J Basic Clin Physiol Pharmacol 11(3):215–230īoudewyns A, Declau F, van den Ende J, Hofkens A, Dirckx S, Van de Heyning P (2016) Auditory neuropathy spectrum disorder (ANSD) in referrals from neonatal hearing screening at a well-baby clinic. Starr A, Sininger YS, Pratt H (2000) The varieties of auditory neuropathy. Kumar UA, Jayaram MM (2006) Prevalence and audiological characteristics in individuals with auditory neuropathy/auditory dys-synchrony. Lepcha A, Chandran RK, Alexander M, Agustine AM, Thenmozhi K, Balraj A (2015) Neurological associations in auditory neuropathy spectrum disorder: results from a tertiary hospital in South India. Shivashankar N, Satishchandra P, Shashikala HR, Gore M (2003) Primary auditory neuropathy - an enigma. Prabhu P, Avilala VKY, Manjula PP (2012) Predisposing factors in individuals with late-onset auditory dys-synchrony. Xoinis K, Weirather Y, Mavoori H, Shaha SH, Iwamoto LM (2007) Extremely low birth weight infants are at high risk for auditory neuropathy. Int J Pediatr Otorhinolaryngol 75(2):151–158 Manchaiah VK, Zhao F, Danesh AA, Duprey R (2011) The genetic basis of auditory neuropathy spectrum disorder (ANSD). Int J Audiol 49(1):30–43īielecki I, Horbulewicz A, Wolan T (2012) Prevalence and risk factors for auditory neuropathy spectrum disorder in a screened newborn population at risk for hearing loss. Pediatrics 116(4):933–938īerlin CI, Hood LJ, Morlet T, Wilensky D, Li L, Mattingly KR et al (2010) Multi-site diagnosis and management of 260 patients with auditory Neuropathy/Dys-synchrony (auditory neuropathy spectrum Disorder*). NeuroReport 10(16):3429–3435īerg AL, Spitzer JB, Towers HM, Bartosiewicz C, Diamond BE (2005) Newborn hearing screening in the NICU: profile of failed auditory brainstem response/passed otoacoustic emission. Zeng FG, Oba S, Garde S, Sininger Y, Starr A (1999) Temporal and speech processing deficits in auditory neuropathy. ![]() Zeng FG, Kong YY, Michalewski HJ, Starr A (2005) Perceptual consequences of disrupted auditory nerve activity. Rance G, McKay C, Grayden D (2004) Perceptual characterization of children with auditory neuropathy. Rance G, Cone-Wesson B, Wunderlich J, Dowell R (2002) Speech perception and cortical event related potentials in children with auditory neuropathy. ![]() Kraus N, Bradlow AR, Cheatham MA, Cunningham J, King CD, Koch DB et al (2000) Consequences of neural asynchrony: a case of auditory neuropathy. Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI (1996) Auditory neuropathy. ![]() Sininger Y, Starr A (2001) Auditory neuropathy: a new perspective on hearing disorders. Rance G, Beer DE, Cone-Wesson B, Shepherd RK, Dowell RC, King AM et al (1999) Clinical findings for a group of infants and young children with auditory neuropathy. Hood LJ, Berlin CI, Bordelon J, Rose K (2003) Patients with auditory neuropathy/dys-synchrony lack efferent suppression of transient evoked otoacoustic emissions. Rance G, Starr A (2015) Pathophysiological mechanisms and functional hearing consequences of auditory neuropathy. Often young females are affected causing significant impairment in speech perception and disability. 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. The clinical characteristics of ANSD shows impairment in speech perception irrespective of degree of hearing loss, preserved cochlear functions and abnormal ABR. The prevalence of ANSD in adult population with sensory neural hearing loss in our study is 1.32% per 1000 adults. Out of 1343 adults with sensory neural hearing loss, 24 (1.78%) adults were diagnosed as ANSD. Out of 8682 patients, 1343 (15.46%) of them had sensory neural hearing loss of varying degrees. A total of 8682 adult population was evaluated during the period of 2017 to 2018. Patients with indications of ANSD in case history and routine audiological evaluation were further evaluated using distortion product otoacoustic emissions and ABR. 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). A prospective study was conducted on the adult population ( ≥ 18 years) attending ENT outpatients clinic at Rajiv Gandhi Government General Hospital, Chennai. ![]() 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. 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.
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