Observational study on mitochondrial hearing loss.

UMIN ID: UMIN000055532

Registered date:01/10/2024

Basic Information

• Recruitment status: Pending
• Health condition(s) or Problem(s) studied: mitochondrial hearing loss
• Date of first enrollment: 2024/08/01
• Target sample size: 12
• Countries of recruitment: Japan
• Study type: Observational

Outcome(s)

• Primary Outcome: When subjects attend for routine medical care, their hearing is measured with the ABR test for cerebral auditory response, the ASSR test for auditory steady-state response, standard pure tone audiometry and the DPOAE test for otoacoustic emissions (DPOAE) and is assessed for changes in hearing during the observation period.

Key inclusion & exclusion criteria

• Age minimum: 16years-old
• Age maximum: 59years-old
• Gender: Male and Female
• Exclude criteria: 1. Subjects with cognitive impairment who, in the opinion of the investigator or sub-investigator, may have difficulty with JMDRS or NMDPS/NMDAS scoring. 2. Subjects with concomitant hearing loss due to causes other than mitochondrial disease 3. Subjects with cochlear implants 4. Subjects with severe hearing loss 5. Subjects on a respirator 6. Subjects with epileptic seizures (convulsions) and severe coma 7. Subjects with septic complications 8. Subjects with renal impairment (estimated glomerular filtration rate eGFR less than 30 mL/min/1.73 m2). 9. Subjects who have participated in other clinical trials within 6 months prior to consent. Other subjects deemed inappropriate by the principal investigator or sub-investigator.

Related Information

• Primary Sponsor: Tohoku University
• Source(s) of Monetary Support: AMED