Hidden hearing and speaking problems in CMT

CMT1 is a muscle disorder that affects approximately 1 in 1,500 people. In CMT1, the myelin sheath (protective layer) surrounding the nerves is affected. This project investigates the extent to which CMT1 affects listening and speaking.

Duration: December 2025 until now
Project member(s): Dr M.A.M. van de Ven (Marco) Prof. P.C.J. Segers (Eliane) Dr E. Janse (Esther) Dr M.M. van Wanrooij (Marc) N.C. Haukes (Nina) B. Caddeo (Beatrice) MSc , Gary Rance - University of Melbourne , Julien Zanin - University of Melbourne , Simone Knuijt - RadboudUMC , Marloes Lagarde - RadboudUMC , José Marques - DCCN , Cris Lanting - RadboudUMC , Alexandra Jesse - UMass Amherst
Project type: Research
Organisation: Learning & Learning Problems, Faculty of Social Sciences, Donders Institute for Brain, Cognition and Behaviour, Behavioural Science Institute

This research project consists of four sub-studies, the first two of which have now been launched. A description of these two sub-studies follows below.

Hidden hearing problems in adults with CMT1 (HMSN type 1)

In this study, we are investigating how well listeners with CMT1 can determine where sounds are coming from and what is being said in the presence of background noise.

Hidden hearing and speech problems in children with CMT1 (HMSN type 1)

In this study, we are investigating how well children (aged 8–16) with CMT1 can localise the source of a sound and understand what is being said in the presence of background noise. We are also assessing how well they can perform speech tasks (such as quickly repeating words or speaking loudly, etc.).

Both studies are taking place in a soundattenuated room in the Huygens Building on our campus.

Results

Results from an initial, exploratory questionnaire study (van de Ven, 2025) show that patients with CMT1 experience more listening difficulties in daily life than people with normal hearing. As a result of these listening difficulties, patients with CMT1 may tend to avoid social situations or feel insecure during social interactions. Listening difficulties can also cause CMT1 patients to have trouble concentrating while listening.

As part of the current research project, we will be conducting a series of experimental studies (involving sound localisation, speech processing in noise, speech tests and MRI) to investigate in greater detail how CMT1 affects listening and speaking.