Mondini Deformity Differential Diagnoses

1. Mondini C. Anatomica surdi nati sectio. De Bononiensi Scientiarum et Artium Instituto atque Academia Commentarii. 1791. 7:28–9:419–31.

2. Lo WW. What is a 'Mondini' and what difference does a name make?. AJNR Am J Neuroradiol. 1999 Sep. 20(8):1442-4. [Medline].

3. Hartley, G. The Minor Works of Carlo Mondini (Carlo Mondini). The American Journal of Otology. 1996. 18:288–293.

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

5. Sennaroglu L, Saatci I. Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol. 2004 Jul. 25(4):520-9; discussion 529. [Medline].

6. Sennaroglu L, Sarac S, Ergin T. Surgical results of cochlear implantation in malformed cochlea. Otol Neurotol. 2006 Aug. 27(5):615-23. [Medline].

7. Everett LA, Glaser B, Beck JC, Idol JR, Buchs A, Heyman M. Pendred syndrome is caused by mutations in a putative sulphate transporter gene (PDS). Nat Genet. 1997 Dec. 17(4):411-22. [Medline].

8. Molecular Otolaryngology Research Labs. Pendred/BOR Homepage. Available at http://www.healthcare.uiowa.edu/labs/pendredandbor. Accessed: Oct 12, 2011.

9. Azaiez H, Yang T, Prasad S, Sorensen JL, Nishimura CJ, Kimberling WJ. Genotype-phenotype correlations for SLC26A4-related deafness. Hum Genet. 2007 Dec. 122(5):451-7. [Medline].

10. Wu CC, Yeh TH, Chen PJ, Hsu CJ. Prevalent SLC26A4 mutations in patients with enlarged vestibular aqueduct and/or Mondini dysplasia: a unique spectrum of mutations in Taiwan, including a frequent founder mutation. Laryngoscope. 2005 Jun. 115(6):1060-4. [Medline].

11. Campbell C, Cucci RA, Prasad S, Green GE, Edeal JB, Galer CE. Pendred syndrome, DFNB4, and PDS/SLC26A4 identification of eight novel mutations and possible genotype-phenotype correlations. Hum Mutat. 2001 May. 17(5):403-11. [Medline].

12. Wang QJ, Zhao YL, Rao SQ, Guo YF, Yuan H, Zong L. A distinct spectrum of SLC26A4 mutations in patients with enlarged vestibular aqueduct in China. Clin Genet. 2007 Sep. 72(3):245-54. [Medline].

13. Fitoz S, Sennaroglu L, Incesulu A, Cengiz FB, Koc Y, Tekin M. SLC26A4 mutations are associated with a specific inner ear malformation. Int J Pediatr Otorhinolaryngol. 2007 Mar. 71(3):479-86. [Medline].

14. Wu CC, Chen PJ, Hsu CJ. Specificity of SLC26A4 mutations in the pathogenesis of inner ear malformations. Audiol Neurootol. 2005 Jul-Aug. 10(4):234-42. [Medline].

15. Incesulu A, Adapinar B, Kecik C. Cochlear implantation in cases with incomplete partition type III (X-linked anomaly). Eur Arch Otorhinolaryngol. 2008 Nov. 265(11):1425-30. [Medline].

16. Mylanus EA, Rotteveel LJ, Leeuw RL. Congenital malformation of the inner ear and pediatric cochlear implantation. Otol Neurotol. 2004 May. 25(3):308-17. [Medline].

17. Kontorinis G, Goetz F, Giourgas A, et al. Radiological diagnosis of incomplete partition type I versus type II: significance for cochlear implantation. Eur Radiol. 2012 Mar. 22(3):525-32. [Medline].

18. Sennaroglu L. Cochlear implantation in inner ear malformations--a review article. Cochlear Implants Int. 2010 Mar. 11(1):4-41. [Medline].

19. Chen X, Yan F, Liu B, et al. The development of auditory skills in young children with Mondini dysplasia after cochlear implantation. PLoS One. 2014. 9 (9):e108079. [Medline]. [Full Text].

Adam E Singleton, MS George Washington University School of Medicine and Health Sciences

Disclosure: Nothing to disclose.

Ashkan Monfared, MD Associate Professor of Surgery and Neurosurgery, Division of Otolaryngology-Head and Neck Surgery, George Washington University School of Medicine and Health Sciences

Disclosure: Nothing to disclose.

Arlen D Meyers, MD, MBA Professor of Otolaryngology, Dentistry, and Engineering, University of Colorado School of Medicine

Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Head and Neck Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cerescan;Cliexa, eMedevents, Neosoma, MI10<br/>Received income in an amount equal to or greater than $250 from: , Cliexa;;Neosoma<br/> Received stock from RxRevu; Received ownership interest from Cerescan for consulting; for: Neosoma, eMedevents, MI10.

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