Acta Oto-Laryngologica. 2015; 135: 226–232
ORIGINAL ARTICLE
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
Surgical treatment of congenital aural atresia – is it still justified?
LENNART EDFELDT & KARIN STRÖMBÄCK Department of Surgical Sciences, Section of Otorhinolaryngology and Head and Neck Surgery, Uppsala University, Uppsala, Sweden
Abstract Conclusion: Surgery candidacy based on the surgical accessibility of the middle ear seems more valuable than the use of a preoperative grading system. Also patients with severe malformations can benefit from surgical reconstruction. Objective: To evaluate the long-term results of the primary surgical treatment of patients with congenital auricular atresia (CAA). Methods: One hundred patients with CAA underwent surgical reconstruction between 1985 and 2010. The mean follow-up time was 40 months. All patients were retrospectively scored using the Jahrsdoerfer grading scale and divided into two groups according to the grade of their malformation. Group 1 included 20 patients with scores of 4–6 and group 2 included 80 patients with scores of 7–10. Pre- and postoperative air conduction (AC), bone conduction (BC), pure-tone average ‘air–bone gap’ (PTA4-ABG), surgical findings, postoperative complications, and revision surgeries performed were determined and compared between the two groups. Results: For 90% of the patients in group 1 and 79% of the patients in group 2, the postoperative ABG was within 0 and 30 dB. The most common complications were recurrent infection, lateralization of the tympanic membrane, and restenosis of the ear canal.
Keywords: atresiaplasty, grade of malformation, long-term results
Introduction Preoperative grading systems are based on information gained from preoperative CT scans of the temporal bone and are regularly used for atresia surgery candidacy and the prediction of expected hearing outcome. The Jahrsdoerfer grading scale [1] is the most commonly used classification system and has a best possible score of 10. The stapes is weighted most heavily, with two points compared with one point for the other parameters. A score of ‡ 6 allows for the consideration of surgical reconstruction, whereas patients with a score of < 6 are considered to have a poor chance of a successful surgical outcome [1]. The combined reconstruction of the external ear, ear canal, and middle ear is intricate and involves a comprehensive multidisciplinary approach. The timing of surgical repair in unilateral and bilateral congenital auricular atresia (CAA) differs greatly. While surgical and hearing restoration is controversial in unilateral
cases, hearing restoration in bilateral cases is essential. Although surgical repair of CAA is one of the most complex otosurgical procedures and is associated with an abundance of complications, it is possible to achieve serviceable and unaided hearing [2]. The introduction of implantable devices for hearing rehabilitation has renewed the management of CAA. As inner ear hearing usually is preserved, numerous authors have utilized bone anchored hearing devices (BAHAs) [3,4], especially in cases not suitable for surgical reconstruction. Active middle ear implants represent another alternative and have increasingly been used for hearing rehabilitation in patients with CAA [5,6]. In light of these novel optional treatment alternatives, the justification of functional surgery is uncertain, and an evaluation of patient outcome is warranted. Here, we assessed long-term surgical and hearing results, incidence of complications, and
Correspondence: Lennart Edfeldt, Department of Surgical Sciences, Section of Otorhinolaryngology and Head and Neck Surgery, Uppsala University, Uppsala, Sweden. E-mail: [email protected]
(Received 18 August 2014; accepted 6 October 2014) ISSN 0001-6489 print/ISSN 1651-2251 online 2015 Informa Healthcare DOI: 10.3109/00016489.2014.979437
Atresiaplasty number of revision surgeries after primary surgical treatment of 100 patients with CAA divided into two groups according to the grade of their malformations.
Material and methods
Table I. Patient demographics. Characteristic
Group 1
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
Group 2
Number
20
80
Mean age (years)
25.6
20
Male
12
43
Female
A total of 116 patients with CAA were treated with surgical reconstruction between 1985 and 2010. The inclusion criteria for surgical treatment were the audiometric confirmation of serviceable cochlear function and the ability to surgically access the middle ear defined by the position of the facial nerve and the size of the middle ear and mastoid determined on the preoperative CT scan. The size of the middle ear was evaluated on the preoperative CT scan. If the distance between the atretic plate and the oval window at the level of the long process of the incus in the coronal projection exceeded 6 mm, the middle ear was defined as normal. If the distance was less than 6 mm the middle ear was defined as small. Cases in which the facial nerve completely covered the oval window did not undergo surgical treatment. In bilateral cases, the surgical reconstruction of the ear canal and the middle ear was accomplished at the age of 5–6 years without prior cosmetic correction. In unilateral cases, cosmetic repair, when applicable, was performed at the age of 5–6 years, and reconstruction of the ear canal and middle ear was performed at the age of 12–15 years to allow the patients to understand the consequences of the split skin transplantation. Surgical and audiological data were pre- and postoperatively entered into our database (Microsoft Access). After ethical approval, the data were extracted and evaluated. Eleven patients had undergone previous surgery and were excluded from the study. Due to more severe malformations, which precluded conventional surgical reconstruction, five patients were treated with a fenestration of the lateral semicircular canal and were also excluded from the study. Retrospectively, all patients were scored using the Jahrsdoerfer grading scale according to the findings on the preoperative CT scan and were divided into two groups corresponding to the grade of their malformations. Group 1 included 20 patients with scores from 4 to 6. Three patients scored 4, 11 patients scored 5, and 6 patients scored 6. No patient scored less than 4. The mean age of the patients was 25.6 years (range 6–62, median 24); 8 patients were female and 12 were male. Group 2 included 80 patients with scores from 7 to 10. Eleven patients scored 7, 21 patients scored 8, 23 patients scored 9, and 25 patients scored 10. The
227
8
37
Follow-up (months)
41
39
Bilateral CAA
11
33
2
12
Previous surgery CAA: Congenital auricular atresia.
mean age of the patients was 20 years (range 5–69, median 18); 37 patients were female and 43 were male. Eleven patients in group 1 and 33 patients in group 2 had bilateral CAA. Two patients in group 1 and 12 patients in group 2 had undergone a previous cosmetic reconstruction of the auricle. Three patients in group 2 had Treacher-Collins syndrome. The follow-up time for both groups varied between 9 and 105 months, with a mean of 39 months. The mean follow-up time for group 1 was 41 months and for group 2 it was 39 months. Patient demographics are presented in Table I. Surgical technique After a retro-auricular incision, a double musculoperiosteal flap was created, and cortical bone chips were harvested. The middle ear was exposed through removal of the atretic bone with drills and curettes, starting from the linea temporalis and following the middle fossa tegmen. To facilitate the access to the middle ear, the fused malleus-incus complex (MIC) was removed in all cases, and the mobility of the stapes was verified. When possible, the frame of the tympanic membrane was widened. To prevent formation of granulations, the novel ear canal was formed with care taken to limit the exposure of the mastoid air cells. The ossicular chain was reconstructed using autologous material (incus, MIC, malleus head, or cortical bone). In cases with a fixed stapes footplate the malleus handle was left at the primary operation and a staged stapedotomy or stapedectomy was performed using a wire piston or Fisch prosthesis (Olympus, Solna, Sweden). For myringoplasty, a piece of temporal fascia was used. The opened mastoid air cells were obliterated with bony pate and the musculo-periosteal flap and the reformed ear canal were lined with two or three pieces of 0.2 mm thick split skin obtained from the inside of the upper part of the arm. To prevent restenosis and match the width of the reformed ear canal, a large meatoplasty was performed by removing cartilage and soft tissue.
228
L. Edfeldt & K. Strömbäck
A rosebud made of strips of rayon silk and small pieces of Tacryl (Lohmann & Rauscher, Neuwied, Germany ) shaped the temporal fascia and the thin split skin, particularly the anterior tympanomeatal angle. Finally a bag of rayon silk and Tacryl was placed in the ear canal and left for 10 days.
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
Audiometry Psycho-acoustic air conduction (AC) and bone conduction (BC) hearing threshold levels were determined using calibrated audiometers pre- and postoperatively. Pure-tone average (PTA) values for BC thresholds levels and for AC thresholds levels were assessed at 0.5, 1, 2, and 3 kHz. Additionally, the AC and BC for 4 kHz were evaluated separately. PTA air–bone gap (ABG) was assessed at 0.5–3 kHz. Audiometric data were reported according to the guidelines published by the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology-Head and Neck Surgery [7]. Results Surgical findings Prognostic factors. The auricular anomaly was severe in seven cases (35%) and the auricle had been previously operated on in seven cases (35%) in group 1. In group 2 it was severe in 13 cases (16%) and the auricle had been previously operated on in 12 cases (15%). The ear canal was totally atretic in 7 cases (35%) in group 1 and 42 cases (53%) in group 2. The size of the middle ear was normal in 12 cases (60%) in group 1 and 52 cases (65%) in group 2. The size of the mastoid was normal in 16 cases (80%) in group 1 and 71 cases (89%) in group 2. The entire ossicular chain was fixed in 14 cases (70%), the lateral chain was fixed in 4 cases (20%), and the stapes was fixed in 2 cases (10%) in group 1. In group 2 the entire chain was fixed in 36 cases (45%), the lateral chain was fixed in 18 cases (23) , and the stapes was fixed in 25 cases (31%). The malleus was anomalous in 7 cases (35%) and fused as MIC in 12 cases (60%) in group 1. In group 2, it was anomalous in 28 cases (35%) and fused as MIC in 46 cases (58%). The incus was fused in 12 cases (60%) and in 1 case data regarding the incus were missing in group 1. In group 2, the incus was fused as MIC in 15 cases (29%), and in 28 cases data regarding the incus were missing. The anomaly of the stapes was severe in 14 cases (70%) in group 1 and 30 cases (38%) in group 2. The tympanic segment of the facial nerve (II) was normal in 9 cases (45%) in group 1 and 59 cases in
group 2, and it was covering half of the footplate in 50% of the cases in group 1 and 25% of the cases (n = 20) in group 2. The mastoid segment of the facial nerve (III) was severely aberrant in 6 cases (30%) and dehiscent in 11 cases (55%) in group 1. In group 2, it was severely aberrant in 8 cases (11%) and dehiscent in 35 cases (50%). In group 2, data regarding the mastoid segment of the facial nerve (III) were missing for 11 cases. The evaluation and comparison of the prognostic factors in groups 1 and 2 are presented in Table II. Ossicular reconstruction. The ossicular chain was reconstructed in all cases. Ingroup1,theossicularchainwasreconstructedusing 17 partial ossicular replacement prostheses (PORPs: 7 incus, 1 cortical, and 9 MIC). In one case, the stapes suprastucture was missing, and the chain was reconstructed with one total ossicular replacement prosthesis (TORP) made of cortical bone. In two cases, the stapes footplate was fixed, and two Fisch prostheses were used for a staged stapedotomy. In group 2, the ossicular chain was reconstructed using 72 PORPs (33 incus, 14 cortical, and 25 MIC). In three cases, the stapes suprastructure was missing, and the chain was reconstructed with two TORPs made of cortical bone and one malleus head. In five cases, the stapes footplate was fixed and five Fisch prostheses or wire pistons were used for a staged stapedotomy. Complications and revision surgery. In group 1, a lateralization of the tympanic membrane was found in four cases (20%), granulations of the ear canal were found in seven cases (35%), and a restenosis of the ear canal was found in three cases (15%). In group 2, a lateralization of the tympanic membrane was found in 8 cases (10%), granulations of the ear canal were found in 22 cases (28%), and a restenosis of the ear canal was found in 6 cases (20%). In group 1, four revisions of the ossicular chain and three major revisions requiring skin transplantation were performed. In group 2, nine revisions of the ossicular chain, one myringoplasty, and five major revisions requiring skin transplantation were performed. In three cases, the reformed ear canal was permanently closed due to severe restenosis. In a majority of cases restenosis of the ear canal was observed during the first year after surgery. Lateralization of the tympanic membrane and the development of granulations were generally revealed at a later stage of the follow-up. No facial dysfunction or deaf ears were observed postoperatively.
229
Atresiaplasty Table II. Prognostic factors in group 1 and 2. Factor
Group 1
Table II. (Continued). Group 2
Group 1
Group 2
Facial nerve III
Auricle Normal
1 (5%)
19 (24%)
Normal
6 (30%)
40 (55%)
Previous surgery
7 (35%)
12 (15%)
Deviation
8 (40%)
25 (34%)
6 (30%)
8 (11%)
Anomalous +
5 (25%)
36 (45%)
Deviation +++
Anomalous +++
7 (35%)
13 (16%)
Not evaluated
Normal
2 (3%)
Yes
11 (55%)
35 (50%)
9 (45%)
34 (49%)
Atretic
6 (30%)
42 (53%)
No
Very narrow
7 (35%)
11 (14%)
Not evaluated
Cone-shaped
7 (35%)
25 (31%)
Small
8 (40%)
28 (35%)
Large
12 (60%)
52 (65%)
16 (80%)
71 (89%)
Middle ear
Small
2 (10%)
6 (8%)
Missing
2 (10%)
1 (1%)
Not evaluated
2
Chain fixation Entire
14 (70%)
36 (45%)
Lateral
4 (20%)
18 (23%)
Stapes
2 (10%)
25 (31%)
Not evaluated
1
Malleus Normal Anomalous MIC
1 (5%)
6 (8%)
7 (35%)
28 (35%)
12 (60%)
46 (58%)
Incus Not evaluated
1
28
Small
1 (5%)
4 (11%)
Missing
1 (5%)
1 (3%)
5 (26%)
16 (43%)
Anomalous MIC
12 (63%)
Large
16 (43%)
Stapes Normal
3 (15%)
30 (38%)
Missing
1
Anomalous +
1 (2%)
45 (56%)
14 (70%)
5 (6%)
Anomalous +++ Not evaluated
11
Hearing results
Mastoid Normal
7
Facial dehiscence
Ear canal
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
Factor
1
The hearing results after surgery presented in this study were assessed at the latest clinical examination during follow-up. The mean AC improved from 66 dB preoperatively to 26 dB postoperatively in group 1 and from 61 dB preoperatively to 32 dB postoperatively in group 2. The mean BC improved from 13 dB preoperatively to 6 dB postoperatively in group 1 and from 11 dB preoperatively to 8 dB postoperatively in group 2. The mean pre- and postoperative AC and BC for both groups are illustrated in Figure 1, and the AC and BC values are presented in Table III. Additionally, the frequency of 4 kHz was evaluated separately. The mean AC for 4 kHz improved from 61 dB preoperatively to 31 dB postoperatively in group 1 and from 58 dB preoperatively to 40 dB postoperatively in group 2. The mean BC for 4 kHz in group 1 improved from 13 dB preoperatively to 8 dB postoperatively. In group 2, the mean BC declined from 12 dB preoperatively to 13 dB postoperatively. The mean pre- and postoperative ACs (4 kHz) and BCs (4 kHz) for groups 1 and 2 are presented in Table IV. There were no statistically significant differences between the groups. The mean ABG improved from 53 dB preoperatively to 20 dB postoperatively in group 1 and from 50 dB preoperatively to 20 dB postoperatively in group 2. The ABG closure within 0–20 dB was 60% in group 1 and 41% in group 2. The ABG closure within 0–30 dB was 90% in group 1 and 79% in group 2. Values for ABG closure for both groups are presented in Table V. There were no statistically significant differences between the groups.
Facial nerve II Normal
9 (45%)
59 (74%)
Lateral
1(10%)
1 (1%)
10 (50%)
20 (25%)
Half footplate
Statistics Descriptive statistics were presented as observed numbers (percentages) for categorical variables and
230
L. Edfeldt & K. Strömbäck Frequency (Hz)
A 250
500
1000
0
2000
4000
8000 0
Postop BC
10
10
Preop BC
20
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
30
20 30
Postop AC
40
40
50
50
60
60 Preop AC
70
70
80
80
90
90
100
100
B
Frequency (Hz) 250
500
1000
2000
4000
8000
0
0 Postop BC Preop BC
10 20 30
10 20
Postop AC
30
40
40
50
50
60
60
70
Preop AC
70
80
80
90
90
100
100
Figure 1. (A) Mean preoperative and postoperative air conduction (AC) and bone conduction (BC) hearing threshold levels for group 1 (score 4–6) in dB. (B) Mean preoperative and postoperative AC and BC hearing threshold levels for group 2 (score 7–10) in dB.
as mean (standard deviation, SD) for numerical variables. Comparisons of differences between subgroups were performed using unpaired t test for numerical variables and Fisher’s exact test for categorical variables. Significance level was defined as 5%, but as no adjustment for multiplicity has been performed the p values should be interpreted as exploratory rather than confirmatory. All statistical analyses were performed using R version 3.0.1.
Discussion To enable an appropriate comparison to similar studies, all patients were retrospectively graded using the Jahrsdoerfer grading scale. A majority of the cases scored 7–10, while 20% of the cases scored 4–6. A score of £ 5 should principally disqualify a patient from surgery [1]. Surprisingly, no differences in hearing outcome between these two groups were observed.
Atresiaplasty
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
Table III. Mean air conduction (AC), bone conduction (BC), air–bone gap (ABG) and p values for group 1 and 2.
Table V. Air–bone gap (ABG) closure 0–20 dB and 0–30 dB for group 1 and 2.
Parameter
Group 1 (4–6)
Group 2 (7–10)
p Value
Preop AC
66 dB
61 dB
0.159
ABG 0–20 dB
12 (60%)
31 (41%)
Preop BC
13 dB
11 dB
0.438
ABG 0–30 dB
18 (90%)
59 (79%)
Preop ABG
53 dB
50 dB
0.332
Postop AC
26 dB
32 dB
0.124
Postop BC
6 dB
8 dB
0.464
Postop ABG
20 dB
24 dB
0.169
In the majority of cases in both groups, the size of the middle ear and/or the size of the mastoid were evaluated as normal compared to non-malformed cases. This observation was in accordance with previous studies, which found that the size and aeration of the middle ear and mastoid were important predictors for a successful outcome [8–10]. In contrast, the malformation of the auricle, the fixation of the ossicular chain, the position of the facial nerve and, in particular, the anomaly of the stapes varied in both groups. Despite the smaller number of patients in the lower scoring group, which may have skewed the comparison, our results indicate that prediction of outcome is more correlated to the surgical accessibility of the middle ear than to the complete grade of the malformation determined using a scoring system. In a previous study [2], a successful hearing result after surgical reconstruction was defined as a postoperative ABG of 30 dB or less. Our results compare favorably with this study and may be explained by the consistent removal of the fused MIC, which enabled the enlargement of the middle ear and the tympanic membrane and facilitated the visualization of the incudo-stapedial joint and the stapes [8]. This was also a prerequisite for the regularly performed ossicular reconstruction. In contrast to the results of another study [11], we found that ossiculoplasty in patients with CAA provides stable long-term hearing. Another explanation for our results might be that all four surgeons were experienced. A correlation between hearing outcome and surgeon experience has been demonstrated previously [12]. Table IV. Preoperative and postoperative air conduction (AC) and bone conduction (BC) for group 1 and 2 at 4 kHz. Parameter
Group 1
Group 2
Preop AC
61 dB
58 dB
Postop AC
31 dB
40 dB
Preop BC
13 dB
12 dB
Postop BC
8 dB
13 dB
231
Group 1 (score 4–6)
Group 2 (score 7–10)
To investigate if extensive drilling influenced the BC hearing at higher frequencies, the 4 kHz frequency was evaluated separately. The mean preoperative BC hearing for 4 kHz improved by 5 dB in group 1 and declined by 1 dB in group 2. The drill-induced noise was reduced by the regular use of curettes. Similar to other surgical procedures involving ossicular reconstruction, the mean BC (0.5–3 kHz) hearing threshold levels improved in both groups [13]. In the present study, granulations of the ear canal and lateralization of the tympanic membrane followed by restenosis of the ear canal were the most commonly observed complications and indications for revision surgery in both groups. Granulations and lateralization were more common in the lower scoring group 1. Restenosis of the reformed ear canal is one of the most commonly reported complications in other studies [4]. Unexpectedly, this complication was more frequent in the higher scoring group 2. In group 2, in three cases with previous microtia repair, a recurrent growth of fibrotic tissue necessitated a permanent closure of the ear canal. Different surgical techniques have been described to prevent restenosis of the ear canal [14,15]. We performed an extensive meatoplasty in all cases. A disadvantage of extensive meatoplasty is that it makes it difficult to fit patients with conventional hearing aids, which might be required in cases with insufficient hearing restoration. It is generally recommended that microtia repair [16] should be performed before surgical reconstruction of the ear canal and middle ear. We used rib cartilage in accordance with the surgical technique described by Firmin [17]. Pinna reconstruction has the risk of interfering with the formation of an ear canal of sufficient width, which might also increase the risk for restenosis. Consequently, the higher incidence of restenosis in group 2 could be explained by the higher incidence of microtia repair in that group. In cases not suitable for surgical reconstruction, implantation of BAHA has been used successfully for hearing rehabilitation [3,4]. However, BAHA implantation has the risk of skin infection, which might lead to suppuration, loosening of the fixture, and extrusion. Bouhabel et al. [3] compared surgically treated patients with patients undergoing BAHA implantation. If the surgically treated group was additionally aided with conventional hearing aids (CHAs), the
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
232
L. Edfeldt & K. Strömbäck
audiological outcome was comparable with the BAHA-implanted group. Middle ear implantation, in which the floating mass transducer is coupled to the stapes or remaining stapes suprastructure, is increasingly used for hearing restoration in patients with CAA. The hearing outcome of middle ear implantation is well documented [5,18], and it can be similarly combined with microtia repair [19] or a prosthetic ear. Major weaknesses of middle ear implants are incompatibility with MRI [20] and the lack of long-term follow-up in patients with atretic ears. Implantable BC devices are generally MRIcompatible to 1.5 Tesla but cause artefacts on the MRI that interfere with radiological interpretation. Another weakness of hearing implants is their restricted function when the processor is not worn, for example at night or during various sporting activities. We conclude that atresiaplasty remains a justified treatment for CAA, which yields valuable, longstanding hearing restoration even in cases with severe malformations. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References [1] Jahrsdoerfer RA, Yeakley JW, Aguilar EA, Cole RR, Gray LC. Grading system for the selection of patients with congenital aural atresia. Am J Otol 1992;13:6–12. [2] De la Cruz A, Teufert KB. Congenital aural atresia surgery: long-term results. Otolaryngol Head Neck Surg 2003;129: 121–7. [3] Bouhabel S, Arcand P, Saliba I. Congenital aural atresia: bone-anchored hearing aid vs. external auditory canal reconstruction. Int J Pediatr Otorhinolaryngol 2012;76:272–7. [4] Nadaraja GS, Gurgel RK, Kim J, Chang KW. Hearing outcomes of atresia surgery versus osseointegrated bone conduction device in patients with congenital aural atresia: a systematic review. Otol Neurotol 2013;34:1394–9.
[5] Frenzel H, Hanke F, Beltrame M, Wollenberg B. Application of the vibrant soundbridge in bilateral congenital atresia in toddlers. Acta Otolaryngol 2010;130:966–70. [6] Colletti L, Carner M, Mandala M, Veronese S, Colletti V. The floating mass transducer for external auditory canal and middle ear malformations. Otol Neurotol 2011;32:108–15. [7] Monsell EM. New and revised reporting guidelines from the committee on hearing and equilibrium. American academy of otolaryngology-head and neck surgery foundation, Inc. Otolaryngol Head Neck Surg 1995;113:176–8. [8] . 3rd Shonka DC Jr, Livingston WJKesser BW. The jahrsdoerfer grading scale in surgery to repair congenital aural atresia. Arch Otolaryngol Head Neck Surg 2008;134:873–7. [9] Dedhia K, Yellon RF, Branstetter BF, Egloff AM. Anatomic variants on computed tomography in congenital aural atresia. Otolaryngol Head Neck Surg 2012;147:323–8. [10] Oliver ER, Lambert PR, Rumboldt Z, Lee FS, Agarwal A. Middle ear dimensions in congenital aural atresia and hearing outcomes after atresiaplasty. Otol Neurotol 2010;31:946–53. [11] Dobratz EJ, Rastogi A, Jahrsdoerfer RA, Kesser BW. To POP or not: ossiculoplasty in congenital aural atresia surgery. Laryngoscope 2008;118:1452–7. [12] Patel N, Shelton C. The surgical learning curve in aural atresia surgery. Laryngoscope 2007;117:67–73. [13] Lee HS, Hong SD, Hong SH, Cho YS, Chung WH. Ossicular chain reconstruction improves bone conduction threshold in chronic otitis media. J Laryngol Otol 2008;122:351–6. [14] Siegert R. Combined reconstruction of congenital auricular atresia and severe microtia. Adv Otorhinolaryngol 2010;68: 95–107. [15] Moon IJ, Cho YS, Park J, Chung WH, Hong SH, Chang SO. Long-term stent use can prevent postoperative canal stenosis in patients with congenital aural atresia. Otolaryngol Head Neck Surg 2012;146:614–20. [16] Nagata S. Modification of the stages in total reconstruction of the auricle: Part IV. Ear elevation for the constructed auricle. Plast Reconstr Surg 1994;93:254–66. [17] Firmin F. State-of-the-art autogenous ear reconstruction in cases of microtia. Adv Otorhinolaryngol 2010;68:25–52. [18] Claros P, Pujol Mdel C. Active middle ear implants: Vibroplasty in children and adolescents with acquired or congenital middle ear disorders. Acta Otolaryngol 2013;133:612–19. [19] Frenzel H, Schonweiler R, Hanke F, Steffen A, Wollenberg B. The lubeck flowchart for functional and aesthetic rehabilitation of aural atresia and microtia. Otol Neurotol 2012;33:1363–7. [20] Wagner JH, Ernst A, Todt I. Magnet resonance imaging safety of the vibrant soundbridge system: a review. Otol Neurotol 2011;32:1040–6.
ORIGINAL ARTICLE
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
Surgical treatment of congenital aural atresia – is it still justified?
LENNART EDFELDT & KARIN STRÖMBÄCK Department of Surgical Sciences, Section of Otorhinolaryngology and Head and Neck Surgery, Uppsala University, Uppsala, Sweden
Abstract Conclusion: Surgery candidacy based on the surgical accessibility of the middle ear seems more valuable than the use of a preoperative grading system. Also patients with severe malformations can benefit from surgical reconstruction. Objective: To evaluate the long-term results of the primary surgical treatment of patients with congenital auricular atresia (CAA). Methods: One hundred patients with CAA underwent surgical reconstruction between 1985 and 2010. The mean follow-up time was 40 months. All patients were retrospectively scored using the Jahrsdoerfer grading scale and divided into two groups according to the grade of their malformation. Group 1 included 20 patients with scores of 4–6 and group 2 included 80 patients with scores of 7–10. Pre- and postoperative air conduction (AC), bone conduction (BC), pure-tone average ‘air–bone gap’ (PTA4-ABG), surgical findings, postoperative complications, and revision surgeries performed were determined and compared between the two groups. Results: For 90% of the patients in group 1 and 79% of the patients in group 2, the postoperative ABG was within 0 and 30 dB. The most common complications were recurrent infection, lateralization of the tympanic membrane, and restenosis of the ear canal.
Keywords: atresiaplasty, grade of malformation, long-term results
Introduction Preoperative grading systems are based on information gained from preoperative CT scans of the temporal bone and are regularly used for atresia surgery candidacy and the prediction of expected hearing outcome. The Jahrsdoerfer grading scale [1] is the most commonly used classification system and has a best possible score of 10. The stapes is weighted most heavily, with two points compared with one point for the other parameters. A score of ‡ 6 allows for the consideration of surgical reconstruction, whereas patients with a score of < 6 are considered to have a poor chance of a successful surgical outcome [1]. The combined reconstruction of the external ear, ear canal, and middle ear is intricate and involves a comprehensive multidisciplinary approach. The timing of surgical repair in unilateral and bilateral congenital auricular atresia (CAA) differs greatly. While surgical and hearing restoration is controversial in unilateral
cases, hearing restoration in bilateral cases is essential. Although surgical repair of CAA is one of the most complex otosurgical procedures and is associated with an abundance of complications, it is possible to achieve serviceable and unaided hearing [2]. The introduction of implantable devices for hearing rehabilitation has renewed the management of CAA. As inner ear hearing usually is preserved, numerous authors have utilized bone anchored hearing devices (BAHAs) [3,4], especially in cases not suitable for surgical reconstruction. Active middle ear implants represent another alternative and have increasingly been used for hearing rehabilitation in patients with CAA [5,6]. In light of these novel optional treatment alternatives, the justification of functional surgery is uncertain, and an evaluation of patient outcome is warranted. Here, we assessed long-term surgical and hearing results, incidence of complications, and
Correspondence: Lennart Edfeldt, Department of Surgical Sciences, Section of Otorhinolaryngology and Head and Neck Surgery, Uppsala University, Uppsala, Sweden. E-mail: [email protected]
(Received 18 August 2014; accepted 6 October 2014) ISSN 0001-6489 print/ISSN 1651-2251 online 2015 Informa Healthcare DOI: 10.3109/00016489.2014.979437
Atresiaplasty number of revision surgeries after primary surgical treatment of 100 patients with CAA divided into two groups according to the grade of their malformations.
Material and methods
Table I. Patient demographics. Characteristic
Group 1
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
Group 2
Number
20
80
Mean age (years)
25.6
20
Male
12
43
Female
A total of 116 patients with CAA were treated with surgical reconstruction between 1985 and 2010. The inclusion criteria for surgical treatment were the audiometric confirmation of serviceable cochlear function and the ability to surgically access the middle ear defined by the position of the facial nerve and the size of the middle ear and mastoid determined on the preoperative CT scan. The size of the middle ear was evaluated on the preoperative CT scan. If the distance between the atretic plate and the oval window at the level of the long process of the incus in the coronal projection exceeded 6 mm, the middle ear was defined as normal. If the distance was less than 6 mm the middle ear was defined as small. Cases in which the facial nerve completely covered the oval window did not undergo surgical treatment. In bilateral cases, the surgical reconstruction of the ear canal and the middle ear was accomplished at the age of 5–6 years without prior cosmetic correction. In unilateral cases, cosmetic repair, when applicable, was performed at the age of 5–6 years, and reconstruction of the ear canal and middle ear was performed at the age of 12–15 years to allow the patients to understand the consequences of the split skin transplantation. Surgical and audiological data were pre- and postoperatively entered into our database (Microsoft Access). After ethical approval, the data were extracted and evaluated. Eleven patients had undergone previous surgery and were excluded from the study. Due to more severe malformations, which precluded conventional surgical reconstruction, five patients were treated with a fenestration of the lateral semicircular canal and were also excluded from the study. Retrospectively, all patients were scored using the Jahrsdoerfer grading scale according to the findings on the preoperative CT scan and were divided into two groups corresponding to the grade of their malformations. Group 1 included 20 patients with scores from 4 to 6. Three patients scored 4, 11 patients scored 5, and 6 patients scored 6. No patient scored less than 4. The mean age of the patients was 25.6 years (range 6–62, median 24); 8 patients were female and 12 were male. Group 2 included 80 patients with scores from 7 to 10. Eleven patients scored 7, 21 patients scored 8, 23 patients scored 9, and 25 patients scored 10. The
227
8
37
Follow-up (months)
41
39
Bilateral CAA
11
33
2
12
Previous surgery CAA: Congenital auricular atresia.
mean age of the patients was 20 years (range 5–69, median 18); 37 patients were female and 43 were male. Eleven patients in group 1 and 33 patients in group 2 had bilateral CAA. Two patients in group 1 and 12 patients in group 2 had undergone a previous cosmetic reconstruction of the auricle. Three patients in group 2 had Treacher-Collins syndrome. The follow-up time for both groups varied between 9 and 105 months, with a mean of 39 months. The mean follow-up time for group 1 was 41 months and for group 2 it was 39 months. Patient demographics are presented in Table I. Surgical technique After a retro-auricular incision, a double musculoperiosteal flap was created, and cortical bone chips were harvested. The middle ear was exposed through removal of the atretic bone with drills and curettes, starting from the linea temporalis and following the middle fossa tegmen. To facilitate the access to the middle ear, the fused malleus-incus complex (MIC) was removed in all cases, and the mobility of the stapes was verified. When possible, the frame of the tympanic membrane was widened. To prevent formation of granulations, the novel ear canal was formed with care taken to limit the exposure of the mastoid air cells. The ossicular chain was reconstructed using autologous material (incus, MIC, malleus head, or cortical bone). In cases with a fixed stapes footplate the malleus handle was left at the primary operation and a staged stapedotomy or stapedectomy was performed using a wire piston or Fisch prosthesis (Olympus, Solna, Sweden). For myringoplasty, a piece of temporal fascia was used. The opened mastoid air cells were obliterated with bony pate and the musculo-periosteal flap and the reformed ear canal were lined with two or three pieces of 0.2 mm thick split skin obtained from the inside of the upper part of the arm. To prevent restenosis and match the width of the reformed ear canal, a large meatoplasty was performed by removing cartilage and soft tissue.
228
L. Edfeldt & K. Strömbäck
A rosebud made of strips of rayon silk and small pieces of Tacryl (Lohmann & Rauscher, Neuwied, Germany ) shaped the temporal fascia and the thin split skin, particularly the anterior tympanomeatal angle. Finally a bag of rayon silk and Tacryl was placed in the ear canal and left for 10 days.
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
Audiometry Psycho-acoustic air conduction (AC) and bone conduction (BC) hearing threshold levels were determined using calibrated audiometers pre- and postoperatively. Pure-tone average (PTA) values for BC thresholds levels and for AC thresholds levels were assessed at 0.5, 1, 2, and 3 kHz. Additionally, the AC and BC for 4 kHz were evaluated separately. PTA air–bone gap (ABG) was assessed at 0.5–3 kHz. Audiometric data were reported according to the guidelines published by the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology-Head and Neck Surgery [7]. Results Surgical findings Prognostic factors. The auricular anomaly was severe in seven cases (35%) and the auricle had been previously operated on in seven cases (35%) in group 1. In group 2 it was severe in 13 cases (16%) and the auricle had been previously operated on in 12 cases (15%). The ear canal was totally atretic in 7 cases (35%) in group 1 and 42 cases (53%) in group 2. The size of the middle ear was normal in 12 cases (60%) in group 1 and 52 cases (65%) in group 2. The size of the mastoid was normal in 16 cases (80%) in group 1 and 71 cases (89%) in group 2. The entire ossicular chain was fixed in 14 cases (70%), the lateral chain was fixed in 4 cases (20%), and the stapes was fixed in 2 cases (10%) in group 1. In group 2 the entire chain was fixed in 36 cases (45%), the lateral chain was fixed in 18 cases (23) , and the stapes was fixed in 25 cases (31%). The malleus was anomalous in 7 cases (35%) and fused as MIC in 12 cases (60%) in group 1. In group 2, it was anomalous in 28 cases (35%) and fused as MIC in 46 cases (58%). The incus was fused in 12 cases (60%) and in 1 case data regarding the incus were missing in group 1. In group 2, the incus was fused as MIC in 15 cases (29%), and in 28 cases data regarding the incus were missing. The anomaly of the stapes was severe in 14 cases (70%) in group 1 and 30 cases (38%) in group 2. The tympanic segment of the facial nerve (II) was normal in 9 cases (45%) in group 1 and 59 cases in
group 2, and it was covering half of the footplate in 50% of the cases in group 1 and 25% of the cases (n = 20) in group 2. The mastoid segment of the facial nerve (III) was severely aberrant in 6 cases (30%) and dehiscent in 11 cases (55%) in group 1. In group 2, it was severely aberrant in 8 cases (11%) and dehiscent in 35 cases (50%). In group 2, data regarding the mastoid segment of the facial nerve (III) were missing for 11 cases. The evaluation and comparison of the prognostic factors in groups 1 and 2 are presented in Table II. Ossicular reconstruction. The ossicular chain was reconstructed in all cases. Ingroup1,theossicularchainwasreconstructedusing 17 partial ossicular replacement prostheses (PORPs: 7 incus, 1 cortical, and 9 MIC). In one case, the stapes suprastucture was missing, and the chain was reconstructed with one total ossicular replacement prosthesis (TORP) made of cortical bone. In two cases, the stapes footplate was fixed, and two Fisch prostheses were used for a staged stapedotomy. In group 2, the ossicular chain was reconstructed using 72 PORPs (33 incus, 14 cortical, and 25 MIC). In three cases, the stapes suprastructure was missing, and the chain was reconstructed with two TORPs made of cortical bone and one malleus head. In five cases, the stapes footplate was fixed and five Fisch prostheses or wire pistons were used for a staged stapedotomy. Complications and revision surgery. In group 1, a lateralization of the tympanic membrane was found in four cases (20%), granulations of the ear canal were found in seven cases (35%), and a restenosis of the ear canal was found in three cases (15%). In group 2, a lateralization of the tympanic membrane was found in 8 cases (10%), granulations of the ear canal were found in 22 cases (28%), and a restenosis of the ear canal was found in 6 cases (20%). In group 1, four revisions of the ossicular chain and three major revisions requiring skin transplantation were performed. In group 2, nine revisions of the ossicular chain, one myringoplasty, and five major revisions requiring skin transplantation were performed. In three cases, the reformed ear canal was permanently closed due to severe restenosis. In a majority of cases restenosis of the ear canal was observed during the first year after surgery. Lateralization of the tympanic membrane and the development of granulations were generally revealed at a later stage of the follow-up. No facial dysfunction or deaf ears were observed postoperatively.
229
Atresiaplasty Table II. Prognostic factors in group 1 and 2. Factor
Group 1
Table II. (Continued). Group 2
Group 1
Group 2
Facial nerve III
Auricle Normal
1 (5%)
19 (24%)
Normal
6 (30%)
40 (55%)
Previous surgery
7 (35%)
12 (15%)
Deviation
8 (40%)
25 (34%)
6 (30%)
8 (11%)
Anomalous +
5 (25%)
36 (45%)
Deviation +++
Anomalous +++
7 (35%)
13 (16%)
Not evaluated
Normal
2 (3%)
Yes
11 (55%)
35 (50%)
9 (45%)
34 (49%)
Atretic
6 (30%)
42 (53%)
No
Very narrow
7 (35%)
11 (14%)
Not evaluated
Cone-shaped
7 (35%)
25 (31%)
Small
8 (40%)
28 (35%)
Large
12 (60%)
52 (65%)
16 (80%)
71 (89%)
Middle ear
Small
2 (10%)
6 (8%)
Missing
2 (10%)
1 (1%)
Not evaluated
2
Chain fixation Entire
14 (70%)
36 (45%)
Lateral
4 (20%)
18 (23%)
Stapes
2 (10%)
25 (31%)
Not evaluated
1
Malleus Normal Anomalous MIC
1 (5%)
6 (8%)
7 (35%)
28 (35%)
12 (60%)
46 (58%)
Incus Not evaluated
1
28
Small
1 (5%)
4 (11%)
Missing
1 (5%)
1 (3%)
5 (26%)
16 (43%)
Anomalous MIC
12 (63%)
Large
16 (43%)
Stapes Normal
3 (15%)
30 (38%)
Missing
1
Anomalous +
1 (2%)
45 (56%)
14 (70%)
5 (6%)
Anomalous +++ Not evaluated
11
Hearing results
Mastoid Normal
7
Facial dehiscence
Ear canal
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
Factor
1
The hearing results after surgery presented in this study were assessed at the latest clinical examination during follow-up. The mean AC improved from 66 dB preoperatively to 26 dB postoperatively in group 1 and from 61 dB preoperatively to 32 dB postoperatively in group 2. The mean BC improved from 13 dB preoperatively to 6 dB postoperatively in group 1 and from 11 dB preoperatively to 8 dB postoperatively in group 2. The mean pre- and postoperative AC and BC for both groups are illustrated in Figure 1, and the AC and BC values are presented in Table III. Additionally, the frequency of 4 kHz was evaluated separately. The mean AC for 4 kHz improved from 61 dB preoperatively to 31 dB postoperatively in group 1 and from 58 dB preoperatively to 40 dB postoperatively in group 2. The mean BC for 4 kHz in group 1 improved from 13 dB preoperatively to 8 dB postoperatively. In group 2, the mean BC declined from 12 dB preoperatively to 13 dB postoperatively. The mean pre- and postoperative ACs (4 kHz) and BCs (4 kHz) for groups 1 and 2 are presented in Table IV. There were no statistically significant differences between the groups. The mean ABG improved from 53 dB preoperatively to 20 dB postoperatively in group 1 and from 50 dB preoperatively to 20 dB postoperatively in group 2. The ABG closure within 0–20 dB was 60% in group 1 and 41% in group 2. The ABG closure within 0–30 dB was 90% in group 1 and 79% in group 2. Values for ABG closure for both groups are presented in Table V. There were no statistically significant differences between the groups.
Facial nerve II Normal
9 (45%)
59 (74%)
Lateral
1(10%)
1 (1%)
10 (50%)
20 (25%)
Half footplate
Statistics Descriptive statistics were presented as observed numbers (percentages) for categorical variables and
230
L. Edfeldt & K. Strömbäck Frequency (Hz)
A 250
500
1000
0
2000
4000
8000 0
Postop BC
10
10
Preop BC
20
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
30
20 30
Postop AC
40
40
50
50
60
60 Preop AC
70
70
80
80
90
90
100
100
B
Frequency (Hz) 250
500
1000
2000
4000
8000
0
0 Postop BC Preop BC
10 20 30
10 20
Postop AC
30
40
40
50
50
60
60
70
Preop AC
70
80
80
90
90
100
100
Figure 1. (A) Mean preoperative and postoperative air conduction (AC) and bone conduction (BC) hearing threshold levels for group 1 (score 4–6) in dB. (B) Mean preoperative and postoperative AC and BC hearing threshold levels for group 2 (score 7–10) in dB.
as mean (standard deviation, SD) for numerical variables. Comparisons of differences between subgroups were performed using unpaired t test for numerical variables and Fisher’s exact test for categorical variables. Significance level was defined as 5%, but as no adjustment for multiplicity has been performed the p values should be interpreted as exploratory rather than confirmatory. All statistical analyses were performed using R version 3.0.1.
Discussion To enable an appropriate comparison to similar studies, all patients were retrospectively graded using the Jahrsdoerfer grading scale. A majority of the cases scored 7–10, while 20% of the cases scored 4–6. A score of £ 5 should principally disqualify a patient from surgery [1]. Surprisingly, no differences in hearing outcome between these two groups were observed.
Atresiaplasty
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
Table III. Mean air conduction (AC), bone conduction (BC), air–bone gap (ABG) and p values for group 1 and 2.
Table V. Air–bone gap (ABG) closure 0–20 dB and 0–30 dB for group 1 and 2.
Parameter
Group 1 (4–6)
Group 2 (7–10)
p Value
Preop AC
66 dB
61 dB
0.159
ABG 0–20 dB
12 (60%)
31 (41%)
Preop BC
13 dB
11 dB
0.438
ABG 0–30 dB
18 (90%)
59 (79%)
Preop ABG
53 dB
50 dB
0.332
Postop AC
26 dB
32 dB
0.124
Postop BC
6 dB
8 dB
0.464
Postop ABG
20 dB
24 dB
0.169
In the majority of cases in both groups, the size of the middle ear and/or the size of the mastoid were evaluated as normal compared to non-malformed cases. This observation was in accordance with previous studies, which found that the size and aeration of the middle ear and mastoid were important predictors for a successful outcome [8–10]. In contrast, the malformation of the auricle, the fixation of the ossicular chain, the position of the facial nerve and, in particular, the anomaly of the stapes varied in both groups. Despite the smaller number of patients in the lower scoring group, which may have skewed the comparison, our results indicate that prediction of outcome is more correlated to the surgical accessibility of the middle ear than to the complete grade of the malformation determined using a scoring system. In a previous study [2], a successful hearing result after surgical reconstruction was defined as a postoperative ABG of 30 dB or less. Our results compare favorably with this study and may be explained by the consistent removal of the fused MIC, which enabled the enlargement of the middle ear and the tympanic membrane and facilitated the visualization of the incudo-stapedial joint and the stapes [8]. This was also a prerequisite for the regularly performed ossicular reconstruction. In contrast to the results of another study [11], we found that ossiculoplasty in patients with CAA provides stable long-term hearing. Another explanation for our results might be that all four surgeons were experienced. A correlation between hearing outcome and surgeon experience has been demonstrated previously [12]. Table IV. Preoperative and postoperative air conduction (AC) and bone conduction (BC) for group 1 and 2 at 4 kHz. Parameter
Group 1
Group 2
Preop AC
61 dB
58 dB
Postop AC
31 dB
40 dB
Preop BC
13 dB
12 dB
Postop BC
8 dB
13 dB
231
Group 1 (score 4–6)
Group 2 (score 7–10)
To investigate if extensive drilling influenced the BC hearing at higher frequencies, the 4 kHz frequency was evaluated separately. The mean preoperative BC hearing for 4 kHz improved by 5 dB in group 1 and declined by 1 dB in group 2. The drill-induced noise was reduced by the regular use of curettes. Similar to other surgical procedures involving ossicular reconstruction, the mean BC (0.5–3 kHz) hearing threshold levels improved in both groups [13]. In the present study, granulations of the ear canal and lateralization of the tympanic membrane followed by restenosis of the ear canal were the most commonly observed complications and indications for revision surgery in both groups. Granulations and lateralization were more common in the lower scoring group 1. Restenosis of the reformed ear canal is one of the most commonly reported complications in other studies [4]. Unexpectedly, this complication was more frequent in the higher scoring group 2. In group 2, in three cases with previous microtia repair, a recurrent growth of fibrotic tissue necessitated a permanent closure of the ear canal. Different surgical techniques have been described to prevent restenosis of the ear canal [14,15]. We performed an extensive meatoplasty in all cases. A disadvantage of extensive meatoplasty is that it makes it difficult to fit patients with conventional hearing aids, which might be required in cases with insufficient hearing restoration. It is generally recommended that microtia repair [16] should be performed before surgical reconstruction of the ear canal and middle ear. We used rib cartilage in accordance with the surgical technique described by Firmin [17]. Pinna reconstruction has the risk of interfering with the formation of an ear canal of sufficient width, which might also increase the risk for restenosis. Consequently, the higher incidence of restenosis in group 2 could be explained by the higher incidence of microtia repair in that group. In cases not suitable for surgical reconstruction, implantation of BAHA has been used successfully for hearing rehabilitation [3,4]. However, BAHA implantation has the risk of skin infection, which might lead to suppuration, loosening of the fixture, and extrusion. Bouhabel et al. [3] compared surgically treated patients with patients undergoing BAHA implantation. If the surgically treated group was additionally aided with conventional hearing aids (CHAs), the
Acta Otolaryngol Downloaded from informahealthcare.com by Biblioteka Uniwersytetu Warszawskiego on 02/09/15 For personal use only.
232
L. Edfeldt & K. Strömbäck
audiological outcome was comparable with the BAHA-implanted group. Middle ear implantation, in which the floating mass transducer is coupled to the stapes or remaining stapes suprastructure, is increasingly used for hearing restoration in patients with CAA. The hearing outcome of middle ear implantation is well documented [5,18], and it can be similarly combined with microtia repair [19] or a prosthetic ear. Major weaknesses of middle ear implants are incompatibility with MRI [20] and the lack of long-term follow-up in patients with atretic ears. Implantable BC devices are generally MRIcompatible to 1.5 Tesla but cause artefacts on the MRI that interfere with radiological interpretation. Another weakness of hearing implants is their restricted function when the processor is not worn, for example at night or during various sporting activities. We conclude that atresiaplasty remains a justified treatment for CAA, which yields valuable, longstanding hearing restoration even in cases with severe malformations. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References [1] Jahrsdoerfer RA, Yeakley JW, Aguilar EA, Cole RR, Gray LC. Grading system for the selection of patients with congenital aural atresia. Am J Otol 1992;13:6–12. [2] De la Cruz A, Teufert KB. Congenital aural atresia surgery: long-term results. Otolaryngol Head Neck Surg 2003;129: 121–7. [3] Bouhabel S, Arcand P, Saliba I. Congenital aural atresia: bone-anchored hearing aid vs. external auditory canal reconstruction. Int J Pediatr Otorhinolaryngol 2012;76:272–7. [4] Nadaraja GS, Gurgel RK, Kim J, Chang KW. Hearing outcomes of atresia surgery versus osseointegrated bone conduction device in patients with congenital aural atresia: a systematic review. Otol Neurotol 2013;34:1394–9.
[5] Frenzel H, Hanke F, Beltrame M, Wollenberg B. Application of the vibrant soundbridge in bilateral congenital atresia in toddlers. Acta Otolaryngol 2010;130:966–70. [6] Colletti L, Carner M, Mandala M, Veronese S, Colletti V. The floating mass transducer for external auditory canal and middle ear malformations. Otol Neurotol 2011;32:108–15. [7] Monsell EM. New and revised reporting guidelines from the committee on hearing and equilibrium. American academy of otolaryngology-head and neck surgery foundation, Inc. Otolaryngol Head Neck Surg 1995;113:176–8. [8] . 3rd Shonka DC Jr, Livingston WJKesser BW. The jahrsdoerfer grading scale in surgery to repair congenital aural atresia. Arch Otolaryngol Head Neck Surg 2008;134:873–7. [9] Dedhia K, Yellon RF, Branstetter BF, Egloff AM. Anatomic variants on computed tomography in congenital aural atresia. Otolaryngol Head Neck Surg 2012;147:323–8. [10] Oliver ER, Lambert PR, Rumboldt Z, Lee FS, Agarwal A. Middle ear dimensions in congenital aural atresia and hearing outcomes after atresiaplasty. Otol Neurotol 2010;31:946–53. [11] Dobratz EJ, Rastogi A, Jahrsdoerfer RA, Kesser BW. To POP or not: ossiculoplasty in congenital aural atresia surgery. Laryngoscope 2008;118:1452–7. [12] Patel N, Shelton C. The surgical learning curve in aural atresia surgery. Laryngoscope 2007;117:67–73. [13] Lee HS, Hong SD, Hong SH, Cho YS, Chung WH. Ossicular chain reconstruction improves bone conduction threshold in chronic otitis media. J Laryngol Otol 2008;122:351–6. [14] Siegert R. Combined reconstruction of congenital auricular atresia and severe microtia. Adv Otorhinolaryngol 2010;68: 95–107. [15] Moon IJ, Cho YS, Park J, Chung WH, Hong SH, Chang SO. Long-term stent use can prevent postoperative canal stenosis in patients with congenital aural atresia. Otolaryngol Head Neck Surg 2012;146:614–20. [16] Nagata S. Modification of the stages in total reconstruction of the auricle: Part IV. Ear elevation for the constructed auricle. Plast Reconstr Surg 1994;93:254–66. [17] Firmin F. State-of-the-art autogenous ear reconstruction in cases of microtia. Adv Otorhinolaryngol 2010;68:25–52. [18] Claros P, Pujol Mdel C. Active middle ear implants: Vibroplasty in children and adolescents with acquired or congenital middle ear disorders. Acta Otolaryngol 2013;133:612–19. [19] Frenzel H, Schonweiler R, Hanke F, Steffen A, Wollenberg B. The lubeck flowchart for functional and aesthetic rehabilitation of aural atresia and microtia. Otol Neurotol 2012;33:1363–7. [20] Wagner JH, Ernst A, Todt I. Magnet resonance imaging safety of the vibrant soundbridge system: a review. Otol Neurotol 2011;32:1040–6.
