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Aesthetic and hearing rehabilitation in patients with bilateral microtia-atresia
International Journal of Pediatric Otorhinolaryngology 101 (2017) 150e157
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International Journal of Pediatric Otorhinolaryngology journal homepage: http://www.ijporlonline.com/
Aesthetic and hearing rehabilitation in patients with bilateral microtia-atresia Xinmiao Fan a, Yibei Wang a, Pu Wang a, Yue Fan a, Yu Chen b, Yuanli Zhu b, Xiaowei Chen a, * a b
Department of Otolaryngology, Peking Union Medical College Hospital, Beijing, PR China Department of Radiology, Peking Union Medical College Hospital, Beijing, PR China
a r t i c l e i n f o
a b s t r a c t
Article history: Received 22 June 2017 Received in revised form 7 August 2017 Accepted 8 August 2017 Available online 10 August 2017
Objectives: To evaluate the safety and efficacy of auricle reconstruction and active transcutaneous boneconduction implantation in patients with bilateral microtia-atresia. Design: Patients were chosen prospectively, with each being his/her own control. Setting: The setting was a tertiary referral center. Participants: Twelve patients, aged 6e18 years, with bilateral microtia-atresia suffering from bilateral conductive hearing loss. All had an upper bone conduction threshold limit of 45 dB HL at frequencies of 0.5e4 kHz. Main outcome measures: Patient satisfaction with the reconstructed auricle was rated as highly satisfactory, basically satisfactory, or unsatisfactory. Mean pure-tone thresholds and speech audiometry test results were compared among patients unaided, with a soft-band Bonebridge, and with an implanted Bonebridge. Subjective satisfaction was analyzed using three questionnaires: the Abbreviated Profile of Hearing Aid Benefit (APHAB), the Glasgow children's benefit inventory (GCBI), and the International Outcome Inventory for Hearing Aids (IOI-HA). Results: All patients who underwent auricle reconstruction expressed satisfaction with their appearance. The mean pure-tone thresholds of unaided patients and those with soft-band and implanted Bonebridge were 55.25 ± 3.43 dBHL, 31.37 ± 3.03 dBHL, and 21.25 ± 2.16 dBHL, respectively. The mean speech discrimination scores measured in a sound field with a presentation level of 65 dB SPL under these three conditions were 46.0 ± 0.11%, 80.0 ± 0.09%, and 94.0 ± 0.02%, respectively. Questionnaires demonstrated patients' benefits and satisfaction with this surgery. Conclusions: The surgical procedure involving auricle reconstruction and Bonebridge implantation was safe and effective for patients with bilateral microtia-atresia, solving both appearance and hearing problems. © 2017 Published by Elsevier Ireland Ltd.
Keywords: Congenital microtia Auricle reconstruction Hearing rehabilitation Bonebridge
1. Introduction Microtia-atresia is characterized by abnormalities of the auricle (microtia) and aplasia or hypoplasia of the external auditory canal, often associated with middle ear abnormalities. The incidence of congenital microtia-atresia has been estimated to be one in 10,000
* Corresponding author. Department of Otolaryngology, Peking Union Medical College Hospital, #1 shuaifuyuan, Beijing, 100730, PR China. E-mail addresses: [email protected] (X. Fan), wangyibei19910115@163. com (Y. Wang), [email protected] (P. Wang), [email protected] (Y. Fan), [email protected] (Y. Chen), [email protected] (Y. Zhu), chenxw_pumch@ 163.com (X. Chen). http://dx.doi.org/10.1016/j.ijporl.2017.08.008 0165-5876/© 2017 Published by Elsevier Ireland Ltd.
births, with about one-quarter being bilateral [1]. This condition is frequently associated with various syndromes, including TreacherCollins, Goldenhar syndromes and hemifacial microsomia [2]. Congenital microtia-atresia affects patients in two specific ways. The first is severe conductive hearing loss (HL) with an air-bone gap of 50e60 dB, which, if not corrected in a timely manner, may delay speech development. The second is feelings of inferiority and problems integrating into social environments due to auricle malformation [3]. Resolution of microtia-atresia therefore requires both hearing rehabilitation and appearance improvement. Patient appearance may be improved by two-stage auricle reconstruction surgery, including implantation of a skin soft tissue expander and auricle reconstruction using autogenous rib cartilage.
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These patients also require hearing rehabilitation to improve their hearing. Methods of hearing rehabilitation include surgical reconstruction of the external aural canal (atresiaplasty), conventional bone conduction hearing aids, and percutaneous bone anchored hearing aids (BAHA). Despite good rehabilitation results, these procedures have been associated with various complications, including canal restenosis, chronic infections, salivary fistula, local inflammation, skin-overgrowth, and implant extrusion [4,5]. Although transcutaneous passive skin-drive magnetic devices were developed to overcome the limitations of percutaneous bone anchored hearing aids, these devices have the disadvantage of a transcutaneous attenuation of 10e15 dB [6]. The Bonebridge is an active transcutaneous bone-conduction implant (MED-EL Corporation, Innsbruck, Austria) which consists of two major parts, a magnetic implant and an external audio processor. The external processor provides active direct-drive transcutaneous conduction to the magnetic receiver under the skin, directly stimulating the bone via an electromagnetic transducer screwed onto the mastoid [7]. This system was found to have fewer complications compared with percutaneous bone conduction implants and showed proven auditory benefits [8]. Therefore, unilateral Bonebridge should theoretically benefit patients with bilateral microtia-atresia. Determining the position of the Bonebridge implant is important, as the audio processor should not interfere with auricle reconstruction. If the audio processor touches the skin flaps or ear rim, it can potentially impair the blood supply to the reconstructed auricle, which may lead to necrosis in the reconstructed ear and produce acoustic feedback [9]. A three-stage surgical procedure involving auricle reconstruction and Bonebridge implantation was therefore developed. The Bonebridge implantation was performed after the auricle was reconstructed, or before implantation of the skin soft tissue expander. The first aim of this study was to describe the surgical procedure of Bonebridge implantation. Although Bonebridge implantation has shown good outcomes [3,7,8,10], few studies have assessed the efficacy of Bonebridge in speakers of Mandarin. The second aim of this study was to evaluate the benefits of unilateral Bonebridge implantation, using Mandarin Speech Test Materials (MSTMs) and three questionnaires, in 12 Mandarin-speaking patients with bilateral microtia-atresia treated at Peking Union Medical College Hospital (PUMCH).
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2. Materials and methods 2.1. Participants This single center prospective study involved patients with bilateral microtia-atresia treated at PUMCH in Beijing, China, and was approved by the Institutional Review Board of PUMCH. Patients were included if they were aged >6 years, of height >1.28 m, had bone conduction hearing thresholds >45 dB HL at frequencies of 0.5e4 kHz, and were psychologically and emotionally stable. Patients with unilateral microtia-atresia, malformation of the inner ear (sensorineural hearing loss) or concomitant diagnosed conditions such as cerebral palsy and intellectual disability were excluded from this study. Twelve patients (Nine boys, three girls) with bilateral microtiaatresia were enrolled in the study. Degrees of auricular dysplasias were evaluated according to Max's classification [11]. Their mean (SD) age was 11.0 (5.0) years (range, 6e18 years). All had conductive hearing loss due to bilateral atresia. All had used a soft-band Bonebridge for at least 6 months prior to unilateral Bonebridge implantation surgery, performed at PUMCH between March 2016 and October 2016. The detailed characteristics of these patients are shown in Table 1. 2.2. Surgical techniques involving auricle reconstruction and Bonebridge implantation Before Bonebridge implantation, patients underwent a temporal bone high-resolution computed tomography (HRCT) scan to evaluate the structure of their temporal bone and middle ear. These CT datasets were imported into the three-dimensional simulation software to determine the optimal site of the Bonebridge-FMT (Fig. 1) [12]. Patients were graded by the Jahrsdoerfer grading scale [13]. Based on the results of three-dimensional simulations, all patients underwent Bonebridge implantation via a transmastoid (TM) approach. Patients who wish aesthetic and hearing rehabilitation were performed three-stage surgery. During the first stage, a soft skin expander was implanted in the mastoid region. During the second stage, the auricle was reconstructed using autogenous rib cartilage. During the third stage, performed 6 months later, the Bonebridge was implanted. The surgical procedures are shown in Fig. 2.
Table 1 Demographics and clinical data of patients. Item Gendery Age (years)y
Degree of auricularmicrotia (right ear/left ear)*z
Syndrome Performed surgery Before BB implantation
BB implantation pre or post auricle reconstruction
Js Grading
1 2
M M
6.5 9
III/III III/II
post post
6 6
3 4 5 6 7 8 9 10 11 12
M F M M F M M F F F
7.5 6 7 7 18 6 18 13 16 18
III/III II/III III/III III/III III/III II/III III/II III/III III/III III/III
Left auricle reconstruction þ right skin expander implantation Right auricle reconstruction þ left Vibrant Sound-bridge implantation Right auricle reconstruction þ right atresiaplasty Goldenhar none Right auricle reconstruction þ left skin expander implantation Bilateral auricle reconstruction Right atresiaplasty none Right skin expander implantation none none none
post pre post post post pre post pre post post
7 6 5 6 7 6 6 6 6 6
yF, female; M, male; Age, age in years at the time of Bonebridge implantation. zDegrees of auricular dysplasias were evaluated according to Max's classification.
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Fig. 1. Location of Bonebridge-FMT on the three-dimensional simulation software. A. The axial image to avoid touch ear canal and sigmoid sinus. B. The coronal image to avoid touch dura of brain. C. The sagittal views to make sure weather the screws are fully embedded for at least 2e3 mm. D. The three-dimensional reconstructed views of the Bonebridge-FMT location, with the blue arrow indicating skull fissure, the orange arrow indicating temporal line, and the green arrow indicating mastoid apex. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
Patients who did not wish auricle reconstruction underwent Bonebridge implantation surgery alone. The C-shaped incision was more posterior (about 1 cm beyond the hairline) to protect sufficient skin flap for future auricle construction surgery. This was followed by the separation of the inferiorly pedicled muscular periosteal flap to expose the region for the coil. The surgical procedures are illustrated in Fig. 3. 2.3. Audiometric data Pure-tone audiograms and speech perception tests were performed unaided, with the soft-band Bonebridge and with the implanted Bonebridge. Air conduction thresholds were evaluated through loudspeakers 1 m in front of the subject at 0.25, 0.5, 1, 2, 4 and 8 kHz, using the Mandarin Speech Test Materials (MSTM) [14]. Speech discrimination scores (in quiet) were measured using disyllabic tests, which consisted of 10 lists containing 50 Chinese characters or spondaic words. Speech stimuli were presented at 65 dB SPL. All test materials were presented without repetition. The average hearing gains at 0.5, 1, 2, 4 kHz were calculated, and thresholds unaided, with the soft-band Bonebridge, and with the implanted Bonebridge were compared. 2.4. Questionnaires The reconstructed auricles were evaluated by the patients and/ or their parents 6 months postoperatively. Degree of satisfaction was rated by an ordinal category (highly satisfactory, basically
satisfactory, and unsatisfactory) and a numerical scale from 1 (very bad) to 5 (excellent). Patients' subjective satisfaction was evaluated using three questionnaires, administered via face-to-face interviews 6 months postoperatively. The first questionnaire was the Chinese version of the Abbreviated Profile of Hearing Aid Benefit (APHABCH) questionnaire [15], which was first utilized in 1995 to evaluate the disability associated with hearing loss and the reduction of this disability by hearing aids. This questionnaire consisted of 24 items in four subscales: Ease of Communication (EC), Reverberation (RV), Background Noise (BN), and Aversiveness of Sounds (AV). All items addressed communication difficulties in daily life and patients were asked to indicate their agreement on a sevenpoint scale. The effectiveness of the Bonebridge was evaluated using the validated Chinese version of the International Outcome Inventory for Hearing Aids questionnaire [16]. This questionnaire is comprised of seven items: hearing aid use (Use) and benefit (Ben), residual limitation in activity (RAL), satisfaction (Sat), residual participation restriction (RPR), impact on others (Ioth) and quality of life (QoL). Each item was evaluated on a five-point scale. All patients aged <18 years were assessed with the Glasgow Children's Benefit Inventory (GCBI) questionnaire [17], translated into Chinese by two of the authors of this study (Zurich, Switzerland). The Glasgow children's benefit inventory (GCBI) is comprised of 18 items in two subscales, evaluating improvements in patients' quality of life.
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Fig. 2. Three-stage surgical procedure involving auricle reconstruction and Bonebridge (BB) implantation. A. The lateral view of the microtia patient with a soft-band Bonebridge (BB). B. The lateral view of the patient after skin soft tissue expander implantation. C. The lateral view of the reconstructed auricle at 6th month postoperatively. D. The C-shaped incision of Bonebridge (BB) implantation. E. The skin and subcutaneous tissue was separated to expose the mastoid bone. F. The FMT was fixed in the bone using screws and a lift, and the coil was placed under the skin.
2.5. Statistics All data were analyzed using SPSS (V 21, the International Business Machines Corp). Continuous variables were presented as mean ± standard deviation and compared by paired t tests with Bonferroni corrections. Statistical significance was defined as a p value < 0.05. 3. Results Nine of the 12 patients underwent the three-stage surgery, whereas three underwent Bonebridge implantation alone. Followup data was obtained 6 months postoperatively. Of the nine patients who underwent auricle reconstruction, eight regarded the reconstructed auricle as “highly satisfactory” and the ninth as “basically satisfactory”. All nine patients had scores of 4 (good) to 5 (excellent). None of these patients experienced major complications, such as traumatic extrusion or skin necrosis. The mean sound field thresholds at 0.5, 1, 2 and 4 kHz were 55.25 ± 1.75 dB HL unaided, 31.38 ± 1.29 dB HL with the soft-band Bonebridge, and 21.25 ± 1.48 dB HL with the implanted Bonebridge. The mean sound field threshold was significantly better with the soft-band Bonebridge than unaided (t ¼ 22.15, p < 0.01), and with the implanted than with the soft-band Bonebridge (t ¼ 16.81, p < 0.01). Fig. 4 lists the average hearing thresholds and the average hearing thresholds of frequencies from 0.25 KHZ to 8KHZ under
three conditions. The mean speech discrimination scores, evaluated by disyllabic tests (presentation level ¼ 65 dB SPL), were 46.0 ± 11.2% unaided, 80.0 ± 9.2% with the soft-band Bonebridge, and 94.0 ± 2.2% with the implanted Bonebridge, with the soft-band and implanted Bonebridge showing mean gains of 34.0 ± 16.3% (t ¼ 6.5, P < 0.01) and 47.5 ± 12.6% (t ¼ 11.9, P < 0.01), respectively (Fig. 5). The twelve patients answered all three questionnaires. Patients younger than ten years of age completed the questionnaires with the help of their parents. All patients reported considerable benefit from both the soft-band and implanted Bonebridge, and felt they were not restricted in activities. All experienced a very high level (4e5) of impact on their social life(Fig. 6). The Glasgow children's benefit inventory (GCBI) questionnaire score for each patient was divided by the number of questions and then multiplied by 50, thus yielding a final score ranging between 100 and þ 100 [18]. For both implanted and soft-band Bonebridge, the final scores were positive, with means of 39.6 ± 3.6 and 33.0 ± 3.8, respectively. 4. Discussion 4.1. Surgical procedure involving aesthetic and hearing rehabilitation Because sufficient skull thickness and autogenous rib cartilage are required, auricle reconstruction and Bonebridge implantation
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Fig. 3. The surgical procedure of Bonebridge (BB) implantation before skin soft tissue expander implantation. A. A more posterior C-shaped incision was performed. B. The T-Sizer and C-Sizer bone recess. C. The FMT was closed with a double-layer muscle-fascia closure. D. The incision was sutured with 5e0 stitch.
Fig. 4. (I) Mean hearing thresholds of twelve patients under three different conditions., Blue, pre-intervention hearing thresholds; Orange, 6 months after using soft-band Bonebridge (BB); Green, postoperative hearing thresholds evaluated at 6 months after activation of the implant. (II) Mean frequency hearing thresholds of twelve patients under three different conditions. Diamond, pre-intervention hearing thresholds; Square, 6 months after using soft-band Bonebridge (BB); Triangle, postoperative hearing thresholds evaluated at 6th months after activation of the implant. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
should be performed after age 6 years. This study introduces an overall flow-chart for patients born with bilateral microtia-atresia. A soft-band Bonebridge should be used as early as possible to improve hearing temporarily. This should be followed, after age 6 years, by soft expander implantation into the skin and auricle reconstruction. Bonebridge implantation surgery should be performed 6 months later, thereby avoiding any effect on the blood supply of the transferred skin flap and assuring rapid recovery. Seven patients were thus treated, solving both their appearance and hearing problems. In addition, the incision used for Bonebridge implantation was the same as that of the transferred skin flap,
eliminating the need for an additional incision. This series of procedures may be optimal for patients with bilateral microtia-atresia. Some patients who do not wish to undergo auricle reconstruction may later decide to undergo auricle reconstruction. These patients may therefore undergo Bonebridge implantation before auricle reconstruction, thus requiring a posterior retroauricular incision to avoid impairing the local flap essential for auricle reconstruction surgery. Considering skin soft expander implantation surgery requires only about 20 min, we intend to combine Bonebridge implantation with contralateral skin expander implantation into one stage, thereby reducing anesthesia time.
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Fig. 5. Speech discrimination scores at 65 dB SPL. Diamonds pre-intervention, square 6th month after using soft-band Bonebridge (BB), triangle 6th month after Bonebridge (BB) implantation.
Fig. 6. (I) Mean Abbreviated Profile of Hearing Aid Benefit (APHAB) scores in the following subscales: ease of communication (EC), background noise (BN), reverberation (RV), and aversiveness of sound (AV) for unaided, aided with soft-band Bonebridge (BB) at 6th month, and implanted Bonebridge (BB) at 6th month. (II) Mean scores for each item of the International Outcome Inventory for Hearing Aids, including hearing aid use (Use), benefit (Ben), residual limitation in activity (RAL), satisfaction (Sat), residual participation restriction (RPR), impact on others (Ioth) and quality of life (QoL), under soft-band Bonebridge (BB) at 6th month, and implanted Bonebridge (BB) at 6th month.
4.2. Bonebridge may be an optimal option for patients with bilateral microtia-atresia Atresiaplasty was one of the most common method for hearing improvement in patients with congenital aural atresia [19]. The lack of normal land marks, however, makes atresiaplasty one of the most challenging types of surgery in otology [20]. The Jahrsdoerfer CT grading system has been used to select candidates for atresiaplasty [21], with surgery recommended for patients with a Jahrsdoerfer score higher than 6. Based on these criteria, ten of our patients scored less than 7 points with both ears and were considered poor candidates for hearing reconstruction and therefore did not undergo atresiaplasty. The other two patients underwent atresiaplasty but experienced canal stenosis within 6 months. These two patients did not wish to undergo revision surgery for aural canal reconstruction. The Bonebridge implant was totally secured under closed skin, avoiding most complications, such as skin reactions, skin overgrowth, and implant extrusion. A study of complications after Bonebridge implantation reported skin reaction and revision surgery rates of 5.12% and 0.85% respectively [22]. In contrast, none of our patients experienced any complications 6-months after
Bonebridge implantation. Thus, Bonebridge may be considered an important technological advancement, with its functional outcomes similar to those of bone anchored hearing aids (BAHA). The mean speech discrimination score for patients using the soft-band Bonebridge was 80.0 ± 9.2%, 34% higher than that in unaided patients. This finding suggests that use of a soft-band Bonebridge before age 6 years can improve speech perception in patients with bilateral microtia-atresia until Bonebridge implantation surgery is warranted. Speech discrimination scores following Bonebridge implantation was 94.00 ± 0.02%, similar to that in normal-hearing subjects. A previous study of four subjects with bilateral microtia-atresia reported a mean postoperative dissyllabic speech discrimination scores (at 65 dB SPL) of 86.2% [23]. The discrepancy may have been due to these patients having mixed hearing loss, whereas patients in our study had relatively better hearing capability. In general, our results demonstrated the audiological benefits of Bonebridge, consistent with previous findings. 4.3. Questionnaires used for the subjective evaluation of Bonebridge The subjective evaluation provided another perspective on the advantages of Bonebridge in treating patients with bilateral
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microtia-atresia. It has been suggested that children older than 8 years can accurately report on subjective issues [24]. In this study, patients younger than ten years of age completed the questionnaires with the help of their parents, but some patients aged 8e10 years also replied the questionnaires by themselves if they can coordinate well. The reliability of results may be lower for patients who completed the questionnaires with their parents' help, because the patients may be influenced by their parents' thought, which had been mostly considered when the results were analyzed. Results on the Abbreviated Profile of Hearing Aid Benefit (APHAB) indicate that Bonebridge markedly improved the ability of patients to communicate, both in quiet and noisy environments. AV was greater when wearing either a soft-band or an implanted Bonebridge. If unaided patients did not recognize loud sounds as annoying, Bonebridge-aided patients heard sounds they had not heard for a long period of time, which may be helpful for hearing restoration. Thus, an increase in AV may be a positive sign of hearing recovery [10]. The International Outcome Inventory for Hearing Aids questionnaire found that most patients were very satisfied with their device. Both soft-band and implanted Bonebridge enhanced subject participation, quality of life and satisfaction. The average scores on subscales of daily use were lower for the soft-band than for the implanted Bonebridge, indicating that patients tend to choose smaller, less conspicuous devices. The Glasgow children's benefit inventory (GCBI) is a subjective, child-oriented, post-interventional questionnaire developed to evaluate outcomes of pediatric otorhinolaryngologic surgery and therapy, with three subdomains including general, social and physical performance [25]. All of our patients showed benefits with Bonebridge in these three aspects, with a mean score of 39.6 ± 3.6, similar to the results of previous studies [18,25,26]. In addition, In terms of quality of life, all the patients showed a high satisfaction of Bonebridge in our study. The shortcoming of the Glasgow children's benefit inventory (GCBI) questionnaire we used was that it was not a validated Chinese version, so the reliability of the results may be poorer than that of the original questionnaire. 4.4. Limitations of Bonebridge The Bonebridge was released in 2011, but hadn't introduced into China until 2016, which was about two years delay compared with BAHA. The Bonebridge is semi-implantable, with a BonebridgeFMT 8.7 mm in thickness and 15.8 mm in diameter. Because the BB-FMT must be positioned completely in a narrow mastoid field close to delicate structures, including the dura of the brain, the sigmoid sinus and the ear canal, preoperative simulation of the location is necessary to determine the optimal position and avoid impairing the above structures. If the implant cannot be located on the mastoid, because the sigmoid sinus is too anterior, the dura mater is too low, or the patient has previously undergone mastoidectomy surgery, Bonebridge implantation may not be a proper choice. In addition, touching the dura mater or sigmoid sinus may be unavoidable when positioning the BB-FMT, but long-term follow-up is required to determine whether the depression could lead to sequelae [8,10,22]. 5. Conclusion In conclusion, the surgical procedure involving aesthetic and hearing rehabilitation improves both appearance and hearing in patients with bilateral microtia-atresia. Moreover, this method assists in proper positioning of the implant, avoiding complications and protecting the skin flap for possible future usage. The set of procedures outlined, starting with soft-band bone conduction
hearing aid usage followed by the three-stage surgical procedure, may be an optimal option for patients with bilateral microtiaatresia. Authors' contributions Xiaowei Chen acted as head surgeon for the Bonebridge (BB) implantations performed in this study and collected data. Xinmiao Fan analyzed data, composed the manuscript, and participated in some of the operations as an assistant. Yibei Wang, Pu Wang and Yue Fan participated in some of the operations as assistants, and collected and analyzed data. Yu Chen and Yuanli Zhu assisted in conducting the CT scans and collecting CT data. All authors read and approved the final manuscript. Declaration of interest The authors report no conflicts of interest and have received no payment in the preparation of this manuscript. The authors alone are responsible for the content and writing of this paper. Acknowledgements We thank Prof. Shu-man He from the Center for Hearing Research of Boys Town National Research Hospital for her useful technical comments and suggestions. This work was supported by grant to Xiaowei Chen from the General Programs of National Natural Science Foundation of China (81271053) and The National Key Research and Development Program of China (2016YFC0901501). References [1] F. Declau, C. Cremers, P. Van de Heyning, Diagnosis and management strategies in congenital atresia of the external auditory canal. Study Group on Otological Malformations and Hearing Impairment, Br. J. Audiol. 33 (5) (1999) 313e327. [2] B.J. McKinnon, et al., Vibrant soundbridge in aural atresia: does severity matter? Eur. Arch. Otorhinolaryngol. 271 (7) (2014) 1917e1921. [3] A. Jovankovicova, et al., Surgery or implantable hearing devices in children with congenital aural atresia: 25 years of our experience, Int. J. Pediatr. Otorhinolaryngol. 79 (7) (2015) 975e979. [4] C.A. Dun, et al., Assessment of more than 1,000 implanted percutaneous bone conduction devices: skin reactions and implant survival, Otol. Neurotol. 33 (2) (2012) 192e198. [5] R.S. Miller, et al., Diagnosis and management of salivary fistula after surgery for congenital aural atresia, Otol. Neurotol. 27 (2) (2006) 189e192. [6] O. Mulla, F. Agada, P.G. Reilly, Introducing the Sophono Alpha 1 abutment free bone conduction hearing system, Clin. Otolaryngol. 37 (2) (2012) 168e169. [7] R.F. Bento, P.T. Lopes, C. Cabral Junior Fda, Bonebridge bone conduction implant, Int. Arch. Otorhinolaryngol. 19 (4) (2015) 277e278. [8] M.E. Zernotti, A.B. Sarasty, Active bone conduction prosthesis: bonebridge(TM), Int. Arch. Otorhinolaryngol. 19 (4) (2015) 343e348. [9] Y. Bajaj, et al., How we do it: BAHA positioning in patients with microtia requiring auricular reconstruction, Clin. Otolaryngol. 30 (5) (2005) 468e471. [10] S. Schmerber, et al., Safety and effectiveness of the Bonebridge transcutaneous active direct-drive bone-conduction hearing implant at 1-year device use, Eur. Arch. Otorhinolaryngol. 274 (4) (2017) 1835e1851. [11] S. Bartel-Friedrich, Congenital auricular malformations: description of anomalies and syndromes, Facial Plast. Surg. 31 (6) (2015) 567e580. [12] I. Todt, et al., A computed tomographic data-based vibrant bonebridge visualization tool, Cochlear Implants Int. 15 (Suppl 1) (2014) S72eS74. [13] R.A. Jahrsdoerfer, et al., Grading system for the selection of patients with congenital aural atresia, Am. J. Otol. 13 (1) (1992) 6e12. [14] K. Badran, et al., Patient satisfaction with the bone-anchored hearing aid: a 14-year experience, Otol. Neurotol. 27 (5) (2006) 659e666. [15] R.M. Cox, G.C. Alexander, The abbreviated profile of hearing aid benefit, Ear Hear 16 (2) (1995) 176e186. [16] R.M. Cox, D. Stephens, S.E. Kramer, Translations of the International Outcome inventory for Hearing Aids (IOI-HA), Int. J. Audiol. 41 (1) (2002) 3e26. [17] J. Hendry, et al., The Glasgow Benefit Inventory: a systematic review of the use and value of an otorhinolaryngological generic patient-recorded outcome measure, Clin. Otolaryngol. 41 (3) (2016) 259e275. [18] K.M. Holgers, et al., Soft tissue reactions around percutaneous implants: a clinical study of soft tissue conditions around skin-penetrating titanium
X. Fan et al. / International Journal of Pediatric Otorhinolaryngology 101 (2017) 150e157 implants for bone-anchored hearing aids, Am. J. Otol. 9 (1) (1988) 56e59. [19] R.R. Cole, R.A. Jahrsdoerfer, Congenital aural atresia, Clin. Plast. Surg. 17 (2) (1990) 367e371. [20] A. de Alarcon, D.I. Choo, Controversies in aural atresia repair, Curr. Opin. Otolaryngol. Head. Neck Surg. 15 (5) (2007) 310e314. [21] D.C. Shonka Jr., W.J. Livingston 3rd, B.W. Kesser, The Jahrsdoerfer grading scale in surgery to repair congenital aural atresia, Arch. Otolaryngol. Head. Neck Surg. 134 (8) (2008) 873e877. [22] G.M. Sprinzl, A. Wolf-Magele, The Bonebridge Bone Conduction Hearing Implant: indication criteria, surgery and a systematic review of the literature, Clin. Otolaryngol. 41 (2) (2016) 131e143.
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International Journal of Pediatric Otorhinolaryngology journal homepage: http://www.ijporlonline.com/
Aesthetic and hearing rehabilitation in patients with bilateral microtia-atresia Xinmiao Fan a, Yibei Wang a, Pu Wang a, Yue Fan a, Yu Chen b, Yuanli Zhu b, Xiaowei Chen a, * a b
Department of Otolaryngology, Peking Union Medical College Hospital, Beijing, PR China Department of Radiology, Peking Union Medical College Hospital, Beijing, PR China
a r t i c l e i n f o
a b s t r a c t
Article history: Received 22 June 2017 Received in revised form 7 August 2017 Accepted 8 August 2017 Available online 10 August 2017
Objectives: To evaluate the safety and efficacy of auricle reconstruction and active transcutaneous boneconduction implantation in patients with bilateral microtia-atresia. Design: Patients were chosen prospectively, with each being his/her own control. Setting: The setting was a tertiary referral center. Participants: Twelve patients, aged 6e18 years, with bilateral microtia-atresia suffering from bilateral conductive hearing loss. All had an upper bone conduction threshold limit of 45 dB HL at frequencies of 0.5e4 kHz. Main outcome measures: Patient satisfaction with the reconstructed auricle was rated as highly satisfactory, basically satisfactory, or unsatisfactory. Mean pure-tone thresholds and speech audiometry test results were compared among patients unaided, with a soft-band Bonebridge, and with an implanted Bonebridge. Subjective satisfaction was analyzed using three questionnaires: the Abbreviated Profile of Hearing Aid Benefit (APHAB), the Glasgow children's benefit inventory (GCBI), and the International Outcome Inventory for Hearing Aids (IOI-HA). Results: All patients who underwent auricle reconstruction expressed satisfaction with their appearance. The mean pure-tone thresholds of unaided patients and those with soft-band and implanted Bonebridge were 55.25 ± 3.43 dBHL, 31.37 ± 3.03 dBHL, and 21.25 ± 2.16 dBHL, respectively. The mean speech discrimination scores measured in a sound field with a presentation level of 65 dB SPL under these three conditions were 46.0 ± 0.11%, 80.0 ± 0.09%, and 94.0 ± 0.02%, respectively. Questionnaires demonstrated patients' benefits and satisfaction with this surgery. Conclusions: The surgical procedure involving auricle reconstruction and Bonebridge implantation was safe and effective for patients with bilateral microtia-atresia, solving both appearance and hearing problems. © 2017 Published by Elsevier Ireland Ltd.
Keywords: Congenital microtia Auricle reconstruction Hearing rehabilitation Bonebridge
1. Introduction Microtia-atresia is characterized by abnormalities of the auricle (microtia) and aplasia or hypoplasia of the external auditory canal, often associated with middle ear abnormalities. The incidence of congenital microtia-atresia has been estimated to be one in 10,000
* Corresponding author. Department of Otolaryngology, Peking Union Medical College Hospital, #1 shuaifuyuan, Beijing, 100730, PR China. E-mail addresses: [email protected] (X. Fan), wangyibei19910115@163. com (Y. Wang), [email protected] (P. Wang), [email protected] (Y. Fan), [email protected] (Y. Chen), [email protected] (Y. Zhu), chenxw_pumch@ 163.com (X. Chen). http://dx.doi.org/10.1016/j.ijporl.2017.08.008 0165-5876/© 2017 Published by Elsevier Ireland Ltd.
births, with about one-quarter being bilateral [1]. This condition is frequently associated with various syndromes, including TreacherCollins, Goldenhar syndromes and hemifacial microsomia [2]. Congenital microtia-atresia affects patients in two specific ways. The first is severe conductive hearing loss (HL) with an air-bone gap of 50e60 dB, which, if not corrected in a timely manner, may delay speech development. The second is feelings of inferiority and problems integrating into social environments due to auricle malformation [3]. Resolution of microtia-atresia therefore requires both hearing rehabilitation and appearance improvement. Patient appearance may be improved by two-stage auricle reconstruction surgery, including implantation of a skin soft tissue expander and auricle reconstruction using autogenous rib cartilage.
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These patients also require hearing rehabilitation to improve their hearing. Methods of hearing rehabilitation include surgical reconstruction of the external aural canal (atresiaplasty), conventional bone conduction hearing aids, and percutaneous bone anchored hearing aids (BAHA). Despite good rehabilitation results, these procedures have been associated with various complications, including canal restenosis, chronic infections, salivary fistula, local inflammation, skin-overgrowth, and implant extrusion [4,5]. Although transcutaneous passive skin-drive magnetic devices were developed to overcome the limitations of percutaneous bone anchored hearing aids, these devices have the disadvantage of a transcutaneous attenuation of 10e15 dB [6]. The Bonebridge is an active transcutaneous bone-conduction implant (MED-EL Corporation, Innsbruck, Austria) which consists of two major parts, a magnetic implant and an external audio processor. The external processor provides active direct-drive transcutaneous conduction to the magnetic receiver under the skin, directly stimulating the bone via an electromagnetic transducer screwed onto the mastoid [7]. This system was found to have fewer complications compared with percutaneous bone conduction implants and showed proven auditory benefits [8]. Therefore, unilateral Bonebridge should theoretically benefit patients with bilateral microtia-atresia. Determining the position of the Bonebridge implant is important, as the audio processor should not interfere with auricle reconstruction. If the audio processor touches the skin flaps or ear rim, it can potentially impair the blood supply to the reconstructed auricle, which may lead to necrosis in the reconstructed ear and produce acoustic feedback [9]. A three-stage surgical procedure involving auricle reconstruction and Bonebridge implantation was therefore developed. The Bonebridge implantation was performed after the auricle was reconstructed, or before implantation of the skin soft tissue expander. The first aim of this study was to describe the surgical procedure of Bonebridge implantation. Although Bonebridge implantation has shown good outcomes [3,7,8,10], few studies have assessed the efficacy of Bonebridge in speakers of Mandarin. The second aim of this study was to evaluate the benefits of unilateral Bonebridge implantation, using Mandarin Speech Test Materials (MSTMs) and three questionnaires, in 12 Mandarin-speaking patients with bilateral microtia-atresia treated at Peking Union Medical College Hospital (PUMCH).
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2. Materials and methods 2.1. Participants This single center prospective study involved patients with bilateral microtia-atresia treated at PUMCH in Beijing, China, and was approved by the Institutional Review Board of PUMCH. Patients were included if they were aged >6 years, of height >1.28 m, had bone conduction hearing thresholds >45 dB HL at frequencies of 0.5e4 kHz, and were psychologically and emotionally stable. Patients with unilateral microtia-atresia, malformation of the inner ear (sensorineural hearing loss) or concomitant diagnosed conditions such as cerebral palsy and intellectual disability were excluded from this study. Twelve patients (Nine boys, three girls) with bilateral microtiaatresia were enrolled in the study. Degrees of auricular dysplasias were evaluated according to Max's classification [11]. Their mean (SD) age was 11.0 (5.0) years (range, 6e18 years). All had conductive hearing loss due to bilateral atresia. All had used a soft-band Bonebridge for at least 6 months prior to unilateral Bonebridge implantation surgery, performed at PUMCH between March 2016 and October 2016. The detailed characteristics of these patients are shown in Table 1. 2.2. Surgical techniques involving auricle reconstruction and Bonebridge implantation Before Bonebridge implantation, patients underwent a temporal bone high-resolution computed tomography (HRCT) scan to evaluate the structure of their temporal bone and middle ear. These CT datasets were imported into the three-dimensional simulation software to determine the optimal site of the Bonebridge-FMT (Fig. 1) [12]. Patients were graded by the Jahrsdoerfer grading scale [13]. Based on the results of three-dimensional simulations, all patients underwent Bonebridge implantation via a transmastoid (TM) approach. Patients who wish aesthetic and hearing rehabilitation were performed three-stage surgery. During the first stage, a soft skin expander was implanted in the mastoid region. During the second stage, the auricle was reconstructed using autogenous rib cartilage. During the third stage, performed 6 months later, the Bonebridge was implanted. The surgical procedures are shown in Fig. 2.
Table 1 Demographics and clinical data of patients. Item Gendery Age (years)y
Degree of auricularmicrotia (right ear/left ear)*z
Syndrome Performed surgery Before BB implantation
BB implantation pre or post auricle reconstruction
Js Grading
1 2
M M
6.5 9
III/III III/II
post post
6 6
3 4 5 6 7 8 9 10 11 12
M F M M F M M F F F
7.5 6 7 7 18 6 18 13 16 18
III/III II/III III/III III/III III/III II/III III/II III/III III/III III/III
Left auricle reconstruction þ right skin expander implantation Right auricle reconstruction þ left Vibrant Sound-bridge implantation Right auricle reconstruction þ right atresiaplasty Goldenhar none Right auricle reconstruction þ left skin expander implantation Bilateral auricle reconstruction Right atresiaplasty none Right skin expander implantation none none none
post pre post post post pre post pre post post
7 6 5 6 7 6 6 6 6 6
yF, female; M, male; Age, age in years at the time of Bonebridge implantation. zDegrees of auricular dysplasias were evaluated according to Max's classification.
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Fig. 1. Location of Bonebridge-FMT on the three-dimensional simulation software. A. The axial image to avoid touch ear canal and sigmoid sinus. B. The coronal image to avoid touch dura of brain. C. The sagittal views to make sure weather the screws are fully embedded for at least 2e3 mm. D. The three-dimensional reconstructed views of the Bonebridge-FMT location, with the blue arrow indicating skull fissure, the orange arrow indicating temporal line, and the green arrow indicating mastoid apex. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
Patients who did not wish auricle reconstruction underwent Bonebridge implantation surgery alone. The C-shaped incision was more posterior (about 1 cm beyond the hairline) to protect sufficient skin flap for future auricle construction surgery. This was followed by the separation of the inferiorly pedicled muscular periosteal flap to expose the region for the coil. The surgical procedures are illustrated in Fig. 3. 2.3. Audiometric data Pure-tone audiograms and speech perception tests were performed unaided, with the soft-band Bonebridge and with the implanted Bonebridge. Air conduction thresholds were evaluated through loudspeakers 1 m in front of the subject at 0.25, 0.5, 1, 2, 4 and 8 kHz, using the Mandarin Speech Test Materials (MSTM) [14]. Speech discrimination scores (in quiet) were measured using disyllabic tests, which consisted of 10 lists containing 50 Chinese characters or spondaic words. Speech stimuli were presented at 65 dB SPL. All test materials were presented without repetition. The average hearing gains at 0.5, 1, 2, 4 kHz were calculated, and thresholds unaided, with the soft-band Bonebridge, and with the implanted Bonebridge were compared. 2.4. Questionnaires The reconstructed auricles were evaluated by the patients and/ or their parents 6 months postoperatively. Degree of satisfaction was rated by an ordinal category (highly satisfactory, basically
satisfactory, and unsatisfactory) and a numerical scale from 1 (very bad) to 5 (excellent). Patients' subjective satisfaction was evaluated using three questionnaires, administered via face-to-face interviews 6 months postoperatively. The first questionnaire was the Chinese version of the Abbreviated Profile of Hearing Aid Benefit (APHABCH) questionnaire [15], which was first utilized in 1995 to evaluate the disability associated with hearing loss and the reduction of this disability by hearing aids. This questionnaire consisted of 24 items in four subscales: Ease of Communication (EC), Reverberation (RV), Background Noise (BN), and Aversiveness of Sounds (AV). All items addressed communication difficulties in daily life and patients were asked to indicate their agreement on a sevenpoint scale. The effectiveness of the Bonebridge was evaluated using the validated Chinese version of the International Outcome Inventory for Hearing Aids questionnaire [16]. This questionnaire is comprised of seven items: hearing aid use (Use) and benefit (Ben), residual limitation in activity (RAL), satisfaction (Sat), residual participation restriction (RPR), impact on others (Ioth) and quality of life (QoL). Each item was evaluated on a five-point scale. All patients aged <18 years were assessed with the Glasgow Children's Benefit Inventory (GCBI) questionnaire [17], translated into Chinese by two of the authors of this study (Zurich, Switzerland). The Glasgow children's benefit inventory (GCBI) is comprised of 18 items in two subscales, evaluating improvements in patients' quality of life.
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Fig. 2. Three-stage surgical procedure involving auricle reconstruction and Bonebridge (BB) implantation. A. The lateral view of the microtia patient with a soft-band Bonebridge (BB). B. The lateral view of the patient after skin soft tissue expander implantation. C. The lateral view of the reconstructed auricle at 6th month postoperatively. D. The C-shaped incision of Bonebridge (BB) implantation. E. The skin and subcutaneous tissue was separated to expose the mastoid bone. F. The FMT was fixed in the bone using screws and a lift, and the coil was placed under the skin.
2.5. Statistics All data were analyzed using SPSS (V 21, the International Business Machines Corp). Continuous variables were presented as mean ± standard deviation and compared by paired t tests with Bonferroni corrections. Statistical significance was defined as a p value < 0.05. 3. Results Nine of the 12 patients underwent the three-stage surgery, whereas three underwent Bonebridge implantation alone. Followup data was obtained 6 months postoperatively. Of the nine patients who underwent auricle reconstruction, eight regarded the reconstructed auricle as “highly satisfactory” and the ninth as “basically satisfactory”. All nine patients had scores of 4 (good) to 5 (excellent). None of these patients experienced major complications, such as traumatic extrusion or skin necrosis. The mean sound field thresholds at 0.5, 1, 2 and 4 kHz were 55.25 ± 1.75 dB HL unaided, 31.38 ± 1.29 dB HL with the soft-band Bonebridge, and 21.25 ± 1.48 dB HL with the implanted Bonebridge. The mean sound field threshold was significantly better with the soft-band Bonebridge than unaided (t ¼ 22.15, p < 0.01), and with the implanted than with the soft-band Bonebridge (t ¼ 16.81, p < 0.01). Fig. 4 lists the average hearing thresholds and the average hearing thresholds of frequencies from 0.25 KHZ to 8KHZ under
three conditions. The mean speech discrimination scores, evaluated by disyllabic tests (presentation level ¼ 65 dB SPL), were 46.0 ± 11.2% unaided, 80.0 ± 9.2% with the soft-band Bonebridge, and 94.0 ± 2.2% with the implanted Bonebridge, with the soft-band and implanted Bonebridge showing mean gains of 34.0 ± 16.3% (t ¼ 6.5, P < 0.01) and 47.5 ± 12.6% (t ¼ 11.9, P < 0.01), respectively (Fig. 5). The twelve patients answered all three questionnaires. Patients younger than ten years of age completed the questionnaires with the help of their parents. All patients reported considerable benefit from both the soft-band and implanted Bonebridge, and felt they were not restricted in activities. All experienced a very high level (4e5) of impact on their social life(Fig. 6). The Glasgow children's benefit inventory (GCBI) questionnaire score for each patient was divided by the number of questions and then multiplied by 50, thus yielding a final score ranging between 100 and þ 100 [18]. For both implanted and soft-band Bonebridge, the final scores were positive, with means of 39.6 ± 3.6 and 33.0 ± 3.8, respectively. 4. Discussion 4.1. Surgical procedure involving aesthetic and hearing rehabilitation Because sufficient skull thickness and autogenous rib cartilage are required, auricle reconstruction and Bonebridge implantation
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Fig. 3. The surgical procedure of Bonebridge (BB) implantation before skin soft tissue expander implantation. A. A more posterior C-shaped incision was performed. B. The T-Sizer and C-Sizer bone recess. C. The FMT was closed with a double-layer muscle-fascia closure. D. The incision was sutured with 5e0 stitch.
Fig. 4. (I) Mean hearing thresholds of twelve patients under three different conditions., Blue, pre-intervention hearing thresholds; Orange, 6 months after using soft-band Bonebridge (BB); Green, postoperative hearing thresholds evaluated at 6 months after activation of the implant. (II) Mean frequency hearing thresholds of twelve patients under three different conditions. Diamond, pre-intervention hearing thresholds; Square, 6 months after using soft-band Bonebridge (BB); Triangle, postoperative hearing thresholds evaluated at 6th months after activation of the implant. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
should be performed after age 6 years. This study introduces an overall flow-chart for patients born with bilateral microtia-atresia. A soft-band Bonebridge should be used as early as possible to improve hearing temporarily. This should be followed, after age 6 years, by soft expander implantation into the skin and auricle reconstruction. Bonebridge implantation surgery should be performed 6 months later, thereby avoiding any effect on the blood supply of the transferred skin flap and assuring rapid recovery. Seven patients were thus treated, solving both their appearance and hearing problems. In addition, the incision used for Bonebridge implantation was the same as that of the transferred skin flap,
eliminating the need for an additional incision. This series of procedures may be optimal for patients with bilateral microtia-atresia. Some patients who do not wish to undergo auricle reconstruction may later decide to undergo auricle reconstruction. These patients may therefore undergo Bonebridge implantation before auricle reconstruction, thus requiring a posterior retroauricular incision to avoid impairing the local flap essential for auricle reconstruction surgery. Considering skin soft expander implantation surgery requires only about 20 min, we intend to combine Bonebridge implantation with contralateral skin expander implantation into one stage, thereby reducing anesthesia time.
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Fig. 5. Speech discrimination scores at 65 dB SPL. Diamonds pre-intervention, square 6th month after using soft-band Bonebridge (BB), triangle 6th month after Bonebridge (BB) implantation.
Fig. 6. (I) Mean Abbreviated Profile of Hearing Aid Benefit (APHAB) scores in the following subscales: ease of communication (EC), background noise (BN), reverberation (RV), and aversiveness of sound (AV) for unaided, aided with soft-band Bonebridge (BB) at 6th month, and implanted Bonebridge (BB) at 6th month. (II) Mean scores for each item of the International Outcome Inventory for Hearing Aids, including hearing aid use (Use), benefit (Ben), residual limitation in activity (RAL), satisfaction (Sat), residual participation restriction (RPR), impact on others (Ioth) and quality of life (QoL), under soft-band Bonebridge (BB) at 6th month, and implanted Bonebridge (BB) at 6th month.
4.2. Bonebridge may be an optimal option for patients with bilateral microtia-atresia Atresiaplasty was one of the most common method for hearing improvement in patients with congenital aural atresia [19]. The lack of normal land marks, however, makes atresiaplasty one of the most challenging types of surgery in otology [20]. The Jahrsdoerfer CT grading system has been used to select candidates for atresiaplasty [21], with surgery recommended for patients with a Jahrsdoerfer score higher than 6. Based on these criteria, ten of our patients scored less than 7 points with both ears and were considered poor candidates for hearing reconstruction and therefore did not undergo atresiaplasty. The other two patients underwent atresiaplasty but experienced canal stenosis within 6 months. These two patients did not wish to undergo revision surgery for aural canal reconstruction. The Bonebridge implant was totally secured under closed skin, avoiding most complications, such as skin reactions, skin overgrowth, and implant extrusion. A study of complications after Bonebridge implantation reported skin reaction and revision surgery rates of 5.12% and 0.85% respectively [22]. In contrast, none of our patients experienced any complications 6-months after
Bonebridge implantation. Thus, Bonebridge may be considered an important technological advancement, with its functional outcomes similar to those of bone anchored hearing aids (BAHA). The mean speech discrimination score for patients using the soft-band Bonebridge was 80.0 ± 9.2%, 34% higher than that in unaided patients. This finding suggests that use of a soft-band Bonebridge before age 6 years can improve speech perception in patients with bilateral microtia-atresia until Bonebridge implantation surgery is warranted. Speech discrimination scores following Bonebridge implantation was 94.00 ± 0.02%, similar to that in normal-hearing subjects. A previous study of four subjects with bilateral microtia-atresia reported a mean postoperative dissyllabic speech discrimination scores (at 65 dB SPL) of 86.2% [23]. The discrepancy may have been due to these patients having mixed hearing loss, whereas patients in our study had relatively better hearing capability. In general, our results demonstrated the audiological benefits of Bonebridge, consistent with previous findings. 4.3. Questionnaires used for the subjective evaluation of Bonebridge The subjective evaluation provided another perspective on the advantages of Bonebridge in treating patients with bilateral
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microtia-atresia. It has been suggested that children older than 8 years can accurately report on subjective issues [24]. In this study, patients younger than ten years of age completed the questionnaires with the help of their parents, but some patients aged 8e10 years also replied the questionnaires by themselves if they can coordinate well. The reliability of results may be lower for patients who completed the questionnaires with their parents' help, because the patients may be influenced by their parents' thought, which had been mostly considered when the results were analyzed. Results on the Abbreviated Profile of Hearing Aid Benefit (APHAB) indicate that Bonebridge markedly improved the ability of patients to communicate, both in quiet and noisy environments. AV was greater when wearing either a soft-band or an implanted Bonebridge. If unaided patients did not recognize loud sounds as annoying, Bonebridge-aided patients heard sounds they had not heard for a long period of time, which may be helpful for hearing restoration. Thus, an increase in AV may be a positive sign of hearing recovery [10]. The International Outcome Inventory for Hearing Aids questionnaire found that most patients were very satisfied with their device. Both soft-band and implanted Bonebridge enhanced subject participation, quality of life and satisfaction. The average scores on subscales of daily use were lower for the soft-band than for the implanted Bonebridge, indicating that patients tend to choose smaller, less conspicuous devices. The Glasgow children's benefit inventory (GCBI) is a subjective, child-oriented, post-interventional questionnaire developed to evaluate outcomes of pediatric otorhinolaryngologic surgery and therapy, with three subdomains including general, social and physical performance [25]. All of our patients showed benefits with Bonebridge in these three aspects, with a mean score of 39.6 ± 3.6, similar to the results of previous studies [18,25,26]. In addition, In terms of quality of life, all the patients showed a high satisfaction of Bonebridge in our study. The shortcoming of the Glasgow children's benefit inventory (GCBI) questionnaire we used was that it was not a validated Chinese version, so the reliability of the results may be poorer than that of the original questionnaire. 4.4. Limitations of Bonebridge The Bonebridge was released in 2011, but hadn't introduced into China until 2016, which was about two years delay compared with BAHA. The Bonebridge is semi-implantable, with a BonebridgeFMT 8.7 mm in thickness and 15.8 mm in diameter. Because the BB-FMT must be positioned completely in a narrow mastoid field close to delicate structures, including the dura of the brain, the sigmoid sinus and the ear canal, preoperative simulation of the location is necessary to determine the optimal position and avoid impairing the above structures. If the implant cannot be located on the mastoid, because the sigmoid sinus is too anterior, the dura mater is too low, or the patient has previously undergone mastoidectomy surgery, Bonebridge implantation may not be a proper choice. In addition, touching the dura mater or sigmoid sinus may be unavoidable when positioning the BB-FMT, but long-term follow-up is required to determine whether the depression could lead to sequelae [8,10,22]. 5. Conclusion In conclusion, the surgical procedure involving aesthetic and hearing rehabilitation improves both appearance and hearing in patients with bilateral microtia-atresia. Moreover, this method assists in proper positioning of the implant, avoiding complications and protecting the skin flap for possible future usage. The set of procedures outlined, starting with soft-band bone conduction
hearing aid usage followed by the three-stage surgical procedure, may be an optimal option for patients with bilateral microtiaatresia. Authors' contributions Xiaowei Chen acted as head surgeon for the Bonebridge (BB) implantations performed in this study and collected data. Xinmiao Fan analyzed data, composed the manuscript, and participated in some of the operations as an assistant. Yibei Wang, Pu Wang and Yue Fan participated in some of the operations as assistants, and collected and analyzed data. Yu Chen and Yuanli Zhu assisted in conducting the CT scans and collecting CT data. All authors read and approved the final manuscript. Declaration of interest The authors report no conflicts of interest and have received no payment in the preparation of this manuscript. The authors alone are responsible for the content and writing of this paper. Acknowledgements We thank Prof. Shu-man He from the Center for Hearing Research of Boys Town National Research Hospital for her useful technical comments and suggestions. This work was supported by grant to Xiaowei Chen from the General Programs of National Natural Science Foundation of China (81271053) and The National Key Research and Development Program of China (2016YFC0901501). References [1] F. Declau, C. Cremers, P. Van de Heyning, Diagnosis and management strategies in congenital atresia of the external auditory canal. Study Group on Otological Malformations and Hearing Impairment, Br. J. Audiol. 33 (5) (1999) 313e327. [2] B.J. McKinnon, et al., Vibrant soundbridge in aural atresia: does severity matter? Eur. Arch. Otorhinolaryngol. 271 (7) (2014) 1917e1921. [3] A. Jovankovicova, et al., Surgery or implantable hearing devices in children with congenital aural atresia: 25 years of our experience, Int. J. Pediatr. Otorhinolaryngol. 79 (7) (2015) 975e979. [4] C.A. Dun, et al., Assessment of more than 1,000 implanted percutaneous bone conduction devices: skin reactions and implant survival, Otol. Neurotol. 33 (2) (2012) 192e198. [5] R.S. Miller, et al., Diagnosis and management of salivary fistula after surgery for congenital aural atresia, Otol. Neurotol. 27 (2) (2006) 189e192. [6] O. Mulla, F. Agada, P.G. Reilly, Introducing the Sophono Alpha 1 abutment free bone conduction hearing system, Clin. Otolaryngol. 37 (2) (2012) 168e169. [7] R.F. Bento, P.T. Lopes, C. Cabral Junior Fda, Bonebridge bone conduction implant, Int. Arch. Otorhinolaryngol. 19 (4) (2015) 277e278. [8] M.E. Zernotti, A.B. Sarasty, Active bone conduction prosthesis: bonebridge(TM), Int. Arch. Otorhinolaryngol. 19 (4) (2015) 343e348. [9] Y. Bajaj, et al., How we do it: BAHA positioning in patients with microtia requiring auricular reconstruction, Clin. Otolaryngol. 30 (5) (2005) 468e471. [10] S. Schmerber, et al., Safety and effectiveness of the Bonebridge transcutaneous active direct-drive bone-conduction hearing implant at 1-year device use, Eur. Arch. Otorhinolaryngol. 274 (4) (2017) 1835e1851. [11] S. Bartel-Friedrich, Congenital auricular malformations: description of anomalies and syndromes, Facial Plast. Surg. 31 (6) (2015) 567e580. [12] I. Todt, et al., A computed tomographic data-based vibrant bonebridge visualization tool, Cochlear Implants Int. 15 (Suppl 1) (2014) S72eS74. [13] R.A. Jahrsdoerfer, et al., Grading system for the selection of patients with congenital aural atresia, Am. J. Otol. 13 (1) (1992) 6e12. [14] K. Badran, et al., Patient satisfaction with the bone-anchored hearing aid: a 14-year experience, Otol. Neurotol. 27 (5) (2006) 659e666. [15] R.M. Cox, G.C. Alexander, The abbreviated profile of hearing aid benefit, Ear Hear 16 (2) (1995) 176e186. [16] R.M. Cox, D. Stephens, S.E. Kramer, Translations of the International Outcome inventory for Hearing Aids (IOI-HA), Int. J. Audiol. 41 (1) (2002) 3e26. [17] J. Hendry, et al., The Glasgow Benefit Inventory: a systematic review of the use and value of an otorhinolaryngological generic patient-recorded outcome measure, Clin. Otolaryngol. 41 (3) (2016) 259e275. [18] K.M. Holgers, et al., Soft tissue reactions around percutaneous implants: a clinical study of soft tissue conditions around skin-penetrating titanium
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