Prevalence and Auditory Characteristics of Auditory Neuropathy Spectrum Disorder in Adult Population with Sensory Neural Hearing Loss: A Hospital Based Study in South India

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.