NEURO-OTOLOGICAL ABNORMALITIES IN XERODERMA PIGMENTOSUM WITH PARTICULAR REFERENCE TO DEAFNESS
- 1 September 1985
- journal article
- case report
- Published by Oxford University Press (OUP) in Brain
- Vol. 108 (3) , 771-784
- https://doi.org/10.1093/brain/108.3.771
Abstract
The neuro-otological findings are described in 3 unrelated patients who had xeroderma pigmentosum. All had impaired hearing. Routine audiometric assessment suggested that the hearing loss was of cochlear origin; brainstem evoked potentials in 2 patients and electrocochleography in 1 support this conclusion. Two adult patients had a supranuclear ophthalmoplegia. Vestibular function was mildly deranged and visual suppression of the vestibulo-ocular reflex impaired.Keywords
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