Congenital unilateral severe hearing loss or an acquired unilateral severe hearing loss unilateral severe hearing loss
Conditions
Interventions
Patients with unilateral severe hearing loss (SSD) are assigned to a congenital SSD group and an acquired SSD group, and AMPA-PET scans using an unapproved investigational drug are performed in both g
AMPA-PET
Sponsors
Kashio Akinori
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: (1) Males and females aged >= 18 and = 70 dB HL (4-frequency average) in standard pure tone audiometry. (3) Individuals who have had a hearing level of >= 70 dB HL (severe hearing loss) in the worse ear for 6 months or more. (4) Patients with unilateral severe hearing loss whose hearing level in the worse ear became >= 70 dB HL between birth and 6 years of age (congenital SSD), or patients whose hearing level in the worse ear became >= 70 dB HL at the age of 6 years or older (acquired SSD). (5) Right-handed individuals with a Laterality Quotient of >= +40 in the Edinburgh Handedness Inventory. (6) Individuals capable of independent walking and answering questionnaires. (7) Individuals from whom written consent regarding participation in this study can be obtained.
Exclusion criteria
Exclusion criteria: (1) Individuals unable to undergo head MRI imaging due to implanted metal objects (pacemakers, deep brain stimulators, etc.). (2) Individuals with severe claustrophobia who cannot enter the MRI scanner. (3) Individuals with tattoos or permanent makeup whom the investigator judges that undergoing MRI may pose a safety risk. (4) Women who are pregnant, breastfeeding, or wish to become pregnant during the study period. (5) Individuals with hypersensitivity to alcohol. (6) Individuals with either AST or ALT >= 150 IU/L, or serum Cr >= 2.0 mg/dL in blood tests. (7) Individuals with a history of neurosurgery or those scheduled for neurosurgery during the study period. (8) Individuals with significant brain structural malformations (congenital or traumatic). (9) Individuals currently being treated for schizophrenia, depression, bipolar disorder, or autism spectrum disorder (ASD). (10) Individuals requiring continued oral administration of perampanel (antiepileptic drug) or topiramate (antiepileptic drug). (11) Individuals currently undergoing treatment for active inflammation in the middle ear of the better ear side. (12) Individuals likely to receive unapproved treatments or examinations in other clinical trials/clinical studies during the participation period of this study. (13) Any other individuals whom the principal investigator judges to be unsuitable as study subjects.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Between-group comparison of the laterality index(pSTG = pSTG_ipsi - pSTG_contra)based on white matter-corrected SUVR of the posterior superior temporal gyrus (pSTG) measured by[11C]K-2 PET, in adults with congenital unilateral severe hearing loss and those with acquired unilateral severe hearing loss. Ipsilateral (ipsi) and contralateral (contra) sides are defined by re-labeling each subject's hemispheres according to the side of hearing loss (ex, in right-sided hearing loss, the right pSTG is defined as ipsi and the left pSTG as contra). | — |
Secondary
| Measure | Time frame |
|---|---|
| (1)Voxel-wise comparison of AMPA receptor density (SUVR) measured by [11C]K-2 PET in adults with congenital unilateral severe hearing loss and those with acquired unilateral severe hearing loss, using healthy control data obtained from an independent study (jRCTs031200083). To ensure comparability, healthy controls will be selected by 1:1 nearest-neighbor matching without replacement, based on sex and age (+- 5 years), as pre-specified. Primary analysis will be conducted on the matched cohorts. Voxel-wise analyses will be performed across the whole brain, and appropriate correction for multiple comparisons (ex, false discovery rate [FDR]) will be applied. (2)Voxel-wise comparison of AMPA receptor density (SUVR) measured by [11C]K-2 PET between adults with unilateral severe hearing loss (congenital and acquired) and adults with bilateral severe sensorineural hearing loss, using data obtained from an independent study (jRCTs031230617). Given the limited sample size of the external control group, no matching will be performed, and all available cases (n = 30) will be included in the analysis. Group comparisons will be conducted using voxel-wise analyses with adjustment for age and sex as covariates. Appropriate correction for multiple comparisons (ex, FDR) will be applied. (3)Voxel-wise correlation between AMPA receptor density (SUVR) measured by [11C]K-2 PET and speech perception performance in noise (OLSA) in adults with congenital and acquired unilateral severe hearing loss. (4)Voxel-wise correlation between AMPA receptor density (SUVR) measured by [11C]K-2 PET and sound localization performance in adults with congenital and acquired unilateral severe hearing loss. | — |
Contacts
Public ContactToshihito Sahara
The University of Tokyo Hospital
Outcome results
None listed