olivopontocerebellar atrophy-deafness syndrome olivopontocerebellar atrophy-deafness syndrome olivopontocerebellar atrophy-deafness syndrome is a rare genetic disorder characterized by olivopontocerebellar atrophy which begins in infancy, sensorineural hearing loss, and speech delay. additional findings include cerebellar ataxia. inheritance pattern varies among families. olivopontocerebellar atrophy-deafness syndromespecialtymedical geneticscomplicationshearing impairment, cerebellar ataxiausual onsetinfancydurationlifelongcausesgenetic mutationrisk factorsbeing of omani ancestrypreventionnonedeaths- it has been described in 11 omani children. the pedigrees of these children couldn't identify a solid mode of inheritance: 8 of the 11 children were suspected to be sporadic cases, while the pedigrees of 3 of the 11 children pointed to autosomal recessive inheritance.