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A very rare neurological disorder, first described by Reardon, comprised of ataxia, deafness, and mental retardation.

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Ataxia Deafness Reardon type; Ataxia-Deafness-Retardation (ADR) Syndrome.

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Fewer than 20 cases worldwide. Autosomal recessive.

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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.

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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 30:694, 1993.  [PubMed: 8411058]

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