title: The incudostapedial articulation in Down's syndrome (trisomy 21): a temporal bone study.
abstract: OBJECTIVE
  Anomaly in the incudostapedial articulation (IS) may be responsible for persistent conductive hearing loss (HL) in Down's syndrome (DS) patients. Our goal was to perform a detailed histopathologic assessment of the role of the IS joint in the conductive HL.
  
  
  BACKGROUND
  In addition to other disabilities, 38% to 78% of patients with Down's syndrome (Trisomy 21) have CHL, some of which are persistent despite maximum treatment. The cause of the persistent conductive HL remains unclear.
  
  
  METHODS
  Retrospective analysis of temporal bone histopathology. Analyses of the midsection through the IS joint of 21 temporal bones (13 subjects) patients with DS and 31 temporal bones (18 subjects) from healthy subjects, performed by 2 blinded authors. The length of 7 different parameters of the width of the IS joint was measured and compared between the 2 groups.
  
  
  RESULTS
  The IS joint of patients with DS was significantly wider than the normal control. As opposed to the control, it did not decrease because of age.
  
  
  CONCLUSIONS
  The persistent conductive HL in some patients with DS might be caused by anomaly of the IS joint. In severe cases, corrective surgery should be considered.
authors.affiliation: *Department of Otorhinolaryngology, Complexo Hospitalar Santa Casa de Porto Alegre, Porto Alegre, Brazil; †Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital Zurich, Zurich, Switzerland; ‡Department of Otolaryngology, Tufts University School of Medicine; and §The Temporal Bone Foundation, Boston, Massachusetts, U.S.A.
authors.forename: Patricia Barcelos
authors.initials: PB
authors.lastname: Ogando
authors.forename: Christof
authors.initials: C
authors.lastname: Röösli
authors.forename: Collin S
authors.initials: CS
authors.lastname: Karmody
authors.forename: Clarinda C
authors.initials: CC
authors.lastname: Northrop
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D004314:Down Syndrome / Q000473:pathology*; D005260:Female; D006314:Hearing Loss, Conductive / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007188:Incus / Q000002:abnormalities* / Q000473:pathology; D007223:Infant; D008297:Male; D008875:Middle Aged; D013199:Stapes / Q000002:abnormalities* / Q000473:pathology; D013701:Temporal Bone / Q000002:abnormalities* / Q000473:pathology