Skip to main content

Advertisement

Log in

Appearance of hypoplastic cochleae in CT and MRI: a new subclassification

  • Head and Neck Radiology
  • Published:
Neuroradiology Aims and scope Submit manuscript

Abstract

Introduction

Hypoplastic cochleae are among the inner ear malformations more frequently encountered by radiologists; little detailed information is available on these, however. We present the first large series of hypoplastic cochleae and document imaging findings to better characterize this anomaly radiologically.

Methods

We used our electronic database to search for inner ear malformations described between 1995 and 2009 and extracted 81 ears (of 47 patients) with hypoplastic cochleae out of 289 patients with inner ear malformations. Two neuroradiologists evaluated the available CT and MRI data. Measurements of all inner ear structures were performed. Accompanying findings were listed.

Results

Cochlear hypoplasia (58 ears, 32 patients) often involves not only the apical turn being reduced in size but also the basal turn being smaller in length. Additionally, 11 ears (eight patients) of hypoplastic cochleae with only a basal turn and five ears (four patients) of cochleae with only a small bud were identified. Non-classifiable hypoplastic cochleae (seven ears, five patients) were those with either a rudimentary or an absent basal turn or a “dwarf appearance” with no further partition.

Conclusions

The term “hypoplastic cochlea” is very general; a further division into severe and less severe forms based on the length and existence of cochlea turns is possible and can help enhance the comparison of CI outcome data. Measurements can help the less experienced radiologist to detect them more easily.

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
Fig. 6

Similar content being viewed by others

References

  1. Lo WW (1999) What is a ‘Mondini’ and what difference does a name make? AJNR Am J Neuroradiol 20:1442–1444

    CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  3. Mondini C (1997) Minor works of Carlo Mondini: the anatomical section of a boy born deaf. Am J Otol 18:288–293

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  5. Purcell D, Johnson J, Fischbein N et al (2003) Establishment of normative cochlear and vestibular measurements to aid in the diagnosis of inner ear malformations. Otolaryngol Head Neck Surg 128:78–87

    Article  PubMed  Google Scholar 

  6. Krombach GA, van den Boom M, Di Martino E et al (2005) Computed tomography of the inner ear: size of anatomical structures in the normal temporal bone and in the temporal bone of patients with Meniere’s disease. Eur Radiol 15:1505–1513

    Article  PubMed  Google Scholar 

  7. Romo LVC, JW RCD (2003) Temporal bone: congenital anomalies. In: Som P, Curtin H (eds) Head and neck imaging. Mosby, St Louis, pp 1109–1171

    Google Scholar 

  8. Schuknecht HF (1993) Pathology of the ear. Lea & Febiger, Malvern

    Google Scholar 

  9. Casselman JW, Offeciers FE, Govaerts PJ et al (1997) Aplasia and hypoplasia of the vestibulocochlear nerve: diagnosis with MR imaging. Radiology 202:773–781

    CAS  PubMed  Google Scholar 

  10. Shim HJ, Shin JE, Chung JW et al (2006) Inner ear anomalies in cochlear implantees: importance of radiologic measurements in the classification. Otol Neurotol 27:831–837

    Article  PubMed  Google Scholar 

  11. Streeter GL (1948) Developmental horizons in human embryos. Description of age groups XV, XVI, XVII, and XVIII, being the third issue of a survey of the Carnegie Collection. Contrib Embryol 32:133–203

    Google Scholar 

  12. Papsin BC (2005) Cochlear implantation in children with anomalous cochleovestibular anatomy. Laryngoscope 115:1–26

    Article  PubMed  Google Scholar 

  13. Park AH, Kou B, Hotaling A et al (2000) Clinical course of pediatric congenital inner ear malformations. Laryngoscope 110:1715–1719

    Article  CAS  PubMed  Google Scholar 

  14. Ma H, Han P, Liang B et al (2008) Multislice spiral computed tomography imaging in congenital inner ear malformations. J Comput Assist Tomogr 32:146–150

    Article  PubMed  Google Scholar 

  15. Koch BL (2007) Labyrinthine aplasia. In: Barkovitch AJ (ed) Diagnostic imaging pediatric neuroradiology. Amirsys, Salt Lake City, pp II1-2–II1-3

    Google Scholar 

  16. Hudgins PA (2004) Labyrinthine aplasia. In: Harnsberger HR (ed) Diagnostic imaging head and neck. Amirsys, Salt Lake City, pp I2-96–I2-97

    Google Scholar 

  17. Rodriguez K, Shah RK, Kenna M (2007) Anomalies of the middle and inner ear. Otolaryngol Clin North Am 40:81–96, vi

    Article  PubMed  Google Scholar 

  18. Robson CD, Robertson RL, Barnes PD (1999) Imaging of pediatric temporal bone abnormalities. Neuroimaging Clin N Am 9:133–155

    CAS  PubMed  Google Scholar 

  19. Zheng Y, Schachern PA, Cureoglu S et al (2002) The shortened cochlea: its classification and histopathologic features. Int J Pediatr Otorhinolaryngol 63:29–39

    Article  PubMed  Google Scholar 

  20. Romo LV, Curtin HD (2001) Anomalous facial nerve canal with cochlear malformations. AJNR Am J Neuroradiol 22:838–844

    CAS  PubMed  Google Scholar 

  21. Davidson HC, Harnsberger HR, Lemmerling MM et al (1999) MR evaluation of vestibulocochlear anomalies associated with large endolymphatic duct and sac. AJNR Am J Neuroradiol 20:1435–1441

    CAS  PubMed  Google Scholar 

  22. Jackler RK, De La Cruz A (1989) The large vestibular aqueduct syndrome. Laryngoscope 99:1238–1242, discussion 1242–1233

    Article  CAS  PubMed  Google Scholar 

  23. Valvassori GE (1983) The large vestibular aqueduct and associated anomalies of the inner ear. Otolaryngol Clin North Am 16:95–101

    CAS  PubMed  Google Scholar 

  24. Koesling S, Rasinski C, Amaya B (2006) Imaging and clinical findings in large endolymphatic duct and sac syndrome. Eur J Radiol 57:54–62

    Article  PubMed  Google Scholar 

  25. Phelps PD (1990) Mondini and ‘pseudo Mondini’. Clin Otolaryngol Allied Sci 15:99–101

    Article  CAS  PubMed  Google Scholar 

  26. Cock E (1838) A contribution to the pathology of congenital deafness. Guys Hosp Rep 7:289–307

    Google Scholar 

Download references

Conflict of interest statement

We declare that we have no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Anja Maria Giesemann.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Giesemann, A.M., Goetz, F., Neuburger, J. et al. Appearance of hypoplastic cochleae in CT and MRI: a new subclassification. Neuroradiology 53, 49–61 (2011). https://doi.org/10.1007/s00234-010-0777-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00234-010-0777-3

Keywords

Navigation