Auditory Neuropathy: Challenges and Significant Progress in Diagnosis and Treatment

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.

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Publication Details

Journal
Advanced Therapeutics
Published
2026-02-28
DOI
https://doi.org/10.1002/adtp.202500513
Primary Topic
Hearing, Cochlea, Tinnitus, Genetics
Type
article
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0.00
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article

Auditory Neuropathy: Challenges and Significant Progress in Diagnosis and Treatment

Jie Yang, Xiang Gao, Qingqing Dai, Jieyu Qi et al.
Advanced Therapeutics
Hearing, Cochlea, Tinnitus, Genetics
article

Auditory Neuropathy: Challenges and Significant Progress in Diagnosis and Treatment

Jie Yang, Xiang Gao, Qingqing Dai, Jieyu Qi, Renjie Chai, Ling Lü, Busheng Tong, Maoli Duan, Xiaoyun Qian, XiaoWan Chen, Wen Xie, Lian Fang
article en

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.

Advanced TherapeuticsVol. 9(3)
Beijing Institute of Technology (CN), Karolinska University Hospital (SE), Nanchang University (CN), University of Electronic Science and Technology of China (CN), Anhui Medical University (CN), Nantong University (CN), Beijing Electronic Science and Technology Institute (CN), Sichuan University (CN), Wenzhou Medical University (CN), XinHua Hospital (CN), First Affiliated Hospital of Wenzhou Medical University (CN), Karolinska Institutet (SE), West China Hospital of Sichuan University (CN), Zhongda Hospital Southeast University (CN), Second Affiliated Hospital of Nanchang University (CN), Jinan Institute of Quantum Technology (CN), Nanjing Drum Tower Hospital (CN), First Affiliated Hospital of Anhui Medical University (CN), First Hospital of Lanzhou University (CN), State Key Laboratory of Digital Medical Engineering (CN), Lanzhou University (CN), Southeast University (CN)
Good health and well-being
Openalex Percentile: Top 26%
Hearing, Cochlea, Tinnitus, Genetics
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