Olivopontocerebellar atrophy-deafness syndrome is characterized by infancy-onset olivopontocerebellar atrophy, sensorineural deafness and speech impairment. It has been described in less than 15 children. Most cases were sporadic, but autosomal recessive inheritance was suggested in three cases. The disease is olivopontocerebellar atrophy-deafness syndrome (MONDO_0017135, a Monarch Disease Ontology identifier).