Mitochondrial disease hearing loss Nervous System Diseases
Conditions
Interventions
Baseline will consist of a number of audiology assessments and completion of a number of questionnaires as measures of quality of life and hearing satisfaction. Clinical data relating to each particip
those randomised to Group 2 will not use the ALD over the next two weeks.
Participants will return for further assessments (audiology tests and questionnaires) after two weeks (Week 2 Visit) at which
Sponsors
Newcastle upon Tyne Hospitals NHS Foundation Trust
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1. Be between 16 years and 70 years (aged =16 to = 70 years) 2. Have ability, in the opinion of the trial team, to participate in trial activities 3. Be capable of providing informed consent 4. Have a genetically confirmed diagnosis of mitochondrial disease 5. Have confirmed hearing loss- via NMDAS hearing score of: 2 (mild), 3 (moderate), or 4 (severe) 6. Have an NMDAS speech score of: 0 (normal), 1 (communication unaffected), or 2 (mild communication difficulties). 7. Have evidence of impaired speech perception in background noise due to: 7.1. Significant sound detection defect measured by a 4-frequency average hearing detection threshold level (pure-tone-audiometry, =60 dBHL in the poorer ear) and/or 7.2. Impaired auditory processing (=2 SDs from the normative mean) assessed by the ‘Speech Reception Threshold’ subtest of the Listening in Specialised Noise-Sentences (LiSN-S) test most reflective of everyday listening 8. If hearing aid users: Use Phonak hearing aids that are compatible with the remote microphone ALD and be willing to continue using hearing aid(s) during the trial. 9. If non-hearing aid users: Be willing and able to use a receiver device e.g., ‘Roger Focus’ which will be paired to the ALD during the device use periods of the trial. This receiver will be provided by the trial alongside the ALD.
Exclusion criteria
Exclusion criteria: 1. Have an NMDAS hearing score of 0 or 1 - no communication problems; tinnitus or deterioration from prior ‘normal’ 2. Have an NMDAS speech score of 3 (moderate difficulties) or above 3. Have profound/end stage hearing loss i.e. NMDAS hearing score of 5 4. Have conductive hearing loss (=10-dB air-bone gap at 500 - 3000 Hz) 5. Have current local ear intolerances or issues preventing hearing aid/ALD effectiveness or use i.e., ear infection, significant earwax 6. Are currently using, or have previously used, a Roger On remote microphone assistive listening device 7. If hearing aid users: 8. Use of non-compatible hearing aids (non-Phonak) 9. Planning a change to their hearing aid(s) during the course of the trial 10. Any other medical issues, which in the opinion of the investigator would preclude involvement
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Tolerability and usability of a Roger On™ remote microphone device assessed via: 1. Tolerability, acceptability and satisfaction measured via an investigator-designed questionnaire at Week 2, Week 4, Week 24. 2. Usability measured via System Usability Scale at Week 2, Week 4, Week 24. 3. Hearing aid satisfaction measured by the International Outcomes Inventory Hearing Aid (IOI-HA) Questionnaire at Week 2, Week 4, Week 24. 4. Compliance measured by participant diary from Week 2 – through to Week 24. 5. Device-related adverse effects and undesirable effects measured by participant diary measured from Week 2 – through to Week 24. 6. Participant withdrawal number and reason(s) for trial withdrawal (assessed as applicable and/or at Withdrawal visit). | — |
Secondary
| Measure | Time frame |
|---|---|
| The efficacy of a Roger On™ remote microphone in adults with mitochondrial disease hearing loss will be assessed via: 1. Spatial processing measured using the Listening in Spatialised Noise-Sentences test (LiSN-S) at Screening and Week 24. 2. Disease severity measured using the Newcastle Mitochondrial Disease Adult Scale at Screening and Week 24. 3. Speech perception measured using the Consonant-Nucleus-Consonant (CNC) test at Baseline, Week 2, Week 4 and Week 24. 4. Hearing and listening ability measured using the Speech, Spatial & Qualities of Hearing Scale (SSQ)-12 at Baseline, Week 2, Week 4 and Week 24. 5. Social participation measured using the Assessment of Life Habits (LIFE-H- Short Form) at Baseline and Week 24. 6. Participation restriction measured using the Social Participation Restrictions Questionnaire (SPaRQ) at Baseline and Week 24. 7. Verbal communication measured using the Communicative Participation Item Bank (CPIB) at Baseline and Week 24. 8. Health-related Quality of Life (QoL) measured using World Health Organisation QoL-BREF at Baseline and Week 24. 9. Perceived fatigue measured using the Modified Fatigue Impact Scale (MFIS) at Baseline and Week 24. 10. Caregiver burden measured using Significant Other Scale for Hearing Disability (SOS-HEAR) at Baseline and Week 24. 11. Speech perception measured using the Consonant-Nucleus-Consonant (CNC) test at Baseline, Week 2, Week 4 and Week 24. 12. Spatial processing measured using the Listening in Spatialised Noise-Sentences test (LiSN-S) at Screening and Week 24. 13. Disease severity measured using the Newcastle Mitochondrial Disease Adult Scale at Screening and Week 24. | — |
Countries
England, United Kingdom
Contacts
Public ContactLaura Brown
Outcome results
None listed