Repair Of Congenital Aural Atresia Research Articles

Surgical repair of congenital aural atresia

Despite the ease, efficacy, predictability, and widespread availability of osseointegrated bone conduction hearing devices, surgical repair of congenital aural atresia (CAA) remains a viable and rewarding operation for patients, parents, and surgeons. Careful selection of patients using appropriate audiometry, detailed radiographic interpretation, and assessment of family understanding and child cooperability are the first steps toward a successful outcome. Intimate knowledge of outer, middle, and inner ear anatomy – both in the normally and abnormally developed ear - coupled with meticulous technique at each stage of the operation can yield outstanding hearing results with a clean, skin-lined, dry, patent ear canal. This chapter reviews the preoperative evaluation and steps of the atresia repair operation, including postoperative management, and provides both predictive and published data on hearing outcomes.

Audiometric Outcomes After Surgical Repair of Congenital Aural Atresia: Does Age Matter?

To determine if there is an age-based difference in audiometric outcomes for patients undergoing primary congenital aural atresia (CAA) repair. Retrospective chart review. Single academic, high-volume, tertiary care hospital. Individuals undergoing primary CAA repair by a single surgeon between 2004 and 2020. CAA repair. Preoperative and postoperative four tone (500, 1,000, 2,000, 4,000 Hz) air-conduction pure-tone average (PTA), bone-conduction PTA, air-bone gap and speech reception threshold, and preoperative to postoperative change in values. We identified 247 patients (262 ears) who underwent repair. The mean and median ages were approximately 12 and 8.5 years, respectively, both of which served as cutoff ages to compare younger versus older patients. The average preoperative to postoperative improvement values in air-conduction PTA, air-bone gap, and speech reception threshold for individuals younger than 12 years were 26.6 ± 10.2, 23.8 ± 12.6, and 30.1 ± 12.1 dB hearing level (HL), respectively, and those for individuals 12 years or older were 25.9 ± 15.7, 26.2 ± 10.3, and 31.3 ± 12.8 dB HL, respectively. For individuals younger than 8.5 years, the values were 25.8 ± 9.5, 24.9 ± 9.4, and 30.0 ± 10.6 dB HL, respectively, and those for individuals 8.5 years or older were 27.1 ± 13.5, 25.7 ± 11.0, and 30.0 ± 14.6 dB HL, respectively. The improvement did not differ significantly between the younger and older groups, using both cutoff ages. There was no difference in revision surgery rates or complications between groups. An individual at any age can enjoy audiometric improvement from atresia repair.

When to Say When-Reasons to Abort Surgery for Congenital Aural Atresia.

To determine reasons for premature termination in surgery for congenital aural atresia (CAA). Retrospective chart review. High-volume tertiary care center. Patients undergoing primary surgical repair of CAA whose surgeries were aborted for various indications. Patients with CAA who underwent elective aural atresia surgery at the University of Virginia from July 2004 to January 2020 were identified from the medical record and an institutional database of patients with CAA. Patients undergoing revision surgery or surgery not for hearing correction were excluded. All included candidates were noted to be good surgical candidates for atresia repair based on preoperative Jahrsdoerfer score of 6 or greater. Reasons for terminating surgery prematurely were categorized by anatomical constraints (inability to identify middle ear space, position of the facial nerve, or other anatomic abnormality), stapes fixation, and middle ear and/or mastoid inflammatory disease. Reasons for premature termination in CAA surgery. Over the interval, 319 patients underwent primary surgery for CAA. Surgery was terminated prematurely without completing the primary objective in 14 patients (4.4%); surgery was terminated in 8 (57%) due to anatomic constraints, 4 (29%) due to stapes fixation, and 2 (14%) due to middle ear space and/or mastoid inflammatory disease. Premature termination in surgery for CAA is a rare occurrence with careful study of the preoperative high-resolution computed tomography of the temporal bone. Of the reasons for premature termination, anatomic constraints were the most common. Knowing limitations in surgery-both technical and anatomic-is critical to optimize outcomes and prevent complications.

Long-term Audiometric Outcomes After Atresiaplasty for Congenital Aural Atresia.

Describe long-term trends and stability of hearing outcomes for patients undergoing primary congenital aural atresia (CAA) repair. Retrospective chart review. Single academic, tertiary referral center. Children and adults who underwent primary CAA repair between 1980 and 2017. CAA repair. Long-term (> 1 yr) postoperative three-tone (500, 1000, 2000 Hz) air conduction pure-tone average (AC PTA) compared with the immediate (within 90 d of surgery) postoperative audiogram. The mean preoperative AC PTA was 59.6 dB HL. CAA repair improved hearing an average of 30.5 dB, but hearing declined by 8.2 dB over the long-term follow-up period (mean 4.4 yr; range 1-15.7 yr), leaving a final mean improvement of 22.2 dB (final mean AC PTA 37.3 dB HL). Two-thirds (92 of 138 ears) had an AC PTA ≤ 30 dB HL recorded in the first year after surgery. At the last follow-up test, 64% had "stable" hearing defined as no more than a 10 dB decline in AC PTA compared with the immediate postoperative audiogram; 21% had a 10 to 20 dB decline, 8% a 20 to 30 dB decline, and 7% declined > 30 dB. All patients enjoyed improvement in AC PTA after surgery (preoperative minus the best postoperative PTA, mean = 34 dB, range = 3.3-52 dB). Hearing declined by an average 8.2 dB over the long-term leaving a final average AC PTA of 37.4 dB HL. Sixty-four percent of patients exhibited stable (< 10 dB loss) hearing over time; 36% lost 10 dB or more over the long-term follow-up period. Hearing results typically stabilize over time with most of the change occurring in the first 3 years after CAA repair. Bone conduction thresholds remained stable over the study period.

Relationship Between Middle Ear Volume and Long-term Audiological Outcomes in Congenital Aural Atresia Repair.

To assess the association of middle ear volume with long-term hearing outcomes in congenital aural atresia (CAA) repair. Retrospective chart and radiological review. Single academic tertiary referral center. Children and adults who underwent CAA repair between 1995 and 2016. Patients were divided into "best" and "worst" audiometric groups, based on stability of postoperative air conduction pure-tone average (AC PTA) results. Ten patients were included for study in the "best" group, and 12 in the "worst" group. CAA repair. Long-term (> 1 yr) postoperative three-tone (500, 1000, 2000 Hz) AC PTA, speech reception threshold (SRT), air bone gap, and semiautomated calculated middle ear volume from preoperative computed tomography (CT) scans. Statistically significant differences were noted between "best" and "worst" groups in AC PTA, SRT, and air bone gap (p < 0.001). Mean middle ear volume in the "best" group was 434.6 mm (range 326.3-602.1 mm) and 339.5 mm (range 199.4-502.1 mm) in the "worst" group (p = 0.02). The majority in both groups were right ears (p = 0.38), and males outnumbered females in the "best" group (9 out of 10; p = 0.018). Preoperative Jahrsdoerfer grading scores were similar between groups (p = 0.31). Mean follow-up for the "best" and "worst" groups was approximately 3.5 and 4.5 yr, respectively. For patients undergoing CAA repair, larger middle ear volume is associated with stable and better long-term audiometric outcomes.

Surgical and Audiometric Outcomes for Repair of Congenital Aural Atresia and Hypoplasia.

Surgical repair of congenital aural atresia and hypoplasia (CAAH) is technically challenging. Long-term surgical and audiologic outcomes of atresiaplasty are incompletely understood. To review the surgical outcomes for CAAH and analyze the hearing results. A retrospective medical record review of CAAH outcomes was performed during an 11-year period from January 1, 2004, through December 31, 2014. The data analysis was undertaken from December 1, 2014, through January 31, 2015. The mean clinic follow-up time was 3.9 years, and the mean audiologic follow-time was 2.8 years. The study included 98 patients aged 5 to 66 years (mean age, 16.6 years) with CAAH who underwent a total of 104 operations. Surgical repair of CAAH. Preoperative and postoperative pure-tone averages (PTAs), speech reception thresholds (SRTs), air-bone gaps (ABGs), and interaural PTA and SRT differences were compared. Factors that affect hearing outcomes were analyzed. The complication rates were reviewed and compared with results from similar studies. In the 98 patients with CAAH, the mean improvement in ABGs and SRTs was 26.7 and 25.9 dB, respectively, resulting in a postoperative ABG of 30 dB or less in 4 of 5 cases. The mean postoperative PTAs and SRTs were 36.9 and 34.3 dB, respectively. Patients with a functional native ossicular chain (36 of 104 [34.6%]) had significantly superior audiometric outcomes when compared with patients in whom a reconstruction prosthesis was required during primary or revision operations. Audiometric results from hypoplasia surgery were not significantly different from those of atresia surgery; results in patients with craniofacial syndromes were similarly not significantly different from those in patients with sporadic CAAH. We report a low incidence of meatal stenosis. The mean hearing outcomes for this group compared favorably with other series. The need for ossicular chain reconstruction was associated with poorer audiometric outcomes. The safety profile and the demonstrated hearing improvement of CAAH surgery suggest that it remains a favorable option for patients.

Fibrous Incudostapedial Joint in Congenital Aural Atresia

(1) Determine the prevalence of a non-bony or fibrous incudostapedial (IS) joint in the setting of congenital aural atresia. (2) Assess this anomaly's impact on surgical management and associated hearing outcomes. Retrospective chart review. Subspecialty private practice. Operative reports and audiometric data of patients who underwent congenital aural atresia repair by a single surgeon from 2007 to 2011 were reviewed for operative anatomic findings and audiometric outcomes. Two hundred twenty-eight operations on 206 ears were performed. Median age was 5 years old. Fifty-five (26.7%) of these ears had a fibrous IS joint. The severity of this anomaly was graded as mild in 23 ears, moderate in 20 ears, and severe in 12 ears. Mean postoperative pure tone air conduction (PTA2) in the severely fibrous group was 51 compared to 46 in the moderate group (P = .03) and 41 in the mild group (P = .006). Patients with a fibrous IS joint who underwent successful ossicular chain reconstruction (OCR) had a mean postoperative PTA2 of 30, which was a significantly better outcome than in patients with moderately or severely fibrous IS joints who did not have OCR (P < .05). A fibrous IS joint was seen in 27% of patients undergoing repair of congenital aural atresia. The severity of this anomaly has important implications for postoperative hearing results. These findings suggest that ossicular chain reconstruction should be performed in moderately or severely fibrous cases.

Fibrous Incudostapedial Joint in Congenital Aural Atresia

Objectives: 1) Determine the prevalence of a non-bony or fibrous incudostapedial (IS) joint in the setting of congenital aural atresia. 2) Assess this anomaly’s impact on surgical management and associated hearing outcomes. Methods: Operative reports and audiometric data of patients who underwent congenital aural atresia repair by a single surgeon from 2007-2011 were reviewed for operative anatomic findings and audiometric outcomes. Results: Two hundred and twenty-eight operations on 206 ears were performed. Median age was 5 years old. Fifty-five (26.7%) of these ears had a fibrous IS joint. The severity of this anomaly was graded as mild in 23 ears, moderate in 20 ears, and severe in 12 ears. Average postoperative PTA2 in the severely fibrous group was 51 compared to 46 in the moderate group ( P = 0.03) and 41 in the mild group ( P = 0.006). Patients with a fibrous IS joint who underwent successful ossicular chain reconstruction (OCR) had an average postoperative PTA2 of 30, which was a significantly better outcome than in patients with moderately or severely fibrous IS joints who did not have OCR ( P &lt; 0.05). Conclusions: A fibrous IS joint was seen in 27% of patients undergoing repair of congenital aural atresia. The severity of this anomaly has important implications for postoperative hearing results. These findings suggest that ossicular chain reconstruction should be performed in moderately or severely fibrous cases.

A Case of Image-Guided Surgery in Congenital Aural Atresia

Received April 10, 2013 Revised May 23, 2013 Accepted May 28, 2013 Address for correspondence Myung-Koo Kang, MD, PhD Department of OtolaryngologyHead and Neck Surgery, College of Medicine, Dong-A University, 26 Daesingongwon-ro, Seo-gu, Busan 602-715, Korea Tel +82-51-240-5428 Fax +82-51-253-0712 E-mail mgkang@dau.ac.kr The repair of congenital aural atresia remains one of the most challenging otologic procedures because of the scarcity of surgical landmarks, the complexity and the variety of the temporal bone anatomy, and the limited space for reconstruction. The risks of facial nerve injury and profound sensorineural hearing loss following atresia surgery are common concerns. Furthermore, the rarity of the disease makes it difficult to improve surgical learning. Image-guided surgery may aid otologic surgeons in repairing atresia as it allows identifying the exact position of surgical instruments in relation to the specific anatomy. To our knowledge, there have been no published reports on image-guided atresia surgery in Korea. Herein, we report a case of congenital aural atresia, which was repaired using image-guided surgical technique. Korean J Otorhinolaryngol-Head Neck Surg 2013;56:659-63 Key WordsZZCongenital aural atresia ᆞImage-guided surgery ᆞNavigation.

Repair of congenital aural atresia

While challenging, surgery to repair congenital aural atresia can be successful in the carefully selected patient. This article describes the preoperative evaluation, surgical technique, and postoperative care that have evolved to produce the most favorable results for patients undergoing surgical repair of aural atresia. Results and complications of surgery and their management are also reviewed.

The course of the facial nerve in evaluation of Congenital aural atresia repair candidacy

The course of the facial nerve in evaluation of Congenital aural atresia repair candidacy

Malleus Bar

Malleus bar is a rare cause of conductive hearing loss and has yet to be described in the setting of congenital aural atresia. This study aimed to define the anomaly and review the surgical management and outcomes of patients found to have a malleus bar in the setting of congenital aural atresia. Retrospective case review of patients with malleus bar and congenital aural atresia. Tertiary otologic referral center. Patients found to have a malleus bar at the time of surgery for congenital aural atresia. Charts of patients who underwent surgery for congenital aural atresia repair with a malleus bar were reviewed for demographic data, preoperative Jahrsdoerfer score, surgical findings, and audiometric data. Seven subjects (8 ears) were identified to have a malleus bar and congenital aural atresia. In all 8 ears, the malleus bar proved to be a second point of attachment of the ossicular chain in addition to the atretic plate. The chorda tympani nerve was found in the bony bar in 4 patients. Closure of the air-bone gap within 35 dB hearing level (HL) was achieved in 83% of patients. All patients showed improved speech reception thresholds and stable bone-conduction thresholds. All but 1 patient had stable or improved word recognition scores. Malleus bar in the setting of congenital aural atresia represents a rare combination of causes of congenital conductive hearing loss. Patients with these 2 abnormalities obtain good short-term results with surgical repair of congenital aural atresia.

Diagnosis and Management of Salivary Fistula after Surgery for Congenital Aural Atresia

Salivary fistula is an uncommon and unreported yet meaningful complication associated with the repair of congenital aural atresia. The capsule of the parotid gland may be violated during two steps of the operation: the initial dissection around the glenoid fossa or while aligning the auricle with the bony canal at the end of the procedure. We present the first described series of patients with salivary fistula after repair of congenital aural atresia. Retrospective case review from 1985 to 2004. Tertiary referral center. We included all patients who were diagnosed with a salivary fistula after congenital aural atresia repair. The diagnosis of a salivary fistula or salivary tissue in the external auditory canal after atresia repair was based on one of the following criteria: 1) identification of a fistula tract or salivary tissue in the external auditory canal, 2) otorrhea positive for amylase, or 3) intermittent otorrhea associated with eating. Of 1,500 patients operated on for aural atresia, we identified 6 with salivary fistula after atresia repair. Salivary fistulas were diagnosed from 15 days to 10 years postoperatively, and the duration ranged from 6 months to 14 years. Treatment included observation, medical management, and surgical intervention. Salivary fistulas in the external auditory canal may present with granulation tissue, persistent crusting, or persistent otorrhea; it is therefore necessary to consider salivary fistula when managing these findings in postoperative congenital aural atresia patients. Salivary fistula secondary to repair of congenital aural atresia may be managed conservatively or surgically.

Computer-aided virtual surgery for congenital aural atresia.

Computer-enhanced three-dimensional (3D) computed tomography (CT) provides accurate spatial representation of the complex surgical anatomy of congenitally atretic ears, and is superior to conventional CT for surgical planning. The surgical repair of congenital aural atresia is challenging. Conventional CT, routinely used for surgical planning, is limited in its ability to represent spatial relationships between important structures. Because of the lack of density differences between bony structures in the ear, 3D CT has thus far been useful for representing surface contour but not internal anatomy. A two-level segmentation scheme was developed to distinguish structures in the temporal bone. 3D CT reconstructions of congenital ears were produced with a high-resolution helical scanner. An interactive tool was used to mark the ossicles and facial nerve. The segmentation scheme was used to color-enhance the ossicles and otic capsule, and render the surrounding bone translucent. "Virtual surgery" was then performed by subtracting a cylindrical volume of bone lateral to the atresia plate. The enhanced 3D CT reconstructions were correlated with intraoperative video recordings. In four congenital ears, computer-enhanced 3D CT was highly predictive of the actual anatomy. Surgery was avoided in two anatomically unfavorable cases. Computer-enhanced 3D CT is a major advance over conventional CT for demonstrating the complex spatial relationships in congenitally atretic ears.

Surgical Aspects of Congenital Aural Atresia

Surgical repair of congenital aural atresia is a formidable task. The preoperative evaluation, embryology, surgical approaches, postoperative results, complications of surgery, and alternative methods of auditory rehabilitation of these patients are discussed in this article.