Comprehensive auditory evaluations were conducted on OPA1-related patients, and some of them were performed a follow-up study.<h4>Results</h4>We identified seven probands (1.55%, 7/452) with OPA1 variants in seven unrelated families, demonstrating distinct genetic patterns, including one family with rare autosomal recessive (AR) inheritance, six families with autosomal dominant (AD) inheritance (three were AD de novo). The gene discussed is OPA1; the disease is Alzheimer disease.