Perrault syndrome (PS) is characterized by the association of ovarian dysgenesis in females with sensorineural hearing impairment. In more recent PS reports, some authors have described neurologic abnormalities, notably progressive cerebellar ataxia and intellectual deficit. The disease is Perrault syndrome (MONDO_0017312, a Monarch Disease Ontology entry). Also known as: XX gonodal dysgenesis-deafness syndrome.