Sclerosing bone dysplasias: neurologic assessment and management

Hofmeyr, Louis Murray; Hamersma, Herman

doi:10.1097/01.moo.0000134441.84110.9e

Cited by 10 publications

(36 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The symptoms and complications of excessive bony overgrowth are currently managed by surgical treatment (Hofmeyr & Hamersma, 2004). Proptosis was managed by orbital decompression (Hofmeyr & Hamersma, 2004).…”

Section: Managementmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

“…Proptosis was managed by orbital decompression (Hofmeyr & Hamersma, 2004). Postoperative blindness had however been encountered in several cases after orbital decompression (Hofmeyr & Hamersma, 2004). Other cosmetic surgeries were done on the mandible, syndactyly, and skeletal deformities for functional purposes (Hofmeyr & Hamersma, 2004).…”

Section: Managementmentioning

confidence: 99%

“…One of the most significant challenges was the management of hearing loss, which had limited success (Hofmeyr & Hamersma, 2004). The encroachment of the dense bone on the round and oval windows needed to be drilled away, which might cause sensorineural damage to the high frequencies of hearing (Hofmeyr & Hamersma, 2004).…”

Section: Managementmentioning

confidence: 99%

“…The encroachment of the dense bone on the round and oval windows needed to be drilled away, which might cause sensorineural damage to the high frequencies of hearing (Hofmeyr & Hamersma, 2004).…”

Section: Managementmentioning

confidence: 99%

See 4 more Smart Citations

An auditory profile of sclerosteosis

et al. 2014

View full text Add to dashboard Cite

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.

show abstract

Section: Managementmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

See 3 more Smart Citations

An auditory profile of sclerosteosis

et al. 2014

View full text Add to dashboard Cite

show abstract

Decompressive surgery in a patient with hyperostosis corticalis generalisata for relief of cognitive disability and dysaesthesia

et al. 2015

View full text Add to dashboard Cite

A 28-year-old man with genetically confirmed hyperostosis corticalis generalisata (Van Buchem disease) suffered from headache and progressive cognitive and sensibility disorders. Bone formation of the skull was ongoing, leading to narrowing of the intracranial space and foramen magnum. A large bilateral frontoparietal craniotomy and decompression of the foramen magnum resulted in almost complete relief of his symptoms. This is the first report on successful decompressive surgery as a treatment of cognitive impairment and dysaesthesia.

show abstract