Screening

The Joint Committee on Infant Hearing (JCIH) has endorsed hearing screening no later than 1 month of age. Most babies have their hearing screened shortly after birth. Babies who do not pass the newborn hearing screening should be referred immediately for a comprehensive audiological evaluation . The goal is to have hearing loss confirmed by 3 months of age.

Children who have passed the newborn hearing screen should have their hearing screened anytime there is a concern or prior to school entry.

Audiologic Assessment

Confirmation of hearing status in children birth through 5 years of age typically includes a battery of audiologic test procedures to:

• assess the integrity of the auditory system in each ear;
• estimate hearing sensitivity across the speech frequency zone;
• determine the type of hearing loss;
• establish a baseline for future monitoring; and to
• provide ear specific information collected using insert earphones needed to initiate amplification device fitting.*

*It is preferable to obtain thresholds using insert earphones because the child's real-ear-to-coupler difference (RECD) can be used to convert threshold measures to real-ear SPL. The click ABR provides insufficient information regarding both the degree and configuration of hearing loss-information that is critical for use with prescriptive selection and evaluation procedures.

ASHA's evidence map on Assessment for Permanent Childhood Hearing Loss shows the available scientific evidence, expert opinion and client/caregiver perspectives pertaining to this topic.

Audiologic Evaluation- Chronologic/Developmental Age of Birth - 6 months

The following assessment procedures are based on the work of the ASHA working group responsible for the development of guidelines for the audiological assessment of children from birth to 5 years of age, and the JCIH Position Statement. Audiologic evaluation for children birth through six months of age should include:

Case History: The case history will often guide the selection of a strategy for the audiological evaluation, and should consider several areas.

• Accurate diagnosis of hearing loss relies on interpretation of a test battery within the context of the child's medical and/or developmental history.
• Case history information may suggest 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 case history should be recorded using a standard form.

Otoscopy: Otoscopy in this population is used to ensure that there are no contraindications to placing an earphone or probe in the ear canal. 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. Because of the size and anatomy of the newborn ear, it may be difficult to identify the tympanic membrane or any landmarks.

Audiologic Test Battery: Auditory Evoked Potentials. ABR is an appropriate test for children who are too young for reliable behavioral testing. There is not sufficient evidence to recommend using Auditory Steady State Responses (ASSR) for threshold testing in this population at this time. 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. However, active or older infants may require sedation to allow adequate time for acquisition of high-quality recordings and sufficient frequency-specific information. (See section regarding Monitored Conscious Sedation).

Threshold Assessment Auditory Brainstem Response (ABR)

Stimuli: Frequency-specific stimuli (tone bursts of low, mid, and high frequency)

Transducer: A complete audiologic evaluation should include both an air-conduction and bone-conduction ABR

• Insert earphones are recommended, unless contraindicated, for air-conduction testing.
• Use caution when interpreting bone conduction ABR results as these 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.

Protocol: Responses are typically attempted down to 20 dB nHL in at least 10 dB stepsLink to Map: See available scientific evidence, expert opinion and client/caregiver perspectives pertaining to ABR.

Assessment of VIIIth Nerve Integrity (ABR)

Stimuli: Click stimuli at a high level (e.g., 70 dB nHL) will be adequate in most situations to identify Waves I, III, and V. If no response is obtained at the maximum output level, obtain one run of rarefaction clicks and one of condensation clicks to distinguish between cochlear and neural dysfunction. Use a catch trial (i.e., no signal) to rule out a stimulus artifact that may be misinterpreted as the cochlear microphonic (CM)Transducer: Insert earphones

Protocol: Compare interpeak latencies with corrected age norms.

Otoacoustic Emissions (OAE). 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; 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

Acoustic Immittance: Tympanometry and acoustic reflex testing should be used in conjunction to assess middle ear function. Under the age of approximately 4 months, interpretation of tympanograms and acoustic reflex findings may be compromised when a conventional low-frequency (220- or 226-Hz) probe tone is used. Between 5 and 7 months of 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
• Acoustic reflex thresholds

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

Audiologic Evaluation- Chronologic/Developmental Age 6 months - 3 years

The following assessment procedures are expert recommendations, based on the work of the ASHA working group responsible for the development of guidelines for the audiological assessment of children from birth to 5 years of age. Audiologic evaluation for children 6 months through 3 years of age typically should include the elements listed below.

Case History: The case history will often guide the selection of a strategy for the audiological evaluation.

• Accurate diagnosis of hearing loss relies on interpretation of a test battery within the context of the child's medical and/or developmental history.
• Case history information may suggest 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 case history should be recorded using a standard form.

Otoscopy: At a minimum, a limited examination consisting of visual inspection of the entrance to the ear canal will be sufficient prior to insertion of insert earphones. However, a complete otoscopic examination is recommended prior to immittance testing, OAE testing, or the insertion of probe microphones.

Audiologic Test Battery

Behavioral Assessment:

Visual reinforcement audiometry (VRA) and/or conditioned play audiometry (CPA) as developmentally appropriate. Because VRA requires that the infant have the developmental ability to respond to conditioned procedures, sit, maintain head control, and turn his or her head, it should only be performed on infants that are at a developmental age of at least 6 months. For children from approximately 6 months through 2 years of age, VRA is the recognized method of choice. As children mature beyond their second birthday, CPA may be attempted.

Stimuli: Frequency-specific (octave intervals from 250 to 6000Hz)

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.

Protocol: Minimum response levels (MRL) are typically obtained down to 20 dB HL; consider alternating between ears when testing.

Speech audiometry: Speech audiometry results are helpful for planning treatment and monitoring the child's ability to understand speech. Given that there are speech audiometry procedures that have been developed for infants and young children, it is recommended that an audiologic evaluation include these measures, such as:

• Speech detection threshold (SDT) or speech awareness threshold (SAT)
• Speech reception threshold (SRT) for spondees or body part identification
• Speech recognition of common words/sentences within the child's vocabulary

Auditory Evoked Potentials (EBP Recommendations): ABR should be a part of the test battery for this age population under the following circumstances:

• When behavioral audiometric tests are judged to be unreliable, ear-specific thresholds cannot be obtained, or when 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 two-month period; or
• If the neurological integrity of the auditory systems through the level of the brainstem is in question.

Otoacoustic Emissions (OAE): 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, 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

Acoustic Immittance: Tympanometry and acoustic reflex testing should be 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 through 7 months of age.

• Tympanograms using a low frequency (226-Hz) probe tone
• Acoustic reflex thresholds at 500, 2000, and 1000 Hz

Audiologic Evaluation- Chronologic/Developmental Age 3 years - 5 years

The following assessment procedures are expert recommendations, based on the work of the ASHA working group responsible for the development of guidelines for the audiological assessment of children from birth to 5 years of age. Audiologic evaluation for children age 3 -5 years typically includes the elements listed below.

Case History: The case history will often guide the selection of a strategy for the audiological evaluation and should include multiple elements.

• Accurate diagnosis of hearing loss relies on interpretation of a test battery within the context of the child's medical and/or developmental history.
• Case history information may suggest 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 case history should be recorded using a standard form.

Otoscopy: At a minimum, a limited examination consisting of visual inspection Otoscopy. Otoscopic examination of the external auditory canal (EAC) and tympanic membrane is necessary prior to the audiological evaluation. At the least, verification that the EAC is free of obstructions (e.g., foreign objects, impacted cerumen) and that there is no drainage from the middle ear is essential. To the extent possible, examination of the tympanic membrane with regard to color, position, and abnormalities should be attempted.

Audiologic Test Battery:

Behavioral Assessment: The abilities of children in the age range from 3 years to 5 years vary widely. The assessment method used is dependent to a large extent on the developmental level of the individual child.

Frequency-Specific Thresholds (VRA, CPA, conventional audiometric testing)

Stimuli: Speech and frequency-specific (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. Given that there are speech audiometry procedures that have been developed for infants and young children, it is recommended that an audiologic evaluation include these measures, such as:

• Speech detection threshold (SDT) or speech awareness threshold (SAT)
• Speech reception threshold (SRT) for spondees or body part identification
• Speech recognition of common words/sentences within the child's vocabulary

Auditory Evoked Potentials: ABR should be a part of the test battery for this age population under the following circumstances:

• when behavioral audiometric tests are judged to be unreliable, ear-specific thresholds cannot be obtained, or when 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 two-month period; or
• if the neurological integrity of the auditory systems through the level of the brainstem is in question.

Otoacoustic Emissions (OAE): 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, 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

Acoustic Immittance: Tympanometry and acoustic reflex testing should be used in conjunction to assess middle ear function

• Tympanograms using a low frequency (226-Hz) probe tone.
• Acoustic reflex thresholds 500, 1000, and 2000 Hz

Monitored Conscious Sedation

To gain the cooperation of some infants and young children during physiologic assessments of auditory function, sedation may be required. However sedation of pediatric patients 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. Additionally, the oversight by skilled medical personnel and the availability of age- and size-appropriate equipment, medications, and continuous monitoring are essential during procedures and in rescuing the child should an adverse sedation event occur.

Developmental and Communication Assessments

Pediatric audiologists are involved in developmental screening and functional auditory assessment in their patients. Examples of screening tools can be found in ASHA's Directory of Screening and Assessment Tools. Children with hearing loss should receive a complete developmental assessment and be evaluated across various domains including cognition, social, motor, self-help/adaptive.

The following areas that are typically monitored include:

• developmental screening;
• prelinguistic communication;
• receptive and expressive language status;
• auditory skill development;
• functional auditory performance; and
• social skills.

Documentation and Follow-Up of Assessment

Documentation for the audiologic assessment of children from birth to 5 years of age typically contains:

• pertinent background information;
• associated conditions (e.g. medical diagnosis, disability, home programs);
• assessment procedures used;
• interpretation of test results;
• type and severity of the hearing loss, and
• specific recommendations for follow-up

Follow-up: Newly Confirmed Hearing Loss

The following should be completed by 3 months of age for infants with confirmed hearing loss:

Review of the results and implications of the audiologic testing and recommendations for intervention with the parents/caregivers, including:

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

Children diagnosed with hearing loss of any type should be referred to an otolaryngologist for a medical and otologic evaluation. It is important for this evaluation to include a thorough review of the child's medical and family history; a physical examination of the ears, head, and neck; and possibly a neurotological evaluation . Additional audiologic, radiologic, and serum laboratory tests and evaluation by a medical geneticist or other specialists may be requested as indicated , Children with hearing loss should have ongoing otologic and audiologic monitoring because hearing loss can fluctuate or progress, and medical conditions can change or evolve over time

A family assessment should be conducted to determine the resources, concerns, and priorities of the family Initiate the amplification process if appropriate and ensure that medical clearance for amplification has been obtained. Hearing aids should be fit within one month of diagnosis.

Refer the family to the community's infant-toddler service coordinator for specific information regarding early intervention options and local resources. If not part of the infant-toddler services referral, contact the educational audiologist in the child's school district. Report, with consent, to the family/caregiver, to the infant's primary care provider, and to the referral source.

Audiologists must be vigilant in observing the few parents who demonstrate severe emotional responses to their child's diagnosis, particularly when those responses continue for lengthy periods of time and/or become more acute over time. In such cases, the parent's need for counseling or other supports may be beyond the scope of practice for audiologists, who should be prepared to refer families to appropriate mental health care professionals.

Because of their role in the 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.

Follow-up: Normal Hearing

• Review results of the audiologic assessment and provide information about risk indicators for progressive and delayed-onset hearing loss, as well as typical speech, language, and listening developmental milestones.
• Recommend re-evaluation if concerns about hearing or speech and language development arise.
• Report should be provided (with parental consent) to the infant's primary care provider, and to the referral source. If the child was referred from a newborn hearing screening program, results should also be provided to the state, based on state guidelines.

Follow-up: Children with Risk Factors

The timing and number of hearing reevaluations for children with risk factors should be 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 should have at least 1 diagnostic audiology assessment by 24 to 30 months of age.

Early and more frequent assessment may be indicated for children with 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; and when there is caregiver concern or a family history of hearing loss.