Auditory Neuropathy: Challenges and Significant Progress in Diagnosis and Treatment

Wen Xie; Ling Lu; Busheng Tong; Lian Fang; Qingqing Dai; Xiaowan Chen; Jieyu Qi; Xiaoyun Qian; Xia Gao; Renjie Chai; Jun Yang; Maoli Duan

doi:10.1002/adtp.202500513

Auditory Neuropathy: Challenges and Significant Progress in Diagnosis and Treatment

Wen Xie
, Ling Lu^*
, Busheng Tong
, Lian Fang
, Qingqing Dai
, Xiaowan Chen
, Jieyu Qi
, Xiaoyun Qian
, Xia Gao
, Renjie Chai^*
, Jun Yang^*
, Maoli Duan^*

^*Corresponding author for this work

School of Life Science

Nanchang University
Southeast University, Nanjing
Anhui Medical University
The First Affiliated Hospital of Wenzhou Medical University
Sichuan University
Lanzhou University
Beijing Institute of Technology
Nanjing University
Nantong University
University of Electronic Science and Technology of China
Southeast University Shenzhen Research Institute
Shanghai Jiao Tong University
Karolinska Institutet

Research output: Contribution to journal › Review article › peer-review

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.

Original language	English
Article number	e00513
Journal	Advanced Therapeutics
Volume	9
Issue number	3
DOIs	https://doi.org/10.1002/adtp.202500513
Publication status	Published - Mar 2026

Keywords

auditory neuropathy
diagnosis
pathophysiology
treatment

Access to Document

10.1002/adtp.202500513

Cite this

@article{e2c6f799cb9046c2a9d70954c9c20eeb,

title = "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.",

keywords = "auditory neuropathy, diagnosis, pathophysiology, treatment",

author = "Wen Xie and Ling Lu and Busheng Tong and Lian Fang and Qingqing Dai and Xiaowan Chen and Jieyu Qi and Xiaoyun Qian and Xia Gao and Renjie Chai and Jun Yang and Maoli Duan",

note = "Publisher Copyright: {\textcopyright} 2026 The Author(s). Advanced Therapeutics published by Wiley-VCH GmbH.",

year = "2026",

month = mar,

doi = "10.1002/adtp.202500513",

language = "English",

volume = "9",

journal = "Advanced Therapeutics",

issn = "2366-3987",

publisher = "Wiley-Blackwell Publishing Ltd",

number = "3",

}

TY - JOUR

T1 - Auditory Neuropathy

T2 - Challenges and Significant Progress in Diagnosis and Treatment

AU - Xie, Wen

AU - Lu, Ling

AU - Tong, Busheng

AU - Fang, Lian

AU - Dai, Qingqing

AU - Chen, Xiaowan

AU - Qi, Jieyu

AU - Qian, Xiaoyun

AU - Gao, Xia

AU - Chai, Renjie

AU - Yang, Jun

AU - Duan, Maoli

PY - 2026/3

Y1 - 2026/3

N2 - 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.

AB - 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.

KW - auditory neuropathy

KW - diagnosis

KW - pathophysiology

KW - treatment

UR - https://www.scopus.com/pages/publications/105031438296

U2 - 10.1002/adtp.202500513

DO - 10.1002/adtp.202500513

M3 - Review article

AN - SCOPUS:105031438296

SN - 2366-3987

VL - 9

JO - Advanced Therapeutics

JF - Advanced Therapeutics

IS - 3

M1 - e00513

ER -

Auditory Neuropathy: Challenges and Significant Progress in Diagnosis and Treatment

Abstract

Keywords

Access to Document

Other files and links

Fingerprint

Cite this