![]() Cochlear implantation of auditory neuropathy. ![]() Speech and language outcomes in children with auditory neuropathy/dys-synchrony managed with either cochlear implants or hearing aids. A dominantly inherited progressive deafness affecting distal auditory nerve and hair cells. ![]() Auditory synaptopathy, auditory neuropathy, and cochlear implantation. ATP1A3 mutations can cause progressive auditory neuropathy: a new gene of auditory synaptopathy. Pathology and physiology of auditory neuropathy with a novel mutation in the MPZ gene (Tyr145->Ser). Mutations in the gene encoding pejvakin, a newly identified protein of the afferent auditory pathway, cause DFNB59 auditory neuropathy. Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy. Loss of ca(v)1.3 (CACNA1D) function in a human channelopathy with bradycardia and congenital deafness. Increased activity of diaphanous homolog 3 (DIAPH3)/diaphanous causes hearing defects in humans with auditory neuropathy and in drosophila. Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene. Auditory nerve disease of both ears revealed by auditory brainstem responses, electrocochleography and otoacoustic emissions. Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI. Moreover, we review previous reports of EABR waveforms and propose a new system for classification of EABR patterns in patients with auditory neuropathy by combining the different pathophysiological mechanisms of this heterogeneous disease. Herein, we present examples of EABR in patients with auditory neuropathy and discuss the importance of EABR in this field. While they lack ordinal sound-evoked ABR, cochlear implant-mediated EABR may be present in several patients. Some patients with auditory neuropathy have severe-to-profound hearing loss and undergo cochlear implantation surgery. Clinically, patients with auditory neuropathy lack the auditory brainstem response (ABR) induced by auditory stimulation. In this chapter, we review the applications of EABR in hearing loss and auditory neuropathy.Īuditory neuropathy (or auditory neuropathy spectrum disease) is a heterogeneous disease characterized by absent or abnormal auditory nerve function but normal hair cell function. Recording electrical auditory brainstem responses (EABRs) is useful for investigating the pathophysiology of hearing loss as well as for predicting the outcomes of cochlear implantation.
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