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 shortly after birth. Because hearing loss in childhood is often delayed-onset or progressive in nature, it is important to provide audiologic monitoring over time for children who are considered to be "at risk" for hearing loss. It is also important to note that some mild hearing losses will not be identified through newborn hearing screening due to the current limitations of the test equipment.

Hearing is critical to speech and language development, communication and learning. Early identification and intervention of hearing loss can lessen the impact on a child's development. The Joint Committee on Infant Hearing (JCIH, 2007) recommends all children be screened for hearing loss by one month of age, that hearing and medical evaluations be completed by 3 months of age and that for infants with confirmed hearing loss early intervention services begin by 6 months of age.

Family Centered Practice

Families should be actively involved in the assessment process to the extent they desire and to the extent feasible given the nature of the audiologic test procedure. The audiologist must engage the family in the case history and testing session(s), and the family must fully participate in deciding on intervention strategies. The family's rights (including informed consent and confidentiality issues), reasonable expectations, reasonable needs, and preferences are paramount and must be considered.

Incidence and Prevalence

The term 'prevalence' refers to the estimated population living with permanent childhood hearing loss at any given time. Estimates of the prevalence of permanent hearing loss in children under five vary according to the source of data and the criteria for defining hearing loss. Most estimates suggest a prevalence of 1 to 3 per 1000 children based on screening and/or medical records. Prevalence estimates based on parental surveys are significantly higher, as is the prevalence in neonatal intensive care units.

Signs and Symptoms

The sooner hearing loss is diagnosed and intervention is initiated, the better the outcomes for speech and language development. 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.

Infants and young children with normal hearing sensitivity generally develop listening and communication skills in a typical developmental sequence. Children should acquire skills (milestones) by a certain age. A delay in these milestones may be a sign of hearing loss or other developmental delay.

Causes

Non-genetic factors can account for about 25% of permanent hearing loss in children. Non-genetic factors known to cause permanent hearing loss can include:

• maternal infections, such as rubella (German measles), cytomegalovirus, or herpes simplex virus;
• prematurity;
• low birth weight;
• birth injuries;
• toxins including drugs and alcohol consumed by the mother during pregnancy ;
• complications associated with the Rh factor in the blood/jaundice;
• maternal diabetes;
• toxemia during pregnancy;
• lack of oxygen (anoxia);
• cochlear malformations (Mondini's dysplasia); and.
• outer/middle ear malformations (atresia, stenosis).

Genetic factors (hereditary) are thought to cause more than 50% of all hearing loss. Hearing loss from genetic defects can be present at birth or develop later on 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. There are many genetic syndromes that include hearing loss as one of the symptoms. In fact, 20% of babies with genetic hearing loss have a syndrome. Examples include:

• Down syndrome;
• Usher syndrome;
• Treacher Collins syndrome;
• Crouzon syndrome;
• Alport syndrome;
• Sickle cell disease;
• Tay Sachs;
• Waardenburg syndrome;
• Pendred's syndrome (Large Vestibular Aqueduct).

Acquired hearing loss is a hearing loss that occurs after birth. The following are examples of conditions that can cause permanent acquired hearing loss in children:

• ear infections;
• ototoxic medications;
• meningitis;
• measles;
• encephalitis
• chicken pox;
• flu;
• mumps;
• large vestibular aqueduct;
• head injury; and
• noise exposure.

Roles and Responsibilities

Roles and Responsibilities of Audiologists

Audiologists play a critical role in the identification, assessment and rehabilitation of patients with permanent childhood hearing loss. The professional roles and activities in audiology include clinical/education services (identification, assessment, planning, and treatment), prevention and advocacy, and education, administration and research.

Professional roles and responsibilities for audiologists include, but are not limited to:

• prevention, including monitoring of the acoustic environment and promotion of hearing wellness;
• identification, including early detection and screening program development, management, quality assessment and service coordination;
• comprehensive assessment, including behavioral, electroacoustic and/or electrophysiologic methods to assess hearing, auditory function, balance, and related systems;
• evaluation for candidacy for amplification and other sensory devices and assistive technology, and the fitting and maintenance of such devices for optimal use;
• rehabilitation, including the development and implementation of an appropriate audiologic rehabilitative management plan ;
• documentation, including interpretation of data, summarization of findings, recommendations and the development of an audiologic treatment/management plan;
• referral to other professions, agencies, and/or consumer organizations;
• counseling on the psychosocial aspects of hearing loss and other auditory dysfunction, and processes to enhance communication competence;
• advocacy for communication needs of all individuals that may include advocating for rights/funding of services for those with and without hearing loss, auditory, and/or vestibular disorders.;
• consultation with professionals of related and/or allied services when needed, including educators, public and private agencies, and governmental bodies regarding communication management, educational implications, accessibility, and legal implications of hearing loss and/or other auditory dysfunction;
• education, supervision, and administration for audiology graduate and other professional education programs; and
• research to increase the knowledge base, to develop new methods and programs, and to determine the efficacy, effectiveness, and efficiency of assessment and treatment paradigms, and to disseminate research findings to other professionals and the public.

See ASHA's Scope of Practice in Audiology for more information.

Roles and Responsibilities of Speech Language Pathologists

Speech Language Pathologists play a critical role in the identification, assessment and rehabilitation of patients with Permanent Childhood Hearing Loss. Professional roles and responsibilities for speech language pathologists include, but are not limited to:

• provision of clinical services including: prevention and prereferral; screening; assessment/evaluation; consultation; diagnosis; treatment, intervention, management; counseling; collaboration; documentation; and referral.
• prevention and advocacy activities related to human communication and swallowing including: promoting healthy lifestyle practices; presenting primary prevention information; early identification and early intervention services; advocacy for individuals and families; advising regulatory and legislative agencies; and advocacy;
• serving as educators, administrators, and researchers, including
• educating public regarding communication and swallowing; providing in-service training to families, caregivers and other professionals; educating, supervising and mentoring current and future speech-language pathologists; fostering public awareness of communication and swallowing disorders and their treatment; serving as expert witnesses; administering and managing clinical and academic programs; developing policies, operational procedures and professional standards; and conducting research related to communication sciences and disorders.

See ASHA's Scope of Practice in Speech-Language Pathology for more information.

Assessment

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.

Treatment

Service provision for children with PCHL begins soon after the diagnosis is made and is comprised of audiologic re/habilitation including the selection, fitting and evaluation of technology and a comprehensive early intervention program that is family-centered and interdisciplinary. See ASHA's evidence map on Treatment for pertinent evidence, clinical expertise and patient perspectives.

Hearing Aids

Attempts should be made to fit hearing aids within one month of diagnosis. See ASHA's evidence map on Hearing Aids for pertinent evidence, clinical expertise and patient perspectives.

Candidacy

A child with any degree of hearing loss that has the potential to impede access to speech is a potential candidate. Specific considerations include:

• chronic hearing loss due to otitis media may have detrimental long-term effects (ref); often, however, short-term hearing loss can more readily be ameliorated and/or managed by medical intervention;
• permanent bilateral hearing loss; and
• unilateral hearing loss has also been shown to impact speech and language, psychosocial, and academic success. Children with unilateral hearing loss that is within a range where speech audibility can be improved should be considered candidates for amplification.

Preselection considerations

The behind-the-ear (BTE) style is most commonly recommended for infants and young children for the following reasons:

• earmolds will need frequent replacement to account for rapid ear growth through age 7 or 8;
• vinyl or silicone earmold materials that are not easily cracked or broken and that are easy to modify;
• binaural amplification should always be provided to young children with bilateral hearing loss unless there is a clear contraindication;
• connectivity considerations, depending upon the age and involvement of the child; and
• Direct Audio Input (DAI), or built in FM receiver to allow compatibility with Hearing Assistive Techology (HAT) as required;
• flexible electroacoustic characteristics to accommodate changes in hearing over time;
• multiple memories to allow the parent to adjust the hearing aid for different listening conditions; and
• safety-related features such as tamper resistant battery doors.

Selection

A wide range of signal processing strategies are available in hearing aids. Selection of these features should be based on the child's listening needs and existing evidence.

Feedback management: Acoustic feedback can result in limited high-frequency gain and parent or caregiver frustration. Feedback management is recommended for children as long as the processing does not impose significant limitations on the bandwidth of the device or aided audibility of speech.

Digital noise reduction: Limited evidence with school-age children suggests that DNR does not negatively impact speech understanding or complex learning tasks. DNR is recommended for school-age children as long as the DNR strategy does not result in significant reduction in audibility for speech.

Directional microphones: Directional microphones can improve the signal-to-noise ratio in situations where speech and noise are spatially separated and the child is able to orient towards the signal of interest. Directional microphones may be appropriate for school-age children with hearing loss.

Frequency-lowering: Limitations in hearing-aid bandwidth can impact speech perception and communication development. Frequency-lowering signal processing strategies alter the spectrum of the signal to increase the audibility of sounds that occur beyond the audible bandwidth of the hearing aid. Frequency lowering should be used in cases where verification demonstrates that this type of signal processing can provide greater bandwidth than conventional processing.

Verification

In order to quantity the audibility of the speech signal, probe microphone measures of real-ear performance should be obtained on children 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 ( Seewald et al, 1999).

Primary goals of probe microphone measures for verifying a hearing aid fitting are: to optimize the audibility of speech across frequencies ; to protect the child from loudness discomfort or potential damage to hearing from amplified sound; to provide quick, repeatable and valid measures of "goodness of fit"; and to estimate the impact of signal processing strategies on audibility.

A prescriptive approach can aid the clinician in optimizing the fit to ensure important speech cues are sufficiently amplified. 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 [i/o] and NAL-NL (National Acoustics Laboratories-Non-Linear) 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 (Stelmachowicz et al ). 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 estimate the real ear responses.

RECD measurements should be completed

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

The hearing aid settings should be verified in the child's ear (when possible) or simulated test box measures for soft, average, and loud speech stimuli, and 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 limitations of a child's aided listening. Validation typically takes the form of parent-reports.

In the audiology clinic, hearing aids are often validated using developmentally appropriate speech perception materials in an aided sound field condition. Hearing aids should also be validated in the home/ daycare or school setting by parents/teachers/EI providers using questionnaires that measure functional auditory performance based on age.

Follow-up for Children with Hearing Aids

All family members and any professionals who will be working with the child should receive hearing aid orientation. Counseling and orientation should include routine troubleshooting and realistic expectations for the child's performance with the devices.

Fitting of personal amplification in an infant or young child is an on-going process. Minimally, an audiologist should see the child every three months during the first two years of using amplification and every 4-6 months thereafter if there are no concerns. Children with fluctuating or progressive hearing loss may need more frequent monitoring. (The Pediatric Working Group, 1996).

Follow-up appointments should include:

• audiologic evaluation to monitor hearing status;
• periodic assessment of functional measures to document auditory skills;
• electroacoustic evaluation and listening checks of the hearing aids/s;
• re-evaluation of the RECD* and other probe-microphone measures as appropriate

*RECD should be remeasured whenever earmolds are replaced.

Cochlear Implants

See the available scientific evidence, expert opinion and client/caregiver perspectives pertaining toCochlear Implants in ASHA's evidence map. t http://www.ncepmaps.org/PCHL-Treatment-Cochlear-Implants.php The following are expert recommendations, based on the work of the ASHA working group responsible for the development of the technical report on Cochlear Implants.

Candidacy

Specific considerations for cochlear implant candidacy include:

• bilateral severe to profound SNHL with a pure-tone average of 90 dB in the better ear;
• auditory neuropathy/dyssynchrony;
• 12 months of age or older (exceptions dependent on etiology);
• limited or no benefit from binaural hearing aids coupled with intensive auditory training;
• no medical contraindications;
• realistic expectations for cochlear implant use;
• family commitment to habilitation/rehabilitation process;

Pre-implant Process

Steps in the pre-implant process include;

• audiologic evaluation;
• medical evaluation/radiologic studies;
• vestibular evaluation;
• psycho-social evaluation;
• discussion regarding expectations

Device Selection

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

• FDA indications for the device;
• age of the patient;
• family preference;
• availability of devices at the CI center the family selects

Bilateral Implantation

Research continues to assess the efficacy of bilateral cochlear implantation. Binaural cochlear implantation can be performed simultaneously or sequentially. Suggested advantages to binaural CI are better sound localization and marginally improved speech understanding scores.

Activation of the device

Device activation occurs 2-6 weeks after the surgery. After a listening check of the microphone, the impedances of each electrode are checked through telemetry.

Programming (mapping) the speech processor

The speech processor is programmed to provide sufficient stimulation of the internal electrodes so that speech is audible but below a threshold of discomfort. Objective measures for mapping include electrically evoked auditory brainstem response testing (EABR) and neural response telemetry (NRT). These measures, available through the programming software, are noninvasive and do not require a response from the patient.

Behavioral measures for mapping can be employed as children become older and are able to respond appropriately. Additionally, sound field testing will assist in verifying the appropriateness of the map for functional detection of sound.

Validation

Validation is a critical component of the 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.

Cochlear implants are validated using developmentally appropriate speech perception materials in an aided sound field condition. Speech perception testing should include a hierarchy of listening skills that range from detection, discrimination through recognition of speech.

Validation should also be accomplished in the home/ daycare or school setting using questionnaires that measure functional auditory performance.

Follow-up for Children with Cochlear Implants

All family members and any professionals who will be working with the child should receive orientation to the cochlear implant. Counseling and orientation should 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 then 12 months). During this time the sensitivity of the electrodes is refined. Over time, children are taught to provide feedback on soft, comfortable and loud sounds.

Follow-up appointments should include:

• equipment check;
• continued programming of electrodes;
• behavioral evaluation including threshold and speech perception tests;
• feedback from parents and providers regarding functional listening skills in the home or daycare environment; and
• discussion regarding progress made in auditory and communication development

Hearing Assistive Technology: Personal FM Systems

In many situations, distance, background noise and reverberation effects may interfere with optimal audibility. Hearing Assistive Technology (HAT) 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 earlevel FM-only receiver.

See ASHA's evidence map on Hearing Assistive Technology Systems (HATS) Permanent Childhood Hearing Loss: Treatment - Hearing Assistive Techonology Systems (HATS)), showing the available scientific evidence, expert opinion and client/caregiver perspectives.

Candidacy

Children who may be candidates for personal FM use include those with the following audiologic, listening and/or learning concerns:

• hearing loss (including current or potential hearing aid and cochlear implant users);
• auditory neuropathy spectrum disorder;
• auditory processing deficits;
• learning disability;
• language deficit;
• attention deficit; and
• English Language Learner

Selection

Selection of FM technology for this age group may take the following into consideration:

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

Verification

Careful verification of the performance of the system is necessary when fitting a child with an FM system. Specific procedures will vary based on the unique characteristics of the listener and the device configuration, but typically include one or more of the following:

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

Validation

Validation is an ongoing process with the purpose of demonstrating the benefits and limitations of a child's listening abilities for perception of speech. Objective validation for FM systems should be 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 their customary environment.

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 should be performed in the room(s) where the FM system will be used so that any interference will be detected.

Periodic comprehensive monitoring of the FM system by the audiologist may include electroacoustic analysis, probe microphone measurements, and other in-depth troubleshooting measures. These comprehensive procedures should be performed at least annually. Monitoring procedures should be completed 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 hearing status and a child's performance with the FM device are necessary to monitor stability of hearing, appropriate device settings, function, 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 (bone anchored hearing aids, auditory brainstem implants)

Osseointegrated device, egbone-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 five years of age; however, children under five can wear the external processor in a soft band until they are old enough for implantation.

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 now 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.

Resources

ASHA- Information for Audiologists and Speech-Language Pathologists

ASHA- IDEA Part C Issue Brief: Implications for Audiologists and Speech-Language Pathologists Who Provide Services for Infants and Toddlers With Hearing Loss and Their Families

ASHA's Fact Sheet on Natural Environments

ASHA IDEA Part C Issue Briefs

American Speech-Language-Hearing Association. (2008). Loss to Follow-Up in Early Hearing Detection and Intervention [Technical Report].

American Speech-Language-Hearing Association. (2008). Guidelines for Audiologists Providing Informational and Adjustment Counseling to Families of Infants and Young Children With Hearing Loss Birth to 5 Years of Age [Guidelines].

Guidelines for Pediatric Medical Home Providers

CDC- Hearing Loss in Children

IDEA Part C Final Regulations (2011) [PDF]

Loud and Clear- Clinical Red Flags-Amy McConkey Robbins (2005)

My Baby's Hearing

NIDCD- Communication Options for Children Who Are Deaf or Hard‐ of‐Hearing

Joint Committee on Infant Hearing (JCIH)

National Early Childhood Technical Assistance Center (NECTAC)

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

References

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