Clin Exp Otorhinolaryngol.  2019 May;12(2):176-180. 10.21053/ceo.2018.00857.

Analysis of Inner Ear Anomalies in Unilateral Congenital Aural Atresia Combined With Microtia

Affiliations
  • 1Department of Otolaryngology-Head and Neck Surgery, Korea University College of Medicine, Seoul, Korea. logopas@korea.ac.kr

Abstract


OBJECTIVES
The aim of this study was to analyze the incidence of inner ear anomalies in patients with unilateral congenital aural atresia (CAA) combined with microtia.
METHODS
We retrospectively reviewed 61 patients with unilateral CAA combined with microtia who underwent high-resolution temporal bone computed tomography (TBCT) and hearing examination. Inner ear anomalies were analyzed using TBCT and evaluated according to the Jahrsdoerfer grading system, Marx classification, and extent of inferior displacement of the mastoid tegmen.
RESULTS
Inner ear anomalies were observed in 14 patients (23.0%). Lateral semicircular canal (LSCC) dysplasia was the most common inner ear anomaly, with an incidence of 16.4%. The incidence was significantly higher on the pathologic side than on the unaffected side (P=0.002). All vascular anomalies were observed in the high-riding jugular bulb, with an incidence of 24.6%. The incidence was significantly higher on the pathologic side than on the unaffected side (P<0.001). LSCC dysplasia was significantly more common in patients with a lower Jahrsdoerfer score (odds ratio, 0.66; P=0.004).
CONCLUSION
The incidence of inner ear anomalies was relatively high in patients with unilateral CAA combined with microtia; LSCC dysplasia was the most common anomaly and the probability of coexistence was higher in patients with a lower Jahrsdoerfer score.

Keyword

Inner Ear; Anomalies; Aural Atresia, Congenital; Congenital Microtia; Semicircular Canals

MeSH Terms

Classification
Congenital Microtia*
Ear, Inner*
Hearing
Humans
Incidence
Mastoid
Retrospective Studies
Semicircular Canals
Temporal Bone

Figure

  • Fig. 1. The extent of inferior displacement of the mastoid tegmen was calculated as the ratio of middle ear height to mastoid tegmen displacement (yellow arrows, middle ear height; red arrow, mastoid tegmen displacement).

  • Fig. 2. High-resolution temporal bone computed tomography images of unilateral congenital aural atresia combined with microtia. Axial (A) and coronal (B) views showing the dysmorphic features of the lateral semicircular canal (arrows).

  • Fig. 3. High-resolution temporal bone computed tomography images of unilateral congenital aural atresia combined with microtia. Axial (A) and coronal (B) views showing the dilated vestibular morphology (arrows).

  • Fig. 4. High-resolution temporal bone computed tomography images of unilateral congenital aural atresia combined with microtia. Axial (A) and coronal (B) views showing the medio-inferiorly displaced tympanic segment of the facial nerve (arrows).

  • Fig. 5. High-resolution temporal bone computed tomography images of unilateral congenital aural atresia combined with microtia. Axial (A) and coronal (B) views showing high-riding jugular bulb (arrows).


Cited by  1 articles

Increased Risk of Psychopathological Abnormalities in Subjects With Unilateral Hearing Loss: A Cross-Sectional Study
Jae-Jin Song, Eu Jeong Ku, Seoyoung Kim, Euitae Kim, Young-Seok Choi, Hahn Jin Jung
Clin Exp Otorhinolaryngol. 2021;14(1):82-87.    doi: 10.21053/ceo.2020.00283.


Reference

1. Schuknecht HF. Congenital aural atresia. Laryngoscope. 1989; Sep. 99(9):908–17.
Article
2. Genc S, Kahraman E, Ozel HE, Arslan IB, Demir A, Selcuk A. Microtia and congenital aural atresia. J Craniofac Surg. 2012; Nov. 23(6):1733–5.
Article
3. Vrabec JT, Lin JW. Inner ear anomalies in congenital aural atresia. Otol Neurotol. 2010; Dec. 31(9):1421–6.
Article
4. Mayer TE, Brueckmann H, Siegert R, Witt A, Weerda H. High-resolution CT of the temporal bone in dysplasia of the auricle and external auditory canal. AJNR Am J Neuroradiol. 1997; Jan. 18(1):53–65.
5. Yuen HY, Ahuja AT, Wong KT, Yue V, van Hasselt AC. Computed tomography of common congenital lesions of the temporal bone. Clin Radiol. 2003; Sep. 58(9):687–93.
Article
6. Naunton RF, Valvassori GE. Inner ear anomalies: their association with atresia. Laryngoscope. 1968; Jun. 78(6):1041–9.
Article
7. Phelps PD. Congenital lesions of the inner ear, demonstrated by tomography. Arch Otolaryngol. 1974; Jul. 100(1):11–8.
8. Halle TR, Soares BP, Todd NW. Inner ear anomalies in children with isolated unilateral congenital aural atresia. Int J Pediatr Otorhinolaryngol. 2017; Apr. 95:5–8.
Article
9. Balaker AE, Roberson JB Jr, Goldsztein H. Fibrous incudostapedial joint in congenital aural atresia. Otolaryngol Head Neck Surg. 2014; Apr. 150(4):673–6.
Article
10. Yellon RF, Branstetter BF 4th. Prospective blinded study of computed tomography in congenital aural atresia. Int J Pediatr Otorhinolaryngol. 2010; Nov. 74(11):1286–91.
Article
11. 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; Jan. 13(1):6–12.
Article
12. Dedhia K, Yellon RF, Branstetter BF, Egloff AM. Anatomic variants on computed tomography in congenital aural atresia. Otolaryngol Head Neck Surg. 2012; Aug. 147(2):323–8.
Article
13. Kim DW, Lee JH, Song JJ, Chang H, Choi YS, Jang JH, et al. Continuity of the incudostapedial joint: a novel prognostic factor in postoperative hearing outcomes in congenital aural atresia. Acta Otolaryngol. 2011; Jul. 131(7):701–7.
Article
14. Ju YH, Park E, Park S, Jung K, Lee K, Im GJ. The extent of inferior displacement of the mastoid tegmen is related to the severity of congenital aural atresia. Acta Otolaryngol. 2014; Mar. 134(3):244–9.
Article
15. Sennaroglu L, Saatci I. A new classification for cochleovestibular malformations. Laryngoscope. 2002; Dec. 112(12):2230–41.
Article
16. Brook CD, Buch K, Kaufmann M, Sakai O, Devaiah AK. The prevalence of high-riding jugular bulb in patients with suspected endolymphatic hydrops. J Neurol Surg B Skull Base. 2015; Dec. 76(6):471–4.
17. Frenzel H, Sprinzl G, Widmann G, Petersen D, Wollenberg B, Mohr C. Grading system for the selection of patients with congenital aural atresia for active middle ear implants. Neuroradiology. 2013; Jul. 55(7):895–911.
Article
18. Yu Z, Han D, Gong S, Wang Z, Dai H, Zhao S, et al. Facial nerve course in congenital aural atresia: identified by preoperative CT scanning and surgical findings. Acta Otolaryngol. 2008; 128(12):1375–80.
19. Takegoshi H, Kaga K, Kikuchi S, Ito K. Facial canal anatomy in patients with microtia: evaluation of the temporal bones with thin-section CT. Radiology. 2002; Dec. 225(3):852–8.
Article
Full Text Links
  • CEO
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr