title: Bilateral cochlear nerve absence in a 3 year old child with VACTERL association.
abstract: We report a case of a 3 year old boy with bilateral profound sensorineural hearing loss diagnosed from New Born Hearing Screening, with severe form of VACTERL association. He was referred to our Cochlear Implant Unit for assessment with regard to the possibility of cochlear implantation. MRI findings have showed bilateral vestibulocochlear cystic abnormalities. Only single nerve noted within the IAM on the right and likely single nerve within the IAM on the left. Hence, decision was made not to offer cochlear implantation. This is the first report of severe bilateral cochleovestibular nerve abnormalities to be associated with VACTERL.
authors.affiliation: Yorkshire Auditory Implant Service (YAIS), Bradford Royal Infirmary, Listening for Life Centre, Bradford Teaching Hospitals NHS Foundation Trust, UK. Electronic address: Milan.Rudic@bthft.nhs.uk.
authors.forename: Milan
authors.initials: M
authors.lastname: Rudić
authors.affiliation: Yorkshire Auditory Implant Service (YAIS), Bradford Royal Infirmary, Listening for Life Centre, Bradford Teaching Hospitals NHS Foundation Trust, UK.
authors.forename: Winson
authors.initials: W
authors.lastname: Wong
authors.affiliation: Radiology Department, Bradford Royal Infirmary, Bradford Teaching Hospitals NHS Foundation Trust, UK.
authors.forename: Stuart
authors.initials: S
authors.lastname: Viner
authors.affiliation: Yorkshire Auditory Implant Service (YAIS), Bradford Royal Infirmary, Listening for Life Centre, Bradford Teaching Hospitals NHS Foundation Trust, UK.
authors.forename: David
authors.initials: D
authors.lastname: Strachan
authors.affiliation: Yorkshire Auditory Implant Service (YAIS), Bradford Royal Infirmary, Listening for Life Centre, Bradford Teaching Hospitals NHS Foundation Trust, UK.
authors.forename: Christopher
authors.initials: C
authors.lastname: Raine
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis*; D001003:Anal Canal / Q000002:abnormalities*; D002675:Child, Preschool; D003056:Cochlear Nerve / Q000002:abnormalities* / Q000000981:diagnostic imaging; D004947:Esophagus / Q000002:abnormalities*; D006319:Hearing Loss, Sensorineural / Q000151:congenital* / Q000175:diagnosis; D006330:Heart Defects, Congenital / Q000175:diagnosis*; D006801:Humans; D007668:Kidney / Q000002:abnormalities*; D017880:Limb Deformities, Congenital / Q000175:diagnosis*; D008279:Magnetic Resonance Imaging; D008297:Male; D013131:Spine / Q000002:abnormalities*; D014132:Trachea / Q000002:abnormalities*
keywords: Cochlear implantation; Congenital anomalies; VACTERL; Vestibulocochlear nerve