First branchial cleft cyst accompanied by external auditory canal atresia and middle ear malformation: A case report

Abstract

Background: We report a rare case of first branchial cleft anomaly (FBCA) accompanied by bony atresia of the external auditory canal, middle ear malformation, and location malformation of the facial nerve according to the intraoperative findings.

Case summary: A 19-year-old male patient presented to our department with a mass behind the right earlobe and recurrent postauricular swelling and pain since childhood, he also had severe hearing loss in the right ear since birth. The patient underwent surgery including mass removal, mastoidectomy, and simultaneous meatoplasty and ossiculoplasty under microscopy. No facial palsy or recurrence was noted during postoperative follow-up.

Conclusion: FBCAs are rare, and to our knowledge, this is the first report of FBCA accompanied by external auditory canal bony atresia, middle ear malformation, and location malformation of the facial nerve. An effective postauricular approach under microscopy facilitated complete lesion removal and simultaneous otologic reconstruction.

Keywords: Case report; External auditory canal atresia; First branchial cleft anomaly; Middle ear malformation.

Figure 1

Temporal bone computed tomography findings. A: The first branchial cleft cyst; B: The white arrow indicates the lesion tightly adhered to the facial nerve; C: The orange arrow indicates that the incus is fused with the lateral bony plank.

Figure 2

Intraoperative findings and surgical strategy. A: The lesion; B: The white arrow indicates the fusion of the malleus head and incus body, the black arrow indicates that the malleus handle was fused to the lateral bony plank, the yellow arrow shows the origin of the lesion; C: The white arrow indicates the hearing reconstruction with a partial ossicular replacement prosthesis and tragus cartilage graft, the black arrow shows the location variation of the facial nerve, which migrated to the anteposition. a: The lesion; b: The atretic bony plank outside the closed tympanic cavity. In: Incus; MH: Malleus head; PG: Parotid gland; FN (t): Tympanic segment of the facial nerve; FN (v): Vertical segment of the facial nerve; LSC: Lateral semicircular canal; St: Stapes; ET: Eustachian tube.

Figure 3

Comparison of preoperative and postoperative pure tone audiometry in the right ear. A: Shows the preoperative pure tone audiometry, the preoperative air-bone conduction gap was 56 decibels; B: Shows postoperative pure tone audiometry, the postoperative air-bone gap was 21 decibels, and the pure-tone average gain was 35 decibels.