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  • Title: Electrophysiological study of Norrie's disease. An X-linked recessive trait with hearing loss.
    Author: Parving A, Elberling C, Warburg M.
    Journal: Audiology; 1978; 17(4):293-8. PubMed ID: 567472.
    Abstract:
    3 patients with Norrie's diseas were examined with electrocochleography and brain-stem-evoked responses. The examination revealed a hearing loss of pure cochlear origin with no involvement of the brain stem. The disease is inherited as an X-linked recessive trait only affecting males with unaffected female carriers. The results are thus in agreement with previous investigations of the topical lesion in genetic hearing loss.
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