Assessment of speech in early-onset ataxia: a pilot study.

AIM: The aim of the study was to determine whether paediatric ataxia speech subscores are reliably applicable for international early-onset ataxia (EOA) databases. If so, we reasoned that ataxia speech subscores should be associated with ataxia scores and involve high interobserver agreement, including those for internationally applicable Scale for Assessment and Rating of Ataxia (SARA) syllable repetition tasks (SARASRT). METHOD: Three independent paediatric neurologists and a speech therapist scored speech in 52 healthy children (mean age 10y, range 4-16y) and 40 individuals with EOA (mean age 15y, range 5-34y). We compared ataxia speech subscores for the association with age and ataxia scores as well as interobserver reliability. RESULTS: In healthy children, ataxia speech subscores were moderately associated with age (International Cooperative Ataxia Rating Scale [ICARS]: r=-0.515; SARA: r=-0.321; p<0.05) and with ataxia scores (ICARS: r=0.552; SARA: r=0.336; p<0.05), and revealed slight to moderate interobserver agreement (ICARS-intraclass correlation coefficient [ICC]: 0.380; SARA-ICC: 0.185; SARASRT-ICC: 0.509). In EOA, speech subscores have a strong association with ataxia scores (ICARS: r=0.735; SARA: r=0.730; p<0.001) and revealed substantial to nearly perfect interobserver agreement (ICARS-ICC: 0.812; SARA-ICC: 0.854; SARASRT-ICC: 0.724). INTERPRETATION: Early-onset ataxia speech subscores are associated with ataxia and also reveal high interobserver agreement, including those internationally applicable to SARASRT. We conclude that SARASRT appears to be applicable for EOA databases. However, before syllable repetition tasks are included, we would advise to wait for the results published by the international Childhood Ataxia and Cerebellar Group.

The discrepancy hypothesis in children with language disorders: does it work?

OBJECTIVES: Analysis of the relationship between verbal and nonverbal development in children with language problems. METHODS: From 134 children enrolled in a multidisciplinary diagnostic procedure in a speech and hearing clinic and diagnosed as having a language disorder, the language comprehension score (LCQ) and the nonverbal IQ score (SON-IQ) were compared. t-Tests were used to test whether the children's mean LCQ differs from their mean SON-IQ and to test whether the children with an inadequate LCQ differ from children with an adequate LCQ with respect to discrepancy. Plots inspired by Bland and Altman [18] display the measurement of mean value of verbal and nonverbal development against the discrepancy between these scores. RESULTS: All children had a language production problem (inadequate GDS). Out of the 57 children with an adequate language comprehension (LCQ>80), 16 children (28%) show a discrepancy of 10 quotient points or more between their LCQ and SON-IQ. Out of the 77 children with an inadequate language comprehension (LCQ80 the discrepancy is not significant (p=0.084). CONCLUSIONS: The discrepancy hypothesis, in our opinion, must be modified. There is not only verbal and nonverbal functioning but there is language production, language comprehension and nonverbal learning abilities. Between these three aspects discrepancies can be found. In 43% of the children there is a discrepancy between language production and language comprehension. When children also show language comprehension problems, 58% of these children show a discrepancy with nonverbal functioning.