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Contribution of fetal MRI to the diagnosis of inner ear abnormalities: report of two cases

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Abstract

We report two cases of fetal inner ear abnormalities diagnosed by MRI. Cerebral MRI was performed on two fetuses, at 32 and 30 weeks gestation, following US that demonstrated multiple malformations suggestive of CHARGE syndrome in one fetus and ventriculomegaly and poor visibility of the posterior fossa in the other. MRI revealed vestibular hypoplasia and agenesis of the semicircular canals in one fetus and cystic cochleas, partial vermian agenesis and an occipital meningocele in the second fetus. Both pregnancies were terminated and there was good correlation between fetal MRI, ex utero CT and fetopathological findings. The inner ears should be carefully examined when performing fetal cerebral MRI because abnormalities of the inner ear may be associated with cerebral anomalies.

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References

  1. Isaacson G, Mintz MC (1986) Prenatal sonographic visualisation of the inner ear. J Ultrasound Med 5:409–410

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  3. Jackler RK, Luxford WM, House WF (1987) Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope 97 (3Pt 2 Suppl 40):2–14

    PubMed  CAS  Google Scholar 

  4. Sennaroglu L, Saatci I (2002) A new classification for cochleovestibular malformations. Laryngoscope 112:2230–2241

    Article  PubMed  Google Scholar 

  5. Hall BD (1979) Choanal atresia and associated multiple anomalies. J Pediatr 95:395–398

    Article  PubMed  CAS  Google Scholar 

  6. Pagon RA, Graham JM Jr, Zonana J et al (1981) Coloboma, congenital heart disease and choanal atresia with multiple anomalies: CHARGE association. J Pediatr 99:223–227

    Article  PubMed  CAS  Google Scholar 

  7. Tellier AL, Cormier-Daire V, Abadie V et al (1998) CHARGE syndrome: report of 47 cases and review. Am J Med Genet 76:402–409

    Article  PubMed  CAS  Google Scholar 

  8. Siebert JR, Graham JM Jr, MacDonald C (1985) Pathologic features of the Charge association: support for the involvement of the neural crest. Teratology 31:331–336

    Article  PubMed  CAS  Google Scholar 

  9. Amiel J, Attié-Bitach T, Marianowski R et al (2001) Temporal bone anomaly proposed as a major criteria for the diagnosis of CHARGE syndrome. Am J Med Genet 99:124–127

    Article  PubMed  CAS  Google Scholar 

  10. Guyot JP, Gacek RR, Di Raddo P (1987) The temporal bone anomaly in CHARGE association. Arch Otolaryngol Head Neck Surg 113:321–324

    PubMed  CAS  Google Scholar 

  11. Abadie V, Wiener-Vacher S, Morisseau-Durand MP et al (2000) Vestibular anomalies in CHARGE syndrome: investigations on and consequences for postural development. Eur J Pediatr 159:569–574

    Article  PubMed  CAS  Google Scholar 

  12. Smith OD, Neumann AM, Sirimanna KS et al (2001) Occipital meningocele and Mondini deformity of the cochlea. J Laryngol Otol 115:71–73

    Article  PubMed  CAS  Google Scholar 

  13. Becker R, Stiemer B, Neumann L et al (2001) Mild ventriculomegaly, mild cerebellar hypoplasia and dysplastic choroid plexus as early prenatal signs of CHARGE association. Fetal Diagn Ther 16:280–283

    Article  PubMed  CAS  Google Scholar 

  14. Hertzberg B, Kliewer MA, Lile RL (1994) Antenatal ultrasonographic findings in the CHARGE association. J Ultrasound Med 13:238–242

    PubMed  CAS  Google Scholar 

  15. Ruano R, Molho M, Roume J et al (2004) Prenatal diagnosis of fetal skeletal dysplasias by combining two-dimensional and three-dimensional ultrasound and intrauterine three-dimensional helical computer tomography. Ultrasound Obstet Gynecol 24:134–140

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Catherine Garel.

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Tilea, B., Garel, C., Menez, F. et al. Contribution of fetal MRI to the diagnosis of inner ear abnormalities: report of two cases. Pediatr Radiol 36, 149–154 (2006). https://doi.org/10.1007/s00247-005-0026-9

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  • DOI: https://doi.org/10.1007/s00247-005-0026-9

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