Batten disease

In CLN2 and CLN3 Batten disease, progressive speech and language decline is a sensitive marker of neurodegeneration. Redenlab’s digital speech analytics offer objective, functional endpoints to assess disease progression and treatment effects in paediatric trials.

Speech, language, and social communication in Batten disease

Batten disease, including CLN2 and CLN3 subtypes, is a group of rare, inherited lysosomal storage disorders that cause progressive neurodegeneration in children. Early impairments in speech, language, and motor coordination are hallmark features, often preceding cognitive and visual decline and have profound effects on social engagement and quality of life. In conditions like CLN2, rapid deterioration over months necessitates sensitive tools to monitor therapeutic response. Redenlab delivers validated speech and language assessments to track functional change across disease stages, supporting natural history studies, gene therapy trials, and real-world clinical endpoints.

Speech analytics in Batten disease

Captures neurocognitive impact

Measures speech rate, fluency, prosody, and voice quality to index executive function, affective state, and neurotoxicity.

Supports intervention and relapse studies

Tracks change across detoxification, behavioural therapy, and pharmacological trials in substance use disorder (SUD) populations.

Passive and active data collection

Enables frequent, low-burden capture of speech samples via mobile or in-clinic environments.

Linked to real-world function

Speech changes align with impairments in decision-making, emotional regulation, and social interaction, which are core domains in SUD recovery.

Validated in psychiatric and neurologic comorbidities

Tools applicable across populations with polysubstance use, dual diagnoses, and long-term neurocognitive sequelae.

Redenlab’s experience in Batten disease (CLN2 & CLN3)

Redenlab has contributed to advancing clinical research in Batten disease, particularly the CLN2 and CLN3 subtypes, through the development and implementation of objective speech and language assessments to capture meaningful functional change. In a key collaborative study published in the Journal of Inherited Metabolic Disease (Morison et al., 2025), Redenlab supported the evaluation of disease progression in CLN2 using standardized clinical and speech-based outcome measures. The study demonstrated that expressive language decline is not only an early indicator of disease but also a sensitive and reliable endpoint for therapeutic monitoring.

Working alongside the Murdoch Children’s Research Institute and through close engagement with families and advocates, including the Batten Disease Support and Research Association (BDSRA) Australia, Redenlab has helped design protocols that balance scientific rigour with family-centred practicality. These partnerships emphasise the importance of functional outcomes that reflect real-world challenges faced by children and caregivers, such as intelligibility, communication independence, and swallowing function.

Redenlab’s tools are now used to support natural history studies, enzyme replacement therapy (ERT) and gene therapy trials, and early-intervention monitoring in CLN2 and CLN3. By leveraging our expertise in paediatric neurodegeneration, we help trial sponsors and clinicians quantify meaningful change over time, particularly in populations where traditional neurocognitive assessments may be limited or insensitive.