Dysplasia of the cerebellum in Waardenburg syndrome: outcomes following cochlear implantation

International Journal of Pediatric Otorhinolaryngology
Lisa KaufmannDaniel J Lee

Abstract

This study provides the first description of isolated cerebellar dysplasia associated with Waardenburg syndrome (WS) and includes a review of cochlear implant outcomes in 42 WS patients. A 1-year-old male infant presented with speech delay, iris heterochromia, profound hearing loss, and an asymmetric, underdeveloped right occipital skull on CT imaging. Brain MRI demonstrated a hypoplastic right cerebellum, no hydrocephalus, normal auditory nerves and brainstem. He underwent successful bilateral sequential cochlear implantation. Cochlear implants remain a reasonable habilitative option for WS patients with congenital deafness, including those with cerebellar abnormalities.

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Citations

Dec 14, 2011·European Archives of Oto-rhino-laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery·Susan AmirsalariYasaman Ghazavi
Feb 22, 2012·International Journal of Pediatric Otorhinolaryngology·Bita NajafiyanSusan Amirsalari
Sep 4, 2013·Cochlear Implants International·Jane BlackAsaduzzaman Khan
Jun 2, 2016·Audiology & Neuro-otology·Josephine W I van NieropHenricus P M Kunst
Apr 23, 2011·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Georgios KontorinisAnke Lesinski-Schiedat
Jan 1, 2020·European Journal of Radiology·Felice D'ArcoGiacomo Talenti

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