Unilateral Congenital Aural Atresia Research Articles

Changes in Listening Effort Through Pupil Response After Atresioplasty in Children With Congenital Aural Atresia.

This study aimed to determine whether the improvement of hearing by surgical treatment alleviates cognitive demands through pupil response in patients with unilateral congenital aural atresia (CAA). A prospective study was performed on patients with unilateral CAA who were scheduled to undergo primary atresioplasty between November 2017 and May 2020. Pure-tone audiometry, auditory digit span test, Korean Speech Perception in Noise test, pupil measurement during speech tests, and questionnaires (Sound-Spatial-Qualities of Hearing Scale; subjective listening effort rating) were performed before and 6 months after surgery. Of 30 consecutive patients who initially enrolled, only 18 patients (12 males and 6 females) were included in the analysis. When the improvement of the air-bone gap and interaural difference of air conduction within 30 dB was defined as a successful hearing outcome, successful hearing improvement was achieved in 50% of the 18 patients. In pupil measurement, the success group had a significantly smaller mean pupil dilation response than the nonsuccess group at 0 and -3 dB signal to noise ratio (SNR) (all p < 0.01). In addition, significant differences were identified between the two groups for peak dilation and peak latency at all noise levels (all p < 0.01). When analyzing the change in pupil response before and after surgery, the difference in relative mean pupil dilation in the success group was significantly greater than that in the nonsuccess group at -3 dB SNR (p = 0.02). In addition, the success group showed a significantly greater change in peak latency than the nonsuccess group at the -3 dB SNR (p < 0.01). The difference in peak dilation tended to be greater in the success group than in the nonsuccess group, but the difference was not statistically significant. Patients with unilateral CAA who achieved surgically improved hearing had a smaller pupil dilation response than those who did not. These results suggest that successful hearing outcomes after surgery in patients with unilateral CAA may reduce the cognitive effort required to understand speech under difficult listening conditions.

A pilot study on spatial hearing in children with congenital unilateral aural atresia.

Despite normal hearing in one ear, individuals with congenital unilateral aural atresia may perceive difficulties in everyday listening conditions typically containing multiple sound sources. While previous work shows that intervention with bone conduction devices may aid spatial hearing for some children, testing conditions are often arranged to maximize any benefit and are not very similar to daily life. The benefit from amplification on spatial tasks has been found to vary between individuals, for reasons not entirely clear. This study has sought to expand on the limited knowledge on how children with unilateral aural atresia recognize speech masked by competing speech, and how horizontal sound localization accuracy is affected by the degree of unilateral hearing loss and by amplification using unilateral bone conduction devices when fitted before 3 years of age. In a within-subject, repeated measures design, including 11 children (mean age = 7.9 years), bone conduction hearing device (BCD) amplification did not negatively affect horizontal sound localization accuracy. The effect on speech recognition scores showed greater inter-individual variability. No benefit from amplification on a group level was found. There was no association between age at fitting and the benefit of the BCD. For children with poor unaided sound localization accuracy, there was a greater BCD benefit. Unaided localization accuracy increased as a function of decreasing hearing thresholds in the atretic ear. While it is possible that low sound levels in the atretic ear provided access to interaural localization cues for the children with the lowest hearing thresholds, the association has to be further investigated in a larger sample of children.

Unilateral Congenital Aural Atresia from an Ychsma Group Burial at the Site of Pachacamac, Peruvian Central Coast

Congenital malformations of the human skeleton are a major area of palaeopathological and bioarchaeological interest, although our understanding of such conditions in the ancient world is hampered by their often extreme rarity, and inconsistent reporting in the field. A cranium from a group burial at the Peruvian site of Pachacamac was found to display almost complete absence of the right external auditory meatus, styloid process, vaginal process and tympanic plate of the right temporal bone. Following a differential diagnosis, it was determined that the skeletal pathology likely represents an instance of congenital aural atresia, a developmental anomaly resulting in the partial or complete aplasia of the external acoustic meatus. This condition is often associated with other congenital abnormalities and syndromes. However, as the postcranial remains of this individual could not be distinguished from other remains in the group burial, no further associated skeletal anomalies could be detected. This is the first instance of congenital aural atresia recovered from a defined funerary context in Peru, complementing other bioarchaeological reports of this rare congenital anomaly. The pathology of the condition is described, and compared with clinical and bioarchaeological data. The potential social implications concerning social attitudes of the Pachacamac population towards this individual are also considered, in reference to both historical and archaeological contexts.

Acoustic Reflex After Surgical Repair in Patients with Congenital Aural Atresia.

This study aimed to examine the plasticity of nerves indirectly by acoustic reflex after surgical repair of unilateral congenital aural atresia. This study is a retrospective study including 80 patients who had undergone surgical repairs of congenital aural atresia before 18 years old and for whom acoustic reflex testing was performed postoperatively. Several variables correlated with acoustic reflex were analyzed to find factors affecting the presence of acoustic reflex. Among 80 patients, 44 were positive for acoustic reflex. As a result of multivariate analysis, good postoperative hearing (P = .017), younger age at surgery (P = .028), and the longer time interval between surgery and acoustic reflex test (P = .040) were revealed as factors affecting the acoustic reflex. Acoustic reflex was present in 55% of surgically managed patients with congenital aural atresia. The results of this study imply that the reflex arc of acoustic reflex may be restored after proper surgical reconstruction and prolonged use of reconstructed ear in patients with unilateral congenital aural atresia.

Long-term outcomes of retrospective case series of middle ear implantation with Vibrant Soundbridge in children with congenital aural atresia.

to assess audiological performance in quiet and noise, quality of life and side effects of Vibrant Soundbridge (VSB) in children with congenital aural atresia (CAA). A retrospective study including consecutive patients with unilateral or bilateral CAA implanted with VSB from 2009 to 2020 in a tertiary referral centre. 18 patients with CAA and a present stapes were included (3 simultaneous bilateral VSB implants) and 21 ears: 17 VSB were attached to the short incus process, and four to the stapes. Age at implantation ranged from 4.7 to 15.8years. Average follow-up was 6.5years (± 3.7years). In unilateral VSB, air conduction pure tone average (ACPTA) thresholds increased from 75.3 ± 15.2 to 32.6 ± 8.3dB post-operatively (VSB activated) (n = 15; p < 0.01). The speech reception threshold (SRT) and the word recognition score (WRS) were significantly improved from 81.5 ± 10.4 to 43.9 ± 7.6dB and 0% to 84.8 ± 8.5% postoperatively (n = 15; p < 0.01). The signal to noise ratio (SNR) was significantly improved from 2.1 ± 2.9dB VSB inactivated to 0.3 ± 2.7dB VSB activated (n = 15; p < 0.01). There was no significant difference in performance according to floating mass transducer (FMT) placement. 5/15 children were non-users at last follow-up in unilateral VSB and 0/3 in bilateral. CAA ears with VSB activated had a significant improvement of ACPTA, WRS, SRT and SNR. A third of patients with unilateral CAA became non-users at last follow-up. The main challenge is to target the indications for the implantation of the VSB to avoid its discontinuation.

Using the external auditory canal skin graft of the healthy side to treat unilateral congenital aural atresia: preliminary results of clinical research

Objective: To investigate whether the contralateral normal external auditory canal (EAC) skin graft can maintain the ear canal health after EAC reconstruction in unilateral congenital aural atresia (CAA) cases. Methods: A Zelen design randomized controlled study was used to collect unilateral CAA patients for EAC reconstruction prospectively (clinical trial registration number: ChiCTR2000032103). The patients were randomly divided into the control group and the trial group. The trial group used the contralateral normal EAC skin graft group (transplant part of the contralateral normal EAC skin to repair the atresia side for unilateral CAA patients), the control group all used scalp blade thick skin. We observed the EAC health and hearing results of the two groups after EAC reconstruction. Results: A total of 13 cases were enrolled from July 2020 to August 2021. There were eight patients in the trial group, including six males and two females, with an average age of 22.3 years (14-36 years). There were two patients with CAA on the left and six patients on the right. The average follow-up time was 8.8 months (4-14 months). There were five patients in the control group, all cases were male with an average age of 16.2 years (12-20 years). There were four patients with CAA on the left and one patient on the right. The average follow-up time was 7.0 months (2-14 months). In the trial group, eight cases of reconstructed EAC epithelium were healthy, one patient had cicatricial stenosis of EAC opening and lateralization of the tympanic membrane. The other patient had cicatricial stenosis of reconstructed EAC, this case also had scar hyperplasia of the contralateral EAC opening but recovered after soft packing and triamcinolone acetonide injection treatment. The healthy side EAC of the rest trial group had no scarring stenosis or local bone hyperplasia during long-term follow-up. In the control group, one patient was lost to follow-up and the other four patients had dry ears of reconstructed EAC, but easily to form crusts and needed to be cleaned repeatedly, one patient had lateralization of the tympanic membrane, the EAC epithelium was not healthy for long-term follow-up. The incidence of complications related to EAC reconstruction was lower than previous studies (χ²=5.55, P=0.018), and the average postoperative Air-Bone Gap increased (18.8±10.0)dB. Conclusion: By optimizing the EAC reconstruction technology, the health of the reconstructed EAC is improved compared with the previous study. After active intervention and treatment, there should be no scarring stenosis or local bone hyperplasia on the contralateral side EAC.

Two cases of unilateral congenital aural atresia with cholesteatoma

Two cases of unilateral congenital aural atresia with cholesteatoma

Evaluation of adhesive bone conduction hearing aid in pediatric patients with unilateral congenital aural atresia

Objective: To evaluate the auditory efficacy and subjective satisfaction of adhesive bone conduction hearing aid in children with unilateral congenital aural atresia (UCAA). Methods: Ten subjects (5 males and 5 females) diagnosed with UCAA with an average age of 8.3 years old (ranged from 5 to 15) were included in Beijing Tongren Hospital, Capital Medical University from January to August 2019. The free sound field hearing threshold, word recognition score in quiet, speech reception threshold in noise and sound localization ability (results were measured by RMS error) tests were performed in unaided and aided situation, respectively. Subjective satisfaction questionnaires were also distributed to subjects. Paired t test and Wilcoxon signed rank test were used as statistical analysis methods. Results: The average hearing threshold in aided condition was improved by (21.9±4.4) dB (t=15.8，P<0.05). Speech recognition abilities were generally improved both under quiet and noise (P<0.05);however, when the binaural summation, squelch and head shadow effects were analyzed respectively, the binaural squelch effect was not statistically improved (P>0.05), while the other effects were improved in aided condition (P<0.05). In sound localization test, there was no significant difference of the RMS error value between the unaided and aided situation (P>0.05). The subjects got high satisfaction rates in three subjective questionnaires. Conclusion: The adhesive bone conduction hearing aid can provide significant audiological benefit for children with UCAA as well as raising the quality of their life.

Toward Optimal Care for Children With Congenital Unilateral Aural Atresia

Toward Optimal Care for Children With Congenital Unilateral Aural Atresia

Cortical auditory function in children with unilateral congenital aural atresia

ABSTRACT Objective: This study explored cortical auditory evoked potential (CAEPs) elicited by speech sounds in children with unilateral congenital aural atresia (CAA). Design: Twelve children (8 males) aged 7–16 years (M age 10.0, SD 3.1) with unilateral CAA took part in the study. Results were compared to a control group of 15 children 5–16 years (M age 9.5, SD 3.4 years) with normal hearing bilaterally. CAEPs to speech in noise (+5 dB signal-to-noise ratio) were recorded. Results: P1 amplitudes were larger for frontal locations regardless of the stimulus, ear of presentation, group (CAA vs. normal hearing), or age. Younger participants had larger P1 amplitude across electrodes. For N250 responses, CAEPs evoked by /ti/ had the shortest latency, followed by /di/and/gi/. Older children presented with shorter N250 latencies for /ti/, regardless of electrode location or group or ear of presentation. Conclusions: CAEPs in children with a moderate conductive congenital unilateral hearing loss showed typical developmental and stimulus effects. We did not find group differences when CAEPs of children with atresia were compared to control participants. Better understanding of typical development and changes in CAEPs over time in children with CAA is needed to ascertain whether unilateral hearing loss due to CAA has effects on CAEPs that correlate with perceptual effects.

Acoustic Reflexes in Aural Atresia Patients: Evidence of an Intact Efferent System?

To record crossed acoustic reflex thresholds (xART's) postoperatively from patients after surgical repair of unilateral congenital aural atresia (CAA). To seek explanations for when xARTs can and cannot be recorded. We hope to understand the implications for this central auditory reflex despite early afferent deprivation. Patients who underwent surgery to correct unilateral CAA at a tertiary academic medical were prospectively enrolled to evaluate for the presence of xART. Preoperative ARTs in the normal (non-atretic) ear, and postoperative ipsilateral ARTs (stimulus in the normal ear) and contralateral ARTs (stimulus in the newly reconstructed atretic ear; record in the normal ear) were measured at 500, 1000, and 2000 Hz. Four of 11 patients with normal ipsilateral reflex thresholds preoperatively demonstrated crossed acoustic reflexes postoperatively (stimulus in reconstructed ear; record from normal ear). Four other patients demonstrated normal ipsilateral thresholds preoperatively but did not have crossed reflexes postoperatively. No reflexes (pre- or postoperatively) could be recorded in 3 patients. Crossed reflex threshold is significantly correlated with the postoperative audiometric threshold. There was no correlation between ipsilateral and contralateral reflex thresholds. Crossed acoustic reflexes can be recorded from some but not all postoperative atresia patients, and the thresholds for those reflexes correlate with the postoperative pure tone threshold. The presence of acoustic reflexes implies an intact CN VIII-to-opposite CN VII central reflex arc despite early unilateral sound deprivation.

Effects of Presentation Level on Spatial Hearing With and Without Bone-Conduction Amplification in Congenital Unilateral Aural Atresia.

This study assessed the effect of ipsilateral bone-conduction amplification on spatial hearing abilities in subjects with congenital unilateral aural atresia (CUAA). Twelve patients with unilateral conductive hearing loss secondary to CUAA and normal hearing in the contralateral ear were tested. Most (75%) had limited experience with a bone-conduction hearing aid (BCHA). Performance was evaluated with and without a BCHA fitted acutely on a softband. Spatial hearing abilities were evaluated in two paradigms. Spatial release from masking was evaluated by comparing masked sentence recognition with a target and two speech maskers either colocated at 0 degree or with the maskers separated at +90 degrees and -90 degrees. Sound source localization was evaluated in a 180 degrees arc of loudspeakers on the horizontal plane. Performance was evaluated at 50 and 75 dB SPL, and results were compared for patients tested with and without a BCHA. Group level results indicate similar spatial release from masking in the aided and unaided conditions at both presentation levels. Localization at 50 dB SPL was similar aided and unaided, but at 75 dB SPL the root mean square error was lower unaided than aided (17.2 degrees vs 41.3 degrees; p = 0.010). Use of a BCHA in patients with CUAA may interfere with auditory cues required for sound source localization when the signal level is intense enough to overcome the patient's conductive hearing loss. These findings have potential clinical implications in fitting of BCHAs to support optimal spatial hearing in patients with CUAA.

Bone conduction hearing device adherence in relationship to age in pediatric unilateral congenital aural atresia

ObjectiveTo characterize the adherence of bone conduction hearing devices (BCHDs) for hearing management in children with unilateral congenital aural atresia (UCAA) in relation to the age of offer and fitting. BCHD Soft Bands help predict amplification benefits before surgery can be performed beginning around five years when both hearing and parental compliance reach stability. We hypothesized device compliance might decrease with age of fitting from lack of early acclimatization. MethodsRetrospective case series of patients less than five years old at a tertiary pediatric center's microtia clinic database, born between 2014 and 2018 with UCAA. Adherence was assessed through electronic health record note documentation at less than 1 year, 1–2 years, and greater than 2 years from fitting. The ages at offer and fitting, along with the time from offer to fitting, were recorded. ResultsOne hundred and eight patients with UCAA were identified, including 46 patients fit with a BCHD used for further analysis. Adherence rates at 1 year, 1–2 years, and greater than 2 years were 47.8%, 30%, and 43.5%, respectively. However, there was no significant association between age offered, age fit, or time from offer to fit and adherence at all time points. Also, there was no significant difference between ages at the time of BCHD offer for those who chose not to proceed with fitting (20.9 months) compared to the age of offer in patients that were subsequently fit (13.9 months). ConclusionBCHD adherence in patients less than five years old may not be affected by the age offered or fit. The time between offer and fitting was also not associated with usage. BCHDs should be offered to UCAA patients regardless of age. Further investigation in this younger age group would help expand these findings.

Application of retrosigmoid sinus approach in Bonebridge implantation

Background Bonebridge is an suitable option for conductive hearing loss, however, the traditional approach cannot accomplished a satisfying implantation for patients with congenital malformation or radical mastoidectomy. Objectives To evaluate the clinical application of retrosigmoid sinus approach in Bonebridge implantation and postoperative evaluation. Materials and methods 11 patients who underwent retrosigmoid sinus approach Bonebridge implantation from March 2016 to September 2019 were retrospectively analyzed, including 6 males and 5 females, aged 12–54 years old (30.6 in average). Among them, 4 cases had undergone bilateral radical mastoidectomy, 6 cases had bilateral congenital aural atresia or stenosis, and 1 case had unilateral congenital aural atresia. Results All patients underwent Bonebridge implantation through retrosigmoid sinus approach according to the preoperative image reconstruction and plan. There was no surgical injury of sigmoid sinus or cerebrospinal fluid leakage during the operation. The aided threshold obtained an increase of 32.32 dB HL; the speech recognition rates of bisyllabic words, monosyllabic words and sentence were 79.6%, 67.8% and 75.0%, respectively. After 11–53 months of follow-up, the hearing effect was stable and no long-term complications occurred. Conclusion The retrosigmoid sinus approach is an effective surgical approach for patients with congenital ear deformities or radical cavity after mastoidectomy.

Active middle ear implant (vibrant soundbridge) in children with unilateral congenital aural atresia

Background Detailed studies have not been conducted on sound localization and speech perception in noise in patients with unilateral congenital aural atresia (UCAA). Aims/objectives To evaluate the benefits of the use of the Vibrant Soundbridge (VSB) for UCAA by performing audiometric and sound localization tests. Materials and methods Four children with UCAA underwent VSB (VORP 503) implantation from 2018 to 2019. Speech perception tests in noise were conducted using the Japanese monosyllable test. The spatial configuration for speech testing consisted of speech presented from the front and noise presented into the normal ear (S0N90). The sound localization test was conducted using nine loudspeakers equally distributed in a semicircle. Results The children’s speech perception in noise 6 months after VSB activation was significantly better than before activation (p < .05). Additionally, the children’s sound localization ability after VSB activation was significantly better than before VSB activation. Conclusion and significance The benefits of VSB use in children with UCAA were revealed using audiometric and sound localization tests.

Long-term Outcomes of Clip Coupler Implantation in Patients with Unilateral Congenital Aural Atresia.

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). 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). 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. 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.

Acquisition limitations of bone conduction hearing devices in children with unilateral microtia and atresia

ObjectiveTo characterize the use of bone conduction hearing devices (BCHD) for hearing management in children with unilateral congenital aural atresia (CAA) at a tertiary pediatric center's microtia clinic while assessing challenges in acquisition. BCHD generally provides better audiologic outcomes than atresiaplasty in pediatric CAA. BCHD is formally recommended for only bilateral CAA, although literature has begun to show benefit in unilateral CAA. MethodsA retrospective case series of patients born between 2014 and 2018 with unilateral microtia at an urban tertiary care children's hospital collected information on demographics, CAA laterality, hearing loss (HL) severity, management, and acquisition. Statistical analysis aided characterization of BCHD use. Results131 patients (65% males) were included with a mean age of 3.5 years. 108 (82%) patients with unilateral microtia were used for further analysis, and right sided microtia was most common (67.6%). Of patients with auditory brain response (ABR) testing available, 70% demonstrated conductive HL, 16% mixed HL, 1% sensorineural HL, 6% no HL secondary to grade 1 microtia, and 7% were pending evaluation. Overall, 46 (42.6%) patients with unilateral microtia obtained a BCHD. 68.7% of those offered a BCHD after ABR opted for acquisition. The average length of time from offer to fit was about 6 months. ConclusionEven with thorough consultation and follow up, less than half of the patients with unilateral CAA received a BCHD. Missed initial ABR appointments that lead to BCHD recommendation seem to be the greatest limiting factor, while demographics did not play a confounding role. Family education and future research on obstacles preventing BCHD use in unilateral CAA will help standardize management and improve hearing within this critical auditory period.

Location of the stapedius muscle with reference to the facial nerve in patients with unilateral congenital aural atresia: implication for active middle ear implants surgery

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.

Effects of FM system fitted into the normal hearing ear orcartilage conduction hearing aid fitted into the affected ear on speech-in-noise recognition in Japanese children with unilateral congenital aural atresia.

The effects of FM system fitted into the normal hearing ear (NHE) or a cartilage conduction hearing aid (CCHA) fitted into the affected ear (AE) on the speech recognition ability in noise were examined in children with unilateral congenital aural atresia (UCAA). In children with bilateral normal hearing (BNH), speech recognition score (SRS) was significantly decreased in the noisy environment of -5 dB signal-to-noise ratio (SNR), compared with those in quiet. In children with UCAA, SRS was significantly decreased in noisy environments of 0 and -5 dB SNR, compared with those in quiet. In noisy environments of 0 and -5 dB SNR, SRS in children with UCAA was significantly decreased, compared those in children with BNH. In the noisy environment of -5 dB SNR, SRS in UCAA children aided by FM system fitted into NHE was significantly better than those in unaided children in the same group. In the noisy environment of 0 dB SNR, SRS in UCAA children aided by CCHA into AE tended to be higher than those in unaided children in the same group. FM system and CCHA can be recommended as an audiological management for the improvement of speech recognition in children with UCHL in classrooms. J. Med. Invest. 67 : 134-138, February, 2020.

Management of Congenital Aural Atresia : A Case Report

Congenital Aural Atresia (CAA) is the failure of development of the external auditory canal. It usually occurs in conjunction with microtia, which is malformation of the auricle due to the failure of development of the external ear. The evaluation and treatment of aural atresia present a number of challenges to the otologic surgeon. Computed tomography also plays a pivotal role in planning the surgery and grading the outcome of the surgery in congenital aural atresia. We report a case of unilateral congenital aural atresia and microtia and did a canalplasty and tympanoplasty in this patient.