title: Auditory nerve disease and auditory neuropathy spectrum disorders.
abstract: In 1996, a new type of bilateral hearing disorder was discerned and published almost simultaneously by Kaga et al. [1] and Starr et al. [2]. Although the pathophysiology of this disorder as reported by each author was essentially identical, Kaga used the term "auditory nerve disease" and Starr used the term "auditory neuropathy". Auditory neuropathy (AN) in adults is an acquired disorder characterized by mild-to-moderate pure-tone hearing loss, poor speech discrimination, and absence of the auditory brainstem response (ABR) all in the presence of normal cochlear outer hair cell function as indicated by normal distortion product otoacoustic emissions (DPOAEs) and evoked summating potentials (SPs) by electrocochleography (ECoG). A variety of processes and etiologies are thought to be involved in its pathophysiology including mutations of the OTOF and/or OPA1 genes. Most of the subsequent reports in the literature discuss the various auditory profiles of patients with AN [3,4] and in this report we present the profiles of an additional 17 cases of adult AN. Cochlear implants are useful for the reacquisition of hearing in adult AN although hearing aids are ineffective. In 2008, the new term of Auditory Neuropathy Spectrum Disorders (ANSD) was proposed by the Colorado Children's Hospital group following a comprehensive study of newborn hearing test results. When ABRs were absent and DPOAEs were present in particular cases during newborn screening they were classified as ANSD. In 2013, our group in the Tokyo Medical Center classified ANSD into three types by following changes in ABRs and DPOAEs over time with development. In Type I, there is normalization of hearing over time, Type II shows a change into profound hearing loss and Type III is true auditory neuropathy (AN). We emphasize that, in adults, ANSD is not the same as AN.
authors.affiliation: National Institute of Sensory Organs, National Tokyo Medical Center, Japan; Center for Speech and Hearing Disorders, International University of Health and Welfare, Japan. Electronic address: kaga@kankakuki.go.jp.
authors.forename: Kimitaka
authors.initials: K
authors.lastname: Kaga
mesh_terms: D000328:Adult; D001300:Audiometry, Evoked Response; D019929:Cochlear Implantation; D003054:Cochlear Implants; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology*; D020558:GTP Phosphohydrolases / Q000235:genetics; D006310:Hearing Aids; D006313:Hearing Loss, Central / Q000235:genetics / Q000503:physiopathology* / Q000534:rehabilitation; D006801:Humans; D007231:Infant, Newborn; D008565:Membrane Proteins / Q000235:genetics; D015997:Neonatal Screening; D017084:Otoacoustic Emissions, Spontaneous / Q000502:physiology*
chemical_list: D008565:Membrane Proteins; C118457:OTOF protein, human; D020558:GTP Phosphohydrolases; C415198:OPA1 protein, human
keywords: ABR; Auditory nerve disease; Auditory neuropathy; Auditory neuropathy spectrum disorders; DPOAE; OPA1; OTOF