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Auditory synaptopathy — Clinical Guidelines

Overview

Auditory synaptopathy refers to damage to the synapses of the inner ear, particularly affecting the auditory nerve fibers, leading to symptoms such as hearing loss and auditory processing difficulties without significant changes in pure-tone audiometry 1 7 16.

Diagnosis

Behavioral Audiometry: Often normal pure-tone thresholds, but may reveal deficits in speech-in-noise tests or frequency discrimination 1 7 16.

Electrophysiological Tests: Auditory brainstem response (ABR) may show subtle abnormalities; auditory steady-state response (ASSR) and auditory evoked potentials can provide additional insights 1 2 7.

Central Auditory Processing Tests: Utilize tests like dichotic listening, temporal processing, and binaural interaction tasks to identify deficits 6 13 16.

Imaging: Not typically indicated unless central nervous system involvement is suspected 16.

Management

Sedation for Testing:

- Intranasal Dexmedetomidine: Initial dose of 2.5 μg/kg for children undergoing ABR testing 1. - Chloral Hydrate: Oral dose of 40 mg/kg, with repeat doses if necessary, commonly used for sedation in pediatric ABR testing 7 9. - Propofol-Ketamine Regimen: Alternative to chloral hydrate, effective but requires careful monitoring 3.

Auditory Rehabilitation: Tailored interventions focusing on auditory processing strategies and compensatory techniques 6 12.

Special Populations

Pediatrics: Sedation methods like intranasal dexmedetomidine and chloral hydrate are frequently used and generally safe 1 7 9.

Comorbidities: No specific guidelines provided in abstracts; individualized management based on coexisting conditions is recommended 16.

Key Recommendations

Utilize behavioral and electrophysiological assessments to diagnose auditory synaptopathy beyond standard audiometry 1 7 16 (Evidence: Moderate).

Employ sedation strategies such as intranasal dexmedetomidine or chloral hydrate for pediatric ABR testing to ensure compliance and accurate results 1 7 9 (Evidence: Moderate).

Implement individualized auditory rehabilitation programs addressing specific processing deficits identified through comprehensive testing 6 12 (Evidence: Weak).

References

1 Giordano A, Lehner B, Voicu A, Donzeau D, Joulie A, Froissant L et al.. Intranasal dexmedetomidine for sedation in ABR testing in children: No pain, big gain!. International journal of pediatric otorhinolaryngology 2024. link 2 Wong K, Kiringoda R, Kanumuri VV, Barber SR, Franck K, Sahani N et al.. Effect of anesthesia on evoked auditory responses in pediatric auditory brainstem implant surgery. The Laryngoscope 2020. link 3 Abulebda K, Patel VJ, Ahmed SS, Tori AJ, Lutfi R, Abu-Sultaneh S. Comparison between chloral hydrate and propofol-ketamine as sedation regimens for pediatric auditory brainstem response testing. Brazilian journal of otorhinolaryngology 2019. link 4 Skarżyński H, Bieńkowska K, Gos E, Skarżyński PH, Grudzień D, Czajka N et al.. Cross-Cultural Adaptation of the Scale of Auditory Behaviors Questionnaire. Language, speech, and hearing services in schools 2019. link 5 Presacco A, Middlebrooks JC. Tone-Evoked Acoustic Change Complex (ACC) Recorded in a Sedated Animal Model. Journal of the Association for Research in Otolaryngology : JARO 2018. link 6 Richburg CM, Atcherson SR, Findlen UM, Wakefield S, Benafield NJ. (Central) Auditory Processing Disorder Grand Rounds: Multiple Cases, Multiple Causes, Multiple Outcomes. American journal of audiology 2017. link 7 Avlonitou E, Balatsouras DG, Margaritis E, Giannakopoulos P, Douniadakis D, Tsakanikos M. Use of chloral hydrate as a sedative for auditory brainstem response testing in a pediatric population. International journal of pediatric otorhinolaryngology 2011. link 8 Logue-Kennedy M, Lyons R, Carroll C, Byrne M, Dignan E, O'Hagan L. Services for children with central auditory processing disorder in the Republic of Ireland: current and future service provision. American journal of audiology 2011. link) 9 Fávero ML, Ponce FA, Pio MR, Tabith Junior A, Carvalho e Silva FL. Chloral hydrate to study auditory brainstem response. Brazilian journal of otorhinolaryngology 2010. link 10 Foli KJ, Elsisy H. Influence, education, and advocacy: the pediatric nurse's role in the evaluation and management of children with central auditory processing disorders. Journal for specialists in pediatric nursing : JSPN 2010. link 11 Gemma M, de Vitis A, Baldoli C, Calvi MR, Blasi V, Scola E et al.. Functional magnetic resonance imaging (fMRI) in children sedated with propofol or midazolam. Journal of neurosurgical anesthesiology 2009. link 12 McArthur GM. Auditory processing disorders: can they be treated?. Current opinion in neurology 2009. link 13 Jutras B, Lagacé J, Lavigne A, Boissonneault A, Lavoie C. Auditory processing disorders, verbal disfluency, and learning difficulties: a case study. International journal of audiology 2007. link 14 Cardin JA, Schmidt MF. Auditory responses in multiple sensorimotor song system nuclei are co-modulated by behavioral state. Journal of neurophysiology 2004. link 15 Zeng S, Zhang X, Peng W, Zuo M. Immunohistochemistry and neural connectivity of the Ov shell in the songbird and their evolutionary implications. The Journal of comparative neurology 2004. link 16 Gravel JS, Stapells DR. Behavioral, electrophysiologic, and otoacoustic measures from a child with auditory processing dysfunction: case report. Journal of the American Academy of Audiology 1993. link 17 Wolberg SC, Temlett JA, Fritz VU. Pure word deafness. South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde 1990. link 18 Young IM, Zwillenberg D. Decruitment and abnormal auditory adaptation. Transactions - Pennsylvania Academy of Ophthalmology and Otolaryngology 1989. link 19 Moore JK, Moore RY. Glutamic acid decarboxylase-like immunoreactivity in brainstem auditory nuclei of the rat. The Journal of comparative neurology 1987. link 20 Takahashi TT, Carr CE, Brecha N, Konishi M. Calcium binding protein-like immunoreactivity labels the terminal field of nucleus laminaris of the barn owl. The Journal of neuroscience : the official journal of the Society for Neuroscience 1987. link 21 Campbell MJ, Lewis DA, Foote SL, Morrison JH. Distribution of choline acetyltransferase-, serotonin-, dopamine-beta-hydroxylase-, tyrosine hydroxylase-immunoreactive fibers in monkey primary auditory cortex. The Journal of comparative neurology 1987. link 22 Watson CS. Time course of auditory perceptual learning. The Annals of otology, rhinology & laryngology. Supplement 1980. link 23 Fletcher SG, Higgins JM. Performance of children with severe to profound auditory impairment in instrumentally guided reduction of nasal resonance. The Journal of speech and hearing disorders 1980. link

Auditory synaptopathy