A Single-Center, Double-Blind, Randomized Controlled Trial on the Therapeutic Efficacy of NADH in Patients with AIFM1 Mutation-Positive Auditory Neuropathy

INTERVENTION: NADH Group:Oral administration of NADH (40 mg/day) 30 minutes before breakfast each day for 12 weeks. ;Placebo Group: Daily oral administration of placebo, identical in formulation and appearance to the NADH group, for 12 consecutive weeks.; CONDITION: Auditory Neuropathy PRIMARY OUTCOME: Hearing tests;Auditory speech perception;Speech perception in noisy environments;Auditory cortex information processing; SECONDARY OUTCOME: Appraisal of life quality;Cognitive and emotional states; 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 prog