Skip to Main Content

We have a new app!

Take the Access library with you wherever you go—easy access to books, videos, images, podcasts, personalized features, and more.

Download the Access App here: iOS and Android

At a glance

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


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


Fewer than 20 cases worldwide.

Genetic inheritance

Autosomal recessive. Consanguinity.


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 an abnormal gait can also be observed.

Anesthetic considerations

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.

Other condition to be considered

  • Richards Rundle Syndrome: Characterized by mental retardation, underdevelopment of secondary sex characteristics, deafness, ataxia, and peripheral muscle wasting. Increased urinary excretion of beta-aminoisobutyric acid, and low urinary excretion of neutral 17-ketosteroids.


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]

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.