A very rare neurological disorder, first described by
Reardon, comprised of ataxia, deafness, and mental retardation.
Ataxia Deafness Reardon type; Ataxia-Deafness-Retardation
Fewer than 20 cases worldwide.
Diagnosis is clinical on the association of the ataxia,
mental retardation, progressive sensorineural deafness. In addition to the
three principal signs that make diagnosis, hypotonia and abnormal gate can
also be observed.
Evaluate the significance of the muscle weakness and
the degree of deafness. Careful intraoperative positioning should be used because of
hypotonia. Previous knowledge of gait abnormality intensity is necessary
before authorizing board discharge in day surgery. Aminoglycosides should
be used with great caution because of the progressive deafness.
Reardon W, Wilson J, Cavanagh N, et al: A new form of familial ataxia,
deafness, and mental retardation. J Med Genet