Skip to main content
Log in

Prenatal evaluation of the middle ear and diagnosis of middle ear hypoplasia using MRI

  • Original Article
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

Background

Analysis of the middle ear with fetal MRI has not been previously reported.

Objective

To show the contribution of fetal MRI to middle ear imaging.

Materials and methods

The tympanic cavity was evaluated in 108 fetal cerebral MRI examinations (facial and/or cerebral malformation excluded) and in two cases, one of Treacher Collins syndrome (case 1) and the other of oculo-auriculo-vertebral (OUV) spectrum (case 2) with middle ear hypoplasia identified by MRI at 27 and 36 weeks’ gestation, respectively.

Results

In all 108 fetuses (mean gestational age 32.5 weeks), the tympanic cavity and T2 hypointensity related to the ossicles were well visualised on both sides. Case 1 had micro/retrognathia and bilateral external ear deformity and case 2 had retrognathism with a left low-set and deformed ear. MRI made it possible to recognize the marked hypoplasia of the tympanic cavity, which was bilateral in case 1 and unilateral in case 2. Both syndromes are characterized by craniofacial abnormalities including middle ear hypoplasia, which cannot be diagnosed with US.

Conclusion

The middle ear cavity can be visualized with fetal MRI. We emphasize the use of this imaging modality in the diagnosis of middle ear hypoplasia.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. Tilea B, Garel C, Menez F et al (2006) Contribution of fetal MRI to the diagnosis of inner ear abnormalities: report of two cases. Pediatr Radiol 36:149–154

    Article  PubMed  Google Scholar 

  2. Nemzek WR, Brodie HA, Chong BW et al (1996) Imaging findings of the developing temporal bone in fetal specimens. AJNR 17:1467–1477

    PubMed  CAS  Google Scholar 

  3. Fryer A (2003) Syndromes associated with hereditary deafness. In: King SJ, Boothroyd AG (eds) Pediatric ENT radiology. Springer, Berlin, pp 35–54

    Google Scholar 

  4. Edwards SJ, Gladwin AJ, Dixon MJ (1997) The mutational spectrum in Treacher Collins syndrome reveals a predominance of mutations that create a premature-termination codon. Am J Hum Genet 60:515–524

    PubMed  CAS  Google Scholar 

  5. Marszalek B, Wojcicki P, Kobus K et al (2002) Clinical features, treatment and genetic background of Treacher Collins syndrome. J Appl Genet 43:223–233

    PubMed  Google Scholar 

  6. Burglen L, Soupre V, Diner PA et al (2001) Oto-mandibular dysplasias: genetics and nomenclature of syndromes. Ann Chir Plast Esthet 46:400–409

    Article  PubMed  CAS  Google Scholar 

  7. Dixon J, Trainor P, Dixon MJ (2007) Treacher Collins syndrome. Orthod Craniofac Res 10:88–95

    Article  PubMed  Google Scholar 

  8. Cohen J, Ghezzi F, Goncalves L et al (1995) Prenatal sonographic diagnosis of Treacher Collins syndrome: a case and review of the literature. Am J Perinatol 12:416–419

    Article  PubMed  CAS  Google Scholar 

  9. Crane JP, Beaver HA (1986) Midtrimester sonographic diagnosis of mandibulofacial dysostosis. Am J Med Genet 25:251–255

    Article  PubMed  CAS  Google Scholar 

  10. Meizner I, Carmi R, Katz M (1991) Prenatal ultrasonic diagnosis of mandibulofacial dysostosis (Treacher Collins syndrome). J Clin Ultrasound 19:124–127

    Article  PubMed  CAS  Google Scholar 

  11. Milligan DA, Harlass FE, Duff P et al (1994) Recurrence of Treacher Collins’ syndrome with sonographic findings. Mil Med 159:250–252

    PubMed  CAS  Google Scholar 

  12. Ochi H, Matsubara K, Ito M et al (1998) Prenatal sonographic diagnosis of Treacher Collins syndrome. Obstet Gynecol 91:862

    Article  PubMed  CAS  Google Scholar 

  13. Shih JC, Shyu MK, Lee CN et al (1998) Antenatal depiction of the fetal ear with three-dimensional ultrasonography. Obstet Gynecol 91:500–505

    Article  PubMed  CAS  Google Scholar 

  14. Tanaka Y, Kanenishi K, Tanaka H et al (2002) Antenatal three-dimensional sonographic features of Treacher Collins syndrome. Ultrasound Obstet Gynecol 19:414–415

    Article  PubMed  CAS  Google Scholar 

  15. Vento AR, LaBrie RA, Mulliken JB (1991) The O.M.E.N.S. classification of hemifacial microsomia. Cleft Palate Craniofac J 28:68–76, discussion 77

    Article  PubMed  CAS  Google Scholar 

  16. Castori M, Brancati F, Rinaldi R et al (2006) Antenatal presentation of the oculo-auriculo-vertebral spectrum (OAVS). Am J Med Genet A 140:1573–1579

    PubMed  Google Scholar 

  17. De Catte L, Laubach M, Legein J et al (1996) Early prenatal diagnosis of oculoauriculovertebral dysplasia or the Goldenhar syndrome. Ultrasound Obstet Gynecol 8:422–424

    Article  PubMed  Google Scholar 

  18. Bisdas S, Lenarz M, Lenarz T et al (2005) Inner ear abnormalities in patients with Goldenhar syndrome. Otol Neurotol 26:398–404

    Article  PubMed  Google Scholar 

  19. Martinelli P, Maruotti GM, Agangi A et al (2004) Prenatal diagnosis of hemifacial microsomia and ipsilateral cerebellar hypoplasia in a fetus with oculoauriculovertebral spectrum. Ultrasound Obstet Gynecol 24:199–201

    Article  PubMed  CAS  Google Scholar 

  20. Volpe P, Gentile M (2004) Three-dimensional diagnosis of Goldenhar syndrome. Ultrasound Obstet Gynecol 24:798–800

    Article  PubMed  CAS  Google Scholar 

  21. Hattori Y, Tanaka M, Matsumoto T et al (2005) Prenatal diagnosis of hemifacial microsomia by magnetic resonance imaging. J Perinat Med 33:69–71

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Catherine Garel.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Katorza, E., Nahama-Allouche, C., Castaigne, V. et al. Prenatal evaluation of the middle ear and diagnosis of middle ear hypoplasia using MRI. Pediatr Radiol 41, 652–657 (2011). https://doi.org/10.1007/s00247-010-1913-2

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-010-1913-2

Keywords

Navigation