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Auditory neuropathy spectrum disorder

MedGen UID:
751969
•Concept ID:
C2732267
•
Disease or Syndrome
Synonyms: Auditory Neuropathy; Auditory Neuropathy Spectrum Disorder; Auditory neuropathy with dys-synchrony
SNOMED CT: Auditory neuropathy spectrum disorder (443805006); Auditory neuropathy with dys-synchrony (443805006)

Definition

A hearing disorder characterized by impaired transmission of signals through the auditory nerve, resulting in mild to severe hearing loss and poor speech perception. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Auditory neuropathy spectrum disorder

Professional guidelines

PubMed

Twelve years of neonatal hearing screening: audiological and etiological results.
Acke FRE, De Vriese C, Van Hoecke H, De Leenheer EMR
Eur Arch Otorhinolaryngol 2022 Jul;279(7):3371-3378. Epub 2021 Aug 31 doi: 10.1007/s00405-021-07060-5. PMID: 34463816
Hearing screening in neonates with hyperbilirubinemia.
Ezzeldin ZM, Sharaf E, Hamdy HS, Abdelwahab Selim YA
Int J Pediatr Otorhinolaryngol 2021 Mar;142:110591. Epub 2020 Dec 23 doi: 10.1016/j.ijporl.2020.110591. PMID: 33360868
The management of pediatric hearing loss caused by auditory neuropathy spectrum disorder.
Pham NS
Curr Opin Otolaryngol Head Neck Surg 2017 Oct;25(5):396-399. doi: 10.1097/MOO.0000000000000390. PMID: 28708635

Recent clinical studies

Etiology

A 15-Year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: I. Demographic and Diagnostic Characteristics.
Morlet T, Parkes W, Pritchett C, Venskytis E, DeVore B, O'Reilly RC
Ear Hear 2023 Sep-Oct 01;44(5):969-978. Epub 2023 Apr 10 doi: 10.1097/AUD.0000000000001337. PMID: 37036288
A 15-year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: II. Management and Outcomes.
Morlet T, O'Reilly R, Pritchett C, Venskytis E, Parkes W
Ear Hear 2023 Sep-Oct 01;44(5):979-989. Epub 2023 Apr 10 doi: 10.1097/AUD.0000000000001366. PMID: 37036283
Speech and Language outcomes in Auditory Neuropathy Spectrum Disorder (ANSD) children managed with amplification.
Ramanathan D, Mahomva C, Goldberg D, Liu YC, Anne S, Lyle W
Am J Otolaryngol 2023 Mar-Apr;44(2):103753. Epub 2022 Dec 20 doi: 10.1016/j.amjoto.2022.103753. PMID: 36577168
Hearing screening in neonates with hyperbilirubinemia.
Ezzeldin ZM, Sharaf E, Hamdy HS, Abdelwahab Selim YA
Int J Pediatr Otorhinolaryngol 2021 Mar;142:110591. Epub 2020 Dec 23 doi: 10.1016/j.ijporl.2020.110591. PMID: 33360868
Auditory impairment in infants at risk for bilirubin-induced neurologic dysfunction.
Shapiro SM, Popelka GR
Semin Perinatol 2011 Jun;35(3):162-70. doi: 10.1053/j.semperi.2011.02.011. PMID: 21641490

Diagnosis

A 15-year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: II. Management and Outcomes.
Morlet T, O'Reilly R, Pritchett C, Venskytis E, Parkes W
Ear Hear 2023 Sep-Oct 01;44(5):979-989. Epub 2023 Apr 10 doi: 10.1097/AUD.0000000000001366. PMID: 37036283
Speech and Language outcomes in Auditory Neuropathy Spectrum Disorder (ANSD) children managed with amplification.
Ramanathan D, Mahomva C, Goldberg D, Liu YC, Anne S, Lyle W
Am J Otolaryngol 2023 Mar-Apr;44(2):103753. Epub 2022 Dec 20 doi: 10.1016/j.amjoto.2022.103753. PMID: 36577168
Hearing screening in neonates with hyperbilirubinemia.
Ezzeldin ZM, Sharaf E, Hamdy HS, Abdelwahab Selim YA
Int J Pediatr Otorhinolaryngol 2021 Mar;142:110591. Epub 2020 Dec 23 doi: 10.1016/j.ijporl.2020.110591. PMID: 33360868
Auditory Neuropathy Spectrum Disorder (ANSD)-Clinical Characteristics and Pathogenic Variant Analysis of Three Nonsyndromic Deafness Families.
Zhai R, Feng H, Li Q, Lu W, Liu D, Tian Y, Liu H, Li R, Zuo B, Tang W, Xu H, Chen B
Biomed Res Int 2020;2020:8843539. Epub 2020 Dec 21 doi: 10.1155/2020/8843539. PMID: 33426078Free PMC Article
Auditory function in Pelizaeus-Merzbacher disease.
Morlet T, Nagao K, Bean SC, Mora SE, Hopkins SE, Hobson GM
J Neurol 2018 Jul;265(7):1580-1589. Epub 2018 May 3 doi: 10.1007/s00415-018-8884-x. PMID: 29725841

Therapy

Diagnosis of Auditory Neuropathy Spectrum Disorder in the Neonatal Intensive Care Unit Population.
Mahomva C, Liu YC, Raol N, Anne S
Otolaryngol Head Neck Surg 2022 May;166(5):964-969. Epub 2021 Jul 27 doi: 10.1177/01945998211029836. PMID: 34311620
Contemporary surgical issues in paediatric cochlear implantation.
Wasson JD, Briggs RJ
Int J Audiol 2016;55 Suppl 2:S77-87. Epub 2016 Jun 7 doi: 10.1080/14992027.2016.1184765. PMID: 27266370
Children with Auditory Neuropathy Spectrum Disorder Fitted with Hearing Aids Applying the American Academy of Audiology Pediatric Amplification Guideline: Current Practice and Outcomes.
Walker E, McCreery R, Spratford M, Roush P
J Am Acad Audiol 2016 Mar;27(3):204-218. doi: 10.3766/jaaa.15050. PMID: 26967362Free PMC Article
Cortical development and neuroplasticity in Auditory Neuropathy Spectrum Disorder.
Sharma A, Cardon G
Hear Res 2015 Dec;330(Pt B):221-32. Epub 2015 Jun 10 doi: 10.1016/j.heares.2015.06.001. PMID: 26070426Free PMC Article
Aiding and occluding the contralateral ear in implanted children with auditory neuropathy spectrum disorder.
Runge CL, Jensen J, Friedland DR, Litovsky RY, Tarima S
J Am Acad Audiol 2011 Oct;22(9):567-577. doi: 10.3766/jaaa.22.9.2. PMID: 22192602Free PMC Article

Prognosis

Kernicterus Spectrum Disorders Diagnostic Toolkit: validation using retrospective chart review.
Dasari VR, Shapiro SM, Yeh HW, Gelineau-Morel R
Pediatr Res 2022 Mar;91(4):862-866. Epub 2021 Nov 5 doi: 10.1038/s41390-021-01755-5. PMID: 34741107
Audiological Features in 63 Patients With Cochlear Nerve Deficiency.
Matsuura K, Yoshimura H, Shinagawa J, Kurozumi M, Takumi Y
Otol Neurotol 2022 Jan 1;43(1):23-28. doi: 10.1097/MAO.0000000000003365. PMID: 34538855
Cortical Auditory Plasticity Following Cochlear Implantation in Children With Auditory Neuropathy Spectrum Disorder: A Prospective Study.
Saki N, Nikakhlagh S, Moridi B, Karimi M, Aghayi A, Bayat A
Otol Neurotol 2021 Oct 1;42(9):e1227-e1233. doi: 10.1097/MAO.0000000000003257. PMID: 34172662
Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation.
Harrison RV, Gordon KA, Papsin BC, Negandhi J, James AL
Int J Pediatr Otorhinolaryngol 2015 Dec;79(12):1980-7. Epub 2015 Oct 17 doi: 10.1016/j.ijporl.2015.10.006. PMID: 26545793
Cortical development and neuroplasticity in Auditory Neuropathy Spectrum Disorder.
Sharma A, Cardon G
Hear Res 2015 Dec;330(Pt B):221-32. Epub 2015 Jun 10 doi: 10.1016/j.heares.2015.06.001. PMID: 26070426Free PMC Article

Clinical prediction guides

A 15-year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: II. Management and Outcomes.
Morlet T, O'Reilly R, Pritchett C, Venskytis E, Parkes W
Ear Hear 2023 Sep-Oct 01;44(5):979-989. Epub 2023 Apr 10 doi: 10.1097/AUD.0000000000001366. PMID: 37036283
Kernicterus Spectrum Disorders Diagnostic Toolkit: validation using retrospective chart review.
Dasari VR, Shapiro SM, Yeh HW, Gelineau-Morel R
Pediatr Res 2022 Mar;91(4):862-866. Epub 2021 Nov 5 doi: 10.1038/s41390-021-01755-5. PMID: 34741107
Audiological Features in 63 Patients With Cochlear Nerve Deficiency.
Matsuura K, Yoshimura H, Shinagawa J, Kurozumi M, Takumi Y
Otol Neurotol 2022 Jan 1;43(1):23-28. doi: 10.1097/MAO.0000000000003365. PMID: 34538855
Cortical Auditory Plasticity Following Cochlear Implantation in Children With Auditory Neuropathy Spectrum Disorder: A Prospective Study.
Saki N, Nikakhlagh S, Moridi B, Karimi M, Aghayi A, Bayat A
Otol Neurotol 2021 Oct 1;42(9):e1227-e1233. doi: 10.1097/MAO.0000000000003257. PMID: 34172662
Auditory function in Pelizaeus-Merzbacher disease.
Morlet T, Nagao K, Bean SC, Mora SE, Hopkins SE, Hobson GM
J Neurol 2018 Jul;265(7):1580-1589. Epub 2018 May 3 doi: 10.1007/s00415-018-8884-x. PMID: 29725841

Recent systematic reviews

Is auditory neuropathy an appropriate term? A systematic literature review on its aetiology and pathogenesis.
Burdo S, Di Berardino F, Bruno G
Acta Otorhinolaryngol Ital 2021 Dec;41(6):496-506. Epub 2021 Nov 26 doi: 10.14639/0392-100X-N0932. PMID: 34825666Free PMC Article
Cochlear Implant Behavioral Outcomes for Children With Auditory Neuropathy Spectrum Disorder: A Mini-Systematic Review.
Myers K, Nicholson N
Am J Audiol 2021 Sep 10;30(3):777-789. Epub 2021 Jul 23 doi: 10.1044/2021_AJA-20-00175. PMID: 34297601
Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation.
Harrison RV, Gordon KA, Papsin BC, Negandhi J, James AL
Int J Pediatr Otorhinolaryngol 2015 Dec;79(12):1980-7. Epub 2015 Oct 17 doi: 10.1016/j.ijporl.2015.10.006. PMID: 26545793
Performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implant: a systematic review.
Fernandes NF, Morettin M, Yamaguti EH, Costa OA, Bevilacqua MC
Braz J Otorhinolaryngol 2015 Jan-Feb;81(1):85-96. Epub 2014 Oct 22 doi: 10.1016/j.bjorl.2014.10.003. PMID: 25458263Free PMC Article
Audiologic management of auditory neuropathy spectrum disorder in children: a systematic review of the literature.
Roush P, Frymark T, Venediktov R, Wang B
Am J Audiol 2011 Dec;20(2):159-70. Epub 2011 Sep 22 doi: 10.1044/1059-0889(2011/10-0032). PMID: 21940978

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