CSD 425 Final

Congenital HL

Present at birth

Genetic or in-utero factors

Acquired HL

Develops after birth

Infection, trauma, or aging

Behavioral Observation Audiometry (BOA)

Age range: 0-6 months

Observe the child's behavioral responses to sound (e.g., eyes widening, startle reflex).

Presenting various sound stimuli and observing the child's reactions.

Visual Reinforcement Audiometry (VRA)

Age range: 6-24 months

Uses visual stimuli (e.g., lights or toys) to reinforce responses to sound, often using a head-turn response.

Presenting sounds at different intensities and observing if the child turns their head towards the source of the sound, followed by a visual reward.

Play Audiometry

Age range: 2+ years

Involves a game-like activity (e.g., dropping a toy into a bucket) when the child hears a sound.

Playing a game with the child where they perform an action whenever they hear a sound.

AC Thresholds

Measures hearing thresholds through the entire auditory pathway (outer ear, middle ear, inner ear, auditory nerve).

 If air conduction thresholds are abnormal, it suggests a hearing loss that could be either conductive, sensorineural, or mixed.

BC Thresholds

Measures hearing thresholds through the inner ear and beyond, bypassing the outer and middle ear.

If bone conduction thresholds are normal but air conduction thresholds are abnormal, it indicates conductive hearing loss

Pure Tone Average (PTA):

Calculated as the average hearing threshold levels (dB HL) at 500 Hz, 1000 Hz, and
2000 Hz.
If the air conduction thresholds at 500 Hz = 40 dB, 1000 Hz = 45 dB, and
2000 Hz = 50 dB, the PTA would be (40 + 45 + 50) / 3 = 45 dB HL.

Normal Hearing

Air conduction (AC) and bone conduction (BC) thresholds within
normal range (-10 – 20 dB HL)

Conductive HL

Affects the outer or middle ear.

Hearing loss caused by a middle ear infection (otitis media). BC normal, AC elevated (air-bone gap)

Sensorineural HL

Affects the cochlea, auditory nerve, or beyond

Hearing loss caused by noise exposure

AC and BC elevated without significant air-bone gap

Mixed HL

Combination of conductive and sensorineural hearing loss.

A child with a genetic sensorineural hearing loss who also has fluid in the middle ear causing conductive hearing loss. Both AC and BC elevated with an air-bone gap

Effects of HL

Impacts speech, language development, academic performance, and social interactions

Tympanometry, otoacoustic emissions (OAEs), auditory brainstem response (ABR)

Assess middle ear function, cochlear health, and neural pathways.

OAEs and ABR are used widely in newborn hearing screening

Normal

-10-20 dB

Mild

21-40 dB

Moderate

41-55 dB

Moderate Severe

56-70 dB

Severe

71-90 dB

Profound

greater than 90 dB

Hearing aids

Designed to amplify sound for individuals with hearing loss. They work by capturing sound through a microphone and delivering it to the ear through the receiver. 

Behind-the-ear

In-the-ear

BAHA’s

Surgically implanted devices that bypass the outer and middle ear and directly stimulate the cochlea or inner ear by vibrating the bones of the skull.

Conductive hearing loss

*Cochlea needs to be intact in at least one ear

Cochlear Implant

Surgically implanted device that electrically stimulates the auditory nerve, bypassing the external ear, middle ear, and the hair cells and directly stimulates the auditory nerve

CI candidate for adults

>18 years 

Severe-profound bilateral sensorineural hearing loss (>70 dB PTA)

Unilateral serve to profound hearing loss (single sided deafness)

Functional auditory nerve 

Limited or no benefit from hearing aids

CI candidate for children

12 months to 17 years of age

Profound bilateral sensorineural hearing loss (>/= 90 dB PTA)

Unilateral profound hearing loss (single sided deafness), older than 5 years of age

Functional auditory nerve

Limited or no benefit from hearing aids

Brainstem/midbrain Implants

Electrode in the cochlear nucleus (brainstem implant) or in the inferior colliculus (midbrain implant). 

For those with no functioning auditory nerve. 

Bypass the cochlea and directly stimulate the brainstem or midbrain auditory pathways.

EBP principles

Scientific Evidence, Patient Values, Clinical Experience

Scientific Evidence (EBP)

Research data

Patient Values (EBP)

Patient preferences, cultural, and individual needs

Clinical Experience (EBP)

Professional expertise

Scientific Method + EBP

Hypothesis-Driven vs. Data-Driven

Hypothesis-driven: Pre-determined hypothesis guides data collection

Data-driven: Data collection drives analysis

Brainstem

The auditory brainstem, plays a crucial role in transmitting auditory information from the ears to higher brain regions.

It processes basic auditory features such as intensity and timing and helps to localize sound sources

Auditory Cortex

Located in the temporal lobe of the brain, responsible for processing sound information received from the ears.

It analyzes sound frequencies, temporal patterns, and spatial localization to interpret and recognize auditory stimuli

Early exposure to sound

During early infancy and childhood, exposure to auditory stimuli is vital for the development of auditory pathways and neural connections in the brain.

Auditory experiences shape the organization and function of the auditory system, laying the foundation for auditory processing abilities later in life

Cortical decoupling

In cases of hearing loss or auditory deprivation, the auditory cortex may become less responsive to auditory input, a phenomenon known as cortical decoupling. This reduced activity in the auditory cortex reflects the brain's adaptation to the lack of auditory stimulation

Cross-modal reorganization

In the absence of auditory input, other sensory modalities, such as vision or touch, may undergo structural and functional changes to compensate for the deficit.

This cross-modal reorganization involves the recruitment of brain regions typically dedicated to auditory processing for processing information from other sensory modalities

Auditory Neuroplasticity

the brain's ability to reorganize its structure and function in response to sensory experiences, including auditory input. 

This adaptive process allows the brain to optimize its functioning in changing environmental conditions and in the presence of sensory deficits

Sensitive Period

a window of time during early childhood, typically before the age of three, when the brain is particularly receptive to auditory input and experiences.

Optimal auditory development occurs during this period, and early intervention for hearing loss is crucial to capitalize on this window of opportunity

Critical Period

a narrower timeframe, approximately until the age of seven, during which auditory input has the most significant impact on neural development and auditory processing abilities.

After this period, neural plasticity decreases, and it becomes more challenging to achieve significant improvements in auditory function

Connectome Model

a conceptual framework for understanding brain function based on the mapping of neural connections, or "connectivity," within the brain.

identifying and characterizing the complex networks of interconnected brain regions involved in specific cognitive functions, including auditory processing.

helps researchers study how information flows and is integrated across different brain regions during auditory processing tasks

EDHI

By or before 1 month: Hearing screening.

By or before 3 months: Follow-up testing if hearing loss is suspected.

By or before 6 months: Intervention initiated.

Hierarchy of Auditory Skills

Detection, Discrimination, Identification/Recognition, Comprehension

Listening Age

Chronological age minus age of consistent hearing

Oral Communication

Cued Speech

Auditory oral (traditional)

Auditory Verbal

ASL

Sign Only

Bimodal/bicultural

Total communication

oral +Sign

oral + fingerspelling

oral + signed english

Counseling

Emotional support

Coaching

Teaching caregivers

Stages of Grief

Denial, anger, bargaining, depression, acceptance

AVT

Intervention for Children

Education, orientation, advocacy, and family support.

2. Coaching Strategies

Active listening, observation, engagement, demonstration, feedback, guided practice.

3. Communication Strategies

Acoustic highlighting, following the child's lead, parallel talk, expansion/extension, sabotage.

 

EI Transition

Most children with hearing loss arrive at school with appropriate EI treatment.

Some children may be diagnosed or develop hearing loss during school years.

Schools must ensure that children use necessary hearing devices

IEPs + Interdisciplinary Approach

IEPs (Individualized Education Programs): Not all children with hearing loss will need an IEP.

An interdisciplinary team, including audiologists and speech-language pathologists, helps provide comprehensive support

Audiologist Roles

Evaluate hearing loss and provide amplification.

Counsel parents and other professionals on hearing care.

SLP Roles

Evaluate speech, language, and literacy skills.

Provide speech therapy, if needed.

Serve as a bridge of communication between all parties

Classroom Acoustics & Accommodations

Reduce noise and provide assistive listening technologies.

Preferential seating and visual aids support accessibility.

Children HL Effects

May face difficulties in social settings.

Increased risk of listening effort, fatigue, and social isolation.

Normalizing hearing loss and promoting peer advocacy can help.

Minimal HL

Unilateral Hearing Loss (UHL)

Bilateral Minimal Hearing Loss (MBHL)

High-Frequency Hearing Loss 

Outcomes and Challenges of Minimal HL

 - At risk for academic difficulties and increased listening effort and fatigue.

- Providing amplification and/or classroom accommodations is critical

   - Possible challenges with technology, such as over-amplification and circuit noise.

Central Auditory Processing Disorder (CAPD)

Controversial disorder with symptoms overlapping other conditions like ADHD.

No gold standard test available; treatments should be individualized.

HL + Additional Disabilities

Prevalence: 40% of children with hearing loss have additional disabilities.

Etiology: Includes pre-natal, peri-natal, and post-natal factors.

Intervention: Early identification and amplification are key

Auditory Neuropathy

Hearing loss due to impaired signal transmission from inner ear to brain.

Variable outcomes and interventions

Bilingualism and Hearing Loss

Professionals should support home language and tailor interventions accordingly.

However, it is currently unclear how to develop interventions for multilingual families (we need to rely on evidence-informed practices

Can bilingual children with hearing loss learn two languages?

Yes, current research supports bilingual language development in children with hearing loss.

Hearing Assistance Technology (HAT)

Technology to enhance auditory environment, regardless of hearing loss.

Personal Systems (e.g., RM systems): Benefits only one person at a time.

Class Audio Distribution Systems: Provide optimal signal access in classrooms

RM System Benefits

Provide access to speech in noisy and reverberant environments.

May reduce listening effort and fatigue, especially beneficial for children with hearing loss.

RM system use at home has advantages but also limitations (e.g., child being disrupted, lack of localization cues etc.)

Pseudotherapies

Beware of unproven treatments lacking scientific support.

Properly research any therapies before considering them.

AIT therapies are one type of pseudoscientific treatments in our field

Quality of Life

an individual's overall well-being and satisfaction with various aspects of their life, including physical, emotional, and social dimensions

Personal Fulfillment (Psychosocial Well-being)

Statistics indicate lower self-rated health among individuals with hearing loss compared to those with normal hearing.

Communication difficulties impact access to healthcare.

High school dropout rates are higher with hearing loss.

Individuals with hearing loss have lower median net worth compared to those with normal hearing

Hearing Handicap Inventory for the Elderly (HHIE)

Screening tool for assessing hearing-related handicap in the elderly

Hearing Aid Satisfaction (SADL)

Measures satisfaction with hearing aids in daily life

International Outcome Inventory for Hearing Aids (IOI-HA)

Asses outcomes of hearing aid use

Auditory Rehab (Adults)

Sensory management, instruction, perceptual training, counseling

Sensory Management (AR)

Audiometry and objective measures (OAEs, ABRs)

Speech perception assessments

Technology fitting options: hearing aids, cochlear implants, BAHA

Instruction (AR)

Education on hearing loss and available technologies

Understanding communication environments

Importance of informational counseling

Perceptual Training (AR)

Goal and methods of auditory training

Computer-based programs and speech reading

Limitations and considerations

Counseling (AR)

Managing patient expectations

Addressing psychological issues and referrals to professional counselors

Use of Auditory Rehab

Low utilization rates of hearing aids among adults with hearing loss

Reasons for underutilization: perceived limitations, unrealistic expectations, cost, and access issues

Common Cause Theory

Brain degeneration links hearing loss and cognitive decline, possibly influenced by genetics

Cognitive Load Theory

Hearing loss increases cognitive effort, altering brain structure over time

Change in Brain Structure Theory

Plasticity changes trigger compensatory strategies, leading to cognitive decline

Reduced Socialization

Communication difficulties lead to isolation, reducing cognitive stimulation.

Aural Rehabilitation Benefits

Improves well-being, potentially prevents cognitive decline, reduces depression symptoms

Aural Rehabilitation Limitations

Small effect size, doesn't reverse existing cognitive decline, limited evidence for direct cognitive effects of auditory training.

Auditory Perceptual Training

Systematic presentation of sounds to improve speech recognition.

Theoretical framework: Enhance auditory skills, induce brain reorganization.

Evidence regarding AT effectiveness is unclear. Studies investigate different types of training and outcome measures. If anything the degree of the evidence is modest.