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).
The tests were also performed on 130 healthy controls (ages 24 to 76).
After the evaluation, each SCA participant received an ISense FM listening device, a new FM (wireless) receiver specially developed for people with difficulties in understanding what’s being said in complex listening situations. The device helps people with hearing loss to reduce background noise.
Uus explained in her presentation that the device uses a “system that transmits speech signals (detected by a microphone worn by the the speaker) via radio waves to an ear-level receiver worn by the listener.”
Patients tried the device at home for six weeks. Most, regardless of SCA type, entered the test with evident “auditory neural dysfunction and abnormal function hearing,” Uus said.
“What we saw was that quite a proportion of patients had troubles listening to speech in a background noise,” she said, and “the majority also struggles with communication.”
Speech discrimination amid background noise was severely affected, with 12 out of the 14 patients performing below average. Everyday listening skills were impaired in most of the patients.
“Ten of 14 reported extreme difficulty understanding conversation in background noise and 9/14 felt that their hearing challenges adversely affected communication ability,” the researchers wrote.
The FM listening system allowed them to ably overcome these difficulties, improving “the results quite dramatically, in most cases going to the normal ranges,” Uus said.
Speech perception scores rose to normal range in 13 of the 14 while using the device. Ten of the 14 also reported significant improvement in everyday listening, while “7 felt communication was easier” during the six-week trial, Uus said.
“Sadly, there are still those who didn’t reach normal levels,” she added.
Taken together, the results showed that auditory dysfunction is common in SCA1, SCA2 and SCA6 patients, and remote microphone listening devices may be a viable intervention option for them.
“Although a small and preliminary study, we find it quite exciting and promising,” Uus concluded.