title: Hearing loss and the Mayer-Rokitansky-Küster-Hauser syndrome.
abstract: The hearing of 51 female patients with the Mayer-Rokitansky-Küster-Hauser syndrome was examined using otoscopy and standard audiometry. A unilateral or bilateral hearing loss of more than 15 dB Fletcher index was found in 13 of 51 (25.5%). Four of these 13 patients had a hearing loss of less than 20 dB in the worst ear. The remainder had a hearing loss of at least 30 dB in the worst ear. Five of the 13 patients had pure conductive hearing loss; in four of these five, a congenital origin was accepted. Two of the 13 had mixed hearing loss that was a residual symptom from previous otitis media; six had sensorineural hearing loss. A congenital cause was found in one of these six, based on the fact that she had been deaf and dumb since birth. In one other patient, noise-related deafness was likely (i.e., an acquired cause). In the other four cases in this group, the cause was unknown. The results of this study show that hearing loss is a characteristic associated with the Mayer-Rokitansky-Küster-Hauser syndrome.
authors.affiliation: Department of Radiology, Academic Medical Centre, University of Amsterdam, The Netherlands.
authors.forename: E H
authors.initials: EH
authors.lastname: Strübbe
authors.forename: C W
authors.initials: CW
authors.lastname: Cremers
authors.forename: F G
authors.initials: FG
authors.lastname: Dikkers
authors.forename: W N
authors.initials: WN
authors.lastname: Willemsen
mesh_terms: D000328:Adult; D002574:Cervical Vertebrae / Q000002:abnormalities / Q000000981:diagnostic imaging; D005260:Female; D007839:Functional Laterality; D006314:Hearing Loss, Conductive / Q000150:complications / Q000175:diagnosis* / Q000235:genetics*; D006319:Hearing Loss, Sensorineural / Q000150:complications / Q000175:diagnosis* / Q000235:genetics*; D006801:Humans; D007714:Klippel-Feil Syndrome / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D008875:Middle Aged; D013123:Spinal Fusion; D013577:Syndrome; D014567:Urography