Objective To provide recommendations for the workup of hearing loss in the pediatric patient. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group. Results Consensus recommendations include initial screening and diagnosis as well as the workup of sensorineural, conductive and mixed hearing loss in children. The consensus statement discusses the role of genetic testing and imaging and provides algorithms to guide the workup of children with hearing loss. Conclusion The workup of children with hearing loss can be guided by the recommendations provided herein

Carter, John

Carvalho, Daniela

Cheng, Alan

Choo, Daniel

Curotta, John

Germiller, John A.

Hone, Stephen

Kenna, Margaret A.

Liming, Bryan J.

Loundon, Natalie

Preciado, Diego

Reilly, Brian J.

Roman, Stephane

Schilder, Anne

Smith, Richard J.H.

Strychowsky, Julie

Triglia, Jean Michel

Young, Nancy

English

Scholarship@Western

Western University Scholarship@Western Paediatrics Publications Paediatrics Department 11-1-2016 International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient Bryan J. Liming University of Iowa Healthcare John Carter Ochsner Medical Center - New Orleans Alan Cheng Sydney Children's Hospital, Randwick Daniel Choo Cincinnati Children's Hospital Medical Center John Curotta Sydney Children's Hospital, Randwick See next page for additional authors Follow this and additional works at: https://ir.lib.uwo.ca/paedpub Citation of this paper: Liming, Bryan J.; Carter, John; Cheng, Alan; Choo, Daniel; Curotta, John; Carvalho, Daniela; Germiller, John A.; Hone, Stephen; Kenna, Margaret A.; Loundon, Natalie; Preciado, Diego; Schilder, Anne; Reilly, Brian J.; Roman, Stephane; Strychowsky, Julie; Triglia, Jean Michel; Young, Nancy; and Smith, Richard J.H., "International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient" (2016). Paediatrics Publications. 2104. https://ir.lib.uwo.ca/paedpub/2104 Authors Bryan J. Liming, John Carter, Alan Cheng, Daniel Choo, John Curotta, Daniela Carvalho, John A. Germiller, Stephen Hone, Margaret A. Kenna, Natalie Loundon, Diego Preciado, Anne Schilder, Brian J. Reilly, Stephane Roman, Julie Strychowsky, Jean Michel Triglia, Nancy Young, and Richard J.H. Smith This article is available at Scholarship@Western: https://ir.lib.uwo.ca/paedpub/2104 International Pediatric ORL Group (IPOG)   Hearing Loss in the Pediatric Patient Consensus Recommendations    Consensus Objectives To make recommendations on the routine work-up for pediatric patients with hearing loss  Target Population All infants with hearing loss identified by newborn hearing screening and older children with concerns for hearing loss  Intended Users These consensus recommendations are targeted for: 1. Primary care providers and otolaryngologists who take care of children with hearing loss.  Recommendations and Justification The recommendations are outlined in the following sections     Section 1: Diagnostic evaluation considerations  Section 2: Newborn screening and initial workup of the patient with hearing loss  Section 3: Workup of child with auditory neuropathy spectrum disorder (ANSD)  Section 4: Workup of child with sensorineural hearing loss  Section 5: Workup of child with conductive or mixed hearing loss   Disclaimer This report has been prepared by the members of the International Pediatric ORL Group (IPOG). Consensus recommendations are based on the collective opinion of the members of the group. Any person seeking to apply or consult the report is expected to use independent medical judgment in the context of individual patient and institutional circumstances.  Section 1: Diagnostic evaluation considerations The members of the IPOG identified six frequently debated considerations in the management of pediatric hearing loss. Variation in practice among the current group members remains, and the purpose of this section is to provide a list of reasonable options based on expert opinion. Question  1. Which children should be offered genetic testing?  After an audiogram, comprehensive genetic testing has the highest diagnostic rate of any single test for bilateral sensorineural hearing loss.1  Children with unilateral hearing loss should not be offered genetic testing.  Single gene testing is of low diagnostic yield and should not be offered as part of an initial workup.  Directed genetic testing may be considered in consultation with a geneticist if comprehensive genetic testing is negative but suspicion for a genetic cause still exists.  Comprehensive genetic testing should be offered to children with ANSD. 2. Should temporal bone imaging be performed?   Temporal bone imaging is of low diagnostic yield in all children.  Children who are cochlear implant candidates with profound hearing loss may benefit from CT or MRI to assess for cochlear dysplasias and cochlear nerve aplasia.  Temporal bone imaging is optional in unilateral hearing loss and in children with ANSD. 3. Should ophthalmology consult routinely be ordered?  Given the 2-3 fold increased risk of ocular abnormalities in children with nonsyndromic SNHL, ophthalmology evaluation is warranted. 4. Should cardiology consult be routinely requested?  Genetic testing may identify children who should have a cardiac workup.  .  Cardiology consult should not be ordered at the onset of diagnosis of hearing loss. 5. Should amplification be offered for single sided hearing loss?  Children with single-sided hearing loss are at a higher risk for delays in speech and language development and decreased academic performance relative to normal hearing peers.  The decision to amplify unilateral hearing loss is complex and involves factors such as the severity of the hearing loss and socioeconomic factors such as financial resources.  At minimum, a discussion regarding the benefits of amplification should be undertaken. 6. Should testing and treatment for CMV be considered?  Congenital CMV causes up to ¼ of all congenital sensorineural hearing loss.2  CMV saliva/urine PCR is highly specific in the first three weeks of life.  Blood spot CMV testing can be used as an adjunct diagnostic test after the first three weeks of life.  Treatment for CMV associated hearing loss currently only indicated for symptomatic CMV disease.  Treatment with valganciclovir may improve hearing outcomes in children with asymptomatic congenital CMV infection but not indicated at this time.3      Section 2: Newborn screening and initial workup of the patient with hearing loss The screening and initial workup algorithm is designed to guide the evaluation of all newborns with suspected hearing loss based on initial screening methodologies.  The algorithm also applies to children identified later in childhood.   Screening protocols recommended herein are generally derived from consensus recommendations such as those issued by the Joint Committee on Infant Hearing.4   Protocols may vary slightly between institutions.  In general, infants meeting criteria for increased risk for congenital hearing loss should be screened with both OAEs and an automated ABR.  Infants not within the high risk category can be screened with a two stage procedure that utilizes OAEs alone as the initial screening test, with an automated ABR for absent or subthreshold OAE responses.  A child who fails screening protocols should undergo a confirmatory ABR.     Section 3: Workup of child with auditory neuropathy spectrum disorder (ANSD)  The diagnosis of auditory neuropathy spectrum disorder (ANSD) is given when otoacoustic emissions and/or cochlear microphonics are present in the setting of absent or abnormal ABR.  Given that 50% of ANSD has a genetic basis, comprehensive genetic testing should be offered to children in whom ANSD is identified.  Identification of a specific genetic cause may determine candidacy for habilitation options, including cochlear implantation.  Temporal bone imaging is optional in the setting of ANSD and may reveal abnormalities in up to 2/3 of patients. 5    Section 4: Workup of child with sensorineural hearing loss Genetic testing has a limited role in the workup of children with a unilateral sensorineural hearing loss unless syndromic hearing loss (i.e. Branchio-oto-renal syndrome, Waardenburg syndrome) is suspected based on physical exam and/or family history.   Temporal bone imaging should be optional and discussed with parents at the time of diagnosis.  Comprehensive genetic testing should guide subsequent workup in children with bilateral sensorineural hearing loss.  The diagnostic rate is approximately 40-65%.1  The physical examination and presence of other risk factors will dictate additional workup needed in the child with negative genetic testing.  Asymptomatic congenital CMV is a significant contributor to congenital hearing loss and may present as mild to profound, unilateral or bilateral and stable or progressive.  Vestibular dysfunction is also possible.  Testing for congenital CMV is not currently standardized but should be considered in children with identified hearing loss.  Treatment is controversial but has been shown to have some benefit.   Section 5: Workup of child with conductive or mixed hearing loss The management of an effusion in infants is dependent on the age at which it is identified.  In a child who is older than 3 months, a rapid course of action is indicated to ensure that any appropriate rehabilitation occurs prior to 6 months.  For a child under the age of 3 months with purely conductive hearing loss, the effusion may be observed for a period of 3 months for spontaneous resolution.  If the hearing loss is mixed in nature, then a shorter observation period of 4-6 weeks is appropriate to ensure that the sensorineural component is appropriately worked up and diagnosed prior to the age of 6 months.    References 1. Jayawardena AD, Shearer AE, Smith RJH. Sensorineural hearing loss: A changing paradigm for its evaluation.  Otolaryng Head Neck Surg.  2015 Nov; 153(5):843-50 doi: 10.1177/0194599815596727 2. Morton CC, Nance WE. Newborn hearing screening--a silent revolution. N Engl J Med. 2006;354(20):2151–2164. doi:10.1056/NEJMra050700.  3. Kimberlin DW, Jester PM, Sánchez PJ, et al. Valganciclovir for Symptomatic Congenital Cytomegalovirus Disease. N Engl J Med. 2015;372(10):933–943. doi:10.1056/NEJMoa1404599. 4. American Academy of Pediatrics, Joint Committee on Infant Hearing. Year 2007 position statement: principles and guidelines for early hearing detection and intervention programs. Pediatrics. 2007;120 (4):898–921 5. Roche JP, Huang BY, Castillo M, Bassim MK, Adunka OF, Buchman CA. Imaging characteristics of children with auditory neuropathy spectrum disorder. Otol Neurotol. 2010 Jul;31(5):760-8  

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient

https://ir.lib.uwo.ca/cgi/viewcontent.cgi?article=3112&context=paedpub

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient

Abstract

Similar works

Full text

Available Versions

Scholarship@Western