Cause, severity, and efficacy of treatment for hearing loss in children with Trisomy 18: A single institution-based retrospective study.

Trisomy 18 is a common chromosomal aberration syndrome, characterized by variable clinical manifestations, including cardiovascular, pulmonary, genitourinary, and musculoskeletal findings, leading to a shorter survival and severe developmental delay in survivors. However, recently, intensive therapeutic intervention has allowed for prolonging survival. In terms of otological complications, only a limited number of relevant reports have been published. To demonstrate the characteristic of hearing loss (HL) in children with Trisomy 18, we retrospectively evaluated 22 patients (44 ears) by comprehensive auditory evaluation with the auditory steady-state response (ASSR) test and temporal bone computed tomography (CT). ASSR revealed that 20 patients (91%) had bilateral moderate to profound HL, more frequent and severe than that in Trisomy 21; among 42 ears having HL, 12 ears (29%) had conductive HL, and 26 ears (62%) had mixed HL. CT scans of 38 ears revealed that 34 ears (89%) had an external and middle ear malformation. Hearing aids (HA) were fitted in 17 patients (air and bone-conduction HAs). The threshold hearing with HA was improved in all of them. Accurate otological evaluation using ASSR and CT and intervention by HAs could be a feasible choice for children with Trisomy 18.

Efficacy of vibrant sound bridge in congenital aural atresia: an updated systematic review.

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.

Long-term results and quality of life after vibrant soundbridge implantation (VSBs) in children and adults with aural atresia.

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.

Pediatric cholesteatoma associated with congenital aural atresia and stenosis.

OBJECTIVE: Investigate presenting features, associated surgical treatment, and outcomes in patients with cholesteatoma associated with congenital aural atresia (CAA) or stenosis (CAS). METHODS: Colorado Multiple Institution Review Board approval was obtained. A retrospective chart review was performed at a single tertiary care children's hospital of all pediatric patients with congenital aural atresia or stenosis with associated cholesteatoma from January 1, 2003, to October 15, 2018. RESULTS: Of the 278 patients identified with CAA or CAS, twelve (4.3 %) were found to have a canal cholesteatoma. There was a male predominance (8:4). Nine patients (75 %) had conductive loss and three (25 %) had mixed loss. Four patients (33.3 %) exhibited canal cholesteatomas extending into the middle ear or mastoid cavity. All patients underwent surgery, and 25 % of patients required revision canalplasty while 58 % of patients required revision surgery for cholesteatoma recidivism. The average age at the time of surgery was 11.3 ± 3.7 years. CONCLUSION: Fewer than 5 % of pediatric patients with congenital aural atresia or stenosis were diagnosed with an acquired canal cholesteatoma. The need for revision surgery was common, occurring in >50 % of cases. Screening patients with CAA/CAS for cholesteatoma with imaging is recommended to avoid the morbidity of delayed identification.

Search for a genetic cause in children with unilateral isolated microtia and congenital aural atresia.

PURPOSE: Microtia describes a spectrum of auricular malformations ranging from mild dysplasia to anotia. A vast majority of microtia patients demonstrate congenital aural atresia (CAA). Isolated microtia has a right ear predominance (58-61%) and is more common in the male sex. Isolated microtia is a multifactorial condition involving genetic and environmental causes. The aim of this study is to describe the phenotype of children with unilateral isolated microtia and CAA, and to search for a common genetic cause trough DNA analysis. METHODS: Phenotyping included a complete clinical examination. Description on the degree of auricular malformation (Weerda classification-Weerda 1988), assessment for hemifacial microsomia and age-appropriate audiometric testing were documented. Computerized tomography of the temporal bone with 3-D rendering provided a histopathological classification (HEAR classification-Declau et al. 1999). Genetic testing was carried out by single nucleotide polymorphism (SNP) microarray. RESULTS: Complete data are available for 44 children (50% was younger than 33 days at presentation; 59.1% boys; 72.7% right ear). Type III microtia was present in 28 patients. Type 2b CAA existed in 32 patients. All patients had a normal hearing at the non-affected side. Genome wide deletion duplication analysis using microarray did not reveal any pathological copy number variant (CNV) that could explain the phenotype. CONCLUSIONS: Type III microtia (peanut-shell type) in combination with a type 2b CAA was the most common phenotype, present in 23 of 44 (52.3%) patients with isolated unilateral microtia. No abnormalities could be found by copy number variant (CNV) analysis. Whole exome sequencing in a larger sample with a similar phenotype may represent a future diagnostic approach.

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

PURPOSE: 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). METHODS: A retrospective study including consecutive patients with unilateral or bilateral CAA implanted with VSB from 2009 to 2020 in a tertiary referral centre. RESULTS: 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 15.8 years. Average follow-up was 6.5 years (± 3.7 years). In unilateral VSB, air conduction pure tone average (ACPTA) thresholds increased from 75.3 ± 15.2 to 32.6 ± 8.3 dB 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 ± 7.6 dB 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 ± 2.9 dB VSB inactivated to 0.3 ± 2.7 dB 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. CONCLUSIONS: 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.

[Hearing rehabilitation with the Vibrant Soundbridge in patients with congenital middle ear malformation]. / Die Vibrant Soundbridge zur Versorgung von Patienten mit kongenitalen Mittelohrfehlbildungen.

BACKGROUND: Congenital aural atresia, which is usually unilateral, causes hearing loss and aesthetic impairment. Besides tympanoplasty with/without canalplasty and bone conduction devices, active middle ear implants are also available for functional rehabilitation. OBJECTIVE: This article aims to present a contemporary review on the treatment possibilities for middle ear malformations, with a focus on audiological rehabilitation with the Vibrant Soundbridge. MATERIALS AND METHODS: A selective literature search for treatment possibilities was performed in PubMed up to October 2020, and personal clinical experiences are reported. RESULTS: The Vibrant Soundbridge, which is approved for children ≥ 5 years, is suitable for treatment of middle ear malformations with a Jahrsdoerfer score ≥ 5. Although implantation of a Vibrant Soundbridge is surgically more demanding than implantation of a bone conduction device, the method is safe, delivers good auditory results (superior to bone conduction devices in terms of speech understanding and spatial hearing), does not involve intensive postsurgical care, and rarely requires revision surgery. The Vibrant Soundbridge can be coupled to (remnants of) the ossicular chain or the round window. CONCLUSION: The Vibrant Soundbridge is an appropriate treatment method in patients with middle ear malformations who have suitable anatomical preconditions.

Audiological results and subjective benefit of an active transcutaneous bone-conduction device in patients with congenital aural atresia.

PURPOSE: To review functional and subjective benefit after implantation of an active transcutaneous bone conduction device (BCD) in patients with congenital microtia with atresia or stenosis of the external auditory canal. METHODS: Retrospective chart analysis and questionnaire on the subjective impression of hearing ( Speech, Spatial and Qualities of Hearing Scale (SSQ-B) of patients treated between 2012 and 2015. RESULTSRESULTS: 18 patients (24 ears) with conductive or mixed hearing loss in unilateral (n = 10) or bilateral (n = 8) atresia were implanted with a BCD. No major complications occurred after implantation. Preoperative unaided air conduction pure tone average at 0.5, 1, 2 and 4 kHz (PTA 4 ) was 69.2 ± 11.7 dB, while postoperative aided PTA 4 was 33.4 ± 6.3 dB, resulting in a mean functional hearing gain of 35.9 +/- 15.6 dB. Preoperatively, the mean monosyllabic word recognition score was 22.9 % ± 22.3 %, which increased to 87.1 % +/- 15.1 % in the aided condition. The Oldenburger Sentence Test at S0N0 revealed a decrease in signal-to-noise-ratio from - 0.58 ± 4.40 dB in the unaided to - 5.67 ± 3.21 dB in the postoperative aided condition for all patients investigated. 15 of 18 patients had a subjective benefit showing a positive SSQ-B score (mean 1.7). CONCLUSION: The implantation of an active bone conduction device brings along subjective and functional benefit for patients with conductive or combined hearing loss.

Twelve years of neonatal hearing screening: audiological and etiological results.

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.

Speech recognition scores in bilateral and unilateral atretic ears.

OBJECTIVE: Congenital aural atresia causes severe conductive hearing loss disturbing auditory development. The differences in speech recognition were investigated between bilateral and unilateral aural atresia. DESIGN: 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. STUDY SAMPLE: Twenty-four patients with aural atresia (8 bilateral, and 16 unilateral) participated. RESULTS: 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. CONCLUSIONS: Poor unilateral atretic-ear development may induce low speech recognition, and the mechanisms underlying speech-recognition reduction differ from those in SNHL.

Unexplained Variation in Benefit of Treatment of Congenital Unilateral Aural Atresia: A Review of the Literature.

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.

One-Year Follow-Up in Children with Conductive Hearing Loss Using ADHEAR.

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.

Sequential Bilateral Cochlear Implantation in a Child with Severe External, Middle, and Inner Ear Malformations: Surgical Considerations and Practical Aspects.

Cochlear implantation (CI) is a safe and beneficial surgery for children with congenital inner ear malformations, with the exception of cochlear nerve aplasia. The combination of microtia with middle and inner ear abnormalities is extremely uncommon and sufficiently severe to make a surgical approach to the cochlea difficult. We report herein the case of a 2-year-old girl who presented with profound bilateral sensorineural hearing loss, congenital aural atresia, microtia, and inner ear malformations. High-resolution computed tomography revealed poor development of the bilateral middle ear spaces, absence of the incus and stapes, aberrant courses of facial nerves, aplastic lateral semicircular canals, and covered round windows. With intraoperative imaging assistance, sequential bilateral CI was performed using a transmastoid approach with no complication. We propose that CI is feasible in patients with severe external and middle ear malformations. However, major malformations increase the risk of complications. As the facial nerve and cochlea are difficult to locate due to the lack of important anatomical landmarks, detailed planning and adequate preparation, including review of the preoperative imaging data, and the use of facial nerve monitoring and intraoperative imaging are very important. In addition, experienced surgeons should perform CI to ensure the success of the operation.

Anatomic Measurements of Distances from Lateral Surface of Cranium to Cochlea in Congenital Aural Atresia and Stenosis Patients.

INTRODUCTION: Studies have shown that higher response levels can be obtained when the bone conduction stimulation position is closer to the cochlea. However, the morphological characteristics of round window niche and posterior tympanum in congenital aural atresia (CAA) and stenosis (CAS) patients were different from the normal. These affected the position of the cochlea at the cranial base. It was still unknown whether the distances from the cranium of CAA and CAS patients to the cochlea were the same as those of normal patients or not. OBJECTIVE: To measure distances from various points on the lateral surface of the cranium to the cochlea and the cranium thickness on these points among a CAA group, CAS group and normal control group, which may provide valuable information for the better position of bone conduction stimulation. METHODS: CT images of CAA, CAS patients and these patients' healthy sides were analyzed. Firstly, the Frankfurt horizontal plane (Pfrkt) was established. Secondly, a model of part of the cranium was three-dimensionally reconstructed. Then, the Pfrkt plane was rotated down 20, 30 and 40° according to the superior margin of the external auditory canal. At every angle, points 25, 30, 35 and 40 mm away from the superior margin of the external auditory canal were marked out on the surface of the model and recorded as P20A, P30A, P40A, P20B, etc. The spatial distances between the cranium and ipsilateral cochlea were defined as lengths of points on the surface of the model to the cochlea apex (CA), cochlear base (CB) and modiolus midpoint (MM), respectively, recorded as P20A/CA, P20A/CB, P20A/MM, P30A/CA, etc. Results and Conclusions: In all groups, the length of P20D/CA was the shortest compared to P30D/CA and P40D/CA (p < 0.05). The P20A/CB and P20A/MM were also the shortest (p < 0.05). When the Pfrkt plane was rotated down 30 and 40°, the results were the same as at 20° (p < 0.05). However, P20D, P30D and P40D were almost on the mastoid air cells. We suggest that the bone conduction stimulation position is placed closer to the ear, while avoiding the mastoid air cells in the CAA and CAS patients.

Sound localisation ability using cartilage conduction hearing aids in bilateral aural atresia.

OBJECTIVE: Patients with bilateral aural atresia often exhibit poor sound localisation due to bone conduction (BC) features. However, most patients using cartilage conduction (CC) hearing aids reported improvement of sound localisation. DESIGN: CC hearing aids were fitted binaurally. Subsequently, sound localisation was evaluated in three conditions: unaided, aided with previously used hearing aids (air conduction or BC hearing aids), and aided with CC hearing aids. Ears were evaluated with eight loudspeakers positioned in a full-circle at 45-degree interval. Loudspeakers were classified into left and right by midline and front and back by horizontal line. The abilities to distinguish left from right and front from back were compared among three conditions. STUDY SAMPLE: Thirteen patients with bilateral aural atresia participated. RESULTS: The ability to distinguish sounds originating from left or right for participants aided with CC hearing aids was significantly better than that for other conditions (p < 0.05). For distinguishing sounds originating from front or back, unaided ears were significantly better than ears aided with CC hearing aids (p < 0.05). CONCLUSIONS: CC hearing aids provide the ability to distinguish left from right in patients with bilateral aural atresia.

Vibrant Soundbridge® in preschool children with unilateral aural atresia: acceptance and benefit.

The aim of this pilot study was to determine the acceptance and benefit from the middle ear implant system Vibrant Soundbridge® (VSB, MED-EL Corporation, Austria) by means of a questionnaire, compared to a previously used conventional bone conducting hearing device, in preschool children with unilateral congenital aural atresia. Prospective cohort study. All nine children with unilateral congenital aural atresia used the VSB and had previous experience with a bone conducting hearing device. The benefit from the VSB was evaluated by questionnaires concerning acceptance of hearing aids, handling, listening effort, behavior, quality of life, and the duration of daily use and compared to the experience with the bone conducting hearing device. In addition, to quantify the benefit from the VSB use, audiological assessment (pure-tone audiometry via free field testing, speech audiometry, and localization test) was performed with and without VSB. The questionnaires and audiological test results were compared pairwise. According to all questionnaire areas, children benefited significantly more from the VSB compared to bone conducting hearing device (ps <.05). The most important finding was a significant increase in daily use from 2 h for the bone conducting hearing device to 10 h for the VSB. Children performed significantly better with the VSB than without it in the audiological assessment. Children with unilateral aural atresia benefited significantly more from the VSB compared to a conventional bone conducting hearing device according to the parents' questionnaires and yielded better results in the audiometry and localization test with the VSB than without it.

Prevalence of Renal and Cervical Vertebral Anomalies in Patients With Isolated Microtia and/or Aural Atresia.

OBJECTIVE: The objective of this study was to determine whether patients with isolated microtia or aural atresia have an increased prevalence of renal or cervical vertebral anomalies. DESIGN: The study design was a retrospective medical record review. SETTING: The setting was the following four distinct institutions: an urban tertiary care children's hospital, two urban academic medical centers, and a staff-model health maintenance organization. PARTICIPANTS: Patients diagnosed with microtia, aural atresia, or oculoauriculovertebral spectrum were identified. Patients with facial asymmetry, craniofacial microsomia, and other craniofacial abnormalities or syndromes were excluded. MAIN OUTCOME MEASURES: Main outcome measures were the number of patients with isolated microtia or aural atresia who underwent a renal ultrasound or cervical spine X-ray, the results of those studies, and further evaluation or treatment for any abnormalities found. STATISTICAL ANALYSIS: A binomial analysis using a one-sided 95% confidence level was performed. RESULTS: A total of 514 patients with isolated microtia and/or aural atresia were identified. Of these patients, 145 (28%) had undergone a renal ultrasound and 81 (16%) had undergone cervical spine X-rays. A total of 3 patients (2%) had minimal renal pelviectasis, all of which had resolved on repeat ultrasound and required no treatment. There were no structural renal abnormalities identified, and there were no cervical spine abnormalities identified. CONCLUSIONS: The data suggest that there is no increased prevalence of structural renal or cervical vertebral anomalies in patients with isolated microtia and/or aural atresia. Therefore, these patients do not require routine screening renal ultrasound or cervical spine X-rays.

The positional relationship between facial nerve and round window niche in patients with congenital aural atresia and stenosis.

To investigate whether differences existing in the distance between facial nerve (FN) and round window niche opening among congenital aural atresia (CAA), congenital aural stenosis (CAS) and a normal control group and to assess its effect on the round window implantation of vibrant soundbridge, CT images of 10 normal subjects (20 ears), 27 CAS patients (30 ears) and 25 CAA patients (30 ears) were analyzed. The distances from the central point of round window niche opening to the terminal point of the horizontal segment, the salient point of pyramidal segment, the beginning point of the vertical segment, and the vertical segment of the facial nerve (abbreviate as OA, OB, OC, OE, respectively) were calculated based on three-dimensional reconstruction using mimics software. The results suggested that the pyramidal segment of the FN was positioned more closely to round window niche opening in patients with both CAA and CAS groups than that in control group, whereas there was no significant difference between CAA and CAS group (P < 0.05). The vertical portion of the FN was positioned more closely to round window niche opening in the CAA group than those in both the CAS and control groups with statistical significance (P < 0.05). Furthermore, the vertical portion of the FN was positioned more closely to round window niche opening in the CAS group than that in control group (P < 0.05). In conclusion, the dislocation between facial nerve and round window niche in patients with congenital auditory canal malformations could have significant effects on the round window implantation of vibrant soundbridge. Moreover, three-dimensional measurements and assessments before surgery might be helpful for a safer surgical approach and implantation of vibrant soundbridge.

Three-year experience with the Sophono in children with congenital conductive unilateral hearing loss: tolerability, audiometry, and sound localization compared to a bone-anchored hearing aid.

Bone conduction devices (BCDs) are advocated as an amplification option for patients with congenital conductive unilateral hearing loss (UHL), while other treatment options could also be considered. The current study compared a transcutaneous BCD (Sophono) with a percutaneous BCD (bone-anchored hearing aid, BAHA) in 12 children with congenital conductive UHL. Tolerability, audiometry, and sound localization abilities with both types of BCD were studied retrospectively. The mean follow-up was 3.6 years for the Sophono users (n = 6) and 4.7 years for the BAHA users (n = 6). In each group, two patients had stopped using their BCD. Tolerability was favorable for the Sophono. Aided thresholds with the Sophono were unsatisfactory, as they did not reach under a mean pure tone average of 30 dB HL. Sound localization generally improved with both the Sophono and the BAHA, although localization abilities did not reach the level of normal hearing children. These findings, together with previously reported outcomes, are important to take into account when counseling patients and their caretakers. The selection of a suitable amplification option should always be made deliberately and on individual basis for each patient in this diverse group of children with congenital conductive UHL.