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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 28  |  Issue : 1  |  Page : 98-102

Congenital atresia of external auditory canal with choristoma: A rare case report


1 Department of Surgery (Plastic Surgeon), Chettinad Hospital and Research Institute, Kelambakkam, Chennai, Tamil Nadu, India
2 Department of Otorhinolaryngology, Chettinad Hospital and Research Institute, Kelambakkam, Chennai, Tamil Nadu, India

Date of Submission29-Dec-2021
Date of Acceptance06-Jan-2022
Date of Web Publication25-Apr-2022

Correspondence Address:
Prof. Priya Kanagamuthu
Department of Otorhinolaryngology, Chettinad Hospital and Research Institute, Kelambakkam, Chennai, Tami Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.indianjotol_180_21

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  Abstract 


Congenital aural atresia (CAA) is the abnormality of the auricle and/or external auditory canal where there is closure of the external auditory canal which may be associated with microtia and anomalies in the middle ear, inner ear, or temporal bone. It can be solid or membranous type of atresia. To identify the type of defect, high-resolution computed tomography (HRCT) temporal bone is useful. After identifying the defect, the patient can be planned for canaloplasty or atresiaplasty with split skin graft. A 6-year-old boy was brought to the otorhinolaryngology outpatient department with complaints of deformity of the left ear since birth. HRCT temporal bone showed isolated left external auditory canal atresia. Echocardiogram showed dextrocardia with situs solitus and USG abdomen revealed situs inversus of the abdominal organs with no significant abnormality. After proper evaluation of the child, after obtaining preanesthetic fitness, the child had undergone atresiaplasty with split skin graft under general anesthesia. Soft tissue that was excised from the external auditory canal was sent for histopathological examination, which revealed the presence of salivary gland tissue with lymph nodes showing reactive changes. These features were suggestive of possibility of choristoma of the left external auditory canal. CAA occurs in the defect during embryological development which may result in deformity of the auricle and external auditory canal. The surgical management of congenital external auditory canal atresia is atresiaplasty, and hearing rehabilitation (bone anchored hearing aid) should be given in case of no improvement in hearing. The surgery will be challenging because of altered anatomy of surgical landmarks. Choristoma is a tumor-like growth of normal tissue in an abnormal place. It is a benign condition, which may be found commonly in the head-and-neck region such as presence of salivary gland tissue in the middle ear, presence of gastric mucosa in the tongue and presence of osseous or cartilaginous masses in the intraoral soft tissues. Total excision can be done for small and pedunculated tumors, and care should be taken not to injure facial nerve. CAA may not necessarily present with deformities in the external ear (pinna), but Congenital aural atresia cases may not always present with deformities of the pinna,but it is crucial not to miss such cases. These patients may be evaluated by doing High Resolution Computed Tomography temporal bone and audiometric assessment such as. Pediatric evaluation is equally important to rule out for any syndromic association. Atresiaplasty or canaloplasty has shown great result for correcting the cosmetic defect. Hearing can be facilitated further using bone anchored hearing aids or cochlear implantation.

Keywords: Atresia, facial palsy, prosthesis, salivary gland, skin graft


How to cite this article:
Kumar T D, Kanagamuthu P, Ramesh G, Srinivasan R. Congenital atresia of external auditory canal with choristoma: A rare case report. Indian J Otol 2022;28:98-102

How to cite this URL:
Kumar T D, Kanagamuthu P, Ramesh G, Srinivasan R. Congenital atresia of external auditory canal with choristoma: A rare case report. Indian J Otol [serial online] 2022 [cited 2022 Nov 29];28:98-102. Available from: https://www.indianjotol.org/text.asp?2022/28/1/98/343758




  Introduction Top


Congenital aural atresia (CAA) is the abnormality of the auricle and/or external auditory canal where there is closure of the external auditory canal which may be associated with microtia and anomalies in the middle ear, inner ear, or temporal bone. It can be solid or membranous type of atresia. The incidence of CAA is 1–1.5 in 10,000–15,000.[1] Usually, CAA is said to present as unilateral and has a male predominance. Several reports have showed right ear involvement. CAA may be seen in associated with certain syndromes such as Goldenhar syndrome,  Treacher Collins syndrome More Details, and Crouzon syndrome.[2] CAA may also be associated with dysplastic temporomandibular joint.

The etiology is due to the embryological defect in the intrauterine period which could be due to higher maternal parity and age, maternal diabetes mellitus, maternal acute illness (rubella), and teratogens, alcohol intake, or lack of folic acid. Children with CAA will have hearing deficit of the ear involved, usually conductive hearing loss.[3] Speech may be affected in few cases. Behavioral changes have been reported as well such as lack of concentration, agitation, and poor academic performance. Proper evaluation is needed to plan for the right choice of treatment. Audiological and speech assessment must be done. To identify the type of defect, HRCT temporal bone is useful. After identifying the defect, the patient can be planned for canaloplasty or atresiaplasty with split skin graft.


  Case Report Top


A 6-year-old boy was brought to the otorhinolaryngology outpatient department with complaints of deformity of the left ear since birth [Figure 1]. It was associated with hard of hearing of the left ear. There was no history of trauma to the ear. The child had no other symptoms such as otalgia, otorrhea, and facial palsy. The child did not have family history of deafness. Antenatal, perinatal, and postnatal periods of the mother were uneventful. All developmental milestones had been attained up to the child's age.
Figure 1: Congenital aural atresia

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Local examination of the left ear revealed normal preauricular and postauricular region with deepening of the scaphoid fossa and atresia of the external auditory canal. In pure-tone audiometry (PTA), the right ear showed 15 dBHL with a normal hearing sensitivity, while the left ear showed more than 90 dBHL, indicating profound hearing loss. In otoacoustic emission (OAE), the left ear showed no valid OAE. Auditory brainstem response evaluation was completed with air conducted clicks presented to each ear at 11.1 per second. V-wave peaks could be obtained only at 90 dBnHL in the left ear and V-wave peaks were present up to 30 dBnHL in the right ear, thus indicating profound hearing loss. HRCT temporal bone showed isolated left external auditory canal atresia [Figure 2],[Figure 3],[Figure 4]. Echocardiogram showed dextrocardia with situs solitus and USG abdomen revealed situs inversus of the abdominal organs with no significant abnormality.
Figure 2: Computed tomography images showing left external auditory canal atresia

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Figure 3: Computed tomography image showing left external auditory canal atresia

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Figure 4: Computed tomography image showing left external auditory canal atresia

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Management and outcome

After proper evaluation of the child, after obtaining preanesthetic fitness, the child had undergone atresiaplasty with split skin graft under general anesthesia. The skin graft was taken from the left thigh of the child and placed covering the raw area of the left external auditory canal with the support of silicone gel sheet and Ivalon (polyvinyl alcohol) sponge [Figure 5] and [Figure 6]. In view of infection, Ivalon sponge was removed on the 4th postoperative day and replaced by acrylic prosthesis which was designed with the help of the mold [Figure 7] for the support of the graft. Acrylic prosthesis [Figure 8] in the shape of conventional hearing aid was designed specially and placed in the left ear of the child [Figure 9]. This innovative technique gives good support to the graft, so that the graft does not shrink off and fall. After 24 h of surgery, the child developed facial nerve weakness. Incomplete closure of the left eyelid and absence of wrinkling in the left side of forehead were noted. There was no deviation of angle of mouth. The child was treated initially with intravenous steroid followed by oral steroid medication for 10 days. On the 10th postoperative day, the child recovered from facial nerve weakness and left eye closure was improved. Soft tissue that was excised from the external auditory canal was sent for histopathological examination, which revealed presence of salivary gland tissue with lymph nodes showing reactive changes [Figure 10]. These features were suggestive of possibility of choristoma of the left external auditory canal.
Figure 5: Graft placement in external auditory canal

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Figure 6: Graft in external auditory canal

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Figure 7: Mold for acrylic prosthesis

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Figure 8: Acrylic prosthesis

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Figure 9: Acrylic prosthesis placement

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Figure 10: Histopathological image of salivary gland choristoma (A – Serous gland, B – Mucinous gland, C – Duct, D – Adipose tissue)

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  Discussion Top


CAA occurs in the defect during embryological development which may result in deformity of the auricle and external auditory canal. CAA can be identified with clinical examination, and through proper radiological evaluation, the type of defect can be identified. There are grading systems for CAA such as Weerda and Jahrsdoerfer, which will help in accessing the type of defect and also prognosis of surgical treatment if done.

The surgical management of congenital external auditory canal atresia is atresiaplasty and hearing rehabilitation (bone-anchored hearing aid) should be given in case of no improvement in hearing. The surgery will be challenging because of altered anatomy of surgical landmarks. Proper and detailed preoperative counseling should be given to parents. Some additional measures such as special attention and education should be made available to the affected child to improve the academic performance. At times, the patients may opt for reconstruction surgery for cosmetic reasons even though there is normal hearing and speech.[1],[4] In this case, atresiaplasty with split skin grafting was done and acrylic prosthesis was placed. Excess soft tissue was removed from the external auditory canal, and histopathological examination revealed choristoma.
Table 1: Weerda (based on clinical appearance)

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Table 2: Jahrsdoerfer (based on radiological appearance)

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Choristoma is a tumor-like growth of normal tissue in an abnormal place. It is a benign condition, which may be found commonly in the head-and-neck region such as presence of salivary gland tissue in the middle ear, presence of gastric mucosa in the tongue, and presence of osseous or cartilaginous masses in the intraoral soft tissues.[5] In many cases, the etiology of choristoma is unknown. However, any abnormality occurring in the differentiation of the first and second branchial clefts during fetal period may lead to formation of choristoma.[6] Salivary gland choristoma of the ear is more commonly seen in females and more prevalent in the left ear which is consistent with this case report. It may also be associated with congenital profound hearing loss. Other associated abnormalities seen were hemifacial atrophy, microtia, preauricular skin tags, temporal alopecia, and situs inversus totalis.[7] In this report, the child had congenital profound hearing loss and situs inversus totalis. Only a few cases of choristoma were reported in external auditory canal. Choristoma is most commonly observed in the age group of 3–52 years which is consistent with our report. Histopathological examination helps in the diagnosis of choristoma. Differential diagnosis includes schwannoma, glomus tumor, rhabdomyosarcoma, teratoma, dermoid cyst, and lymphomas.[8] Choristoma is a normal tissue, hence total removal is not required when histologically diagnosed. Even when the mass is not removed completely, recurrence is uncommon. Total excision can be done for small and pedunculated tumors, and care should be taken not to injure facial nerve.


  Conclusion Top


CAA may not necessarily present with deformities in the external ear (pinna), Congenital aural atresia cases may not always present with deformities of the pinna, but it is crucial not to miss such cases. These patients may be evaluated by doing High Resolution Computed Tomography temporal bone and audiometric assessment such as such as pure-tone audiometry, otoacoustic emission, and Brainstem evoked response audiometry. Pediatric evaluation is equally important to rule out for any syndromic association. Atresiaplasty or canaloplasty has shown great result for correcting the cosmetic defect. Hearing can be facilitated further using bone anchored hearing aids or cochlear implantation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Gautam R, Kumar J, Pradhan GS, Passey JC, Meher R, Mehndiratta A. High-resolution computed tomography evaluation of congenital aural atresia – How useful is this? J Laryngol Otol 2020;134:610-22.  Back to cited text no. 1
    
2.
Ruhl DS, Kesser BW. Atresiaplasty in congenital aural atresia: What the facial plastic surgeon needs to know. Facial Plast Surg Clin North Am 2018;26:87-96.  Back to cited text no. 2
    
3.
Pellinen J, Vasama JP, Kivekäs I. Long-term results of atresiaplasty in patients with congenital aural atresia. Acta Otolaryngol 2018;138:621-4.  Back to cited text no. 3
    
4.
Gao R, Wang Y, Fan Y, Ai X, Zhang X, Xue H, et al. The role of HRCT and three-dimensional VR CT findings in patients of congenital atresia combined with microtia. Int J Pediatr Otorhinolaryngol 2012;76:1779-84.  Back to cited text no. 4
    
5.
Yamahara K, Katsura Y, Egawa Y, Lee K, Ikegami S. Two cases of cartilaginous choristoma-not chondroma of the bony external auditory canal. Case Rep Otolaryngol 2018;2018:6346453.  Back to cited text no. 5
    
6.
Yamanobe Y, Oishi N, Nishiyama T, Hosoya M, Ogawa K. Inner ear salivary gland choristoma extending to the middle ear with congenital profound hearing loss and facial palsy: A case report. J Otolaryngol Head Neck Surg 2021;50:25.  Back to cited text no. 6
    
7.
Chen S, Li Y. Salivary gland choristoma of the middle ear. Ear Nose Throat J 2015;94:E9-12.  Back to cited text no. 7
    
8.
Toros SZ, Egeli E, Kiliçarslan Y, Gümrükçü G, Gökçeer T, Noşeri H. Salivary gland choristoma of the middle ear in a child with situs inversus totalis. Auris Nasus Larynx 2010;37:365-8.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]
 
 
    Tables

  [Table 1], [Table 2]



 

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