 92% vs. 84%, p = 0.001; SNR -15: 84% vs. 78%, p = 0.005). Analysis of subject responses on blinded questionnaires revealed that 81% found hearing easier with bilateral amplification. Noise was reported to be more disturbing with unilateral amplification by 55% of the subjects and with bilateral amplification, by 9%. Bilateral amplification with bone conduction devices can improve understanding in noise in the binaural squelch setting. Subjective improvements with bilateral bone-conduction aids included better sound quality and reduced noise disturbance. These findings are consistent with the binaural mechanism of spectral release from masking.

A case series of a magnetic bone conduction hearing implant.

The objective of the study was to retrospectively review the postoperative outcomes of patients from a single centre who were implanted with a magnetic bone conduction hearing implant system. The medical and audiological case notes of all patients (n = 34) in a tertiary referral centre ENT department who had been implanted with a Baha® 4 Attract System between October 2013 and April 2015 were reviewed retrospectively. The main outcome measures were use of the aid and complications from implantation or use of the aid. Results indicated that 23 patients had no significant postoperative problems; 5 required an alteration in magnet strength; 1 reported troublesome tinnitus; 1 had the implant changed to an abutment system; 1 had the magnet removed; 1 has ongoing skin problems; 2 have been changed to a newer, lighter sound processor. The majority of patients had good outcomes from the magnetic bone conduction aid. A proportion of patients had significant problems requiring medical attention.

Bone-conduction hearing aids in an elderly population: complications and quality of life assessment.

To determine whether an elderly population with hearing impairment can be adequately rehabilitated with a bone-conduction hearing aid and whether the putative relationship between the elderly and an increased complication rate is justified. The study design was a retrospective case note review with a postal and telephone questionnaire, which was carried out in a tertiary centre. All patients aged 60 or over underwent implantation with a bone-conduction aid between 2009 and 2013 for conductive, SSD or mixed hearing loss. Outcome measures were complication rates and quality of life assessment using the Glasgow Benefit Inventory. The influence of patient and surgical factors on the complication rate was assessed. Fifty-one patients were implanted. Mean age was 67 years (range 60-89 years). The mean benefit, satisfaction and global GBI scores were 70 % (range 0-100 %), 70 % (0-100 %) and 82 % (83-100 %), respectively. The residual disability was 18 % (0-25 %). The adverse skin reaction rate was 16 % and the fixture loss rate was 2 %. There was a demonstrable increase in the complication rate with the dermatome (45 %; 5 patients) compared to the Sheffield 'S' (13 %; 2 patients) or linear incision techniques (29 %; 7 patients). The bone-conduction hearing aids are ideal method of hearing rehabilitation in the elderly for all forms of hearing loss. It provides significant benefit with no increased complication rate, which is imperative if social isolation is to be avoided and cognition preserved in this growing elderly population.

Audiologic and Surgical Outcomes of a Novel, Nonpercutaneous, Bone Conducting Hearing Implant

To assess the selection criteria, surgical technique, audiologic, and quality of life outcomes for a novel, nonpercutaneous bone conductor hearing aid. Retrospective case review. Secondary otology practice. Eighteen patients (16 adults and 2 children). Implantation of unilateral (n = 16) or bilateral (n = 2) devices. Mean preoperative and postoperative air conduction and bone conduction free-field testing, BKB-SIN aided and unaided at 0-degree 70 dB SPL, Speech, Spatial, and Qualities of Hearing Scale (SSQ), aided and unaided measures of localization and discrimination in single-sided deafness (SSD), surgical complications. Implants have been fixed under general or local anesthesia without perioperative complications. Two patients noted minor skin irritation only. Audiologic gain was greatest for those with bilateral conductive loss (21.9 ± 10.4 dB HL). For those with bilateral and unilateral mixed loss, gain was 6.2 ± 5.3 dB HL and 5.5 ± 6.5 dB HL, respectively. A greater improvement was seen with BKB-SIN at 70 dB SPL at 0 with all groups except for SSD, gaining statistically significant benefit. Localization and discrimination studies in patients with SSD or unilateral conductive loss failed to detect benefit from aiding. SSQ scores show an improvement in all domains for each patient group. The surgical procedure requires no specialized equipment and can be performed as a day case. This device complements treatment for patients requiring bone conduction aids and presents as an alternative to conventional percutaneous bone-anchored implants.

New Bone-Anchored Amplification Options for Children

New Bone-Anchored Amplification Options for Children

Considerations in early amplification: Selection, fitting, validation, counseling

The implementation of universal newborn hearing screening programs has increased the number of infants diagnosed with hearing loss and referred for amplification in the first few weeks of life. There is compelling evidence that children born with hearing loss who have been identified and begun intervention by 6 months of age will have significantly better language development than their later-identified peers. 1-3 The challenges of fitting appropriate amplification on this population include limited behavioral response to sound, possible complicating middle ear fluid, parental uncertainties, possible existence of additional handicaps, and limited resources to support and pay for hearing aids. Despite these issues, there is consensus that the diagnosis of congenital hearing loss should be completed in the first 3 months of life and be followed immediately with the consideration of hearing aid use. 4 As access to follow-up from newborn hearing screening improves, pediatric audiologists are faced with recommending technology for infants during their early weeks of life. The options available to them are expanding rapidly as amplification technology grows and diversifies. Outcome studies of children fitted with advanced amplification features are not common and cannot keep up with the features being introduced to the market. Most infants are fitted with the goal of providing audibility for environmental speech to enhance speech and language development and with the expectation that patients will use their hearing aids daily. The pediatric audiologist’s role is to share information on hearing loss and available amplification technology with the parents and their medical and support team. Parents depend on various professionals to acquire the information and tools they need to become empowered, informed consumers. Reaching decisions about the choice of technology for an infant includes examining available features and selecting what might be the best fit for the infant given all the available information and circumstances.

Estimation of the Benefit of Bone-Anchored Hearing Aids

Implantable bone conduction hearing aids are a valuable alternative to conventional aids for those who cannot use a conventional air conduction aid or find it difficult to use because of an aural discharge, most commonly due to chronic otitis media. Previously reported series of the use of a bone-anchored hearing aid (BAHA) come from the originators of this device, and an independent report of their benefit and use, especially in previous air conduction aid users, would be of value. Twenty-three patients were evaluated at least 6 months after implantation of a BAHA. All 7 previous bone conduction aid users were delighted with their BAHA, reporting increased comfort and hearing benefit that was backed by audiometric evidence. Of the 16 individuals who previously used an air conduction aid, 11 (69%) were delighted users of their BAHA. Unfortunately, the other 5 (31%) reverted to solely using their air conduction aid. There was no obvious predictor as to how these individuals might have been identified prior to implantation. In particular, their pure tone thresholds, especially the bone conduction thresholds, were no different from those of the 11 BAHA users. However, in free field audiometry, the users gained superior benefit from their BAHA compared to their air conduction aid, whereas the nonusers did not. In conclusion, in all series to date, previous users of a conventional bone conduction aid have been delighted users of a BAHA and have gained superior audiometric benefit. This is not necessarily the case with previous air conduction aid users.(ABSTRACT TRUNCATED AT 250 WORDS)

Modification of the Traditional Bone Conduction Hearing Aid

The purpose of this technical note is to describe a modification of the traditional bone conduction hearing aid used for the treatment of hearing impairment associated with atresia, microtia, or auditory canal anomalies. This modified unit offers a viable alternative to patients wanting an alternative to the traditional model or an implantable bone conduction aid.

Modification of the Traditional Bone Conduction Hearing Aid

The purpose of this technical note is to describe a modification of the traditional bone conduction hearing aid used for the treatment of hearing impairment associated with atresia, microtia, or auditory canal anomalies. This modified unit offers a viable alternative to patients wanting an alternative to the traditional model or an implantable bone conduction aid.

The British experience of an implantable, subcutaneous bone conduction hearing aid (Xomed Audiant)

Implantable bone conduction aids are potentially an important advance for those with a conductive hearing impairment. One system (Xomed Audiant bone conductor), which uses electromagnetic induction to vibrate a subcutaneous implanted skull magnet, has now been implanted in sufficient patients in the United Kingdom, for enough time, for its indications to be evaluated. Seventeen of the total of 18 patients that have been implanted, satisfied the average threshold criterion for suitability for implantation (average bone conduction over 0.5, 1 and 2 kHz of 25 dB HL or better) yet only 10 of the 17 (59 per cent) currently use their Audiant aid. This was not because of technical reasons but was mainly influenced by the previous type of amplification. Current usage of a body level processor was 100 per cent) (6 of 6) in those that previously could only use a conventional bone conduction aid because of bilateral congenital or acquired atresia of their external auditory canals. In comparison, usage was only 36 per cent (4 of 11) in those that could potentially use a conventional ear level aid albeit with problems such as the discharge from active chronic otitis media. This relative non-use was considered due to a lack of power of the ear level processor and the general unwillingness of patients to change from an ear level to a body level device.

Clinical results with a percutaneous bone-anchored hearing aid.

During a 2-year period ending in July 1987, nine patients were implanted with a percutaneous bone-anchored hearing aid developed at the University of Gothenburg and Chalmers University of Technology, Sweden. Patients selection was based on the presence of conductive or mixed hearing loss in patients who are unable to wear a conventional hearing aid because of infection or ear canal problems. Patients had speech discrimination scores of at least 60% for phonetically balanced monosyllables (CID lists) at 40 dB above threshold, and a pure tone bone conduction average of 45 dB hearing loss or better. Patients were evaluated in sound field, preoperatively and postoperatively using warble tones, speech reception threshold, speech discrimination, and synthetic speech identification. The patient's unaided hearing and hearing with a bone-anchored hearing aid were compared with the patient's hearing with a standard bone conduction hearing aid. Signal to noise ratio testing with the synthetic speech identification test demonstrated that the bone-anchored hearing aid was comparable to a standard bone conduction aid. There were no complications in our series. Five patients who reported otorrhea with conventional hearing aids experienced no otorrhea after implantation. Patient satisfaction, as assessed by questionnaire, revealed that all patients preferred the bone-anchored hearing aid to previously worn hearing aids.