Clinical performance, safety, and patient-reported outcomes of an active osseointegrated bone-conduction hearing implant system at 24-month follow-up.

PURPOSE: To investigate 2-year post-operative hearing performance, safety, and patient-reported outcomes of hearing-impaired adults treated with the Osia® 2 System, an active osseointegrated bone-conduction hearing implant that uses piezoelectric technology. METHODS: A prospective, multicenter, open-label, single-arm, within-subject clinical study conducted at three tertiary referral clinical centers located in Melbourne, Sydney and Hong Kong. Twenty adult recipients of the Osia 2 System were enrolled and followed up between 12 and 24 months post-implantation: 17 with mixed or conductive hearing loss and 3 with single-sided sensorineural deafness. Safety data, audiological thresholds, speech recognition thresholds in noise, and patient-reported outcomes were collected and evaluated. In addition, pre-and 6-month post-implantation data were collected retrospectively for this recipient cohort enrolled into the earlier study (ClinicalTrials.gov NCT04041700). RESULTS: Between 6- and 24-month follow-up, there was no statistically significant change in free-field hearing thresholds or speech reception thresholds in noise (p = > 0.05), indicating that aided improvements were maintained up to 24 months of follow-up. Furthermore, improvements in health-related quality of life and daily hearing ability, as well as clinical and subjective measures of hearing benefit remained stable over the 24-month period. No serious adverse events were reported during extended follow-up. CONCLUSIONS: These study results provide further evidence to support the longer term clinical safety, hearing performance, and patient-related benefits of the Osia 2 System in patients with either a conductive hearing loss, mixed hearing loss, or single-sided sensorineural deafness. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT04754477. First posted: February 15, 2021.

Evaluation of a super powerful bone-anchored hearing system and its users: A retrospective study.

INTRODUCTION: Although the Baha 5SP has been commercially available for six years, very few studies have been performed on the device's efficacy. The current study aims to evaluate the characteristics and audiological results in patients with severe-to-profound mixed hearing loss fitted with this superpower sound processor. METHODS: This retrospective evaluation was conducted at a tertiary referral centre where a series of 82 adult patients with severe-to-profound mixed hearing loss were implanted with a percutaneous bone-anchored hearing system and fitted with a superpower sound processor between 2016 and 2019. Patients with incomplete or unreliable audiological data (n = 24) were excluded, resulting in 58 data sets for analysis. The main outcome measures were unaided and aided pure-tone thresholds and aided free-field speech perception in quiet. RESULTS: The median unaided air conduction (AC) threshold averaged across 0.5, 1 and 2 kHz (PTA0.5-2kHz) of all patients was 75 dB hearing loss (HL); the median unaided AC averaged across 1, 2 and 4 kHz (PTA1-4kHz) was 84 dB HL. For bone conduction and direct bone conduction, the median PTA0.5-2kHz was 52 and 47 dB HL, respectively. With the superpower device, the median free-field speech reception threshold was 54 dB sound pressure level (SPL), and the median speech perception score at 65 dB SPL was 80%. CONCLUSIONS: At least 75% of the patients reached a maximum phoneme score of 70%. For patients with lower scores, the superpower device still provides a substantial hearing benefit. This makes the superpower device particularly suitable for patients with severe-to-profound mixed hearing loss with a contraindication for conventional hearing aids and/or cochlear implants.

Prediction of postoperative speech comprehension with the transcutaneous partially implantable bone conduction hearing system Osia®.

BACKGROUND: The active transcutaneous, partially implantable osseointegrated bone conduction system Cochlear™ Osia® (Cochlear, Sydney, Australia) has been approved for use in German-speaking countries since April 2021. The Osia is indicated for patients either having conductive (CHL) or mixed hearing loss (MHL) with an average bone conduction (BC) hearing loss of 55 dB HL or less, or having single-sided deafness (SSD). OBJECTIVES: The aim of this retrospective study was to investigate the prediction of postoperative speech recognition with Osia® and to evaluate the speech recognition of patients with MHL and in particular an aided dynamic range of less than 30 dB with Osia®. MATERIALS AND METHODS: Between 2017 and 2022, 29 adult patients were fitted with the Osia®, 10 patients (11 ears) with CHL and 19 patients (25 ears) with MHL. MHL was subdivided into two groups: MHL­I with four-frequency pure-tone average in BC (BC-4PTA) ≥ 20 dB HL and < 40 dB HL (n = 15 patients; 20 ears) vs. MHL-II with BC-4PTA ≥ 40 dB HL (n = 4 patients; 5 ears). All patients tested a bone conduction hearing device on a softband preoperatively. Speech intelligibility in quiet was assessed preoperatively using the Freiburg monosyllabic test in unaided condition, with the trial BCHD preoperatively and with Osia® postoperatively with Osia®. The maximum word recognition score (mWRS) unaided and the word recognition score (WRS) with the test system at 65 dB SPL were correlated with the postoperative WRS with Osia® at 65 dB SPL. RESULTS: Preoperative prediction of postoperative outcome with Osia® was better using the mWRS than by the WRS at 65 dB SPL with the test device on the softband. Postoperative WRS was most predictive for patients with CHL and less predictable for patients with mixed hearing loss with BC-4PTA ≥ 40 dB HL. For the test device on a softband, the achievable outcome tended to a minimum, with the mWRS tending to predict the realistically achievable outcome. CONCLUSION: Osia® can be used for the treatment of CHL and MHL within the indication limits. The average preoperative bone conduction hearing threshold also provides an approximate estimate of the postoperative WRS with Osia®, for which the most accurate prediction is obtained using the preoperative mWRS. Prediction accuracy decreases from a BC-4PTA of ≥ 40 dB HL.

Prevalence of hearing loss in patients with Sjögren syndrome: a systematic review and meta-analysis.

Sjögren syndrome (SS) is a multisystem autoimmune disease, primarily targeting salivary and lacrimal glands; skin, nasal and vaginal dryness, along with musculoskeletal pain and fatigue are the most commonly reported symptoms. Hearing loss is hypothesized to be frequent as well. The purpose of this systematic review was to estimate the prevalence of Hearing loss and its different subtypes in patients with Sjögren syndrome. PRISMA guidelines were followed to ensure highest quality for our systematic review. A random effects model meta-analysis and meta-regression was conducted using I2 as heterogeneity indicator. Eleven observational studies were included in this systematic review. Ten of them were cross-sectional, while one study was case-control. Studies were assessed for risk of bias: all were rated to a moderate level, except for two rated to a low level. Pooled prevalence of any type of hearing loss was 52.2%. After excluding studies rated to moderate bias, the pooled prevalence of hearing loss was 36.7%. We also conducted a subgroup analysis depending on type of hearing loss. Pooled prevalence of sensorineural hearing loss was 42.6%., while pooled prevalence of conductive hearing loss and mixed hearing loss were 5% and 2.3%, respectively. Meta-regression was conducted in an effort to identify possible variables capable to explain high heterogeneity between studies. Sample size and year of study were separately found to account for a portion of heterogeneity between studies of sensorineural hearing loss. Year of study was also found to account for a portion of heterogeneity between studies of conductive hearing loss. In conclusion, sensorineural hearing loss, is highly prevalent in patients with Sjögren syndrome. On this basis, early screening and follow-up of patients with Sjögren syndrome by pure tone audiometry is important.

Clinical performance, audiological outcomes, and quality of life of the Cochlear Osia ® system.

OBJECTIVE: The Cochlear Osseointegrated Steady-State Implant Bone Anchored Hearing Device (Osia) is a surgically implanted titanium apparatus that utilizes a piezoelectric actuator under the skin to address conductive and mixed hearing loss as well as single-sided deafness. The purpose of this study is to examine the clinical, audiologic, and quality-of-life outcomes in patients who underwent Osia implantation. METHODS: This is a retrospective study analyzing 30 adult patients (age 27-86) with conductive healing loss (CHL), mixed hearing loss (MHL), or single-sided deafness (SSD) who were implanted with the Osia device from January 2020 to April 2023 at a single institution by the senior author. Preoperative speech score testing (CNC, AzBio in quiet, AzBio in noise) were performed in all subjects while unaided, wearing conventional air conduction hearing aids, and wearing a softband BAHA. These preoperative speech scores were then compared to post-implantation speech scores using paired t-test analysis to assess for degree of speech improvement. In order to analyze quality of life after Osia implantation, each patient filled out the Glasgow Benefit Inventory (GBI) survey. The GBI is a series of 18 questions answered using a five-point Likert scale that addresses the changes in general health status, physical health status, psychosocial health status, and social support after a medical intervention. RESULTS: CHL, MHL, and SSD patients had significant improvement in hearing and speech recognition scores after Osia implantation compared to preoperative unaided hearing: CNC (14 % vs 80 %, p < 0.0001), AzBio in Quiet (26 % vs 94 %, p < 0.0001), and AzBio in Noise (36 % vs 87 %, p = 0.0001). Preoperative speech scores using the softband BAHA were accurate predictors of post-implantation speech scores and can serve to determine surgical candidacy for the Osia. Post-implantation Glasgow Benefit Inventory patient surveys demonstrated significant improvement in quality of life with patients scoring an average increase of +54.1 points in heath satisfaction. CONCLUSION: Adult patients with CHL, MHL, and SSD can receive significant improvement in speech recognition scores after implantation with the Osia device. This translates to improved quality of life, which was confirmed on the post-implantation Glasgow Benefit Inventory patient surveys.

New Osia® OSI200 active transcutaneous bone-anchored hearing device: how I do it.

INTRODUCTION: The new Osia® OSI200 implant incorporates a receiver coil and Piezo Power™ Transducer into one monolithic unit. Appropriate planning and surgical approach is needed for suitable positioning of the device. METHOD: To optimise the surgical field and provide tension-free wound closure our team have adopted a versatile 'Sheffield-S' post-auricular incision which remains hidden within the hairline. CONCLUSION: This incision provides adequate exposure for device placement and bone polishing/recessing. The soft tissue approach has resulted in improved operative efficacy particularly in those patients with irregular cortical bone or where pre-existing osseointegrated implants need to be removed or avoided.

Evaluation of postural stability and vestibulo-ocular reflex in adults with chronic suppurative otitis media.

PURPOSE: To evaluate the vestibulospinal reflex and vestibulo-ocular reflex (VOR) in patients with chronic suppurative otitis media (CSOM) using posturography and the video head impulse test (vHIT). METHODS: Sixty-five patients with CSOM and 65 healthy participants as controls were included. Patients with CSOM were instructed to complete the dizziness handicap inventory (DHI). All participants underwent otoscopy, pure-tone audiometry, posturography sensory organization test (SOT), and vHIT. RESULTS: Patients with CSOM exhibited a high prevalence of dizziness. The CSOM group had poor SOT vestibular scores compared to the control group. Patients with CSOM had worse sways in the antero-posterior and mediolateral planes. The CSOM group was divided into two subgroups according to the type of hearing loss. SOT vestibular scores were significantly poorer in the mixed hearing loss group than those in the conductive hearing loss group. We found a positive correlation between disease duration and poor SOT vestibular scores. Moreover, poor SOT vestibular scores correlated with high DHI scores. We found abnormalities in the vHIT results in the CSOM group in the form of low VOR gain and corrective saccades. CONCLUSION: Our study provides clinical evidence of dizziness, poor postural control, and VOR abnormalities in patients with CSOM. The presence of sensory elements of hearing loss in patients with CSOM appears to be positively associated with vestibular dysfunction.

Multicentric study on surgical information and early safety and performance results with the Bonebridge BCI 602: an active transcutaneous bone conduction hearing implant.

AIM: This European multicentric study aimed to prove safety and performance of the Bonebridge BCI 602 in children and adults suffering from either conductive hearing loss (CHL), mixed hearing loss (MHL), or single-sided sensorineural deafness (SSD). METHODS: 33 patients (13 adults and 10 children with either CHL or MHL and 10 patients with SSD) in three study groups were included. Patients were their own controls (single-subject repeated measures), comparing the unaided or pre-operative to the 3-month post-operative outcomes. Performance was evaluated by sound field thresholds (SF), word recognition scores (WRS) and/or speech reception thresholds in quiet (SRT) and in noise (SNR). Safety was demonstrated with a device-specific surgical questionnaire, adverse event reporting and stable pure-tone measurements. RESULTS: The Bonebridge BCI 602 significantly improved SF thresholds (+ 25.5 dB CHL/MHL/SSD), speech intelligibility in WRS (+ 68.0% CHL/MHL) and SRT in quiet (- 16.5 dB C/MHL) and in noise (- 3.51 dB SNR SSD). Air conduction (AC) and bone conduction (BC) thresholds remained stable over time. All adverse events were resolved, with none unanticipated. Mean audio processor wearing times in hours [h] per day for the CHL/MHL group were ~ 13 h for adults, ~ 11 h for paediatrics and ~ 6 h for the SSD group. The average surgical length was 57 min for the CHL/MHL group and 42 min for the SSD group. The versatility of the BCI 602 (reduced drilling depth and ability to bend the transition for optimal placement) allows for treatment of normal, pre-operated and malformed anatomies. All audiological endpoints were reached. CONCLUSIONS: The Bonebridge BCI 602 significantly improved hearing thresholds and speech understanding. Since implant placement follows the patient's anatomy instead of the shape of the device and the duration of surgery is shorter than with its predecessor, implantation is easier with the BCI 602. Performance and safety were proven for adults and children as well as for the CHL/MHL and SSD indications 3 months post-operatively.

[Prediction of speech understanding with the transcutaneous partially implantable bone conduction hearing system Osia®. German Version]. / Vorhersage des postoperativen Sprachverstehens mit dem transkutanen teilimplantierbaren Knochenleitungshörsystem Osia®.

BACKGROUND: The active transcutaneous, partially implantable osseointegrated bone conduction system Cochlear™ Osia® (Cochlear, Sydney, Australia) has been approved for use in German-speaking countries since April 2021. The Osia is indicated for patients with conductive (CHL) or mixed hearing loss (MHL) with an average bone conduction (BC) hearing loss of 55 dB or less, or with single-sided deafness (SSD). OBJECTIVES: The aim of this retrospective study was to investigate the prediction of postoperative speech recognition with Osia and to evaluate the speech recognition of patients with MHL and an aided dynamic range of less than 30 dB with Osia. MATERIALS AND METHODS: Between 2017 and 2022, 29 adult patients were fitted with the Osia, 10 patients (11 ears) with CHL and 19 patients (21 ears) with MHL. MHL was subdivided into two groups: MHL­I with four-frequency pure-tone average in BC (BC-4PTA) ≥ 20 dB HL and < 40 dB HL (n = 15 patients; 20 ears) vs. MHL-II with BC-4PTA ≥ 40 dB HL (n = 4 patients; 5 ears). All patients tested a bone conduction hearing device on a softband preoperatively. Speech intelligibility in quiet was assessed preoperatively using the Freiburg monosyllabic test unaided and with the test system and postoperatively with Osia. The maximum monosyllabic score (mEV) unaided and the monosyllabic score with the test system at 65 dB SPL were correlated with the postoperative monosyllabic score with Osia at 65 dB SPL. RESULTS: Preoperative prediction of postoperative outcome with Osia was better using the mEV than the EV at 65 dB SPL with the test device on the softband. Postoperative EV was most predictive for patients with CHL and least predictive for patients with mixed hearing loss with 4PTA BC ≥ 40 dB HL. For the test device at softband, results tended to show the minimum achievable outcome and the mEV tended to predict the realistically achievable outcome. CONCLUSION: Osia can be used for the treatment of CHL and MHL within the indication limits. The average preoperative bone conduction hearing threshold also provides an approximate estimate of the postoperative EV with Osia, for which the most accurate prediction is obtained using the preoperative mEV. Prediction accuracy decreases from a BC-4PTA of ≥ 40 dB.

Long-term audiological benefit with an active transcutaneous bone-conduction device: a retrospective cohort analysis.

PURPOSE: To evaluate long-term audiological, surgical and safety outcomes of a complex patient cohort treated with an active transcutaneous bone-conduction device (tBCD). METHODS: This retrospective, monocentric cohort analysis involves 31 adults with conductive (CHL) and mixed hearing loss (MHL). For outcome analysis, study results were divided into short-term follow-up data (< 12 months) and long-term follow-up data (> 12 months). RESULTS: 31 patients with a total person-time of 90.4 years and an average number of 3.2 ± 1.5 preoperatively performed surgeries on the implanted side were investigated. Mean BCD-aided PTA4 thresholds were significantly lower than unaided PTA4AC before surgery (64.7 ± 16.1 dB HL; CHL 50.6 ± 10.6 dB HL; MHL 72.8 ± 12.8 dB HL) and did not change between short-term (42.3 ± 13.1 dB HL; CHL 35.8 ± 6.8 dB HL; MHL 45.2 ± 14.3 dB HL) and long-term (43.4 ± 10.0 dB HL; CHL 35.8 ± 4.3 dB HL; MHL 48.1 ± 9.6 dB HL) follow-up periods. Speech intelligibility in the Freiburg monosyllables test at 65 dB improved significantly, from 16.3 ± 21.5% (CHL 26.8 ± 19.0%; MHL 8.7 ± 20.5%) in the unaided condition to 82.7 ± 15.5% (CHL 90.0 ± 12.2%; MHL 79.4 ± 16.3%) in the short-term and 85.5 ± 13.2% (CHL 93.8 ± 7.9%; MHL 79.5 ± 13.3%) BCD-aided in the long-term follow-up periods. Ten minor procedure-related and 6 implant-related (5 minor, 1 major) AEs occurred over the total follow-up period. CONCLUSION: The device provides satisfactory audiological and speech benefit over long-term follow-up periods, up to 7 years. Explant rates were very low, while the overall rate of manageable AEs was high in this complex patient cohort. The device is considered a safe and effective option in the long-term hearing rehabilitation of patients with CHL and MHL.

Clinical and functional results after implantation of the bonebridge, a semi-implantable, active transcutaneous bone conduction device, in children and adults.

PURPOSE: Aim of the study was to evaluate the surgical, clinical and audiological outcome of 32 implantations of the Bonebridge, a semi-implantable transcutaneous active bone conduction implant. METHODS: In a retrospective cohort study, we analyzed data for 32 implantations in 31 patients (one bilateral case; seven age < 16 years) with conductive or mixed hearing loss, malformations, after multiple ear surgery, or with single-sided deafness as contralateral routing of signal (CROS). RESULTS: Four implantations were done as CROS. Five cases were simultaneously planned with ear prosthesis anchors, and 23 implantations (72%) were planned through three-dimensional (3D) "virtual surgery." In all 3D-planned cases, the implant could be placed as expected. For implant-related complications, rates were 12.5% for minor and 3.1% for major complications. Implantation significantly improved mean sound field thresholds from a preoperative 60 dB HL (SD 12) to 33 dB HL (SD 6) at 3 postoperative months and 34 dB HL (SD 6) at > 11 postoperative months (p < 0.0001). Word recognition score in quiet at 65 dB SPL improved from 11% (SD 20) preoperatively to 74% (SD 19) at 3 months and 83% (SD 15) at > 11 months (p < 0.0001). The speech reception threshold in noise improved from - 1.01 dB unaided to - 2.69 dB best-aided (p = 0.0018). CONCLUSION: We found a clinically relevant audiological benefit with Bonebridge. To overcome anatomical challenges, we recommend preoperative 3D planning in small and hypoplastic mastoids, children, ear malformation, and simultaneous implantation of ear prosthesis anchors and after multiple ear surgery.

Long-term data of the new transcutaneous partially implantable bone conduction hearing system Osia®.

PURPOSE: The new active transcutaneous partially implantable osseointegrated system Cochlear™ Osia® System is indicated in case of conductive or mixed hearing loss (CHL/MHL) with a maximum average bone conduction hearing loss of 55 dB, or in single-sided deafness (SSD). The implant directly stimulates the bone via a piezoelectric transducer and is directed by an external sound processor. We conducted a monocentric retrospective longitudinal within-subject clinical study at our tertiary academic referral center. The aim was to investigate long-term data (2017-2021) on audiological outcomes and hearing-related quality of life for the Osia system. METHODS: Between 2017 and 2020, 22 adults (18: CHL/MHL; 3: SSD) were implanted with the Osia100 implant; seven received bilateral implants. As of 10/2020, the sound processor was upgraded to Osia 2. RESULTS: Mean Osia system use by 04/2021 was 30.9 ± 8.6 months (range 17-40 months). Unaided bone conduction thresholds were unchanged postoperatively. One patient had to be explanted because of prolonged wound infection. Aided hearing thresholds were significantly lower compared to the unaided thresholds preoperatively, along with a marked increase in speech recognition in quiet. Speech processor upgrade resulted in a stable benefit. Patients with CHL/MHL and SSD showed a similar improvement in self-rated hearing performance revealed by SSQ, APHAB, and HUI questionnaires. CONCLUSION: The Osia system is a safe, effective and sustainable option for treatment of conductive and mixed hearing loss or single-sided deafness.

Functional results with active middle ear implant or semi-implantable bone conduction device in patients with comparable hearing loss.

OBJECTIVE: In patients with conductive (CHL) or mixed hearing loss (MHL), hearing rehabilitation with an implantable hearing system, active middle ear implant (AMEI) or a semi-implantable bone-conduction device (SIBCD), is an option when conventional hearing aids are insufficient, or patients are unable to wear them. DESIGN: Retrospective analysis of 20 consecutive patients (24 implants) with a comparison of demographic characteristics and audiometric results (air-bone gap = ABG, effective hearing gain = EHG, functional hearing gain = FHG, Freiburg Monosyllabic Test in quiet, Oldenburg Sentence Test in noise = OLSA). STUDY SAMPLE: Patients, eligible for both devices, who received either AMEI or SIBCD due to CHL or MHL. RESULTS: Analysis showed no significant differences in post-operative functional hearing results between the group of AMEI vs. SIBCD (ABG-reduction: 31.6 ± 12.4 dB HL vs. 28.0 ± 11.8 dB HL; p = 0.702; EHG: -1.6 ± 7.7 dB HL vs. -1.2 ± 4.2 dB HL; p = 0.090; FHG: 33.4 ± 12.6 dB HL vs. 26.1 ± 11.7 dB HL; p = 0.192; Freiburg: 83.0 ± 15.6% vs. 83.6 ± 14.2%; Freiburg-improvement: 57.7 ± 26.8% vs. 68.2 ± 19.7%; p = 0.294; OLSA: -2.7 ± 3.0 SNR vs. -1.4 ± 3.6 SNR; OLSA-improvement: 2.6 ± 2.1 dB vs. 3.7 ± 2.8 dB; p = 0.323). Four patients had the AMEI explanted due to insufficient functioning and later received a SIBCD. CONCLUSIONS: Due to more challenging anatomical conditions, a surgical technique for the AMEI is more complex. However, functional results are comparable to the SIBCD. Therefore, proper patient counselling and cautious choice of the device are mandated before surgery.

Clinical consensus document for fitting non-surgical transcutaneous bone conduction hearing devices to children.

This clinical consensus document addresses the assessment, selection, and fitting considerations for non-surgical bone conduction hearing devices (BCHD) for children under the age of 5 years identified as having unilateral or bilateral, permanent conductive or mixed hearing losses. Children with profound unilateral sensorineural hearing losses are not addressed. The document was developed based on evidence review and consensus by The Paediatric Bone Conduction Working Group, which is composed of audiologists from North America who have experience working with BCHDs in children. The document aims to provide clinical direction for an area of paediatric audiology practice that is under development and is therefore lacking in standard protocols or guidelines. This work may serve as a basis for future research and clinical contributions to support prospective paediatric audiology practices.

Increased maximum power output may improve speech recognition with bone conduction hearing devices.

OBJECTIVE: To investigate the influence of maximum power output of bone conduction hearing devices on speech recognition in quiet and in noise in experienced users of bone conduction hearing devices. DESIGN: Prospective, randomised cross-over investigation comparing speech recognition performance, subjective sound quality, and device preference between two bone conduction hearing devices with different maximum power outputs. STUDY SAMPLE: Sixteen adult subjects with conductive or mixed hearing loss. RESULTS: Both speech recognition in quiet and speech recognition in noise improved significantly when using the device with high vs. lower maximum power output. Mean improvement in word recognition score in quiet was 10.5% and the mean speech reception threshold in noise improved by 0.9 dB SNR. Compared to the device with lower maximum power output, the sound quality was rated significantly higher with the device with high maximum power output, which was also the device of preference for 81% of the subjects. CONCLUSION: Bone conduction hearing devices with higher maximum power output have the potential to improve speech recognition in both quiet and noisy listening environments.

Patient with Aberrant Internal Carotid Artery in the Middle Ear Presenting with Rare Symptoms of Mixed Hearing Loss and Postauricular Pain: A Case Report.

Aberrant internal carotid artery in the middle ear (aICA) is a rare congenital malformation in which the internal carotid artery passes through the tympanic cavity without being separated by bone. A pulsatile vascular mass can be observed in the tympanic cavity of patients with aICA. The diagnosis of aICA may be challenging because improper surgery or treatment can lead to iatrogenic injury, including massive hemorrhage. The aim of this case report was to describe a 39-year-old woman with aICA presenting with mixed hearing loss and postauricular pain. We provided detailed clinical images of the patient to illustrate how aICA can be diagnosed. Because this patient's aICA had no risk of bleeding, close observation, pharmacological therapy, and regular follow-up were applied. The patient's postauricular pain was significantly improved after treatment with oxcarbazepine. At the time of this manuscript's preparation, the patient had been monitored for 10 years and had stable mixed hearing loss with no other complications. Based on the management of this patient, oxcarbazepine can improve aICA-associated postauricular pain, and conservative treatment should be prioritized in cases of aICA without a risk of bleeding. Further studies in a large cohort are required to confirm our findings and recommendations.

Bone-Anchored and Closed Skin Bonebridge Implant in Adults: Hearing Performances and Quality of Life.

INTRODUCTION: Bonebridge® is a novel active bone-anchored hearing implant. The purpose of this study was to evaluate the ease of implantation, the hearing performances, and the patient-reported benefit. MATERIALS AND METHODS: This is a prospective cross-sectional study of 24 consecutive adult patients implanted for a mixed hearing loss (13 chronic otitis media (COM) and 11 other aetiologies). Twenty-one implants were placed in the retrosigmoid position and 3 in the mastoid. Audiometry, Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire, as well as 5 implant-specific questions (analogue visual scale [AVS] 0-10 score), was administered. RESULTS: Surgery lasted 73 ± 29.7 min on average. No major complication occurred. All patients were users at the last follow-up visit (median: 9-month range: 3-25). The average prosthetic gain was similar in COM and other aetiologies (43 ± 4.8 dB and 50 ± 7.2, respectively, not significant, Wilcoxon test). Bone-conduction thresholds were not deteriorated by surgery (Kruskal-Wallis test, not significant). APHAB scores improved in all categories except aversiveness (global score 45 ± 7.0% in COM and 32 ± 10.2% in others, not significant, and Wilcoxon test). Local pain (AVS: 3.23 ± 3.2, n = 16) and manipulation difficulties (3.1 ± 3.69) were low. The device was considered aesthetic (8.3 ± 2.49). Perfectible autonomy (5.0 ± 2.8) and difficulties wearing the implant during sport or at work (5.1 ± 3.47) were the weakest points. CONCLUSIONS: BoneBridge® implant provides reproducible results for the rehabilitation of mixed hearing losses and unilateral hearing loss.

Association of systemic lupus erythematosus with hearing loss: a systemic review and meta-analysis.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease that can affect virtually any organ, including middle and/or inner ear. The objective of the current systematic review and meta-analysis was to investigate the association of SLE with the different subtypes of hearing loss. This systematic review and meta-analysis was conducted in agreement with the PRISMA guidelines. The review protocol was registered in the PROSPERO international prospective register of systematic reviews ( https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=216353 ). A random effects model meta-analysis was carried out while heterogeneity was appraised by I2. Subgroup analysis and sensitivity analysis were also performed. Nine studies comprising 7,654 SLE patients and 37,244 controls were included in this systematic review. Four of them were rated to a moderate rate of bias, while five of them were rated to a low rate of bias. SLE patients had significantly increased odds of sensorineural hearing loss (SNHL) compared with controls (OR 2.31; 95%CI 1.48-3.60; I2 = 0). SLE patients did not have significantly increased odds of Conductive Hearing Loss (CHL) (OR 1.30; 95% CI 0.23-7.45; I2 = 0). Only one study reported on the outcome of Mixed Hearing Loss (MHL) (3 events in SLE group vs. 0 events in control group). Subgroup analysis, based on study design and detection method of hearing loss also showed significantly increased odds of SNHL in SLE patients. The significantly increased odds of SNHL in SLE persisted even after sensitivity analysis. In conclusion, SLE is significantly associated with SNHL; SLE is not associated with CHL, while, due to lack of data, we could not reach a conclusion regarding the odds of MHL in SLE patients. Pure tone audiometry as a screening test and follow-up test in SLE patients could be of essence. Management and prognosis of hearing loss in SLE patients should be discussed.

Diagnosis and patterns of hearing loss in children with severe developmental delay.

INTRODUCTION & OBJECTIVE: Children with cognitive delay often experience challenges with obtaining hearing thresholds through behavioral audiometry (BA). This necessitates sedated Auditory Brainstem Response (sABR) testing. This study aimed to evaluate diagnostic and hearing patterns in children with Down Syndrome (DS), Autism Spectrum Disorder (ASD), Global Developmental delay (GDD), and Cerebral Palsy (CP) who were unable to complete reliable BA testing due to severe cognitive delay. METHODS: Retrospective chart review on a cohort of children aged 0.5-18 years with a diagnosis of DS, ASD, GDD, or CP who underwent sABR due to unsuccessful BA testing. This was performed at a tertiary care institution from 2014 to 2019. Testing patterns and audiometric data were collected. RESULTS: Across 15 DS, 39 ASD, 10 GDD, and 11 CP patients, the average time from first nondiagnostic BA to sABR ranged from 8.6 months (in GDD) to 21.8 months (in DS). The average number of BAs performed before sABR ranged from 1.6 (in ASD and GDD) to 2.7 (in DS). Hearing loss (HL) was diagnosed in 10%, 13%, 36% and 46% of patients with GDD, ASD, CP and DS respectively. Up to 75% of the HL was sensorineural (in CP patients). CONCLUSION: In children with significant cognitive delays, a high incidence of HL (especially SNHL) was identified, therefore high suspicion for HL should be held in these patients. Multiple unsuccessful BAs contribute to prolonged time to diagnosis and treatment, thus prompt sABR should be performed in patients whose severe cognitive delay inhibits reliable testing with BA.

Revision of active middle ear implants (AMEI): causes, surgical issues and rehabilitative transition at a single implanting center.

PURPOSE: To report on failures related to active middle ear implants (AMEI) surgery, within a series of subjects treated at a single Implanting Center. METHODS: A retrospective review of 79 cases of implanted AMEI has been performed to report the failure ratio, the causes for the failure and the selected rehabilitative solution. The AMEI included 25 Vibrant Soundbridge® (Medel, Innsbruck, Austria), 20 as round window vibroplasty (RW-VSB) for mixed hearing loss, 5 as incus-vibroplasty for sensorineural hearing loss; 7 MET/Carina® (Cochlear, Melbourne, Australia), 2 MET for mixed and sensorineural hearing loss, 5 Carina for sensorineural hearing loss; 43 Esteem® (EnvoyMedical, St Paul, USA) for sensorineural hearing loss; 3 Maxum® (Ototronics, Texas, USA) for sensorineural hearing loss; 1 Codacs® (Cochlear, Melbourne, Australia) for severe mixed hearing loss. RESULTS: The overall complication rate affected 20% of the implanted devices, individually ranging from 6.9 to 100%. Hardware system failures were recorded in all the AMEI, ranging from 10 to 50%. The alternative auditory rehabilitation included replacement of the same system in 2 cases, use of a conventional hearing aid in 3 cases, Cochlear implantation in 4 cases and implantation in the contralateral ear in 2 cases. CONCLUSION: The present clinical experience showed that, in spite of the successful functional rate displayed by the majority of the AMEI implantees, complications may occur to a certain percentage of cases and should prompt the professionals to select alternative solutions, starting from the (re)adoption of a conventional hearing aid and ending up to Cochlear implantation.