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Auditory Neuropathy: Challenges and Significant Progress in Diagnosis and Treatment

delete2026-02-28
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OA
AI
W
Wen Xie
L
Ling Lü *
B
Busheng Tong
L
Lian Fang
Q
Qingqing Dai
X
XiaoWan Chen
齐洁玉 cover
齐洁玉 (Jieyu Qi)
X
Xiaoyun Qian
X
Xia Gao
R
Renjie Chai *
J
Jun Yang *
M
Maoli Duan *
DOI:10.1002/adtp.202500513delete
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Abstract

Abstract

En 中文
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.
Keywords:
auditory neuropathy
diagnosis
pathophysiology
treatment
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Advanced Therapeutics
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