title: Familial unilateral deafness and delayed endolymphatic hydrops.
abstract: Delayed endolymphatic hydrops (DEH) is a unique disorder characterized by fluctuating otologic symptoms in the setting of preexisting unilateral deafness. The symptoms include aural fullness, fluctuating hearing, and/or episodes of vertigo similar to those observed in Meniere disease and may occur ipsilateral or contralateral to the previously deafened ear. In most reported cases, the unilateral deafness has been a profound sensorineural hearing loss with a sudden onset that has been variously attributed to bacterial or viral labyrinthitis, acoustic or cranial trauma, otosclerosis, and congenital CMV infection. Familial occurrence of the syndrome has not previously been reported in the literature. In this report, we describe two possible familial instances of delayed DEH. These patients raise the possibility that genetic factors may sometimes be the cause of this unusual syndrome.
authors.affiliation: Department of Human Genetics, Virginia Commonwealth University, Richmond, Virginia, USA. kelleydodson@comcast.net
authors.forename: Kelley M
authors.initials: KM
authors.lastname: Dodson
authors.forename: Tamio
authors.initials: T
authors.lastname: Kamei
authors.forename: Aristides
authors.initials: A
authors.lastname: Sismanis
authors.forename: Walter E
authors.initials: WE
authors.lastname: Nance
mesh_terms: D000328:Adult; D002648:Child; D018159:Endolymphatic Hydrops / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D005192:Family Health; D005260:Female; D046088:Hearing Loss, Unilateral / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D006320:Hearing Tests; D006801:Humans; D008297:Male; D010375:Pedigree; D013701:Temporal Bone / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed