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Permanent Childhood Hearing Loss

Overview

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

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

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.

Incidence and Prevalence

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.

Signs and Symptoms

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.

Causes

Nongenetic Factors

Nongenetic factors of permanent hearing loss include

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

Acquired Hearing Loss

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

Roles and Responsibilities

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, 2004).

Appropriate roles for audiologists include:

As indicated in the Code of Ethics (ASHA, 2010), 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, 2007). As indicated in the Code of Ethics (ASHA, 2010), 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.

Assessment

See the Assessment section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

Identification/Screening

See the Screening section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

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

Expert Opinion

Children should receive a full audiologic assessment-to confirm the presence of a hearing loss and determine the type, configuration, and degree of the loss-if one or more of the following conditions exist.

  • The child fails a physiologic screening.
  • The child has been identified with a speech-language delay.
  • There are multiple clinical clues, known risk factors, or parental/health care provider suspicions of hearing loss.
  • The child has a history of recurrent and persistent otitis media with effusion.

(New York State Department of Health, Early Intervention Program, 2007)

 See the Audiologic Assessment section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

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

Expert Opinion

For infants from birth through a developmental age of 6 months, the test battery should include:

  • child and family history, including an assessment of risk factors and parental reports of the infant's response to sounds,
  • frequency-specific auditory brainstem response (ABR) with air-conducted bursts and bone-conducted bursts as needed,
  • click-evoked ABR,
  • OAEs (transient evoked or distortion product),
  • 1000 Hz tympanometry.

(JCIH, 2007)

See the Audiologic Assessment General Findings section of the permanent childhood hearing loss evidence map for pertinent evidence, expert opinion and client/caregiver perspective.

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.)

Evidence Highlights

Evidence indicates

  • Air-conduction ABR will be abnormal with all types of hearing loss, and bone-conduction ABR results will be abnormal if a mixed or sensorineural hearing loss is present.
  • The "Wave V latency of the ABR is increased with conductive hearing loss as compared to sensorineural hearing loss. Using both bone-conduction and air conduction ABR adds even more information in helping differentiate between a conductive and sensorineural hearing loss" (p. 71).
  • Bone conduction ABR results 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.

(New York State Department of Health, 2007)

See the ABR and ASSR sections of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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.

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.

Expert Opinion

"There is insufficient evidence for the use of auditory steady state response as the sole measure of auditory status in newborn and infant populations" (JCIH, 2007, p. 17).

See the ASSR section of the permanent childhood hearing loss evidence map for pertinent evidence, expert opinion and client/caregiver perspective.

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.

See the ABR section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

Evidence Highlight

Evidence indicates

  • Middle ear pathology, environmental noise, and other factors may affect OAE results.
  • OAE results will not yield information regarding the degree and configuration of hearing loss.
  • Lack of emissions does not verify that permanent hearing loss is present.
  • OAE test results alone will not identify children with hearing loss due to auditory neuropathy.

(New York State Department of Health, 2007)

See the OAE section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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.

See the Tympanometry/Acoustic Reflex section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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.

See the Behavioral Measures section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

Expert Opinion

For children with a developmental age between 6 and 36 months, the test battery should include

  • child and family history, including information on the child's attainment of communication milestones and a parental report of the child's auditory and visual behaviors,
  • behavioral audiometry (visual reinforcement or conditioned-play audiometry) consisting of pure tones across the frequency range as well as speech detection and speech recognition measures,
  • OAE,
  • tympanometry and acoustic reflex thresholds,
  • an ABR, if reliable responses cannot be obtained through audiometry.

(JCIH, 2007)

See the Audiologic Assessment section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/patient perspective.

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

See the Behavioral Measures section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

Auditory Evoked Potentials

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

(JCIH, 2007)

Evidence Highlight

Evidence indicates

  • Air-conduction ABR will be abnormal with all types of hearing loss, and bone-conduction ABR results will be abnormal if a mixed or sensorineural hearing loss is present.
  • The "Wave V latency of the ABR is increased with conductive hearing loss as compared to sensorineural hearing loss. Using both bone-conduction and air conduction ABR adds even more information in helping differentiate between a conductive and sensorineural hearing loss" ( p. 71).
  • Bone conduction ABR results 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.

(New York State Department of Health, 2007)

See the ABR section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

Evidence Highlight

Evidence indicates

  • Middle ear pathology, environmental noise, and other factors may affect OAE results.
  • OAE results will not yield information regarding the degree and configuration of hearing loss.
  • Lack of emissions does not verify that permanent hearing loss is present.
  • OAE test results alone will not identify children with hearing loss due to auditory neuropathy.

(New York State Department of Health, 2007)

See the OAE section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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.

See the Tympanometry/Acoustic Reflex section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

See the Behavioral Measures section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

Auditory Evoked Potentials

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

Evidence Highlights

Evidence indicates

  • Air-conduction ABR will be abnormal with all types of hearing loss, and bone-conduction ABR results will be abnormal if a mixed or sensorineural hearing loss is present.
  • The "Wave V latency of the ABR is increased with conductive hearing loss as compared to sensorineural hearing loss. Using both bone-conduction and air conduction ABR adds even more information in helping differentiate between a conductive and sensorineural hearing loss" (2007, p. 71).
  • Bone conduction ABR results 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.

(New York State Department of Health, 2007).

See the ABR section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

Evidence Highlight

Evidence indicates

  • Middle ear pathology, environmental noise, and other factors may affect OAE results.
  • OAE results will not yield information regarding the degree and configuration of hearing loss.
  • Lack of emissions does not verify that permanent hearing loss is present.
  • OAE test results alone will not identify children with hearing loss due to auditory neuropathy.

(New York State Department of Health, 2007).

See the OAE section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

Acoustic Immittance

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

See the Tympanometry/Acoustic Reflex section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

See the Communication Assessment section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

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

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 EDHI 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

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.

Treatment

See the Treatment section of the permanent childhood hearing loss evidence map 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.

Evidence Highlights

Evidence indicates

  • Use of amplification should "be initiated as soon as possible after the hearing loss is confirmed and that for the majority of children with hearing loss, hearing aids can provide benefit and access to sound and speech."
  • "No child is too young to use some form of amplification device and it is possible to begin hearing aid use as young as 3 to 4 weeks of age."
  • "Consistent and early use of appropriate amplification is critical to the optimal development of spoken language."

(New York State Department of Health, 2007, p. 144).

See the Hearing Aid section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

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

Pre-Selection Considerations

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

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

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

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

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:

Evidence Highlight

Evidence indicates

  • A variety of factors may influence intervention outcomes, including age of identification and initiation of intervention, amount of intervention, benefit gained from amplification, accessibility to intervention and support services, quality of language input, and family participation.
  • "In children with severe-to-profound sensorineural hearing loss, a cochlear implant in conjunction with other interventions can enhance speech perception, enhance speech production and intelligibility, enhance language acquisition, augment education, and increase visual attention" (p. 162).
(New York State Department of Health, 2007).

See the Cochlear Implants section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

Pre-implant Process

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

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).

Evidence Highlight

Evidence indicates "that bilateral implantation is more effective than unilateral or bimodal stimulation and that improved auditory abilities are seen if the implantations are performed simultaneously" (Murphy & O'Donoghue, 2007, p. 1418).

See the Cochlear Implants Placement section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

Hearing Assistive Technology: Personal FM Systems

See the Hearing Assistive Technology section of the permanent childhood hearing loss evidence map for a comprehensive overview of pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

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

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)

See the Aural Habilitation section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective.

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

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

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.

See the Communication Treatment section of the permanent childhood hearing loss evidence map for pertinent scientific evidence, expert opinion, and client/caregiver perspective. A Practice Portal page on SLP involvement in assessment and treatment for permanent childhood hearing loss will be developed.

Resources

ASHA Resources

ASHA IDEA Part C Issue Briefs

Natural Environments for Infants and Children who are Deaf or Hard of Hearing and Their Families

Additional Resources

IDEA Part C Final Regulations (2011) [PDF]

CDC-Hearing Loss in Children (pages for professionals and consumers)

Early Hearing Detection and Intervention - Pediatric Audiology Links to Services

Guidelines for Pediatric Medical Home Providers (flowchart)

Loud and Clear- Clinical Red Flags [PDF]

My Baby's Hearing

NIDCD-Communication Options for Children Who Are Deaf or Hard-of-Hearing [PDF]

The Early Childhood Technical Assistance Center (ECTA Center)

OPTION Schools, Inc.

OSEP TA Community-Part C Settings-Services in Natural Environments

U.S. Department of Education-Opening doors: Technology and communication options for children with hearing loss

Organizations

Alexander Graham Bell Association for the Deaf and Hard of Hearing

American Academy of Audiology

American Society for Deaf Children

Beginnings

Council for Exceptional Children

Hands & Voices

Listening and Spoken Language Knowledge Center

National Association for the Deaf

National Center for Hearing Assessment and Management

National Center on Severe and Sensory Disabilities

National Institute on Deafness and Other Communication Disorders

References

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