title: Large vestibular aqueduct syndrome: a human temporal bone study.
abstract: OBJECTIVE
  Large vestibular aqueduct syndrome (LVAS) is one of the common causes of hearing loss (HL). All prior studies have reported some anomalies associated with LVAS by imaging techniques. This study was undertaken to determine prevalence of LVAS in our temporal bone (TB) collection and its relationship to other systemic or otologic anomalies.
  
  
  METHODS
  Retrospective, TB histopathologic study.
  
  
  METHODS
  Anteroposterior diameters of internal (IA) and external (EA) apertures were measured in 40 normal TBs (40 cases). TBs were considered as large vestibular aqueduct (LVA) if width of apertures was 95% greater than "normals." Systemic and otologic anomalies and histopathology of ears with LVAS were noted.
  
  
  RESULTS
  Of 1,608 non-"normal" TBs, 63 had LVA. There was negative correlation between IA and EA in 48 TBs with only enlarged IA. Fifteen TBs with enlarged EA always had enlarged IA and were therefore considered as LVAS. The most common pathologic condition was congenital heart anomaly. The most common syndrome or dysplasia was Mondini's. The most common anomalies of external and middle ears were dehiscent facial nerves, low set auricles, and ossicular deformities. Inner ear anomalies included modiolar deficiencies, hair cell loss, interscalar septum defects, and strial atrophy. There was no record of family history of HL, head injury, or craniofacial, branchial, or thyroidal abnormalities.
  
  
  CONCLUSIONS
  Because HL associated with LVAS may be attributed to other ear anomalies, it is important to investigate other inner ear problems and system diagnoses that may indicate a syndrome in patients with radiologically diagnosed LVAS.
authors.affiliation: International Hearing Foundation, Minneapolis, Minnesota, USA. shigeo-hirai@hotmail.com
authors.forename: Shigeo
authors.initials: S
authors.lastname: Hirai
authors.forename: Sebahattin
authors.initials: S
authors.lastname: Cureoglu
authors.forename: Patricia A
authors.initials: PA
authors.lastname: Schachern
authors.forename: Hideo
authors.initials: H
authors.lastname: Hayashi
authors.forename: Michael M
authors.initials: MM
authors.lastname: Paparella
authors.forename: Tamotsu
authors.initials: T
authors.lastname: Harada
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D004432:Ear, Middle / Q000002:abnormalities; D004712:Endolymphatic Sac / Q000473:pathology; D005260:Female; D034381:Hearing Loss / Q000209:etiology; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011859:Radiography; D012189:Retrospective Studies; D013577:Syndrome; D013701:Temporal Bone / Q000473:pathology*; D014723:Vestibular Aqueduct / Q000002:abnormalities* / Q000000981:diagnostic imaging