An auditory profile of sclerosteosis

Show simple item record Potgieter, Jenni-Marí Swanepoel, De Wet Heinze, Barbara M. Hofmeyr, Louis Murray Burger, André A.S. Hamersma, Herman 2014-06-10T05:17:19Z 2014-04
dc.description.abstract OBJECTIVE : 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.librarian hb2014 en_US
dc.description.uri en_US
dc.identifier.citation Potgieter, 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.issn 0022-2151 (print)
dc.identifier.issn 1748-5460 (online)
dc.identifier.other 10.1017/S0022215113002648
dc.language.iso en en_US
dc.publisher Cambridge University Press en_US
dc.rights © JLO (1984) Limited, 2014 en_US
dc.subject Audiology en_US
dc.subject Bone dysplasias en_US
dc.subject Hearing loss en_US
dc.subject Conductive en_US
dc.subject Sclerosteosis en_US
dc.subject Sensorineural hearing loss en_US
dc.title An auditory profile of sclerosteosis en_US
dc.type Postprint Article en_US

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