Muthukumar R, Jaya V, Vignesh S S, Thenmozhi K
Upgraded Institute of Otorhinolaryngology and Institute of Speech and Hearing, Madras Medical college and Rajiv Gandhi Government General hospital, Chennai, 600003 India.
Institute of Speech and Hearing, Madras Medical college and Rajiv Gandhi Government General hospital, Chennai, Tamil Nadu 600003 India.
Indian J Otolaryngol Head Neck Surg. 2023 Sep;75(3):1906-1911. doi: 10.1007/s12070-023-03766-6. Epub 2023 Apr 19.
Auditory neuropathy spectrum disorder (ANSD) is a heterogenous group of disorder characterized by abnormalities in auditory brainstem responses (ABR) with preserved otoacoustic emissions and/or cochlear microphonics. The aim of the study is to estimate the prevalence and evaluate the audiological characteristics of ANSD in adult population with sensory neural hearing loss. A prospective study was conducted on the adult population (≥ 18 years) attending ENT outpatients clinic at Rajiv Gandhi Government General Hospital, Chennai. All patients reported to the department with auditory and vestibular symptoms underwent case history, otoscopic examination, and routine audiological evaluation (pure tone audiometry, speech audiometry and immittance audiometry). Patients with indications of ANSD in case history and routine audiological evaluation were further evaluated using distortion product otoacoustic emissions and ABR. A total of 8682 adult population was evaluated during the period of 2017 to 2018. Out of 8682 patients, 1343 (15.46%) of them had sensory neural hearing loss of varying degrees. Out of 1343 adults with sensory neural hearing loss, 24 (1.78%) adults were diagnosed as ANSD. The prevalence of ANSD in adult population with sensory neural hearing loss in our study is 1.32% per 1000 adults. The clinical characteristics of ANSD shows impairment in speech perception irrespective of degree of hearing loss, preserved cochlear functions and abnormal ABR. Hence ANSD is not a rare clinical finding in adults with sensory neural hearing loss, but its prevalence was estimated to be lower in Indian population. Often young females are affected causing significant impairment in speech perception and disability.
听觉神经病谱系障碍(ANSD)是一组异质性疾病,其特征是听觉脑干反应(ABR)异常,而耳声发射和/或耳蜗微音电位正常。本研究的目的是评估成人感音神经性听力损失人群中ANSD的患病率,并评估其听力学特征。对在金奈拉吉夫·甘地政府综合医院耳鼻喉科门诊就诊的成年人群(≥18岁)进行了一项前瞻性研究。所有因听觉和前庭症状前来该科室就诊的患者均接受了病史采集、耳镜检查和常规听力学评估(纯音听力计、言语听力计和声导抗测听)。对病史和常规听力学评估中有ANSD指征的患者,进一步使用畸变产物耳声发射和ABR进行评估。在2017年至2018年期间,共评估了8682名成年人群。在8682名患者中,1343名(15.46%)患有不同程度的感音神经性听力损失。在1343名患有感音神经性听力损失的成年人中,24名(1.78%)被诊断为ANSD。在我们的研究中,成年感音神经性听力损失人群中ANSD的患病率为每1000名成年人中有1.32%。ANSD的临床特征表现为无论听力损失程度如何,言语感知均受损,耳蜗功能正常,ABR异常。因此,ANSD在成年感音神经性听力损失患者中并非罕见的临床发现,但其在印度人群中的患病率估计较低。通常年轻女性易受影响,导致言语感知严重受损和残疾。