Auditory Neuropathy
Conditions
Interventions
case series:NADH(20-80mg/day) and CoQ10 (30-60mg/day)
Sponsors
Chinese PLA General Hospital
Eligibility
Sex/Gender
All
Age
14 Years to No maximum
Inclusion criteria
Inclusion criteria: 1) Meet the current international consensus criteria for the diagnosis of auditory neuropathy, i.e., the main clinical manifestations are that the patient can hear sounds but cannot understand their semantics, the patient's speech recognition rate is disproportionately reduced to the pure tone hearing threshold, the function of the hair cells is normal - Otoacoustic Emissions (OAE) and/or Cochlear Microphonics (CM) can be elicited, while abnormal auditory nerve function - Auditory Brainstem Responses (ABRs) are abnormal or completely absent; 2) AIFM1 gene mutation; 3) the age of onset of the disease is not limited to any age and gender (AIFM1 gene mutation-related auditory neuropathy is an X-linked genetic disorder, so it is prevalent in males). (AIFM1 gene mutation-associated auditory neuropathy is X-linked, so it is predominantly male), and the age of consultation is 14 years old or above; 4) patients and/or guardians voluntarily participate in this program and sign an informed consent form.
Exclusion criteria
Exclusion criteria: 1) pregnant, perinatal, and lactating women; 2) lesions such as middle ear lesions, Meniere's disease, acoustic neuroma, and greater vestibular aqueduct syndrome; 3) severe systemic diseases such as tumors, severe infections, cerebrovascular accidents, uremia, connective tissue disease, and acute stress; 4) cognitive disorders such as psychiatric disorders that interfere with adherence; 5) those who are poorly adherent to the regimen in the judgment of the investigator; 6) those who have had a unilateral or bilateral cochlear implantation; 7) patients who are deemed by the investigator to not be eligible for enrollment in this trial for other factors.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Hearing tests; | — |
Secondary
| Measure | Time frame |
|---|---|
| vestibular function tests;laboratory tests;imaging tests; | — |
Countries
China
Contacts
Public ContactWang Qiuju
Chinese PLA General Hospital
Outcome results
None listed