Auditory Neuropathy: Challenges and Significant Progress in Diagnosis and Treatment
Year: 2026
Type: article
Abstract: ABSTRACT Auditory neuropathy (AN) is a complex disorder where sensorineural hearing impairment occurs due to abnormal neural encoding to sound stimuli. This can be caused by damage to sensory inner hair cells (IHCs)‐presynapse, IHC ribbon synapses, or spiral ganglion neurons via post‐synapse. Genetic factors play a significant role in AN. Audiological assessment such as otoacoustic emissions, auditory brainstem responses, and speech audiometry is crucial in diagnosing auditory neuropathy. Hearing aids and cochlear implants are currently commonly used methods for interventing AN. Owing to the intricate nature of the disease's pathogenesis, a standardized diagnostic approach has yet to be established, and therapeutic options remain significantly constrained. However, it is noteworthy that breakthrough progress has been made in the treatment of AN. In 2024, Chinese researchers used adeno‐associated virus gene therapy for AN patients with OTOF gene mutations, and achieved exceptionally good results in clinical trials. This review focuses on auditory neuropathy, covering its theoretical foundations, epidemiology, diagnostic techniques, treatment strategies, clinical practice, as well as future directions and controversie.
Source: Advanced Therapeutics
Institutions: Second Affiliated Hospital of Nanchang University, Zhongda Hospital Southeast University, Anhui Medical University, First Affiliated Hospital of Anhui Medical University, First Affiliated Hospital of Wenzhou Medical University, Sichuan University, West China Medical Center of Sichuan University, West China Hospital of Sichuan University, First Hospital of Lanzhou University, Beijing Institute of Technology, Zhuhai People's Hospital, Nanjing Drum Tower Hospital, Nanjing University, University of Electronic Science and Technology of China, Nantong University, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, State Key Laboratory of Digital Medical Engineering, Southeast University, Shanghai Jiao Tong University, XinHua Hospital, Karolinska University Hospital, Karolinska Institutet
Language: English
Subfield: Sensory Systems
Field: Neuroscience
Domain: Life Sciences
Open Access status: hybrid
Estimated APC paid: $4,430
Award: G8250856918
OpenAlex ID: W7134167549