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Purpose: The indications of Vibrant Soundbridge (VSB) have been expanded to include patients with conductive and mixed hearing loss due to congenital aural atresia (CAA). However, the current evidence supporting the auditory outcomes of VSB is based mainly on case reports and retrospective chart reviews. Therefore, the present systematic review aims to summarize and critically appraise the current evidence regarding the safety and effectiveness of VSB in children and adult patients with CAA. Methods: A systematic literature search retrieved studies that evaluated the outcomes of unilateral or bilateral implantation of VSB in patients with CAA. The bibliographic search was conducted in PubMed, Scopus, EBSCO, and Cochrane Central Register of Controlled Trials (CENTRAL) databases from January 2000 to December 2022. Results: Twenty-seven studies were included in the present systematic review. Overall, the speech perception after VSB was good, with a mean word recognition score (WRS) score ranging from 60 to 96.7%. The mean postoperative speech recognition threshold (SRT) after implantation ranged from 20.8 to 50 dB. The effective gain was reported in 15 studies, ranging from 31.3 to 45.5 dB. In terms of user satisfaction with VSB, the included studies showed significant improvements in the patient-reported outcomes, such as the Speech Spatial and Qualities of Hearing scale and Glasgow Hearing Aid Benefit Profile. The VSB implantation was generally safe with low incidence of postoperative complications. Conclusion: VSB provides significant benefits to individuals with hearing loss owing to CAA, with very good subjective outcomes and a low risk of complications. [ABSTRACT FROM AUTHOR]

Introduction Atresia of the external auditory canal affects 1 in every 10 thousand to 20 thousand live births, with a much higher prevalence in Latin America, at 5 to 21 out of every 10 thousand newborns. The treatment involves esthetic and functional aspects. Regarding the functional treatment, there are surgical and nonsurgical alternatives like spectacle frames and rigid and softband systems. Active transcutaneous bone conduction implants (BCIs) achieve good sound transmission and directly stimulate the bone. Objective To assess the audiological performance and subjective satisfaction of children implanted with an active transcutaneous BCI for more than one year and to compare the outcomes with a nonsurgical adhesive bone conduction device (aBCD) in the same users. Methods The present is a prospective, multicentric study. The audiological performance was evaluated at 1, 6, and 12 months postactivation, and after a 1-month trial with the nonsurgical device. Results Ten patients completed all tests. The 4-frequency pure-tone average (4PTA) in the unaided condition was of 65 dB HL, which improved significantly to 20 dB HL after using the BCI for 12 months. The speech recognition in quiet in the unaided condition was of 33% on average, which improved significantly, to 99% with the BCI, and to 91% with the aBCD. Conclusion The aBCD demonstrated sufficient hearing improvement and subjective satisfaction; thus, it is a good solution for hearing rehabilitation if surgery is not desired or not possible. If surgery is an option, the BCI is the superior device in terms of hearing outcomes, particularly background noise and subjective satisfaction. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study was to evaluate the long-term effectiveness and acceptance of the active middle ear implant system Vibrant Soundbridge (VSB®, MED-EL, Austria) in patients with aural atresia or aplasia (children and adults). Methods: Data from 51 patients (mean age 13.9 ± 11.3 years), 42 (79.2%) children and adolescents, and 11 (20.8%) adults) who received a VSB implant between 2009 and 2019 at the Department of Otolaryngology at LMU Clinic Großhadern, Munich were included in the study. Pure-tone audiometry, speech recognition in a quiet environment and in a noisy environment were performed preoperatively, during the first fitting of the audio processor, after 1–3 years, after 3–5 years, and after 5 years (if possible). The follow-up period ranged from 11 to 157 months with a mean of 58.6 months (4.8 years). Furthermore, the benefit of the VSB was evaluated by self-assessment questionnaires (Speech, Spatial, and Qualities of Hearing Scale, respectively, for parents). Results: Significant improvements were observed in hearing and speech comprehension immediately after the initial fitting of the VSB system (mean hearing gain 38.4 ± 9.4 dB HL) and at follow-up intervals (1–3, 3–5 and after 5 years) for children and adults (p < 0.01). The values remained stable over the long-term, indicating a sustained functional gain from the VSB (mean hearing gain 38.9 ± 9.2 dB HL). The results of the self-assessments affirm the positive influence on hearing and speech comprehension with the VSB. With the VSB, there was an improvement of 41.3 ± 13.7% in the Freiburg monosyllable test. Conclusion: These results (a stable hearing gain over the long term, a good tolerance of the implant and an improvement in quality of life) affirm the recommendation for using the active middle ear implant VSB as early as permitted for aural atresia and aplasia patients. This study represents the audiometric results with the (to date) largest collective of aural atresia patients and with a long follow-up period. [ABSTRACT FROM AUTHOR]

Introduction Atresia of the external auditory canal affects 1 in every 10 thousand to 20 thousand live births, with a much higher prevalence in Latin America, at 5 to 21 out of every 10 thousand newborns. The treatment involves esthetic and functional aspects. Regarding the functional treatment, there are surgical and nonsurgical alternatives like spectacle frames and rigid and softband systems. Active transcutaneous bone conduction implants (BCIs) achieve good sound transmission and directly stimulate the bone.

A relatively loud sound is audible when a vibrator is attached to the aural cartilage. This form of conduction is referred to as cartilage conduction (CC). In Japan, a new type of hearing aid has been developed using CC and has been available in clinical practice since 2017. A clinical study conducted prior to its launch demonstrated its benefits, particularly in patients with aural atresia who were unable to use air conduction hearing aids. Several studies have been published on the benefits of CC hearing aids since their introduction into clinical practice. Most of the patients included in these studies had canal stenosis or aural atresia, and the purchase rates of CC hearing aids in these patients were relatively high. However, the number of patients with canal-open ears was small, with overall poor results in the trials, with the exception of patients with continuous otorrhea. CC hearing aids are considered a good option for compensating for hearing loss in ears with canal stenosis or atresia in both bilateral and unilateral cases. However, CC hearing aids are not currently considered the first choice for patients with a canal-open ear.

Clinical findings on cartilage conduction hearing aids (CCHAs) have gradually become clear; however, few reports include a large number of cases. This study included 91 ears from 69 patients who underwent CCHA fitting in our hospital. Their ears were divided into six groups (i.e., bilateral aural atresia or severe canal stenosis, unilateral aural atresia or severe canal stenosis, chronic otitis media or chronic otitis externa with otorrhea, sensorineural hearing loss, mixed hearing loss, and conductive hearing loss) according to their clinical diagnosis and type of hearing loss. Most clinical diagnoses were aural atresia or meatal stenosis (bilateral, 21.8%; unilateral, 39.6%). The purchase rate of CCHAs was higher in the closed-ear group (bilateral, 77.3%; unilateral, 62.5%). In the bilateral closed-ear group, air conduction thresholds at 1000, 2000, and 4000 Hz and aided thresholds with CCHAs at 4000 Hz were significantly lower in the purchase group than the non-purchase group. No significant difference was observed between the purchase and non-purchase groups in the unilateral closed-ear group. In the bilateral closed-ear group, air conduction thresholds and aided thresholds were associated with the purchase rate of CCHAs. In the unilateral closed-ear group, factors other than hearing might have affected the purchase rate of CCHAs.

Congenital unilateral hearing loss (UHL) represents a contemporary audiologic challenge. Children with UHL can struggle with understanding speech in noise, localizing sounds, developing language, and maintaining academic performance, leading to low self-esteem, anxiety, and decreased social support. Two specific conditions related to UHL in children are single-sided deafness (SSD) and unilateral auris atresia (UAA). This was a retrospective observational study on a group of children with UHL. The Simplified Italian Matrix Sentence Test was used for the assessment of speech reception threshold (SRT) in different conditions: speech and noise from the front (S0N0), speech at 45° from the side of the better ear and noise at 45° from the opposite side (SbNw), and vice versa (SwNb). Each test was conducted unaided, with a bone-anchored hearing device (BAHD), and with a remote microphone (RM) system. The use of a BAHD and RM led to an improvement in SRT in S0N0 and SwNb conditions. The SSD subgroup demonstrated significant benefits with both devices in SwNb, and the UAA subgroup from the use of BAHD in S0N0. In conclusion, the study underscores the potential benefits of both devices in enhancing speech perception for UHL children, providing insights into effective intervention strategies for these challenging cases. [ABSTRACT FROM AUTHOR]

Abstract Background Congenital aural atresia (CAA) and stenosis is a rare defect affecting the external auditory canal which causes varying degrees of hearing impairment. This malformation may be unilateral or bilateral. Timely hearing rehabilitation in these patients is paramount to ensure appropriate speech and language development. Thus, early detection and hearing assessment with timely intervention in the form of hearing device is crucial. There is a wide range of hearing amplification devices available for these patients, catering to individual needs and preferences. This study investigated the options and choices of hearing rehabilitation among patients with CAA in our center. This study also compared the hearing gain of different types of hearing devices and patient satisfaction levels. Methods This was a retrospective analysis of 55 patients with congenital aural atresia or stenosis under follow-up at the Otorhinolaryngology and Audiology Clinic of a tertiary academic institution. Data on the timing of hearing loss diagnosis and intervention, type of hearing rehabilitation, hearing gain, Jahrsdoerfer score from temporal bone computed tomography, and patient satisfaction level was collected and analyzed. Results Fifty-five patients were recruited with ages between 3.6 months to 58 years old. The majority of patients (63.6%) had moderate hearing loss, followed by severe hearing loss (18.2%), mild hearing loss (9.1%) and profound hearing loss (7.3%). 32.7% of patients had early hearing loss diagnosed by 3 months old, while 67.3% were diagnosed after 3 months of age. Bone anchored hearing implant (BAHA Attract/Connect) gave the highest mean hearing gain of 35.23 dB (SD ± 9.75) compared to air conduction hearing aid, bone conduction hearing aid, and canalplasty. Patients implanted with BAHA Attract/Connect reported the highest average satisfaction scores of 7.74. Conclusion A significant proportion of patients have delayed diagnosis and intervention. Bone-anchored hearing implants provide excellent hearing improvements in patients with CAA with good patient satisfaction.

Clinical findings on cartilage conduction hearing aids (CCHAs) have gradually become clear; however, few reports include a large number of cases. This study included 91 ears from 69 patients who underwent CCHA fitting in our hospital. Their ears were divided into six groups (i.e., bilateral aural atresia or severe canal stenosis, unilateral aural atresia or severe canal stenosis, chronic otitis media or chronic otitis externa with otorrhea, sensorineural hearing loss, mixed hearing loss, and conductive hearing loss) according to their clinical diagnosis and type of hearing loss. Most clinical diagnoses were aural atresia or meatal stenosis (bilateral, 21.8%; unilateral, 39.6%). The purchase rate of CCHAs was higher in the closed-ear group (bilateral, 77.3%; unilateral, 62.5%). In the bilateral closed-ear group, air conduction thresholds at 1000, 2000, and 4000 Hz and aided thresholds with CCHAs at 4000 Hz were significantly lower in the purchase group than the non-purchase group. No significant difference was observed between the purchase and non-purchase groups in the unilateral closed-ear group. In the bilateral closed-ear group, air conduction thresholds and aided thresholds were associated with the purchase rate of CCHAs. In the unilateral closed-ear group, factors other than hearing might have affected the purchase rate of CCHAs. [ABSTRACT FROM AUTHOR]

A relatively loud sound is audible when a vibrator is attached to the aural cartilage. This form of conduction is referred to as cartilage conduction (CC). In Japan, a new type of hearing aid has been developed using CC and has been available in clinical practice since 2017. A clinical study conducted prior to its launch demonstrated its benefits, particularly in patients with aural atresia who were unable to use air conduction hearing aids. Several studies have been published on the benefits of CC hearing aids since their introduction into clinical practice. Most of the patients included in these studies had canal stenosis or aural atresia, and the purchase rates of CC hearing aids in these patients were relatively high. However, the number of patients with canal-open ears was small, with overall poor results in the trials, with the exception of patients with continuous otorrhea. CC hearing aids are considered a good option for compensating for hearing loss in ears with canal stenosis or atresia in both bilateral and unilateral cases. However, CC hearing aids are not currently considered the first choice for patients with a canal-open ear. [ABSTRACT FROM AUTHOR]

Abstract Objectives Microtia and aural atresia are congenital ear anomalies with a wide‐ranging spectrum of phenotypes and varied functional and psychosocial consequences for patients. This study seeks to analyze the management of microtia‐atresia patients at our center over a 20‐year period and to propose recommendations for advancing microtia‐atresia care at a national level. Methods We performed a retrospective analysis of data from patients presenting to the Massachusetts Eye and Ear (Boston, MA) for initial otolaryngology consultation for congenital microtia and/or aural atresia between 1999 and 2018. Results Over the 20‐year study period, 229 patients presented to our microtia‐atresia center at a median age of 7 years. The severity of microtia was most commonly classified as grade III (n = 87, 38%), 61% (n = 140) of patients had complete atresia, the median Jahrsdoerfer grading scale score was 6 (range 0–10), and 81 patients (35%) underwent surgery for microtia repair. For hearing rehabilitation, 30 patients (64%) underwent bone conduction device implantation and 17 patients (36%) underwent atresiaplasty. The implementation of an interdisciplinary, longitudinal care model resulted in an increase in patient (r = 0.819, p

Objective: To compare audiometric outcomes of a new cartilage conduction hearing device (CCD) with traditional bone conduction hearing devices (BCDs). Study Design: Clinical trial and crossover study design. Setting: Tertiary academic center. Methods: Sixteen adults (19 ears) with congenital aural atresia or overclosed ear canals who previously underwent BCD implantation were fitted with a CCD. Audiometric data were collected with use of the BCD and the CCD. Results: The mean pretreatment 4‐frequency pure tone average was 81 dB. The mean aided pure tone averages with the BCD and CCD were 27 and 32 dB (P =.002), and the mean functional gains were 54 and 49 dB (P =.002), respectively. The mean consonant‐nucleus‐consonant scores with the BCD were 90% (best aided) and 80% (aided ear isolated), and those with the CCD were 86% and 76%. Mean AzBio scores were 90% (quiet), 77% (+10 dB SNR [signal to noise ratio]), and 52% (+5 dB SNR) when isolating the BCD ear and 90%, 73%, and 41% when isolating the CCD ear. No difference in speech scores achieved statistical significance except the AzBio isolated to the aided ear in the +5–dB SNR condition, which favored the BCD (P =.01). Conclusion: Pure tone audiometric outcomes with the BCD show a small advantage over the CCD, with the difference driven mainly by high‐frequency responses. Speech outcomes were equivalent apart from the +5–db SNR condition, which favored the BCD. [ABSTRACT FROM AUTHOR]

Abstract Objectives To describe the prevalence and significance of first branchial cleft anomalies in children with congenital aural atresia. Methods Retrospective cohort study and case series. Patients were included if they had ICD‐10 code Q16.0, Q16.1, Q16.9, Q17.2, or Q17.9 in their medical record and were seen at UCSF Benioff Children's Hospital from 2012 to 2020 for aural atresia. Children were categorized as having aural atresia and first branchial cleft anomalies if the presence of a first branchial cleft anomaly was noted in otolaryngology provider notes; otherwise, they were categorized as aural atresia alone. Patients with aural atresia and first branchial cleft anomalies were included in the case series. Results Among 125 children with congenital aural atresia, 5 (4%) were identified with first branchial cleft anomalies. In all cases, an epithelialized tract was noted to originate from the inferior aspect of the middle ear cleft, exiting the temporal bone in an inferomedial position adjacent to the stylomastoid foramen, with a cutaneous exit point inferior to the expected location of the native ear canal. There was no association with sex, microtia grade, or laterality of atresia; however, children with aural atresia and first branchial cleft anomalies were significantly more likely to have syndromes such as Goldenhar and Treacher Collins (p = .04) than those with aural atresia alone. 3/5 (60%) children with aural atresia and first branchial cleft anomalies presented with cholesteatoma compared with 1/120 (0.8%) children with aural atresia alone, a significant difference (p

Objectives: Microtia and aural atresia are congenital ear anomalies with a wide‐ranging spectrum of phenotypes and varied functional and psychosocial consequences for patients. This study seeks to analyze the management of microtia‐atresia patients at our center over a 20‐year period and to propose recommendations for advancing microtia‐atresia care at a national level. Methods: We performed a retrospective analysis of data from patients presenting to the Massachusetts Eye and Ear (Boston, MA) for initial otolaryngology consultation for congenital microtia and/or aural atresia between 1999 and 2018. Results: Over the 20‐year study period, 229 patients presented to our microtia‐atresia center at a median age of 7 years. The severity of microtia was most commonly classified as grade III (n = 87, 38%), 61% (n = 140) of patients had complete atresia, the median Jahrsdoerfer grading scale score was 6 (range 0–10), and 81 patients (35%) underwent surgery for microtia repair. For hearing rehabilitation, 30 patients (64%) underwent bone conduction device implantation and 17 patients (36%) underwent atresiaplasty. The implementation of an interdisciplinary, longitudinal care model resulted in an increase in patient (r = 0.819, p <.001) and surgical volume (microtia surgeries, r = 0.521, p =.019; otologic surgeries, r = 0.767, p <.001) at our center over time. Conclusion: An interdisciplinary team approach to microtia‐atresia patient care may result in increased patient volume, which could improve aesthetic and hearing outcomes over time by concentrating care and surgical expertise. Future work should aim to establish standardized clinical consensus recommendations to guide the creation of high‐quality microtia‐atresia care programs. Level of Evidence: 4. [ABSTRACT FROM AUTHOR]

Cartilage conduction hearing aids (CC-HA), bone anchored hearing aids (Baha), and ADHEAR are good choices to improve hearing in patients who cannot use air conduction hearing aids because of aural atresia or continuous otorrhea. As there are many overlaps in the characteristics of these devices, we conducted a comparative trial of CC-HA, Baha, and ADHEAR. We provided patients with the opportunity to select their devices. The data of 6 patients who underwent comparative trials in our department between October 2021 and August 2022 were retrospectively examined. The gains of Baha and CC-HA outweighed those of ADHEAR. Regarding the sound localization test, there was no significant tendency for any of the hearing devices. Regarding the Glasgow Benefit Inventory, there was no clear tendency among the three devices in the quality of life evaluation. The most satisfactory model was the one subjectively chosen by the patient, regardless of the gain and score of the sound source localization test. Therefore, we believe that it is necessary to provide an opportunity for comparative trials and a consultation with each patient in the process of selecting a device. [ABSTRACT FROM AUTHOR]

Purpose: The objective of this study was to assess and compare the long-term safety and subjective satisfaction levels of Bonebridge (BB) and Vibrant Soundbridge (VSB) in patients with congenital unilateral conductive hearing loss (UCHL)., Methods: The hearing effectiveness was measured using sound field hearing threshold (SFHT) and word recognition score (WRS). Long-term safety and subjective satisfaction levels were measured by questionnaires, including Speech, Spatial, and Qualities of Hearing Scale-12 (SSQ-12) and Abbreviated Profile of Hearing Aid Benefit (APHAB)., Results: Both BB and VSB were effective in improving the hearing of patients with congenital UCHL. Four of the 20 patients discontinued wearing the device postoperatively, and no complications were reported by long-term users. Patients experienced an overall improvement in their hearing experience across most subscales of SSQ-12 and a lower hearing problem rate across most subscales of the APHAB after implantation. However, the 'quality of hearing' subscale in the SSQ-12 did not show any improvement, and the aversiveness subscale in the APHAB exhibited a relatively higher percentage of reported problems after BB or VSB implantation. No significant differences were observed in SFHT, WRS, SSQ-12, and APHAB between the two groups., Conclusions: Both BB and VSB are effective in improving the hearing of UCHL patients, with high subjective satisfaction and low complication rates in the long-term postoperative period, with no significant difference between the two devices. Follow-up device fitting is essential, especially to address increased device sound or noise after surgery, reduce non-usage rates and achieve higher subjective satisfaction levels., (Copyright © 2024 Società Italiana di Otorinolaringoiatria e Chirurgia Cervico-Facciale, Rome, Italy.)

ObjectivesTo present recommendations for the coordinated evaluation and management of the hearing and reconstructive needs of patients with microtia and aural atresia.MethodsA national working group of 9 experts on microtia and atresia evaluated a working document on the evaluation and treatment of patients. Treatment options for auricular reconstruction and hearing habilitation were reviewed and integrated into a coordinated care timeline.ResultsRecommendations were created for children with microtia and atresia, including diagnostic considerations, surgical and non-surgical options for hearing management and auricular reconstruction, and the treatment timeline for each option. These recommendations are based on the collective opinion of the group and are intended for otolaryngologists, audiologists, plastic surgeons, anaplastologists, and any provider caring for a patient with microtia and ear canal atresia. Close communication between atresia/hearing reconstruction surgeon and microtia repair surgeon is strongly recommended.

Congenital unilateral hearing loss (UHL) represents a contemporary audiologic challenge. Children with UHL can struggle with understanding speech in noise, localizing sounds, developing language, and maintaining academic performance, leading to low self-esteem, anxiety, and decreased social support. Two specific conditions related to UHL in children are single-sided deafness (SSD) and unilateral auris atresia (UAA). This was a retrospective observational study on a group of children with UHL. The Simplified Italian Matrix Sentence Test was used for the assessment of speech reception threshold (SRT) in different conditions: speech and noise from the front (S0N0), speech at 45° from the side of the better ear and noise at 45° from the opposite side (SbNw), and vice versa (SwNb). Each test was conducted unaided, with a bone-anchored hearing device (BAHD), and with a remote microphone (RM) system. The use of a BAHD and RM led to an improvement in SRT in S0N0 and SwNb conditions. The SSD subgroup demonstrated significant benefits with both devices in SwNb, and the UAA subgroup from the use of BAHD in S0N0. In conclusion, the study underscores the potential benefits of both devices in enhancing speech perception for UHL children, providing insights into effective intervention strategies for these challenging cases.

Congenital aural atresia causes severe conductive hearing loss disturbing auditory development. The differences in speech recognition were investigated between bilateral and unilateral aural atresia. The maximum speech recognition scores (SRSs) were compared between patients with bilateral and unilateral aural atresia. In patients with unilateral aural atresia, the maximum SRSs were compared between the atretic and unaffected ears. Furthermore, the correct response rates for test material monosyllables were compared with those of patients with sensorineural hearing loss (SNHL), which had been previously obtained. Twenty-four patients with aural atresia (8 bilateral, and 16 unilateral) participated. The maximum SRS in unilateral atretic ears (median: 72%) was significantly lower than that in unaffected ears (median: 89%) (p < 0.05) and in bilateral atretic ears (median: 91%) (p < 0.05). Patients with aural atresia had relatively high correct response rates for monosyllables with low correct response rates by patients with SNHL. Conversely, incorrect responses were obtained for several words for which high correct-response rates were attained by patients with SNHL. Poor unilateral atretic-ear development may induce low speech recognition, and the mechanisms underlying speech-recognition reduction differ from those in SNHL. [ABSTRACT FROM AUTHOR]

Microtia techniques have evolved to improve esthetic outcomes, reduce donor site morbidities, and reduce complications. Patients with microtia commonly have aural atresia associated with conductive hearing loss. We present the evolution of our technique for microtia reconstruction and considerations for hearing management in these patients. [ABSTRACT FROM AUTHOR]

Purpose: Most developed countries have implemented some form of universal newborn hearing screening program. Early identification and rehabilitation of congenital hearing loss is important in functional outcome, and the need to identify the cause of hearing impairment has become clear. We aimed to evaluate audiological and etiological outcomes in a large group of patients with failed neonatal hearing screening. Methods: We performed a retrospective chart analysis of patients who were referred to our tertiary referral center after failing neonatal hearing screening during a 12-year period (2007–2019). Screening was based on automated auditory brainstem response (AABR) or a combined approach of AABR and auditory steady-state response (ASSR) with chirp stimulus. Extensive audiometric testing was performed to confirm and determine the type and degree of hearing loss. In case of permanent hearing loss, a standardized etiological protocol was followed to determine the cause. Results: Of the 802 referred newborns, hearing loss was confirmed by diagnostic ABR in 78%. Main causes of hearing loss included otitis media with effusion (56%, higher in patients screened by AABR/ASSR compared to AABR), a genetic disorder (12%), congenital cytomegalovirus infection (cCMV, 5%) and atresia/stenosis of the external ear canal (5%). Of the patients with permanent hearing loss, 15% showed changes in hearing loss severity over time. Conclusion: In the majority of newborns referred after failing universal neonatal hearing screening, hearing loss could be confirmed. The leading cause was reversible hearing loss due to otitis media with effusion, but hearing loss proved permanent in about 35% of referred newborns, with genetics as predominant cause. Follow-up of congenital hearing loss patients is important as deterioration as well as improvement was observed over time. [ABSTRACT FROM AUTHOR]

Abstract Background Aural atresia (CAA) is a congenital abnormality with hypoplasia or aplasia of the external auditory canal. Surgical procedures of CAA is not only difficult but has still not been embraced by surgeons. Many surgeons are doubtful to perform a repair due to poor hearing results and the risk of new canal stenosis. This article describes the writers’ expertise with surgical management of aural atresia. A retrospective study of 16 cases of CAA was undertaken in the Hearing and Speech Institute between 2015 and 2018. The study consisted of 16 patients, 8 patients for each group, 10 males and 6 females between 4 and 18 years of age with a median age of 6 years. Ten patients had bilateral atresia, and 6 had unilateral atresia. Postoperatively, patients were tested for hearing recovery and follow-up lasting up to 2 years to record any complications. Purpose of the study was to compare hearing effects and risks of anterior and transmastoid approaches to external and middle ear restoration in patients with CAA. Results All patients were satisfied with the surgery by improving the hearing up to 35 dB or less after 12 months. Four patients (25%) developed soft tissue meatal stenosis, with 2 patients in each group. There were no cases of bony canal stenosis. TM perforation was seen in two patients (12.51%), one patient in each group. Lateralization of the graft was seen in one patient in the transmastoid group, and no ossicular chain refixation. Five cases had postoperative otorrhea, one in the anterior mastoid group and four in the transmastoid group. There were no other complications. Conclusions Although the findings of the hearing are close. The previous approach, due to fewer postoperative complications, is now our favorite technique. Proper alignment and soft-tissue strategies are keys to the effective correction of the congenitally atretic ear canal.

Cartilage conduction (CC) is a form of conduction that allows a relatively loud sound to be audible when a transducer is placed on the aural cartilage. The CC transmission mechanism has gradually been elucidated, allowing for the development of CC hearing aids (CC-HAs), which are clinically available in Japan. However, CC is still not fully understood. This review summarizes previous CC reports to facilitate its understanding. Concerning the transmission mechanism, the sound pressure level in the ear canal was found to increase when the transducer was attached to the aural cartilage, compared to an unattached condition. Further, inserting an earplug and injecting water into the ear canal shifted the CC threshold, indicating the considerable influence of cartilage–air conduction on the transmission. In CC, the aural cartilage resembles the movable plate of a vibration speaker. This unique transduction mechanism is responsible for the CC characteristics. In terms of clinical applications, CC-HAs are a good option for patients with aural atresia, despite inferior signal transmission compared to bone conduction in bony atretic ears. The advantages of CC, namely comfort, stable fixation, esthetics, and non-invasiveness, facilitate its clinical use.

Objectives: To describe the prevalence and significance of first branchial cleft anomalies in children with congenital aural atresia. Methods: Retrospective cohort study and case series. Patients were included if they had ICD‐10 code Q16.0, Q16.1, Q16.9, Q17.2, or Q17.9 in their medical record and were seen at UCSF Benioff Children's Hospital from 2012 to 2020 for aural atresia. Children were categorized as having aural atresia and first branchial cleft anomalies if the presence of a first branchial cleft anomaly was noted in otolaryngology provider notes; otherwise, they were categorized as aural atresia alone. Patients with aural atresia and first branchial cleft anomalies were included in the case series. Results: Among 125 children with congenital aural atresia, 5 (4%) were identified with first branchial cleft anomalies. In all cases, an epithelialized tract was noted to originate from the inferior aspect of the middle ear cleft, exiting the temporal bone in an inferomedial position adjacent to the stylomastoid foramen, with a cutaneous exit point inferior to the expected location of the native ear canal. There was no association with sex, microtia grade, or laterality of atresia; however, children with aural atresia and first branchial cleft anomalies were significantly more likely to have syndromes such as Goldenhar and Treacher Collins (p =.04) than those with aural atresia alone. 3/5 (60%) children with aural atresia and first branchial cleft anomalies presented with cholesteatoma compared with 1/120 (0.8%) children with aural atresia alone, a significant difference (p <.001). All four children over the age of two have undergone surgical management. In two of these, excision of the branchial cleft anomaly could be combined with atresiaplasty, with normal hearing results in both cases. Conclusions: Aural atresia can be associated with comorbid anomalies of the head and neck. First branchial cleft anomalies can be suspected based on characteristic clinical appearance and confirmed with computed tomography showing a typical course through the temporal bone. When present in the context of congenital aural atresia, first branchial cleft anomalies are associated with a significantly increased risk for cholesteatoma, often necessitating surgical management with favorable hearing outcomes. Level of evidence: 4. [ABSTRACT FROM AUTHOR]

HT

The Bonebridge active transcutaneous bone conduction implant has been used as an effective treatment for hearing rehabilitation in children with aural atresia. It is currently indicated for children aged five and above. We present a case report of two children implanted with the Bonebridge under the age of five and followed up for more than five years. Both children have had good results and continue to wear their implant for approximately 12 hours per day. To our knowledge, this is the first report of long-term results in children implanted with the Bonebridge under the age of five.

Background Innovated hearing aids (HAs), termed cartilage conduction hearing aids (CC-HAs), show good performance in patients with closed ears and continuous otorrhea. However, factors other than the ear condition that influence the purchase rate of CC-HAs remain unclear. Purpose To identify the factors that influence the purchase rate of CC-HAs. Research Design A correlational study. Study Sample A total of 249 patients were enrolled. Data Collection and Analysis The patients' demographics, clinical characteristics, outcomes, and CC-HA transducer types were compared. The data were analyzed for six groups classified based on the ear condition. Results In the unilateral closed-ear group, the purchase cases were significantly younger than the nonpurchase cases (p < 0.05). Regarding the outcomes in the bilateral closed-ear group, the purchase cases showed significantly better-aided thresholds at 0.25 and 0.5 kHz than the nonpurchase cases. No significant differences in the functional gains and speech recognition scores were found between purchase and nonpurchase cases in all six groups. Regarding the transducer type, the continued- use rate of the simple transducer type was significantly lower in the bilateral chronic continuous otorrhea, bilateral open, and unilateral open groups. Conclusion In the closed ears, no remarkable negative factors were found. Transducer type had a significant influence on the continued-use rate in the nonclosed ears including the ears with chronic continuous otorrhea, although the purchase rate of CC-HAs in the bilateral chronic continuous otorrhea group was comparable to the closed ears. [ABSTRACT FROM AUTHOR]

Abstract Background This case series study is to evaluate the results of our technique for correction of congenital aural atresia. Seventeen patients were done from 2015 till 2018, 4 cases bilateral and 13 unilateral. Surgical canalization of congenital aural atresia with the same session reconstruction of the meatus and TM by split thickness graft (Autograft) was done. Follow-up is done weekly for 3 months then every 2 weeks for another 3 months, till we establish the formation of EAC. All cases were followed up for at least 1 year. Results All patients have now established skin limned external canal. There is no facial nerve or dural or sigmoid injuries. Only one case TMJ was exposed during the surgical procedure. One case had postauricular wound gapping and another one was complicated after 1 year by stenosis. All patients improved subjectively in speech and language postoperatively except one case who has autism and still undertreatment. Conclusion Congenital aural atresia is a challenging surgery. Surgical canalization with the same session grafting of the drum and the formed cavity by split thickness skin graft is effective with minimal minute rate of complications and recurrence.

Background: The ADHEAR™ system (MED-EL, Innsbruck, Austria) is a nonsurgical bone conduction device (BCD) to treat conductive hearing loss (CHL) and single-sided deafness. In contrast to the nonsurgical alternatives on headbands or spectacle frames, the audio processor of ADHEAR is placed retroauricularly on an adhesive adapter. The published evidence on the performance of this system is limited to studies with a trial period of 2–8 weeks. Objective: This study assesses audiological and subjective outcomes over a period of 12 months, on patients with congenital aural atresia (CAA) using the ADHEAR hearing system. Method: Fifteen children (mean age: 9.4 ± 4 years; range: 5–16 years) diagnosed with CAA (7 uni/8 bilateral) were included in this prospective, observational, repeated-measures study. Each subject used ADHEAR for 1 year, and the performance was evaluated after 1, 6, and 12 months. Free-field audiometry and speech discrimination tests were performed, and hearing-, general health- and device-specific questionnaires were used. Results: The unaided sound field threshold improved from an average PTA4 of 63.6 ± 3.4 dB HL to an aided average PTA4 of 29.3 ± 3.0 dB HL after 1 month of device use. The word recognition score (WRS) improved from an average of 27.9 ± 15.9% unaided to an aided average WRS of 91.3 ± 4.4% (p = 0.0003) after 1 month, 92.0 ± 4.1% (p = 0.0002) after 6 months, and 92.7 ± 5.3% (p < 0.0001) after 12 months using the ADHEAR system compared to the unaided condition for all 3 time points. The improvements in the speech in noise at 1, 6, and 12 months were as well consistent over time. The average improvement at the signal to noise ratio (SNR) of +5 dB was 58% and 53% at the SNR of +0 dB. No complications were reported, and all patients continued to use the ADHEAR after the study end. The questionnaire results revealed high user satisfaction and an average wearing time of 12 h per day. Conclusion: This 12-month trial of the nonsurgical adhesive BCD in CAA patients showed sufficient and reliable audiological and subjective outcomes, long wearing time, and high acceptance. The ADHEAR can be considered a suitable option to treat children with CAA for the given indication, without the drawbacks of nonsurgical devices that use pressure for retention of the audio processor or the costs and possible complications involved with a surgical alternative. [ABSTRACT FROM AUTHOR]

Cartilage conduction hearing aids (CC-HA), bone anchored hearing aids (Baha), and ADHEAR are good choices to improve hearing in patients who cannot use air conduction hearing aids because of aural atresia or continuous otorrhea. As there are many overlaps in the characteristics of these devices, we conducted a comparative trial of CC-HA, Baha, and ADHEAR. We provided patients with the opportunity to select their devices. The data of 6 patients who underwent comparative trials in our department between October 2021 and August 2022 were retrospectively examined. The gains of Baha and CC-HA outweighed those of ADHEAR. Regarding the sound localization test, there was no significant tendency for any of the hearing devices. Regarding the Glasgow Benefit Inventory, there was no clear tendency among the three devices in the quality of life evaluation. The most satisfactory model was the one subjectively chosen by the patient, regardless of the gain and score of the sound source localization test. Therefore, we believe that it is necessary to provide an opportunity for comparative trials and a consultation with each patient in the process of selecting a device.

Introduction The transmastoid approach is the most recommended technique to Bonebridge surgery, while in patients with bad anatomy or in the canal wall down technique, retrosigmoid or Middle Fossa Approaches are the alternative surgical options. Objective To describe a novel alternative approach called inverted middle fossa approach (IMFA) and its technique and audiological outcomes. Methods Seven patients submitted to the IMFA were included. All patients presented conductive and mixed hearing loss with bone thresholds of the audiogram > 40 dB. The audiological test was conducted pre- and postoperatively. Results A total of 5 males and 2 females, aged 13,8 years old (range 6–25 years old) were studied. The average follow-up was of 20 months (12 to 32 months). All patients presented aural atresia, except one with severe osseous-fibrous dysplasia of the temporal bone. Two patients showed bilateral compromise, three patients had associated Goldenhar and Treacher Collins syndrome. On the preoperative audiograms, air conduction (AC) thresholds showed a PTA4 (0.5, 1, 2 and 4 kHz) of 66.7 dB (standard deviation [SD] = ± 7.8), while the bone conduction thresholds reached an average of 11.2 dB (SD = ± 6.9). The postoperative thresholds did not change, and additional sensorineural damage was not observed before activation. Four weeks after surgery, all the patients were fitted with the external processor. The postoperative audiological aided exam showed AC PTA 4 thresholds of 18.9 dB (SD = ± 5.9). Conclusion The IMFA allows the nearest position of the microphone to the external auditory canal. The technique is a suitable option to the 3 classical approaches with similar rate of audiological results. More investigation is needed to determine the benefit of the novel approach compared with the others.

Objective: A review of published data regarding binaural hearing after treatment of congenital unilateral conductive hearing loss (UCHL) due to aural atresia. Treatment options concern atresia surgery (reconstructive surgery), application of a bone conduction device (BCD), or application of a middle ear implant (MEI). Data Sources: Database PubMed was searched for articles published in English and German between January 1, 1994, and January 1, 2019. Study Selection: The initial search identified 52 studies, of which 9 met the inclusion criteria. Data Synthesis: Comparison of studies was based on a structured review. Meta-analysis was not feasible because of the heterogeneity of outcome measures, the limited number of relevant papers (9), and diverse types of treatment (5). Conclusions: Treatment of UCHL results in bilateral hearing instead of binaural hearing. The large intersubject variability in benefit of treatment is unexplained with a clear improvement in the minority of listeners and a limited improvement or binaural interference in most listeners after atresia repair or amplification with a BCD or MEI. [ABSTRACT FROM AUTHOR]

Individuals with unilateral hearing loss show poor spatial hearing, but individual variability is high. To investigate if the degree of hearing loss in unilateral ear canal atresia affects horizontal sound localization and speech recognition. Twelve subjects with unilateral ear canal atresia without childhood hearing intervention. Previously published data from eight normal-hearing subjects in normal binaural as well as experimentally induced unilateral hearing loss served as a reference. Horizontal sound localization and recognition of speech in spatially separate competing speech were assessed. Linear regression analysis demonstrated a relationship between sound localization accuracy (SLA) and the air conduction pure tone average of the atretic ear (r = 0.85, p=.007). The large proportion of variability in SLA (72%) explained by the degree of hearing loss of the atretic ear indicates that binaural processing is possible. SLA was worse than for normal hearing individuals (p<.0001), and comparable to moderate simulated unilateral hearing loss (p=.13). Speech discrimination was significantly worse than normal (p<.0001) and not dependent on degree of hearing loss of the atretic ear. Individuals with congenital unilateral ear canal atresia show impaired horizontal SLA and recognition of speech in competing speech. [ABSTRACT FROM AUTHOR]

Introduction The transmastoid approach is the most recommended technique to Bonebridge surgery, while inpatientswithbadanatomyor inthe canalwalldowntechnique, retrosigmoid or Middle Fossa Approaches are the alternative surgical options. Objective To describe a novel alternative approach called inverted middle fossa approach (IMFA) and its technique and audiological outcomes. Methods Seven patients submitted to the IMFA were included. All patients presented conductive andmixed hearing loss with bone thresholds of the audiogram > 40 dB. The audiological test was conducted pre- and postoperatively. Results A total of 5 males and 2 females, aged 13,8 years old (range 6-25 years old) were studied. The average follow-up was of 20 months (12 to 32 months). All patients presented aural atresia, except one with severe osseous-fibrous dysplasia of the temporal bone. Two patients showed bilateral compromise, three patients had associated Goldenhar and Treacher Collins syndrome. On the preoperative audiograms, air conduction (AC) thresholds showed a PTA4 (0.5, 1, 2 and 4 kHz) of 66.7 dB (standard deviation [SD]= ±7.8), while the bone conduction thresholds reached an average of 11.2 dB (SD= ±6.9). The postoperative thresholds did not change, and additional sensorineural damage was not observed before activation. Four weeks after surgery, all the patients were fitted with the external processor. The postoperative audiological aided exam showed AC PTA 4 thresholds of 18.9 dB (SD= ±5.9). [ABSTRACT FROM AUTHOR]

Background Cartilage conduction hearing aids (CCHAs) were newly devised and spread fast in Japan since their launch in 2017. However, little knowledge is available for this new device. Purpose The aim of this study was to establish the knowledge of CCHAs and suggest their indication. Research Design Correlational study. Study Sample A total 256 patients were registered. Data Collection and Analysis The fitting of CCHAs was surveyed in nine institutions. The outcomes were assessed by audiometric tests. The patients were classified into seven groups, depending on the ear conditions. The clinical characteristics, assessment results, and purchase rates were compared among the groups. The assessment results of CCHAs were also compared with those of previously used hearing aids. Results Most patients who used CCHAs were classified into the bilateral closed (aural atresia or severe stenosis) ear (n=65) or unilateral closed ear (n=124) groups. The patients in these groups achieved good benefits that resulted in a high purchase rate. The bilateral continuous otorrhea group also supported a high purchase rate, although the benefits of CCHAs were not always excellent. In contrast, the purchase rate was poor in the patients who could use air conduction hearing aids (ACHAs) without absolute problems. As for using a CCHA as a contralateral routing of signals hearing aid, the benefits depended on the patients. Conclusions CCHAs are considered as a great option not only to the patients with closed ears but also to those who had difficulties in ACHAs usage. [ABSTRACT FROM AUTHOR]

Objective: To explore the behavioural and functional performance of a group of children with conductive unilateral hearing loss (UHL) due to congenital aural atresia.Method: Twelve children aged 7 to 16 years (Mage 10.0, SD 3.1 years) formed the UHL group and 15 age-matched children (Mage 9.5, SD 3.6 years) with normal hearing formed the control group. Auditory skills were assessed using tests of sound localisation, spatial speech perception in noise, and self-ratings of auditory abilities (Listening Inventory for Education; LIFE and Speech, Spatial and Qualities of Hearing scale; SSQ).Results: When speech was directed to the good ear, performance was poorer than for normal hearing controls. Sound localisation abilities were impaired in children with UHL. Children with UHL reported higher levels of difficulties in classroom settings compared to children with normal hearing, particularly for activities involving listening in noise and focused listening activities. Older children self-report and parents report difficulties for their children across all SSQ scales.Conclusions: Children with UHL showed a wide range of auditory difficulties. As expected, speech recognition in noise differed from controls. Sound localisation abilities were variable; greater variability was seen for right ear hearing losses suggesting that some of these children may have developed compensatory mechanisms. Younger children identified listening difficulties for school situations where focussed auditory attention was needed. Older children and parents reported greatest difficulty for activities requiring perception of the direction, distance, and movement of sound. Higher levels of effort and inability to ignore sounds were reported as major difficulties. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the long-term safety and efficacy of the Clip coupler attached to the stapes head in patients with unilateral congenital aural atresia (CAA). Methods: This single-center retrospective study included 16 Mandarin-speaking patients who had unilateral microtia accompanied by CAA. All patients were divided into two groups: the short-term follow-up group (n = 9) and the long-term follow-up group (n = 7). The floating mass transducer of the Vibrant Soundbridge (VSB) was positioned in the stapes head by the Clip coupler. The safety of the VSB was investigated by comparing preoperative and postoperative bone-conduction (BC) thresholds as well as by complications. The effectiveness was evaluated by functional gain (FG), word recognition score (WRS), speech reception threshold (SRT) and signal-to-noise ratio (SNR). Results: Pre- and post-operative BC thresholds were no different in all patients. And no complications developed. VSB-aided thresholds in the free-field had improved significantly in both short- and long-term follow-up groups. The improvements of WRS were observed in two groups. The monosyllabic VSB-aided WRS in the long-term follow-up group was significantly higher than that in the short-term follow-up group. When speech was from the impaired ear and noise presented to the side of normal ear (SVSBNCL), lower SNRs were found in two groups after VSB implantation. However, there was no statistical difference in aided SNR between the two groups at SVSBNCL status. Conclusions: Our results show that the FMT connected to the stapes head is a secure and useful device for patients with unilateral CHL/MHL, not only in terms of improved hearing thresholds, but also improved speech intelligibility in quiet and noisy environments. [ABSTRACT FROM AUTHOR]

Background: Detailed investigations of the stapedial muscle (SM) in congenital aural atresia (CAA) patients have yet to be adequately conducted. Objectives: To assess image variations in the mastoid segment of the facial nerve (FN) and SM in CAA. Materials and methods: A total of nine patients comprising of 9 ears with unilateral CAA were studied. The courses of the FN and SM were evaluated from the basic point to 1 mm intervals between the mastoid portion of FN, and measured from the mean X and Y values in each group. Results: The atresia side of FN among the Y values showed significant differences compared to the contralateral side. In terms of the SM, there were no significant differences in both the X and Y values. The stapedial muscle of the CAA patients was located medially to the FN. Conversely, the distance from the PSC to the FN revealed no significant differences with regard to the X and Y values for each group. Conclusion: The current observations revealed that the SM is located more posterior to the FN in CAA patients, and this is mainly attributed to the laterally and anteriorly displaced FN. [ABSTRACT FROM AUTHOR]

Labyrinthectomy is an effective surgical procedure for the management of poorly compensated unilateral peripheral vestibular dysfunction in the presence of a nonserviceable hearing. It involves removal of labyrinthine portion of the inner ear and exenteration of the neuroepithelium. In our institution, 8 cases underwent surgical labyrinthectomy from a period of 2013–2018 for various extensive disease manifestations, age ranges from 2 to 48 years. Includes, a child of 2 years age presented with bilateral foul smelling otorrhoea with external auditory canal cartilaginous stenosis, 5 cases of extensive cholesteatoma with labrynthitis and 2 cases of purulent labrynthitis among them 1 were suffering from Tuberculosis and was on Category 1 ATT and other one suffering from extensive granulation at the tympanomastoid area which was inconclusive of the diagnosis even after histopathological examination, so treated as tuberculosis and started on prophylactic antitubercular treatment in addition to surgery. All patients except the child gave past history of giddiness, but at the time of presentation they were not having giddiness or noticeable nystagmus and all had profound unilateral sensorineural hearing loss. Thus all the patients underwent a radical mastoidectomy with total labyrinthectomy and blind sac closure in 2 patients. [ABSTRACT FROM AUTHOR]

Objective: Cartilage conduction (CC) is a new transduction form, and hearing devices that utilize CC present a new option for patients with aural atresia. However, in occluded ears, low-tone sounds are transmitted very effectively, resulting in excessive low-tone sound emphasis and speech recognition reduction. This study aimed to clarify low-tone speech recognition for CC in occluded ears, and determine if excessive low-tone sound emphasis decreases maximum speech recognition scores.Methods: Eight volunteers with normal hearing participated. The performance-intensity function and maximum speech recognition scores for CC with an earplug (i.e., occluded) and air conduction (AC) were measured under high- and low-pass filter (HPF and LPF) conditions, respectively.Results: An HPF improved the maximum speech recognition scores for CC in occluded ears. The scores for CC (occluded) under the conditions of the first- and second-order HPF did not differ from that for AC under the no-filter condition. Conversely, an LPF reduced the scores for AC. The scores and confusion matrices under the second-order LPF condition were similar to those for CC (occluded).Conclusion: The conditions under the second-order LPF resembled those of CC in occluded ears. An HPF can compensate excessive low-tone emphasis and improve speech recognition. [ABSTRACT FROM AUTHOR]

CASE REPORT A 12-month-old female Labrador was presented for an absent opening to the right external ear canal. Computed tomography (CT) confirmed a stenotic blind-ending right vertical ear canal and a hypoattenuating fluid-filled right external ear canal and tympanic bulla. Reconstructive surgery was performed to reestablish a patent external auditory meatus. Followup CT 4 weeks post operatively revealed a patent right external ear canal. The external ear canal opening was 50% of the diameter of the clinically normal ear and there was a marked reduction in the fluid within the tympanic bulla. CONCLUSION Functional and cosmetic results were excellent 6 months after surgery. [ABSTRACT FROM AUTHOR]

The aim of this report is to provide international recommendations for functional ear reconstruction in patients with microtia and aural atresia. All patients with microtia and external auditory atresia should be seen in the setting of a multidisciplinary team and agreed treatment outcomes should be measured, so that techniques, approaches, and results can be compared. The methods are expert opinion from the members of the International Microtia and Atresia Workgroup (IMAW). The consensus recommendations reported herein take into account the variability in practice patterns present among experts in the field; the degree of consensus was quantified by presenting the percentage of above authors who agree or partially agree with each statement. Recommendations include the definition and classification of microtia/atresia, treatment of microtia, treatment of congenital aural atresia, flowchart of functional ear reconstruction, and future research directions. Patients with microtia and aural atresia can be guided by the consensus recommendations provided herein. [ABSTRACT FROM AUTHOR]

Bone conduction hearing devices integrated in softbands (BCDSs) are frequently not well accepted by children with conductive hearing loss due to pressure on the head, sweating, or cosmetic stigma. A non-surgical hearing system (ADHEAR) uses a new bone conduction concept consisting of an audio processor connected to an adhesive adapter fixed behind the ear. This study is the first to evaluate the audiological and clinical outcome of this novel system, comparing it with conventional BCDSs in a short- and mid-term follow-up in children under 10 years of age. The ADHEAR was compared to a BCDS in 10 children with conductive hearing loss (age: 0.7–9.7 years). Aided and unaided pure tone/behavioral observational audiometry and, if applicable, speech audiometry in quiet and noise were performed initially with both devices and after 8 weeks with the ADHEAR alone. The subjective hearing gain and usage of the new hearing system, as well as patients' and parents' satisfaction were assessed using questionnaires. The functional gain with the ADHEAR averaged over 0.5, 1, 2, and 4 kHz exceeded that of the conventional BCDS (35.6 dB ± 15.1 vs. 29.9 dB ± 14.6, p =.001, n = 9 ears). Speech perception in quiet and noise (n = 8) improved in the aided situation similarly for both hearing devices. The parents of 8 of 10 children evaluated the ADHEAR system as being useful. Minor wearing problems occurred occasionally. Eight children continued using the ADHEAR after the study, one received an active middle ear implant and one continued to use a BCDS. The ADHEAR system is a promising solution for children with conductive hearing loss or chronically draining ears. [ABSTRACT FROM AUTHOR]