title: Inner ear anatomy in Waardenburg syndrome: radiological assessment and comparison with normative data.
abstract: OBJECTIVE
  As patients with Waardenburg syndrome (WS) represent potential candidates for cochlear implantation, their inner ear anatomy is of high significance. There is an ongoing debate whether WS is related to any inner ear dysplasias. Our objective was to evaluate radiologically the inner ear anatomy in patients with WS and identify any temporal bone malformations.
  
  
  METHODS
  A retrospective case review was carried out in a tertiary, referral center. The high resolution computed tomography (HRCT) scans of the temporal bone from 20 patients (40 ears) with WS who were managed for deafness in a tertiary referral center from 1995 to 2012 were retrospectively examined. Measurements of 15 different inner ear dimensions, involving the cochlea, the vestibule, the semicircular canals and the internal auditory meatus, as well as measurements of the vestibular aqueduct, were performed independently by two neuroradiologists. Finally, we compared the results from the WS group with a control group consisting of 50 normal hearing subjects (100 ears) and with previously reported normative values.
  
  
  RESULTS
  Inner ear malformations were not found in any of the patients with WS. All measured inner ear dimensions were within the normative values compiled by our study group as well as by others.
  
  
  CONCLUSIONS
  Inner ear malformations are not characteristic for all types of WS; however, certain rare subtypes might be related to inner ear deformities. Normative cochleovestibular dimensions that can help in assessing the temporal bone anatomy are provided.
authors.affiliation: Department of Otolaryngology, Hannover Medical School, 1 Carl-Neuberg-Str., Hannover 30625, Germany; Department of Otolaryngology, Glasgow Southern Teaching Hospital, 1345 Govan Road, Glasgow G51 4TF, United Kingdom. Electronic address: gkontorinis@gmail.com.
authors.forename: Georgios
authors.initials: G
authors.lastname: Kontorinis
authors.affiliation: Department of Neuroradiology, Hannover Medical School, 1 Carl-Neuberg-Str., Hannover 30625, Germany.
authors.forename: Friedrich
authors.initials: F
authors.lastname: Goetz
authors.affiliation: Department of Neuroradiology, Hannover Medical School, 1 Carl-Neuberg-Str., Hannover 30625, Germany.
authors.forename: Heinrich
authors.initials: H
authors.lastname: Lanfermann
authors.affiliation: Department of Otolaryngology, Hannover Medical School, 1 Carl-Neuberg-Str., Hannover 30625, Germany.
authors.forename: Stefan
authors.initials: S
authors.lastname: Luytenski
authors.affiliation: Department of Neuroradiology, Hannover Medical School, 1 Carl-Neuberg-Str., Hannover 30625, Germany.
authors.forename: Anja M
authors.initials: AM
authors.lastname: Giesemann
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D007758:Ear, Inner / Q000033:anatomy & histology* / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D013701:Temporal Bone / Q000033:anatomy & histology / Q000000981:diagnostic imaging; D036542:Tomography, Spiral Computed; D014849:Waardenburg Syndrome / Q000150:complications*; D055815:Young Adult
keywords: Cochlear implantation; Computed tomography; Inner ear malformations; Large vestibular aqueduct; Normative/referral cochleovestibular dimensions; Waardenburg syndrome