Enamel defects and ectopic eruption in a child with Usher syndrome and a cochlear implant.
BACKGROUND
Usher syndrome is a genetic disorder consisting of progressive loss of vision and hearing.


METHODS
The paper describes an 8-year-old girl with Usher syndrome type I who presented with generalized defects of the permanent dentition and ectopic eruption of the right maxillary first permanent molar. A cochlear implant had been fitted for her hearing loss, and the report reviews the implications of this device for dental treatment. The impacted first permanent molar was encouraged to erupt into the correct position by shaving the distal surface of the second primary molar.


CONCLUSIONS
This is the first report to describe in detail an association between Usher syndrome and enamel defects.
Balmer
Richard
R
Division Child Dental Health, Leeds Dental Institute, Leeds, UK. r.c.balmer@leeds.ac.uk
Fayle
Stephen A F
SA
D002648:Child; D003054:Cochlear Implants; D003744:Dental Enamel Hypoplasia / Q000209:etiology*; D003818:Dentition, Mixed; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008963:Molar / Q000002:abnormalities*; D014079:Tooth Eruption, Ectopic / Q000209:etiology*; D014095:Tooth, Impacted / Q000628:therapy; D052245:Usher Syndromes / Q000150:complications*