Patients with different types of spinocerebellar ataxias showed significant improvement in their ability to hear and perceive complex sounds — essential to participating in everyday conversations — when wearing a wireless listening device, a small study showed.
The data was presented at IARC 2017 by Kay Uus with the University of Manchester on Friday. The presentation, “Auditory dysfunction and its remediation in individuals with spinocerebellar ataxia,” was part of Session 3: Natural History, Biomarkers and Endpoints at IARC 2o17, the International Ataxia Research Conference, running in Pisa, Italy, through Saturday.
The study was supported by Ataxia UK, a patients’ advocacy group and a conference organizer.
Spinocerebellar ataxias (SCAs) are a heterogeneous group of neurological disorders characterized by degenerative changes in the cerebellum, the part of the brain that controls movement, and in neurons in the spinal cord. There are many different types of SCA, and they are identified according to the mutated (altered) gene responsible for the disease.
In the presentation, Uus discussed the auditory consequences of three specific disease types — SCA1, SCA2 and SCA6 — and reported on the potential for easing difficulties in everyday listening and communicating if those affected wore a remote-microphone listening device.
But, she noted, “sound detection threshold is not a problem for patients, but this is the main test in clinical trials,” emphasizing that this test “just tells a very small part of the story.”
The study enrolled 14 adults (seven with SCA1, two with SCA2, and five with SCA6), ages 44 to 76 at assessment, who underwent a comprehensive evaluation of their hearing capacities, including peripheral hearing mechanisms (sound detection, cochlear mechanics), auditory neural activity (auditory brainstem response), and functional hearing (monosyllabic speech perception, self-reported communication disability).