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Permanent Childhood Hearing Loss

See the Hearing Loss (Early Childhood) and the Hearing Loss (Newborn) Evidence Maps for summaries of the available research on this topic.

Permanent childhood hearing loss can be congenital, delayed-onset, progressive, or acquired in nature. Congenital hearing loss refers to hearing loss that is present at birth and is often identified through a newborn hearing screening conducted shortly after birth. While estimates vary, some hearing loss in childhood is delayed-onset or progressive in nature. As a result, it is important to provide audiologic monitoring over time for children who are considered to be "at risk" for hearing loss. In addition, some mild hearing losses as well as auditory neuropathy may not be identified through newborn hearing screening due to the current limitations of the test equipment or testing methodology used.

Hearing is critical to speech and language development, communication, literacy, and learning. Early identification and intervention of hearing loss can lessen the impact on a child's development (Sininger, Grimes, & Christensen, 2010; Yoshinaga-Itano, Baca, & Sedey, 2010). The Joint Committee on Infant Hearing (JCIH, 2007) recommends that

  • all children be screened for hearing loss no later than 1 month of age,
  • hearing and medical evaluations be completed no later than 3 months of age,
  • infants with confirmed hearing loss are fit with amplification (if the family chooses and if appropriate) within 1 month of diagnosis,
  • early intervention services begin no later than 6 months of age.

Note: The scope of this content is limited to the diagnosis and management of permanent hearing loss for children from birth through 5 years of age from an audiological perspective. Resources for hearing screening and habilitation, as well as hearing loss for school-age and adult populations, are under development.

Family-Centered Practice

Families who are actively involved in the assessment and treatment process achieve better outcomes (DesJardin, 2006). It is paramount that audiologists incorporate family-centered practice into the identification and treatment of young children who are deaf or hard of hearing; family-centered activities include

  • engaging the family and using the child's toys from home in various aspects of the evaluation and therapy sessions,
  • suspending judgment and building rapport with the family about their needs and interests,
  • matching re/habilitation with the family needs and goals,
  • recognizing the family's rights regarding informed consent and confidentiality.

The goal of family-centered practice is to create a partnership with the family so that the family fully participates in all aspects of the child's care.

The "prevalence" of permanent childhood hearing loss refers to the number of children who are living with permanent childhood hearing loss at any given time. Estimates of the prevalence of permanent hearing loss in children under 5 vary according to the source of the data and the criteria for defining hearing loss.

  • Most estimates suggest that 1 to 3 per 1,000 children are born with a hearing loss, based on screening and/or medical records (Centers for Disease Control and Prevention [CDC], 2009; National Institute on Deafness and Other Communication Disorders [NIDCD], 2010).
  • Prevalence estimates based on parental surveys tend to be higher. A recent study by the CDC found that parents of 5 out of every 1,000 children felt their child had a hearing loss. It should be noted, though, that that figure was based on children ages 3-17 (Boulet, Boyle, & Schieve, 2009).
  • Higher still are prevalence estimates from neonatal intensive care units (NICUs). A Dutch study of NICU neonates found the prevalence to be 32 per 1,000 (Hille, van Straaten, & Verkerk, 2007).

Infants and young children with normal hearing sensitivity generally develop listening and communication skills in a predictable developmental sequence and by a certain age. A delay in these communication milestones may be a sign of hearing loss.

The sooner hearing loss is diagnosed and intervention is initiated, the better the outcomes for speech and language development (Sininger et al., 2010; Yoshinaga-Itano et al., 2010). For this reason, it is important for parents/caregivers, physicians, and others who work with young children to be aware of the signs of possible hearing loss.

Nongenetic Factors

Nongenetic factors of permanent hearing loss include

  • maternal infections (e.g., rubella [German measles], cytomegalovirus, or herpes simplex virus),
  • prematurity,
  • low birth weight,
  • birth injuries,
  • toxins, including drugs and alcohol consumed by the mother during pregnancy,
  • complications associated with the Rh factor in the blood/jaundice,
  • maternal diabetes,
  • toxemia during pregnancy,
  • lack of oxygen (anoxia),
  • inner ear malformations (e.g., Mondini's malformation, large vestibular aqueduct),
  • outer/middle ear malformations (Atresia, stenosis).

Genetic Factors

Hearing loss from genetic causes can be present at birth or develop later in life. Most genetic hearing loss can be described as autosomal recessive or autosomal dominant. Other, rarer types of genetic hearing loss include X-linked (related to the sex chromosome) or mitochondrial inheritance patterns.

Genetic syndromes have a group of signs and symptoms that together indicate a specific disease. Many genetic syndromes include hearing loss as one of the symptoms. In fact, 20% of babies with genetic hearing loss have a syndrome (Morton & Nance, 2006). Examples of genetic disorders that include hearing loss are

  • Down syndrome,
  • Usher syndrome,
  • Treacher Collins syndrome,
  • Crouzon syndrome,
  • Alport syndrome,
  • Sickle cell disease,
  • Tay-Sachs disease,
  • Waardenburg syndrome,
  • Pendred syndrome,
  • Goldenhar syndrome,
  • CHARGE syndrome.

Acquired Hearing Loss

Acquired hearing loss occurs after birth. Conditions that may cause permanent acquired hearing loss in children include

Roles and Responsibilities of Audiologists

Audiologists play a central role in the identification, assessment, diagnosis, and re/habilitation of patients with permanent childhood hearing loss. Professional roles and activities in audiology include clinical/education services, prevention and advocacy, and education, administration, and research. See ASHA's Scope of Practice in Audiology (ASHA, 2018).

Appropriate roles for audiologists include:

  • providing prevention information, promoting hearing wellness, and monitoring the acoustic environment;
  • educating other professionals on the needs of children with permanent childhood hearing loss and the role of audiologists in diagnosing and managing permanent childhood hearing loss;
  • identifying permanent childhood hearing loss, including early detection and screening program development, management, quality assessment, and service coordination;
  • conducting a comprehensive assessment, using behavioral, electroacoustic, and/or electrophysiologic methods to assess hearing, auditory function, balance, and related systems;
  • referring the patient to other professionals as needed to facilitate access to comprehensive services;
  • evaluating children with permanent childhood hearing loss for candidacy for amplification and other sensory devices and assistive technology;
  • fitting and maintaining amplification and other sensory devices and assistive technology for optimal use;
  • developing and implementing an audiologic re/habilitation management plan;
  • creating documentation, including interpreting data and summarizing findings and recommendations;
  • counseling the patient and his or her family on the psychosocial aspects of hearing loss and other auditory dysfunction, modes of communication, and processes to enhance communication competence;
  • advocating for the communication needs of all individuals, including advocating for the rights and funding of services for those with hearing loss, auditory, and/or vestibular disorders;
  • consulting and collaborating with professionals of related and/or allied services regarding communication management, educational implications, accessibility, and legal implications of hearing loss and/or other auditory dysfunction;
  • conducting education, supervision, and administration for audiology graduate and other professional education programs;
  • remaining informed of research in the area of permanent childhood hearing loss and helping advance the knowledge base related to the nature, identification, and treatment of permanent childhood hearing loss.

As indicated in the Code of Ethics, audiologists who serve this population should be specifically educated and appropriately trained to do so.

Roles and Responsibilities of Speech-Language Pathologists

Speech-language pathologists (SLPs) play a role in the screening, assessment, and re/habilitation of children with permanent childhood hearing loss. Professional roles and activities in speech-language pathology include clinical/educational services, prevention and advocacy, education, administration, and research. See ASHA's Scope of Practice in Speech-Language Pathology (ASHA, 2016). As indicated in the Code of Ethics, SLPs who serve this population should be specifically educated and appropriately trained to do so.

Note: The scope of this content is limited to the diagnosis and management, from an audiological perspective, of permanent hearing loss for children from birth through 5 years. Resources for SLPs are under development.

See the Assessment sections of the Hearing Loss (Early Childhood) and the Hearing Loss (Newborn) Evidence Maps for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

Identification/Screening

Note: This section is an overview. A Practice Portal page on hearing screening is currently under development.

The JCIH Year 2007 Position Statement endorses hearing screening no later than 1 month of age. Most babies born in the United States now have their hearing screened shortly after birth. Babies who do not pass the newborn hearing screening (and/or rescreening) are referred immediately for a comprehensive audiological evaluation, with a goal of having hearing loss confirmed by 3 months of age. Regardless of previous hearing screening outcomes, all infants are to receive ongoing surveillance of communicative development beginning at 2 months of age (JCIH, 2007).

For all infants, regular surveillance of developmental milestones, auditory skills, parental concerns, and middle-ear status should be performed in the medical home, consistent with the American Academy of Pediatrics (AAP) pediatric periodicity schedule (JCIH, 2007).

Audiologic Assessment

The purpose of the audiologic assessment is to
  • assess the integrity of the auditory system in each ear,
  • measure hearing sensitivity across frequencies,
  • determine the type of hearing loss,
  • establish a baseline for future monitoring,
  • provide ear-specific information needed to initiate amplification device fitting.

Comprehensive assessment is to be performed on both ears even if only one ear fails the screening test.

Audiologic evaluation for children with developmental ages of birth to 5 years includes

  • case history,
  • developmental screening,
  • otosocopy,
  • audiological test battery.

Case History

Accurate diagnosis of hearing loss relies on the audiologist's interpretation of a test battery within the context of the child's medical and/or developmental history. Case history information may indicate a need for modification of evaluation procedures. For example, the audiologist may want to include evaluation of the high-frequency region of the cochlea (above 4000 Hz) for a young child with a history of ototoxic drug exposure. Modification of routine assessment procedures also may be necessary when evaluating a child with multiple disabilities. The practitioner should record case history using a standard form.

Developmental Screening

A complete audiological assessment typically includes a developmental screening to make sure the child is reaching developmental milestones.

Otoscopy

Otoscopy is used to ensure that there are no contraindications to placing an earphone or probe in the ear canal. It is essential for the audiologist to verify that the external auditory canal is free of obstructions (e.g., foreign objects, impacted cerumen, vernix) and that there is no drainage from the middle ear. To the extent possible, the audiologist examines the tympanic membrane with regard to color, position, and abnormalities. Additionally, visual inspection for obvious structural abnormalities (e.g., ear pits, ear tags, atresia, and low-set ears) of the pinna and/or ear canal is typically included.

Audiologic Test Battery

The order and selection of tests to be included in the audiological test battery vary based on the age of the child.

Audiological Test Battery-Developmental Age of Birth-to-6 Months

Auditory Evoked Potentials

Auditory brainstem response (ABR) is an appropriate test for children who are too young for reliable behavioral testing. Many children in this age group can be tested during natural sleep, without sedation, using sleep deprivation with nap and feeding times coordinated around the test session. Active or older infants may require monitored conscious sedation or general anesthesia to allow adequate time for acquisition of high-quality recordings and sufficient frequency-specific information. (See Monitored Conscious Sedation/General Anesthesia.)

Threshold Assessment

Frequency-Specific ABR

Stimuli: Frequency-specific stimuli are tone bursts of low, mid, and high frequencies.

Transducer: A complete audiologic evaluation includes both an air-conduction and bone-conduction ABR, when indicated.

  • Insert earphones are recommended, unless contraindicated, for air-conduction testing.
  • Bone conduction ABR may be influenced by the pressure of the bone oscillator against the skull and the developmental age of the child when the skull bones are fully ossified.

Notes: Responses are typically attempted down to 20 dB nHL in at least 10 dB steps.

ASSR

At this time, the evidence does not support the use of auditory steady state response (ASSR) as the sole test to acquire frequency specific information, but it may be used as a supplemental test in addition to frequency-specific ABR testing.

Stimuli: Frequency-specific stimuli are amplitude and frequency modulated pure tones with carrier frequencies of 500, 1000, 2000, and 4000 Hz.

Transducer: Insert earphones are recommended, unless contraindicated, for air-conduction testing. A bone-conduction transducer will be needed if air conduction is elevated (i.e., if air-conduction thresholds are greater than 20 dB nHL, bone-conduction testing should be completed to assess the type of hearing loss).

Note: ASSR analysis is mathematically based. The specific method of analysis to define threshold is dependent on the manufacturer's statistical detection algorithm.

Assessment of VIIIth Nerve Integrity

ABR

Stimuli: Click stimuli at a high level (e.g., 80-90 dB nHL) is adequate in most situations to identify Waves I, III, and V. If no response is obtained at the maximum output level, the audiologist obtains one run of rarefaction clicks and one of condensation clicks to distinguish between cochlear and neural dysfunction. A catch trial (i.e., signal running but not delivered to the ear) can rule out a stimulus artifact that may be misinterpreted as the cochlear microphonic [CM]).

Transducer: Insert earphones are recommended.

Note: Compare interpeak latencies with corrected age norms and look for abnormal waveform morpohology.

Otoacoustic Emissions (OAEs)

Otoacoustic emmisions (OAEs) are used to assess cochlear function. OAEs are best measured in a quiet environment (e.g., in a quiet room with no one speaking and minimal background noise). A snug probe fit is essential for valid and reliable recordings. Ears are typically tested one at a time, with the infant placed on his/her side and the ear being screened facing up. The infant should be resting quietly. Acceptable OAE protocols include

  • Transient Evoked Otoacoustic Emission (TEOAE): One level (e.g., 80 dB pSPL) click stimulus. Normal distributions for this condition for normal hearing are documented in the literature (Hussain, Gorga, Neely, Keefe, & Peters, 1998).
  • Distortion Product Otoacoustic Emission (DPOAE): One level of L1 and L2 65/55 dB SPL at least at four frequencies. Normal distributions for this condition for normal hearing are documented in the literature (Gorga et al., 1997).

Acoustic Immittance

Tympanometry and acoustic reflex testing are used in conjunction to assess middle ear function. Interpretation of tympanograms and acoustic reflex findings may be compromised when a conventional low-frequency (220 or 226 Hz) probe tone is used on patients under the developmental age of approximately 4 months. With patients between 5 and 7 months of corrected age, there is still a possibility of false-negative tympanograms in ears with middle ear effusion. A higher probe-tone frequency (e.g., 1000 Hz) appears to provide a more valid indication of middle-ear function in this age group. Wideband acoustic reflectance is an area of interest as a clinical tool to assess middle-ear status in young infants, but further investigation is needed.

  • Tympanometry: Normative data for 1000 Hz tympanometry are available for neonates and young infants (Margolis et al., 2003).
  • Acoustic reflex thresholds: Normative data are available for tonal or broadband noise stimuli (Kei, 2012).

Behavioral Assessment

Behavioral observation alone is not adequate for determining whether hearing loss is present in this age group and is not adequate for the fitting of amplification devices. The clinician's observation of the infant's auditory behavior may be used as a cross-check in conjunction with electrophysiologic measures.

Audiologic Test Battery-Developmental Age of 6-to-36 Months

Behavioral Assessment

The audiologist conducts visual reinforcement audiometry (VRA) and/or conditioned play audiometry (CPA) as developmentally appropriate. Because VRA requires that a child have the developmental ability to respond to conditioned procedures, sit, maintain head control, and turn his or her head, VRA is only performed on infants with a developmental age of 6 months or older. It is the recognized method of choice for infants and toddlers with a developmental age of approximately 6 through 24 months. As children mature beyond the second birthday, CPA or a combination of VRA and CPA may be attempted to maintain the child's interest.

Stimuli: Frequency-specific stimuli at octave intervals from 250 to 4000 Hz.

Transducer: Insert earphones are recommended, unless contraindicated, followed by bone conduction as needed; sound-field testing may be necessary or useful with some children, but every attempt should be made to acquire ear-specific information.

Note: Minimum response levels (MRL) are typically obtained down to 20 dB HL; consider alternating between ears and frequencies (high and low frequencies and fill in the gap) when testing.

Speech Audiometry

Speech audiometry results are helpful for planning treatment and monitoring a child's ability to understand speech. Speech audiometry procedures include

  • speech detection threshold (SDT) or speech awareness threshold (SAT),
  • speech reception threshold (SRT) for spondees or body-part identification,
  • speech recognition using age and linguistically appropriate closed- and open-set speech perception tests.
Auditory Evoked Potentials

ABR is a part of the test battery for this age population when

  • behavioral audiometric tests are judged to be unreliable, ear-specific thresholds cannot be obtained, or results are inconclusive regarding type, degree, or configuration of hearing levels;
  • two attempts at behavioral audiometry are not successful in testing the hearing status of a child within a 2-month period;
  • the neurological integrity of the auditory systems through the level of the brainstem is in question;
  • an AABR/ABR has never been performed and the child is under 3 years of age and identified with hearing loss.

(JCIH, 2007)

Otoacoustic Emissions (OAEs)

Otoacoustic emissions (OAEs) are used to assess cochlear function. OAEs are best measured in a quiet environment (e.g., in a quiet room with no one speaking and minimal background noise). A snug probe fit is essential for valid and reliable recordings. Acceptable OAE protocols include

  • Transient Evoked Otoacoustic Emission (TEOAE): One level (e.g., 80 dB pSPL) click stimulus. Normal distributions for this condition for normal hearing are documented in the literature (Hussain et al., 1998) or
  • Distortion Product Otoacoustic Emission (DPOAE): One level of L1 and L2 65/55 dB SPL at least at four frequencies. Normal distributions for this condition for normal hearing are documented in the literature (Gorga et al., 1997)

Acoustic Immittance

Tympanometry and acoustic reflex testing are used in conjunction to assess middle ear. A 226 Hz probe tone is appropriate for most children over 6 months of age, but it is important to note that there is still the possibility of false-negative tympanograms in ears with middle ear effusion with patients less than 7 months of age.

  • Tympanograms should use a low frequency (226 Hz) probe tone.
  • Ipsi- and contralateral acoustic reflex thresholds should be at 500, 1000, and 2000 Hz.

Audiologic Test Battery-Developmental Age of 3-to-5 Years

Behavioral Assessment

Frequency-specific thresholds are obtained through VRA, CPA, or conventional audiometric testing, depending on the developmental level of the child.

Stimuli: Speech and frequency-specific stimuli at octave intervals from 250 to 4000 Hz.

Transducer: Insert earphones are recommended, unless contraindicated, followed by bone conduction as needed; sound-field testing may be necessary or useful with some children, but every attempt should be made to acquire ear-specific information.

Speech Audiometry

Speech audiometry results are helpful for planning treatment and monitoring the child's ability to understand speech. Speech audiometry measures include

  • speech detection threshold (SDT) or speech awareness threshold (SAT),
  • speech reception threshold (SRT) for spondees or body-part identification,
  • speech recognition using age and linguistically appropriate closed- and open-set speech perception tests.
Auditory Evoked Potentials

ABR is a part of the test battery for this age population

  • when behavioral audiometric tests are judged to be unreliable, ear-specific thresholds cannot be obtained, or results are inconclusive regarding type, degree, or configuration of hearing levels;
  • if two attempts at behavioral audiometry are not successful in testing the hearing status of a child within a 2-month period; or
  • if the neurological integrity of the auditory systems through the level of the brainstem is in question.

Otoacoustic Emissions (OAEs)

Otoacoustic emissions (OAEs) are used to assess cochlear function. OAEs are best measured in a quiet environment (e.g., in a quiet room with no one speaking and minimal background noise). A snug probe fit is essential for valid and reliable recordings. Acceptable OAE protocols include

  • Transient Evoked Otoacoustic Emission (TEOAE): One level (e.g., 80 dB pSPL) click stimulus. Normal distributions for this condition for normal hearing are documented in the literature (Hussain et al., 1998).
  • Distortion Product Otoacoustic Emission (DPOAE): One level of L1 and L2 65/55 dB SPL at least at four frequencies. Normal distributions for this condition for normal hearing are documented in the literature (Gorga et al., 1997).

Acoustic Immittance

Tympanometry and acoustic reflex testing are used in conjunction to assess middle ear function and acoustic reflex pathways.

  • Tympanograms should use a low frequency (226 Hz) probe tone.
  • Ipsi- and contralateral acoustic reflex thresholds should be at 500, 1000, and 2000 Hz.

Monitored Conscious Sedation and General Anesthesia

Sedation may be necessary to gain the cooperation of some infants and young children during physiologic assessments of auditory function. Sedation of pediatric patients, however, has serious associated risks, such as hypoventilation, apnea, airway obstruction, and cardiopulmonary impairment. If sedation is required for audiologic testing, the child should undergo testing at a facility with professionals who are experienced in handling adverse or paradoxical responses to sedation. Oversight by skilled medical personnel and the availability of age- and size-appropriate equipment, medications, and continuous monitoring are essential during procedures (AAP, 2006). For some children, use of conscious sedation is contraindicated. In these cases, use of general anesthesia may be necessary. This determination is usually made by the otolaryngologist or the child's primary health care provider in conjunction with a pediatric anesthesiologist.

Developmental and Communication Screening

Pediatric audiologists are involved in developmental screening and functional auditory assessment of their patients. Children with hearing loss also typically receive a complete developmental assessment and are evaluated across various domains, including cognition, social, motor, and self-help/adaptive.

Areas that are monitored include

  • developmental milestones,
  • prelinguistic communication,
  • receptive and expressive language status,
  • auditory skill development,
  • functional auditory performance,
  • social-emotional development.

Documentation and Follow-Up

Follow-Up: Newly Confirmed Hearing Loss

For infants and children with newly confirmed hearing loss, the audiologist discusses audiologic test results, implications of the findings, and recommendations for intervention with the parents/caregivers. Topics discussed include

  • the need for medical evaluation and diagnosis,
  • amplification options,
  • the importance of early intervention,
  • communication options for young children with permanent hearing loss,
  • the availability and importance of parent-to-parent support,
  • sources of funding assistance if necessary.

Although audiologists provide counseling and support to families related to their child's diagnosis, in some cases, the parents' need for counseling or other supports may be beyond the scope of practice for audiologists, who should be prepared to refer families to other professionals as appropriate.

Medical Referral

Infants with confirmed hearing loss and/or middle ear dysfunction are referred for otologic and other medical evaluation to determine the etiology of hearing loss, identify related physical conditions, and provide recommendations for medical/surgical treatment and referrals for other services as needed. The evaluation should include a thorough review of the child's medical and family history; a physical examination of the ears, head, and neck; and a neurological evaluation as indicated. A comprehensive otologic workup often includes radiologic imaging, laboratory studies, and an electrocardiogram. Other studies, such as ophthalmologic evaluation and genetics evaluation and counseling, may be ordered depending on the specific needs of the child.

Ongoing Assessment and Monitoring

Children with hearing loss require ongoing otologic and audiologic monitoring, because hearing loss can fluctuate or progress and medical conditions can change over time. Included in this monitoring is an assessment of the resources, concerns, and priorities of the family.

Mandatory Reporting

For infants and children younger than 3, audiologists have a responsibility to initiate a referral to the Part C program "as soon as possible," but not more than 7 calendar days after the newly confirmed hearing loss. See the state early intervention program (Part C program) for specific information regarding early intervention services and local resources.

Because of their role in the early hearing detection and intervention (EHDI) process, audiologists should be aware of state reporting methods, forms, and requirements. By working closely with EHDI and Part C programs, audiologists can help promote seamless transitions between diagnosis of hearing loss and intervention services.

Children older than 3 with newly confirmed hearing loss should be referred to their respective local area education systems for evaluation and consideration for services. If the child is not part of the infant-toddler services referral program, the audiologist reports to the educational audiologist in the child's school district. With consent, audiologist also reports newly confirmed hearing loss to the family/caregiver, the infant's primary care provider, and the referral source.

Follow-Up: Normal Hearing

For infants and children with normal hearing, the audiologist discusses with parents/caregivers the audiologic test results, including

  • reviewing results of the audiologic assessment and providing information about risk indicators for progressive and delayed-onset hearing loss, as well as typical speech, language, and listening developmental milestones;
  • recommending re-evaluation if concerns about hearing or speech and language development arise;
  • providing a report of the assessment (with parental consent) to the infant's primary care provider and to the referral source. Results should also be provided to the state, based on state guidelines, statutes, and regulations.

Follow-Up: Children With Risk Factors

The timing and number of hearing reevaluations for children with risk factors are customized and individualized depending on the relative likelihood of a subsequent delayed-onset hearing loss.

  • Infants who pass the neonatal screening but have a risk factor are to receive at least one diagnostic audiology assessment by 24 to 30 months of age.
  • Early and more frequent assessment may be indicated
    • for children with congenital cytomegalovirus (CMV) infection, syndromes associated with progressive hearing loss, neurodegenerative disorders, trauma, or culture-positive postnatal infections associated with sensorineural hearing loss;
    • for children who have received extracorporeal membrane oxygenation (ECMO) or chemotherapy;
    • when there is caregiver concern or a family history of hearing loss.

See the Treatment sections of the Hearing Loss (Early Childhood) and the Hearing Loss (Newborn) Evidence Maps for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

Service provision for children with permanent childhood hearing loss begins soon after the diagnosis is made and comprises audiologic re/habilitation-including the selection, fitting, and evaluation of technology (if amplification is selected as the treatment approach)-and a family-centered and interdisciplinary comprehensive early intervention program.

When selecting any treatment approach, the audiologist considers and incorporates the family's goals, preferences, values, beliefs, culture, and linguistic background.

Hearing Aids

Attempts should be made to fit hearing aids within 1 month of diagnosis. Loaner hearing aids can be provided until financing is secured, to avoid a delay in fitting.

Candidacy

Candidates for amplification include children with any degree of hearing loss that has the potential to impede access to speech. Candidates include children with

  • permanent bilateral hearing loss,
  • chronic hearing loss due to otitis media,
  • unilateral hearing loss,
  • auditory neuropathy (trial amplification based on behavioral results and observations).

In addition, cochlear implant candidates often have a trial amplification period.

Pre-Selection Considerations

  • Behind-the-ear (BTE) is the most commonly recommended type of device for infants and young children, because the softer, safer earmold materials are not easily cracked and broken. Earmolds will need frequent replacement to account for rapid ear growth through age 7 or 8 years.
  • Binaural amplification is to be provided to young children with bilateral hearing loss unless there is a clear contraindication (The Pediatric Working Group of the Conference on Amplification for Children With Auditory Deficits, 1996).
  • Connectivity to a wide range of HATs may be provided through direct audio input (DAI), built-in FM receiver, or telecoil.
  • Device selection considerations include the presence of flexible electroacoustic characteristics to accommodate changes in hearing over time; multiple memories to allow caregivers to adjust the hearing aid for different listening conditions; data-logging capability to monitor hearing aid use; volume control and memory button disable options; safety-related features, such as tamper-resistant battery doors; cosmetic considerations, such as earmold and/or hearing aid color; and the inclusion of a parent/caregiver kit.
Selection of Advanced Hearing Aid Features

Children with hearing loss may benefit from the provision of specific advanced signal-processing features in hearing aids, such as digital noise reduction, directional microphones, and frequency lowering strategies. Decisions about the activation or selection of these features, however, are typically based on available peer-reviewed evidence, as well as the age and listening needs of the child. Whereas some technologies have been investigated in older children, studies in this birth-to-5 years of age population have not been conducted at this time.

Verification

The audiologist obtains probe microphone measures of real-ear performance from the child whenever possible. Aided functional gain measured in a sound field for the purpose of setting devices is not sufficient and may result in less than optimal hearing aid fittings (Humes & Kirn, 1990).

The primary goals of probe microphone measures for verifying a hearing aid fitting are to

  • optimize the audibility of speech across frequencies,
  • protect the child from loudness discomfort or potential damage to hearing from amplified sound,
  • provide quick, repeatable, and valid measures of "goodness of fit."

A prescriptive approach is essential. For infants, the most appropriate prescriptive approach is one that is audibility based and accounts for the physical differences between children and adults. The DSL [v5] and NAL-NL2 (National Acoustics Laboratories) fitting procedures can provide this information.

Sound pressure levels and ear canal resonance measured in young ears typically exceeds adult values due to the smaller residual volume between the earmold and tympanic membrane. The real-ear-to-coupler difference (RECD) is the difference between the acoustics measured in the 2cc coupler and the acoustics measured in the child's own ear with the earmold inserted. The hearing aid, using the infant's own earmold, can easily be set in the test box using the RECD combination with test-box measures to predict real-ear responses (The Pediatric Working Group of the Conference on Amplification for Children With Auditory Deficits, 1996).

RECD measurements are completed

  • during or after first fit,
  • during monitoring visits,
  • with all new earmold fittings.

The audiologist verifies hearing aid settings in the child's ear (when possible) or simulated test-box measures for soft, average, and loud speech stimuli and for maximum output limits.

Validation

Validation of the aided auditory function is a critical component of the pediatric amplification-fitting process. The purpose of validating aided auditory function is to demonstrate the benefits and a limitation of a child's aided-listening abilities for perceiving the speech.

In the audiology clinic, hearing aids are often validated using developmentally and linguistically appropriate speech-perception materials in an aided sound-field condition. Speech stimuli, such as the Ling sounds, can be used for functional assessment of the audibility of speech sounds in the clinic or in other settings. Hearing aids are validated in the home/daycare or school setting by parents/teachers/ early intervention providers using questionnaires that measure functional auditory performance based on age.

Counseling and Orientation

Typically, all family members and any professionals who will be working with the child receive hearing aid orientation. Printed handouts in easy-to-understand language are provided to supplement the orientation, because many parents will not remember all of the information presented (Eiser, Parkyn, Havermans, & McNinch, 1994; Reese & Hnath-Chisolm, 2005).

Counseling and orientation typically include information regarding

  • care, maintenance, and troubleshooting, such as
    • cleaning and storage,
    • listening check with a stethoscope,
    • battery checks,
    • acoustic feedback;
  • safety features and issues, such as
    • battery ingestion and tamper-proof battery doors,
    • appropriate ear placement (left versus right);
  • realistic expectations, such as
    • effects of degree and configuration of hearing loss,
    • effects of background noise and distance,
    • environmental awareness;
  • importance of routine hearing aid usage.

Follow-Up Schedule

Fitting personal amplification in an infant or young child is an ongoing process. At a minimum, an audiologist typically sees a child every 3 months during the first 2 years of using amplification and every 4 to 6 months thereafter, with more frequent visits if there are concerns. Children with fluctuating or progressive hearing loss may need more frequent monitoring. Earmolds may need to be replaced every 3 to 4 weeks during the first year of the child's life (The Pediatric Working Group of the Conference on Amplification for Children With Auditory Deficits, 1996).

Follow-up appointments include

  • audiologic evaluation to monitor hearing status,
  • periodic assessment of functional measures to document auditory skills,
  • electroacoustic evaluation and listening checks of the hearing aids,
  • re-evaluation of the RECD and other probe-microphone measures as appropriate. RECD are typically re-measured whenever earmolds are replaced.

Cochlear Implants

Note: The section is an overview. A Practice Portal page on cochlear implants will be developed in the future.

Cochlear implant candidacy criteria have evolved over time with advances in cochlear implant technology and subsequent improvements in performance outcomes. Guidelines for cochlear implant candidacy are given with the FDA approval of each system and are based on the participant criteria used for the clinical investigation of the system's safety and efficacy. These guidelines have also changed substantially over time. As cochlear implant devices continue to improve, the criteria regarding the degree of hearing loss and the performance with a hearing aid that warrants consideration of a cochlear implant will continue to evolve.

Candidacy

Determining candidacy for cochlear implants requires an evaluation of the patient's medical, audiological, and psychosocial/habilitative condition using a team approach. Candidacy factors include:

  • bilateral severe-to-profound sensorineural hearing loss;
  • auditory neuropathy/dyssynchrony;
  • an age of 1 year or older (exceptions dependent on etiology);
  • a history of limited or no benefit from binaural hearing aids coupled with intensive auditory training;
  • an absence of medical contraindications, such as chronic middle ear pathology, some lesions of the VIIIth cranial nerve, some pathologies of the central auditory pathway, and other medical issues that preclude surgical procedures;
  • a family commitment to post-implant appointments and the re/habilitation process and realistic expectations for cochlear implant use and benefit.

Pre-implant Process

  • audiologic evaluation including hearing aid evaluation,
  • medical evaluation/radiologic studies,
  • vestibular evaluation,
  • psychosocial and/or psychological evaluation,
  • speech-language evaluation,
  • discussion of family expectations.

Device Selection

There are three manufacturers of cochlear implants in the United States: Cochlear Ltd., Advanced Bionics Corporation, and MED-EL. The selection of the device depends on a number of factors, including

  • U.S. Food and Drug Administration (FDA) indications for the device,
  • surgeon and audiologist recommendation,
  • family preference,
  • availability of devices at the cochlear implant center the family selects.

Bilateral Implantation

Research continues to assess the efficacy of bilateral cochlear implantation, which can be be performed simultaneously or sequentially. Advantages to bilateral cochlear implants are better sound localization and significantly improved speech recognition performance in the presence of background noise (Litovsky, Johnstone, & Godar, 2006).

Device Activation

Device activation occurs 2 to 6 weeks after implantation of the internal device. At the beginning of each appointment, the components on the external sound processor are inspected, and the impedances of each electrode are checked through telemetry to ensure adequate current flow.

The speech processor is programmed using objective or behavioral measures to provide sufficient stimulation of the internal electrodes so that speech and other auditory stimuli are audible, comfortable, and below a threshold of discomfort. An effort is typically made to restore normal loudness percepts.

Objective measures for mapping include the electrically evoked stapedius reflex threshold (ESRT) conducted with standard immittance equipment and the electrically evoked compound action potential (ECAP) available through the programming software. These measures are noninvasive and do not require a response from the patient.

Behavioral measures for mapping, such as BOA, VRA, and CPA, can be employed for children who are able to give reliable responses. These techniques are often paired with objective measures to ensure an appropriate map. Additionally, sound-field testing assists in verifying the appropriateness of the map for functional detection of sound.

Validation

Validation is a critical component of the cochlear implant (CI) programming process. The purpose of validating auditory function is to demonstrate the benefits and limitations of a child's listening abilities for perception of speech and other auditory stimuli.

Cochlear implant function is validated through speech perception, progress in speech-language therapy, and via questionnaires. Audiologists use developmentally and linguistically appropriate speech-perception materials in an aided sound-field condition to assess a hierarchy of listening skills that range from detection through comprehension of a spoken message. Proper function of the CI is also validated by the child's speech-language pathologist through achievement of age-appropriate goals and objectives. Validation is typically also accomplished in the home, daycare, or school setting using questionnaires that measure functional auditory performance in everyday listening environments.

Follow-Up

All family members and any professionals who will be working with the child typically receive orientation to the cochlear implant. Counseling and orientation include routine troubleshooting and realistic expectations for the child's performance.

Children return often for programming during the first year (e.g., 2, 4, 8, and 12 weeks, 6 months, 12 months). During this time, the programming parameters are refined. During these appointments, children are taught to provide feedback on soft, comfortable, and loud sounds.

Follow-up appointments include

  • impedance and equipment check,
  • updated programs,
  • behavioral assessment, including threshold and speech perception tests,
  • feedback from parents and providers regarding functional listening skills in the home or daycare environment,
  • discussion regarding progress made in auditory and communication development.

Hearing Assistive Technology: Personal FM Systems

In many situations, distance, background noise, and reverberation effects can interfere with optimal audibility. HATs can help a listener overcome the negative consequences of these variables. Remote microphone technology HATs overcome the effects of noise, distance, and reverberation by placing a microphone close to a talker's mouth or sound source. Thus, they can improve communication access for children in adverse listening environments.

While many remote microphone HAT options exist, the focus of this discussion is personal FM (frequency-modulated) systems. Personal FM systems consist of two parts: a wireless transmitter and a small wireless receiver. The transmitter sends a low power FM radio signal. This signal is picked up by the receiver, which may be connected to a hearing aid or cochlear implant processor or worn as an ear level FM-only receiver.

Candidacy

Children with any degree or type of hearing loss- including current or potential hearing aid and cochlear implant users-may be considered candidates for personal FM use.

Selection

Consideration factors for the selection of FM technology for this age include

  • the degree and configuration of the child's hearing loss, his or her current use of hearing technology, chronological and developmental age, and listening environments (home, daycare, car, school, etc.);
  • concerns, such as attention, hyperactivity, sensory integration, behavior, cognition, mobility, auditory processing, learning, vision, and fine motor issues;
  • technology issues (convenience, wearability, reliability, maintenance, compatibility with existing amplification, etc.);
  • parental/child/caregiver/educational motivation and ability to use the device and financial resources.

Verification

It is necessary for the audiologist to conduct verification of the performance of the FM system when fitting a child. Specific procedures vary based on the unique characteristics of the listener and the device configuration, but typically include one or more of the following

  • electroacoustic analysis,
  • real-ear probe microphone measures,
  • behavioral measures, such as sound-field aided speech recognition.

Validation

Validation is an ongoing process whose purpose is to demonstrate the benefits and limitations of a child's listening abilities for perception of speech. Objective validation for FM systems is typically conducted with developmentally appropriate speech-perception materials in conditions that reflect the typical listening environment of the listener. Subjective validation may also be completed using observation questionnaires completed by parents/teachers/caregivers. In addition, IDEA requires that use of assistive technology include a functional evaluation of the listener in his or her customary environment (Federal Register, 2006).

Follow-Up

Daily checks of the FM system are typically performed by the parent, teacher, speech-language pathologist, or anyone who has received appropriate training by the audiologist. A daily check consists of visual inspection of the device and its coupling, followed by listening to the sound quality. If possible, the listening check is performed in the room(s) where the FM system is used so that interference will be detected.

The audiologist's periodic comprehensive monitoring of the FM system may include electroacoustic analysis, probe microphone measurements, and other in-depth troubleshooting measures. Typically, these comprehensive procedures are performed at least annually and more frequently if there is a change in hearing status and/or hearing technology or an unresolved problem is identified during the daily check.

Periodic evaluations of a child's hearing status and performance with the FM device are necessary to monitor stability of hearing, appropriate device settings, and degree of benefit with the FM. These assessments may include, but are not limited to, audiologic evaluations, real-ear probe microphone measurements, behavioral assessment of speech recognition, and observations of performance in normal-use settings.

Other Devices (Osseointegrated Devices, Auditory Brainstem Implants)

Osseointegrated Devices

Osseointegrated devices (e.g., bone-anchored hearing aids) are considered for children with permanent bilateral conductive or mixed hearing loss or single-sided deafness. The FDA has not approved surgical implantation of bone-anchored hearing aids for children less than 5 years of age; however, children under 5 can wear the external processor in a soft band until they are old enough for implantation. There are currently two manufacturers for osseointegrated devices in the United States: Cochlear Corporation and Oticon.

Auditory Brainstem Implants (ABI)

Auditory brainstem implants (ABI) provide electrical stimulation of the cochlear nucleus in the brainstem for patients who do not have a cochlea or auditory nerve. Clinical trials are currently in process and include children who are not candidates for cochlear implantation. Research is needed to better describe the candidacy requirements and long-term outcomes for communication development with ABIs.

Pediatric Audiologic Re/habilitation (AR)

Audiologists' and speech-language pathologists' roles in the provision of pediatric audiologic re/habilitation services may be complementary, interrelated, and, at times, overlapping. It is important to understand that both professions also have distinct areas that are defined by their respective scopes of practice, certification standards, and preferred practice patterns. For example, audiologists are uniquely qualified to perform diagnostic assessment of hearing and to verify and validate hearing aids, cochlear implants, and other technology. Speech-language pathologists are uniquely qualified to perform diagnostic evaluations of communication, speech, language, cognition, and swallowing. Audiologists, however, may screen for speech, language, and communication skills and/or provide aural re/habilitation therapy.

Activities in which both audiologists and SLPs engage include

  • evaluating of functional communication performance;
  • conducting auditory perception training-including activities to improve sound awareness, discrimination, recognition, and comprehension;
  • managing and encouraging consistent use of devices;
  • counseling parents/caregivers regarding modes/methods of communication;
  • determinating optimal training and education settings;
  • monitoring acoustics of home, daycare, and school settings and providing recommendations for modifications;
  • counseling the child with hearing impairment and his or her family regarding peer pressure, stigma, and other issues related to psychosocial adjustment;
  • counseling the child and family regarding behavioral coping strategies;
  • monitoring treatment benefit and outcome;
  • collaborating effectively as part of multidisciplinary teams and communicating relevant information to allied professionals and other appropriate individuals;
  • planning and implementing parent-education programs concerning the management of hearing impairment and subsequent communication difficulties;
  • referring individuals to parent-to-parent support and other consumer-based organizations.

Children under age 3 are referred to both the state Early Hearing Detection and Intervention (EHDI) and Infant and Toddlers Early Intervention (Part C) programs as soon as they have been diagnosed with hearing loss. Children over 3 years of age are referred to their respective local area school systems' special education programs (Part B). Both Part C and Part B of IDEA are federally funded grant programs that assist states in developing, and in many instances funding, comprehensive services for children with disabilities, including hearing loss.

Specific services for children vary state by state and will depend on the individual needs of the child as dictated by the

  • current age of the child,
  • age that hearing loss started,
  • age at which hearing loss was diagnosed,
  • age at which hearing aids or cochlear implants were introduced,
  • age at which therapy and intervention services were started,
  • severity of hearing loss,
  • type of hearing loss,
  • comorbid conditions,
  • family desires and goals for their child.

Audiologists treat children who use a variety of communication methods, including listening and spoken language, cued speech or cued language, sign language, simultaneous communication, and total communication, often working collaboratively with SLPs, teachers of the deaf, and other professionals to provide services.

ASHA Resources

Additional Resources

This list of resources is not exhaustive and the inclusion of any specific resource does not imply endorsement from ASHA.

Organizations

American Academy of Pediatrics. (2006). Guidelines for monitoring and management of pediatric patients during and after sedation for diagnostic and therapeutic procedures: An update Pediatrics, 118(6), 2587-2602.

American Speech-Language-Hearing Association. (2010). Code of ethics [Ethics]. Available from www.asha.org/policy/.

American Speech-Language-Hearing Association. (2018). Scope of practice in audiology [Scope of practice]. Available from www.asha.org/policy/.

Boulet, S. L., Boyle, C. A., & Schieve, L. A. (2009). Health care use and health and functional impact of developmental disabilities of U.S. children, 1997-2005. Archives of Pediatrics and Adolescent Medicine, 163(1), 19-26.

Centers for Disease Control and Prevention. (2009). Summary of 2009 national CDC EHDI data [2009 CDC EHDI Hearing Screening & Follow-up Survey (HSFS)]. Retrieved from www.cdc.gov/ncbddd/hearingloss/2009-Data/2009_EHDI_HSFS_Summary_508_OK.pdf.

DesJardin, J. L. (2006). Family empowerment: Supporting language development in young children who are deaf or hard of hearing. Volta Review, 106(3), 275-298.

Eiser, C., Parkyn, T., Havermans, T., & McNinch, A. (1994). Parent's recall on the diagnosis of cancer in their child. Psycho-oncology, 3(3), 197-203.

Gorga, M. P., Neely, S. T., Olrich, B., Hoover, B., Redner, J., & Peters, J. (1997). From laboratory to clinic: A large scale study of distortion product otoacoustic emissions in ears with normal hearing and ears with hearing loss. Ear and Hearing, 18, 440-455.

Hille, E. T., van Straaten, H. I., & Verkerk, P. H. (2007). Prevalence and independent risk factors for hearing loss in NICU infants. Acta Pediatrica, 96(8), 1155-1158.

Humes, L. E., & Kirn, E. U. (1990). The reliability of functional gain. Journal of Speech and Hearing Disorders, 55, 193-197.

Hussain, D. M., Gorga, M. P., Neely, S. T., Keefe, D. H., & Peters, J. (1998). Transient evoked otoacoustic emissions in patients with normal hearing and in patients with hearing loss. Ear and Hearing, 19, 434-449.

Joint Committee on Infant Hearing. (2007). Year 2007 position statement: Principles and guidelines for early hearing detection and intervention. Pediatrics, 120, 898-921.

Kei, J. (2012). Acoustic stapedial reflexes in healthy neonates: Normative data and test-retest reliability. Journal of the American Academy of Audiology, 23(1), 460.

Litovsky, R., Johnstone, P., & Godar, S. (2006). Benefits of bilateral cochlear implants and/or hearing aids in children. International Journal of Audiology, 45(Suppl. 1), S78-S91.

Margolis, R.H., Bass-Ringdahl, S.B., Hanks, W.D., Holte, L., & Zapala, D.A. (2003). Tympanometry in newborn infants-1kHz norms. Journal of the American Academy of Audiology 14, 383-392.

Morton, C. C., & Nance, W. E. (2006). Newborn hearing screening-A silent revolution. New England Journal of Medicine, 354, 2151-2164.

Murphy, J., & O'Donoghue, G. (2007). Bilateral cochlear implantation: An evidence-based medicine evaluation. Laryngoscope, 117(8), 1412-1418.

National Institute on Deafness and Other Communication Disorders. (n.d.). Quick statistics. Retrieved from http://www.nidcd.nih.gov/health/statistics/Pages/quick.aspx.

New York State Department of Health, Early Intervention Program. (2007). Clinical practice guideline: Report of the recommendations. Hearing loss, assessment and intervention for young children (Age 0-3 Years). (NYS Department of Health, Publication No. 4967). Albany, NY: Author.

The Pediatric Working Group of the Conference on Amplification for Children with Auditory Deficits. (1996). Amplification for infants and children with hearing loss. American Journal of Audiology, 5(1), 53-68.

Reese, J. L., & Hnath-Chisolm, T. (2005). Recognition of hearing aid orientation content by first-time users. American Journal of Audiology, 14(1), 94-104.

Sininger, Y. S., Grimes, A., & Christensen, E. (2010). Auditory development in early amplified children: Factors influencing auditory-based communication outcomes in children with hearing loss. Ear and Hearing, 31(2),166-185.

Yoshinaga-Itano, C., Baca, R. L., &. Sedey, A. L. (2010). Describing the trajectory of language development in the presence of severe to profound hearing loss: A closer look at children with cochlear implants versus hearing aids. Otology and Neurotology, 31(8), 1268-1274.

Acknowledgements 

Content for ASHA's Practice Portal is developed through a comprehensive process that includes multiple rounds of subject matter expert input and review. ASHA extends its gratitude to the following subject matter experts who were involved in the development of the Permanent Childhood Hearing Loss page:

  • Tamala S. Bradham, PhD, CCC-A
  • Winnie Chung, AuD, CCC-A
  • Allan O. Diefendorf, PhD, CCC-A
  • Jeffrey K. Hoffman, PhD, CCC-A
  • Karen G. Munoz, EdD, CCC-A
  • Patricia A. Roush, AuD, CCC-A
  • Christine Yoshinago-Itano, PhD, CCC-A

In addition, ASHA thanks the members of the Working Group on Audiologic Assessment of Children from Birth to 5 Years of Age whose work was foundational to this document.  Members of the working group were Allan O. Diefendorf (coordinator), Kathryn L. Beauchaine, Patricia Connelly, Robert J. Nozza, Jackson Roush, Diane L. Sabo, Anne Marie Tharpe, Judith E. Widen, and Pam Mason (ex officio).  Susan Brannen and Roberta Aungst, served as monitoring officers as vice presidents for professional practice in audiology (2000-2003) and (2004-2007) respectively.

Citing the Practice Portal

The recommended citation for this Practice Portal page is:

American Speech-Language-Hearing Association (n.d). Permanent Childhood Hearing Loss. (Practice Portal). Retrieved month, day, year, from www.asha.org/Practice-Portal/Clinical-Topics/Permanent-Childhood-Hearing-Loss/.

Content Disclaimer: The Practice Portal, ASHA policy documents, and guidelines contain information for use in all settings; however, members must consider all applicable local, state and federal requirements when applying the information in their specific work setting.

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