Cochlear implantation in patient with Dandy-walker syndrome

Summary

Introduction: Dandy Walker Syndrome is a congenital abnormality in the central nervous system, characterized by a deficiency in the development of middle cerebelar structures, cystic dilatation of the posterior pit communicating with the fourth ventricle and upward shift of the transverse sinuses, tentorium and dyes. Among the clinical signs are occipital protuberances, a progressive increase of the skull, bowing before the fontanels, papilledema, ataxia, gait disturbances, nystagmus, and intellectual impairment.

Objectives: To describe a case of female patient, 13 years old with a diagnosis of this syndrome and bilateral hearing loss underwent cochlear implant surgery under local anesthesia and sedation.

Case Report: CGS, 13 years old female was referred to the Otolaryngological Department of Otolaryngology Institute of Parana with a diagnosis of “Dandy-Walker syndrome” for Otolaryngological evaluation for bilateral hearing loss with no response to the use of hearing aids.

Final Comments: The field of cochlear implants is growing rapidly. We believe that the presence of Dandy-Walker syndrome cannot be considered a contraindication to the performance of cochlear implant surgery, and there were no surgical complications due to neurological disorders with very favorable results for the patient who exhibits excellent discrimination. It has less need for lip reading with improvement in speech quality.

Keywords

hearing loss - cochlear implants - Dandy-Walker syndrome

• References

• 1 Diament A. Neurologia infantil 3a ed. São Paulo: Atheneu; 1996
• 2 Rosenberg S. Neuropediatria. São Paulo: Sarvier; 1995
• 3 Yilmaz MA. The site on Dandy Walker Syndrome [homepage on the Internet]. [cited 2005 Aug 29]. Available from: http://www.geocities.com/murat_yil/dandy.html
• 4 Parisi MA, Dobyns WB. Human malformations of the midbrain and hindbrain: review and proposed classification scheme. Mol Genet Metab 2003; 80 (1–2): 36-53
• 5 Chizhikov V, Millen KJ. Development and malformations of the cerebellum in mice. Mol Genet Metab. 2003; 80 (1–2): 54-65
• 6 Ten Donkelaar HJ, Lammens M, Wesseling P, Thijssen HO, Renier WO. Development and developmental disorders of the human cerebellum. J Neurol 2003; 250 (9) 1025-36
• 7 Koren G, Pastuszak A, Ito S. Drugs in pregnancy. N Engl J Med 1998; 338 (16) 1128-37
• 8 Daufenbach DR, Ruttum MS, Pulido JS, Keech RV. Chorioretinal colobomas in a pediatric population. Ophthalmology 1998; 105 (8) 1455-8 . Comment in: Ophthalmology. 1999, 106(4):645–6.
• 9 Niesen CE. Malformations of the posterior fossa: current perspectives. Semin Pediatr Neurol 2002; 9 (4) 320-34
• 10 Coats DK, Paysse EA, Levy ML. PHACE: a neurocutaneous syndrome with important ophthalmologic implications: case report and literature review. Ophthalmology 1999; 106 (9) 1739-41
• 11 Kumandas S, Akcakus M, Coskun A, Gumus H. Joubert syndrome: review and report of seven new cases. Eur J Neurol 2004; 11 (8) 505-10
• 12 Klein O, Pierre-Kahn A, Boddaert N, Parisot D, Brunelle F. Dandy-Walker malformation: prenatal diagnosis and prognosis. Childs Nerv Syst 2003; 19 (78) 484-9
• 13 Boddaert N, Klein O, Ferguson N, Sonigo P, Parisot D, Hertz-Pannier L , et al. Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation. Neuroradiology 2003; 45 (5) 320-4
• 14 Reese V, Paller AS, Esterly NB, Ferriero D, Levy ML , et al. Association of facial hemangiomas with Dandy-Walker and other posterior fossa malformations. J Pediatr 1993; 122 (3) 379-84
• 15 Balci S, Teksam O, Gedik S. Megalocornea, macrocephaly, mental and motor retardation: MMMM syndrome (Neuhauser syndrome) in two sisters with hypoplastic corpus callosum. Turk J Pediatr 2002; 44 (3) 274-7
• 16 Pagon RA, Clarren SK, Milam Jr DF, Hendrickson AE. Autosomal recessive eye and brain anomalies: Warburg syndrome. J Pediatr 1983; 102 (4) 542-6
• 17 Golden JA. Cell migration and cerebral cortical development. Neuropathol Appl Neurobiol 2001; 27 (1) 22-8
• 18 Ruibal Francisco JL, Sánchez Buron P, Piñero Martinez E, Bueno Lozano G. [Turner's syndrome. Relationship between the karyotypes and malformations and associated diseases in 23 patients]. An Esp Pediatr. 1997; 47 (2) 167-71 . Spanish.
• 19 Kawaguchi T, Jokura H, Kusaka Y, Shirane R, Yoshimoto T. Intraoperative direct neuroendoscopic observation of the aqueduct in Dandy-Walker malformation. Acta Neurochir (Wien) 2003; 145 (1) 63-7
• 20 Chakrabarty A, Tarneja VK, Singh VK. Cochlear implant: anesthesia challenges. MJAFI Hournal 1994; 60: 351-6
• 21 Sharon LC, *, Laurie Mb, Evan J, Alok S, Tracy S. Successful cochlear implantation in a child with Keratosis, Icthiosis and Deafness (KID) Syndromeand Dandy-Walker malformation. International Journal of Pediatric Otorhinolaryngology. 2008; 72: 693-698