Speech characteristics associated with three genotypes of ataxia.

PURPOSE Advances in neurobiology are providing new opportunities to investigate the neurological systems underlying motor speech control. This study explores the perceptual characteristics of the speech of three genotypes of spino-cerebellar ataxia (SCA) as manifest in four different speech tasks. METHODS Speech samples from 26 speakers with SCA were perceptually rated by experienced listeners. The genotypes were: SCA1, SCA5, or SCA6. The speech tasks were: diadochokinesis, word repetition, sentence reading, and picture description. The speech samples were rated using two sets of dimensions characterized as primary (e.g., articulation, rate, and rhythm) or secondary (e.g., imprecise consonants, excess and equal stress, and harsh voice). RESULTS On primary dimensions, SCA6 was the most impaired generally. Articulation was the most severely affected dimension and the diadochokinesis task was most effective in revealing speech impairments. On secondary dimensions, picture description was the task most likely to produce abnormal speech. The SCA groups shared articulatory problems but differed with respect to abnormal voice features. CONCLUSIONS These results support previous characterizations of ataxic dysarthria, and provide further information about the speech characteristics of genetic subtypes. Task demands affect perceptual ratings. Voice characteristics may be key to differentiating ataxic subtypes. As the genetic disorders that affect speech become better understood, more detailed characterizations of motor control systems should emerge.