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Detecting Hearing Loss in Infants with a Syndrome or Craniofacial Abnormalities Following the Newborn Hearing Screen

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Title: Detecting Hearing Loss in Infants with a Syndrome or Craniofacial Abnormalities Following the Newborn Hearing Screen
Language: English
Authors: Horn, Philippa (ORCID 0000-0001-6998-4181), Driscoll, Carlie (ORCID 0000-0001-5037-392X), Fitzgibbons, Jane (ORCID 0000-0001-8741-8319), Beswick, Rachael (ORCID 0000-0002-8784-0816)
Source: Journal of Speech, Language, and Hearing Research. Sep 2021 64(9):3594-3602.
Availability: American Speech-Language-Hearing Association. 2200 Research Blvd #250, Rockville, MD 20850. Tel: 301-296-5700; Fax: 301-296-8580; e-mail: slhr@asha.org; Web site: http://jslhr.pubs.asha.org
Peer Reviewed: Y
Page Count: 9
Publication Date: 2021
Document Type: Journal Articles
Reports - Research
Descriptors: Infants, Genetic Disorders, Hearing Impairments, At Risk Persons, Auditory Evaluation, Screening Tests
DOI: 10.1044/2021_JSLHR-20-00699
ISSN: 1092-4388
Abstract: Purpose: The current Joint Committee on Infant Hearing guidelines recommend that infants with syndromes or craniofacial abnormalities (CFAs) who pass the universal newborn hearing screening (UNHS) undergo audiological assessment by 9 months of age. However, emerging research suggests that children with these risk factors are at increased risk of early hearing loss despite passing UNHS. To establish whether earlier diagnostic audiological assessment is warranted for all infants with a syndrome or CFA, regardless of screening outcome, this study compared audiological outcomes of those who passed UNHS and those who referred. Method: A retrospective analysis was performed on infants with a syndrome or CFA born between July 1, 2012, and June 30, 2017 who participated in Queensland, Australia's state-wide UNHS program. Results: Permanent childhood hearing loss (PCHL) yield was higher among infants who referred on newborn hearing screening (51.20%) than in those who passed. Nonetheless, 27.47% of infants who passed were subsequently diagnosed with hearing loss (4.45% PCHL, 23.02% transient conductive), but PCHL was generally milder in this cohort. After microtia/atresia, the most common PCHL etiologies were Trisomy 21, other syndromes, and cleft palate. Of the other syndromes, Pierre Robin sequence featured prominently among infants who passed the hearing screen and were subsequently diagnosed with PCHL, whereas there was a broader mix of other syndromes that caused PCHL in infants who referred on screening. Conclusion: Children identified with a syndrome or CFA benefit from early diagnostic audiological assessment, regardless of their newborn hearing screening outcome.
Abstractor: As Provided
Entry Date: 2021
Accession Number: EJ1313528
Database: ERIC
FullText Links:
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  Data: Detecting Hearing Loss in Infants with a Syndrome or Craniofacial Abnormalities Following the Newborn Hearing Screen
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  Data: <searchLink fieldCode="AR" term="%22Horn%2C+Philippa%22">Horn, Philippa</searchLink> (ORCID <externalLink term="https://orcid.org/0000-0001-6998-4181">0000-0001-6998-4181</externalLink>)<br /><searchLink fieldCode="AR" term="%22Driscoll%2C+Carlie%22">Driscoll, Carlie</searchLink> (ORCID <externalLink term="https://orcid.org/0000-0001-5037-392X">0000-0001-5037-392X</externalLink>)<br /><searchLink fieldCode="AR" term="%22Fitzgibbons%2C+Jane%22">Fitzgibbons, Jane</searchLink> (ORCID <externalLink term="https://orcid.org/0000-0001-8741-8319">0000-0001-8741-8319</externalLink>)<br /><searchLink fieldCode="AR" term="%22Beswick%2C+Rachael%22">Beswick, Rachael</searchLink> (ORCID <externalLink term="https://orcid.org/0000-0002-8784-0816">0000-0002-8784-0816</externalLink>)
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  Data: <searchLink fieldCode="SO" term="%22Journal+of+Speech%2C+Language%2C+and+Hearing+Research%22"><i>Journal of Speech, Language, and Hearing Research</i></searchLink>. Sep 2021 64(9):3594-3602.
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  Data: American Speech-Language-Hearing Association. 2200 Research Blvd #250, Rockville, MD 20850. Tel: 301-296-5700; Fax: 301-296-8580; e-mail: slhr@asha.org; Web site: http://jslhr.pubs.asha.org
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  Data: 9
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  Data: <searchLink fieldCode="DE" term="%22Infants%22">Infants</searchLink><br /><searchLink fieldCode="DE" term="%22Genetic+Disorders%22">Genetic Disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Hearing+Impairments%22">Hearing Impairments</searchLink><br /><searchLink fieldCode="DE" term="%22At+Risk+Persons%22">At Risk Persons</searchLink><br /><searchLink fieldCode="DE" term="%22Auditory+Evaluation%22">Auditory Evaluation</searchLink><br /><searchLink fieldCode="DE" term="%22Screening+Tests%22">Screening Tests</searchLink>
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  Data: 10.1044/2021_JSLHR-20-00699
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  Data: 1092-4388
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Purpose: The current Joint Committee on Infant Hearing guidelines recommend that infants with syndromes or craniofacial abnormalities (CFAs) who pass the universal newborn hearing screening (UNHS) undergo audiological assessment by 9 months of age. However, emerging research suggests that children with these risk factors are at increased risk of early hearing loss despite passing UNHS. To establish whether earlier diagnostic audiological assessment is warranted for all infants with a syndrome or CFA, regardless of screening outcome, this study compared audiological outcomes of those who passed UNHS and those who referred. Method: A retrospective analysis was performed on infants with a syndrome or CFA born between July 1, 2012, and June 30, 2017 who participated in Queensland, Australia's state-wide UNHS program. Results: Permanent childhood hearing loss (PCHL) yield was higher among infants who referred on newborn hearing screening (51.20%) than in those who passed. Nonetheless, 27.47% of infants who passed were subsequently diagnosed with hearing loss (4.45% PCHL, 23.02% transient conductive), but PCHL was generally milder in this cohort. After microtia/atresia, the most common PCHL etiologies were Trisomy 21, other syndromes, and cleft palate. Of the other syndromes, Pierre Robin sequence featured prominently among infants who passed the hearing screen and were subsequently diagnosed with PCHL, whereas there was a broader mix of other syndromes that caused PCHL in infants who referred on screening. Conclusion: Children identified with a syndrome or CFA benefit from early diagnostic audiological assessment, regardless of their newborn hearing screening outcome.
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        Value: 10.1044/2021_JSLHR-20-00699
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        Type: general
      – SubjectFull: Genetic Disorders
        Type: general
      – SubjectFull: Hearing Impairments
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      – SubjectFull: At Risk Persons
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      – SubjectFull: Auditory Evaluation
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      – SubjectFull: Screening Tests
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