CLINICAL TOPICS

Permanent Childhood Hearing Loss

Overview

Incidence and Prevalence

Signs and Symptoms

Causes

Roles and Responsibilities

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
  • 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.

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,
  • 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

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.

  • 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.

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

  • 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).

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.

  • 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).

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

  • 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.

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

  • 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)

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

  • 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)

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.

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

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

  • 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.

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 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.

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

  • 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).

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.

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

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

  • 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 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

  • 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.

Treatment

Resources

References

Content Disclaimer: The Practice Resource Project, 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.