title: Hearing loss in Noonan syndrome.
abstract: A report is presented on a man of Turkish origin, with Noonan Syndrome and unilateral conductive hearing loss since early childhood. There was no history of otitis media. At the age of 23, exploratory tympanotomy revealed a total absence of the long process of the incus and a normal-looking tympanic membrane. The position of the normal-shaped mobile stapes was just medial, and not posteromedial, to the malleus. A congenital ossicular chain anomaly was diagnosed. An allograft malleus head was interposed between the stapes and the malleus. The resulting air-bone gap was less than 10 dB. A review of the literature is given on hearing loss in Noonan Syndrome.
authors.affiliation: Institute of Otorhinolaryngology, University Hospital of Nijmegen, The Netherlands.
authors.forename: C W
authors.initials: CW
authors.lastname: Cremers
authors.forename: C J
authors.initials: CJ
authors.lastname: van der Burgt
mesh_terms: D000328:Adult; D006314:Hearing Loss, Conductive / Q000209:etiology*; D006801:Humans; D007188:Incus / Q000002:abnormalities*; D008297:Male; D009634:Noonan Syndrome / Q000150:complications*