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Cochlear Implantation in Cockayne Syndrome: Our Experience of Two Cases With Different Outcomes
Author(s) -
Morris David P.,
Alian Wael,
Maessen Heather,
Creaser Cathy,
DemmonsO'Brien Stephanie,
Van Wijhe Rene,
Bance Manohar
Publication year - 2007
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1097/mlg.0b013e3180325106
Subject(s) - cockayne syndrome , hearing loss , cochlear implant , audiology , medicine , sensorineural hearing loss , cochlear implantation , visual impairment , biology , genetics , psychiatry , dna repair , xeroderma pigmentosum , dna
Cockayne syndrome is a rare autosomal recessive defect in DNA repair resulting in a classic facies with potential visual and auditory impairment. The hearing loss begins peripherally and may become central as the condition progresses. Coexisting sensory deprivation from visual impairment and the possibility of progressive deterioration in mental function conspire with a lack of published experience to produce many challenges for the cochlear implant team. To the best of our knowledge, we present the first case reports with documented follow‐up of cochlear implantation in two patients with different manifestations of Cockayne syndrome.