title: Correlation of beta-2 transferrin and middle ear abnormalities in congenital perilymphatic fistula.
abstract: Congenital perilymphatic fistula (PLF) as a diagnosis for progressive, fluctuating, or sudden sensorineural hearing loss with or without vertigo is still controversial. Beta-2 transferrin is a protein that is unique to cerebrospinal fluid, aqueous humor, and perilymph. A recent pilot study demonstrated that beta-2 transferrin may be an objective test to determine the existence of a congenital PLF. The authors prospectively evaluated and recommended surgery for 43 children with suspected PLF over the past 3 years. A prospective, blinded study was performed by having the attending otolaryngologist evaluate the middle ear at the time of surgery for a PLF and any middle ear abnormalities. Samples of fluid were collected from the oval and round windows and were tested for beta-2 transferrin. Of 16 patients undergoing tympanoplasty or tympanomastoidectomy who served as controls, none were positive for beta-2 transferrin. Of the 43 patients undergoing exploratory tympanoplasty for PLF, 20 (46.5%) were considered to be negative for PLF on microscopic visualization; 23 (53.5%) were found to be positive. Of the 20 patients thought to be negative for PLF, 18 (90%) tested negative for beta-2, but 2 of these patients were positive for beta-2, and both had a congenital middle ear abnormality. Of the 23 patients found to have a PLF at surgery, 6 (26.1%) tested positive for beta-2, and all of these 6 had middle ear abnormalities. Of the 17 patients negative for beta-2, 9 had normal anatomy; 6 had middle ear abnormalities, and 2 had erosive changes. The authors conclude that beta-2 transferrin, an objective test, confirms the existence of congenital PLF in children, which is associated with middle ear abnormalities.
authors.affiliation: Center for Hearing and Balance Disorders, Medical University of South Carolina, Charleston, USA.
authors.forename: P C
authors.initials: PC
authors.lastname: Weber
authors.forename: C D
authors.initials: CD
authors.lastname: Bluestone
authors.forename: M A
authors.initials: MA
authors.lastname: Kenna
authors.forename: R H
authors.initials: RH
authors.lastname: Kelley
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000032:analysis; D015153:Blotting, Western; D002648:Child; D002675:Child, Preschool; D003052:Cochlear Aqueduct; D004432:Ear, Middle / Q000002:abnormalities*; D005260:Female; D005402:Fistula / Q000151:congenital / Q000175:diagnosis* / Q000378:metabolism; D006801:Humans; D007223:Infant; D007759:Labyrinth Diseases / Q000151:congenital / Q000175:diagnosis* / Q000378:metabolism; D008297:Male; D010046:Oval Window, Ear; D010498:Perilymph; D011446:Prospective Studies; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D014168:Transferrin / Q000032:analysis*
chemical_list: D015415:Biomarkers; D014168:Transferrin