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Review

Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports

Department of Oto-Rhino-Laryngology and Head and Neck Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 10 Avenue Hippocrate, 1200 Brussels, Belgium
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Author to whom correspondence should be addressed.
J. Clin. Med. 2020, 9(4), 1074; https://doi.org/10.3390/jcm9041074
Submission received: 28 February 2020 / Revised: 21 March 2020 / Accepted: 1 April 2020 / Published: 10 April 2020
(This article belongs to the Special Issue Therapies for Hearing Loss)

Abstract

Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by deteriorated speech perception, despite relatively preserved pure-tone detection thresholds. Affected individuals usually present with abnormal auditory brainstem responses (ABRs), but normal otoacoustic emissions (OAEs). These electrophysiological characteristics have led to the hypothesis that ANSD may be caused by various dysfunctions at the cochlear inner hair cell (IHC) and spiral ganglion neuron (SGN) levels, while the activity of outer hair cells (OHCs) is preserved, resulting in discrepancies between pure-tone and speech comprehension thresholds. The exact prevalence of ANSD remains unknown; clinical findings show a large variability among subjects with hearing impairment ranging from mild to profound hearing loss. A wide range of prenatal and postnatal etiologies have been proposed. The study of genetics and of the implicated sites of lesion correlated with clinical findings have also led to a better understanding of the molecular mechanisms underlying the various forms of ANSD, and may guide clinicians in better screening, assessment and treatment of ANSD patients. Besides OAEs and ABRs, audiological assessment includes stapedial reflex measurements, supraliminal psychoacoustic tests, electrocochleography (ECochG), auditory steady-state responses (ASSRs) and cortical auditory evoked potentials (CAEPs). Hearing aids are indicated in the treatment of ANSD with mild to moderate hearing loss, whereas cochlear implantation is the first choice of treatment in case of profound hearing loss, especially in case of IHC presynaptic disorders, or in case of poor auditory outcomes with conventional hearing aids.
Keywords: ANSD; auditory neuropathy spectrum disorder; auditory synaptopathy; hidden hearing loss; genetics; cochlear implant ANSD; auditory neuropathy spectrum disorder; auditory synaptopathy; hidden hearing loss; genetics; cochlear implant

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MDPI and ACS Style

De Siati, R.D.; Rosenzweig, F.; Gersdorff, G.; Gregoire, A.; Rombaux, P.; Deggouj, N. Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports. J. Clin. Med. 2020, 9, 1074. https://doi.org/10.3390/jcm9041074

AMA Style

De Siati RD, Rosenzweig F, Gersdorff G, Gregoire A, Rombaux P, Deggouj N. Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports. Journal of Clinical Medicine. 2020; 9(4):1074. https://doi.org/10.3390/jcm9041074

Chicago/Turabian Style

De Siati, Romolo Daniele, Flora Rosenzweig, Guillaume Gersdorff, Anaïs Gregoire, Philippe Rombaux, and Naïma Deggouj. 2020. "Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports" Journal of Clinical Medicine 9, no. 4: 1074. https://doi.org/10.3390/jcm9041074

APA Style

De Siati, R. D., Rosenzweig, F., Gersdorff, G., Gregoire, A., Rombaux, P., & Deggouj, N. (2020). Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports. Journal of Clinical Medicine, 9(4), 1074. https://doi.org/10.3390/jcm9041074

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