An auditory profile of sclerosteosis

dc.contributor.authorPotgieter, Jenni-Marí
dc.contributor.authorSwanepoel, De Wet
dc.contributor.authorHeinze, Barbara M.
dc.contributor.authorHofmeyr, Louis Murray
dc.contributor.authorBurger, André A.S.
dc.contributor.authorHamersma, Herman
dc.contributor.emaildewet.swanepoel@up.ac.zaen_US
dc.date.accessioned2014-06-10T05:17:19Z
dc.date.issued2014-04
dc.description.abstractOBJECTIVE : To characterise auditory involvement secondary to excessive craniotubular bone growth in individuals with sclerosteosis in South Africa. METHODS : This cross-sectional study assessed the auditory profile of 10 participants with sclerosteosis. An auditory test battery was used and results for each ear were recorded using descriptive and comparative analyses. RESULTS : All participants presented with bilateral, mixed hearing losses. Of the 20 ears, hearing loss was moderate in 5 per cent (n = 1), severe in 55 per cent (n = 11) and profound in 40 per cent (n = 8). Air–bone gaps were smaller in older participants, although the difference was not statistically significant (p > 0.05). Computed tomography scans indicated pervasive abnormalities of the external auditory canal, tympanic membrane, middle-ear space, ossicles, oval window, round window and internal auditory canal. Narrowed internal auditory canals corresponded to poor speech discrimination, indicative of retrocochlear pathology and absent auditory brainstem response waves. CONCLUSION : Progressive abnormal bone formation in sclerosteosis involves the middle ear, the round and oval windows of the cochlea, and the internal auditory canal. The condition compromises conductive, sensory and neural auditory pathways, which results in moderate to profound, mixed hearing loss.en_US
dc.description.librarianhb2014en_US
dc.description.urihttp://journals.cambridge.org/action/displayJournal?jid=JLOen_US
dc.identifier.citationPotgieter, JM, Swanepoel, DW, Heinze, BM, Hofmeyr, LM, Burger, AAS & Hamersma, H 2014, 'An auditory profile of sclerosteosis', Journal of Laryngology and Otology, vol. 128, no. 4, pp. 336-344.en_US
dc.identifier.issn0022-2151 (print)
dc.identifier.issn1748-5460 (online)
dc.identifier.other10.1017/S0022215113002648
dc.identifier.urihttp://hdl.handle.net/2263/40096
dc.language.isoenen_US
dc.publisherCambridge University Pressen_US
dc.rights© JLO (1984) Limited, 2014en_US
dc.subjectAudiologyen_US
dc.subjectBone dysplasiasen_US
dc.subjectHearing lossen_US
dc.subjectConductiveen_US
dc.subjectSclerosteosisen_US
dc.subjectSensorineural hearing lossen_US
dc.titleAn auditory profile of sclerosteosisen_US
dc.typePostprint Articleen_US

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