Do you know "auditory neuropathy"?

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What impact does auditory neuropathy have on hearing? How effective are hearing aid and cochlear implant interventions? Today, with these questions, the editor will take you to understand auditory neuropathy.


Auditory neuropathy (AN) was first proposed by Starr in 1996, and it was speculated that it is a series of symptoms of auditory dysfunction caused by damage to the eighth cranial nerve. In 2008, It is officially named auditory neuropathy spectrum disorder in the guidelines of the International Conference on Diagnosis and Intervention of Auditory Neuropathy.

Principle of sound transmission
Sound enters the external auditory canal from the auricle, and then to the tympanic membrane from the external auditory canal. The tympanic membrane transmits the vibration to the ossicular chain, which vibrates the lymph fluid and hair cells of the inner ear. Shearing motion in the lymph fluid causes the excitement to be transmitted from the auditory nerve to the nerve center. This is a process (air conduction) in which we can hear the sound completely. Problems in any of the links may cause hearing impairment. .






Common Hearing Loss
Common hearing loss is usually caused by damage to the hair cells in the inner ear; Auditory neuropathy, that is, a disorder of the auditory nerve that conducts excitation, results in the inability to completely transmit the bioelectrical signals generated by the inner ear to the auditory center, or abnormal discharge of the hair cells in the inner ear.






What are the clinical characteristics of auditory neuropathy?
The main clinical characteristics of auditory neuropathy are: patients can hear sounds but cannot understand their meaning. The neural coding function of auditory time domain information is weakened, and the speech recognition ability is reduced, showing a disproportionate decrease in speech recognition rate and pure tone hearing threshold.





Therefore, the effect of Hearing Aids used by this type of patients is relatively limited, and it is difficult to hear clearly what the communication partner is saying. But it does not mean that there is no effect. It just means that both the patient and the hearing aid should establish reasonable expectations and try their best to do what they can to maximize the effect of the hearing aid.


What other help can patients seek?


If it is determined that the hearing aid is not working, what help can the patient seek?

Some patients undergo cochlear implantation, but this is related to the specific location of the disease. If the cochlea is compared to a generator, then the auditory nerve is a wire. Implanting a cochlear implant is equivalent to replacing a generator, but the wire is still the same. If the lesion is in the auditory nerve, rather than abnormal discharge of cochlear hair cells, it cannot be obtained. expected result. Specific lesions can be identified through genetic diagnosis and the genetic loci of the patient's lesions can be compared.






Getting sick is not scary! As long as you adopt a positive attitude to deal with it, you will get corresponding improvements. In daily life, relatives and friends around you should provide support and understanding. When communicating, you should slow down your speech speed instead of raising your volume, and combine body and facial expressions to better understand the communication content.

References:
[1] Wang Jie, Zhang Lifang, Chen Jingyuan, Chen Biao, Li Yongxin. Analysis of long-term hearing rehabilitation effects after cochlear implant surgery in patients with auditory neuropathy spectrum disorders[J]. Chinese Journal of Ear Science, 2021,19(01):1-5.
[2] Shu Bo, Lin Chang, Lin Youhui, Lin Shaolian, Hu Jie, Lin Dingding, Chen Xihang. Auditory brainstem response did not elicit children’s Analysis of hearing test results[J/OL]. Journal of Audiology and Speech Diseases: 1-3[2021-05-06].http://kns.cnki.net/kcms/detail/42.1391.R.20210126.1009.054. html.
[3] Shu Bo, Ye Shengnan, Chen Xihang, Lin Youhui, Lin Shaolian, Hu Jie, Lin Dingding. The application value of cochlear microphonic potential in the diagnosis of auditory neuropathy in infants and young children [J]. Audiology and Speech Journal of Diseases, 2020, 28(06): 624-626.

[4] Xu Bin, Fu Yong. Research progress on genes related to auditory neuropathy [J]. Chinese Journal of Otology, 2020, 18( 04):774-777.

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