Amblyaudia

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Amblyaudia (amblyos- blunt; audia-hearing) is a term coined by Dr. Deborah Moncrieff to characterize a specific pattern of performance from dichotic listening tests. Dichotic listening tests are widely used to assess individuals for binaural integration, a type of auditory processing skill. During the tests, individuals are asked to identify different words presented simultaneously to the two ears. Normal listeners can identify the words fairly well and show a small difference between the two ears with one ear slightly dominant over the other. For the majority of listeners, this small difference is referred to as a "right-ear advantage" because their right ear performs slightly better than their left ear. But some normal individuals produce a "left-ear advantage" during dichotic tests and others perform at equal levels in the two ears. Amblyaudia is diagnosed when the scores from the two ears are significantly different with the individual's dominant ear score much higher than the score in the non-dominant ear [1] Researchers interested in understanding the neurophysiological underpinnings of amblyaudia consider it to be a brain based hearing disorder that may be inherited or that may result from auditory deprivation during critical periods of brain development. [2] Individuals with amblyaudia have normal hearing sensitivity (in other words they hear soft sounds) but have difficulty hearing in noisy environments like restaurants or classrooms. Even in quiet environments, individuals with amblyaudia may fail to understand what they are hearing, especially if the information is new or complicated. Amblyaudia can be conceptualized as the auditory analog of the better known central visual disorder amblyopia. The term “lazy ear” has been used to describe amblyaudia although it is currently not known whether it stems from deficits in the auditory periphery (middle ear or cochlea) or from other parts of the auditory system in the brain, or both. A characteristic of amblyaudia is suppression of activity in the non-dominant auditory pathway by activity in the dominant pathway which may be genetically determined [3] and which could also be exacerbated by conditions throughout early development.

Contents

Symptoms and signs

Children with amblyaudia experience difficulties in speech perception, [4] particularly in noisy environments, sound localization, [5] and binaural unmasking [6] [7] [8] [9] [10] (using interaural cues to hear better in noise) despite having normal hearing sensitivity (as indexed through pure tone audiometry). These symptoms may lead to difficulty attending to auditory information causing many to speculate that language acquisition and academic achievement may be deleteriously affected in children with amblyaudia. A significant deficit in a child's ability to use and comprehend expressive language may be seen in children who lacked auditory stimulation throughout the critical periods of auditory system development. A child suffering from amblyaudia may have trouble in appropriate vocabulary comprehension and production and the use of past, present and future tenses. Amblyaudia has been diagnosed in many children with reported difficulties understanding and learning from listening [11] [12] [13] and adjudicated adolescents are at a significantly high risk for amblyaudia (Moncrieff, et al., 2013, Seminars in Hearing).

Risk Factors

Families report the presence of amblyaudia in several individuals, suggesting that it may be genetic in nature. It is possible that abnormal auditory input during the first two years of life may increase a child's risk for amblyaudia, although the precise relationship between deprivation timing and development of amblyaudia is still unclear. Recurrent ear infections (otitis media) are the leading cause of temporary auditory deprivation in young children. [14] [15] [16] During ear infection bouts, the quality of the signal that reaches the auditory regions of the brains of a subset of children with OM is degraded in both timing and magnitude. [17] [18] When this degradation is asymmetric (worse in one ear than the other) the binaural cues associated with sound localization can also be degraded. Aural atresia (a closed external auditory canal) also causes temporary auditory deprivation in young children. Hearing can be restored to children with ear infections and aural atresia through surgical intervention (although ear infections will also resolve spontaneously). Nevertheless, children with histories of auditory deprivation secondary to these diseases can experience amblyaudia for years after their hearing has been restored. [6] [19]

Physiology

Amblyaudia is a deficit in binaural integration of environmental information entering the auditory system. It is a disorder related to brain organization and function rather than what is typically considered a “hearing loss” (damage to the cochlea). It may be genetic or developmentally acquired or both. When animals are temporarily deprived of hearing from an early age, profound changes occur in the brain. Specifically, cell sizes in brainstem nuclei are reduced, [20] [21] [22] [23] the configuration of brainstem dendrites are altered [24] [25] [26] and neurons respond in different ways to sounds presented to both the deprived and non-deprived ears. [27] [28] [29] [30]

An electrophysiologic study demonstrated that children with amblyaudia (referred to then as a "left-ear deficit") were less able to process information from their non-dominant ears when competing information is arriving at their dominant ears. The N400-P800 complex [31] showed a strong and highly correlated response from the dominant and non-dominant ears among normal children while the response from children with amblyaudia was uncorrelated and indicated an inability to separate information arriving at the non-dominant ear from the information arriving at the dominant ear. The same children also produced weaker fMRI responses from their non-dominant left ears when processing dichotic material in the scanner. [32]

Diagnosis

A clinical diagnosis of amblyaudia is made following dichotic listening testing as part of an auditory processing evaluation. Clinicians are advised to use newly developed dichotic listening tests that provide normative cut-off scores for the listener's dominant and non-dominant ears. These are the Randomized Dichotic Digits Test [33] and the Dichotic Words Test. [34] Older dichotic listening tests that provide normative information for the right and left ears can be used to supplement these two tests for support of the diagnosis ( [1] ). If performance across two or more dichotic listening tests is normal in the dominant ear and significantly below normal in the non-dominant ear, a diagnosis of amblyaudia can be made. [35]

Treatments

A number of computer-based auditory training programs exist for children with generalized Auditory Processing Disorders (APD). In the visual system, it has been proven that adults with amblyopia can improve their visual acuity with targeted brain training programs (perceptual learning). [36] A focused perceptual training protocol for children with amblyaudia called Auditory Rehabilitation for Interaural Asymmetry (ARIA) was developed in 2001 [37] which has been found to improve dichotic listening performance in the non-dominant ear and enhance general listening skills.

See also

Related Research Articles

The octave illusion is an auditory illusion discovered by Diana Deutsch in 1973. It is produced when two tones that are an octave apart are repeatedly played in alternation ("high-low-high-low") through stereo headphones. The same sequence is played to both ears simultaneously; however when the right ear receives the high tone, the left ear receives the low tone, and conversely. Instead of hearing two alternating pitches, most subjects instead hear a single tone that alternates between ears while at the same time its pitch alternates between high and low.

<span class="mw-page-title-main">Hearing test</span> Evaluation of the sensitivity of a persons sense of hearing

A hearing test provides an evaluation of the sensitivity of a person's sense of hearing and is most often performed by an audiologist using an audiometer. An audiometer is used to determine a person's hearing sensitivity at different frequencies. There are other hearing tests as well, e.g., Weber test and Rinne test.

<span class="mw-page-title-main">Amblyopia</span> Failure of the brain to process input from one eye

Amblyopia, also called lazy eye, is a disorder of sight in which the brain fails to fully process input from one eye and over time favors the other eye. It results in decreased vision in an eye that typically appears normal in other aspects. Amblyopia is the most common cause of decreased vision in a single eye among children and younger adults.

Sound localization is a listener's ability to identify the location or origin of a detected sound in direction and distance.

Auditory neuropathy (AN) is a hearing disorder in which the outer hair cells of the cochlea are present and functional, but sound information is not transmitted sufficiently by the auditory nerve to the brain. The cause may be several dysfunctions of the inner hair cells of the cochlea or spiral ganglion neuron levels. Hearing loss with AN can range from normal hearing sensitivity to profound hearing loss.

Unilateral hearing loss (UHL) is a type of hearing impairment where there is normal hearing in one ear and impaired hearing in the other ear.

<span class="mw-page-title-main">Cocktail party effect</span> Ability of the brain to focus on a single auditory stimulus by filtering out background noise

The cocktail party effect refers to a phenomenon wherein the brain focuses a person's attention on a particular stimulus, usually auditory. This focus excludes a range of other stimuli from conscious awareness, as when a partygoer follows a single conversation in a noisy room. This ability is widely distributed among humans, with most listeners more or less easily able to portion the totality of sound detected by the ears into distinct streams, and subsequently to decide which streams are most pertinent, excluding all or most others.

<span class="mw-page-title-main">Superior olivary complex</span> Collection of brainstem nuclei related to hearing

The superior olivary complex (SOC) or superior olive is a collection of brainstem nuclei that is located in pons, functions in multiple aspects of hearing and is an important component of the ascending and descending auditory pathways of the auditory system. The SOC is intimately related to the trapezoid body: most of the cell groups of the SOC are dorsal to this axon bundle while a number of cell groups are embedded in the trapezoid body. Overall, the SOC displays a significant interspecies variation, being largest in bats and rodents and smaller in primates.

The auditory brainstem response (ABR), also called brainstem evoked response audiometry (BERA) or brainstem auditory evoked potentials (BAEPs) or brainstem auditory evoked responses (BAERs) is an auditory evoked potential extracted from ongoing electrical activity in the brain and recorded via electrodes placed on the scalp. The measured recording is a series of six to seven vertex positive waves of which I through V are evaluated. These waves, labeled with Roman numerals in Jewett and Williston convention, occur in the first 10 milliseconds after onset of an auditory stimulus. The ABR is considered an exogenous response because it is dependent upon external factors.

Binaural fusion or binaural integration is a cognitive process that involves the combination of different auditory information presented binaurally, or to each ear. In humans, this process is essential in understanding speech as one ear may pick up more information about the speech stimuli than the other.

Echoic memory is the sensory memory that registers specific to auditory information (sounds). Once an auditory stimulus is heard, it is stored in memory so that it can be processed and understood. Unlike most visual memory, where a person can choose how long to view the stimulus and can reassess it repeatedly, auditory stimuli are usually transient and cannot be reassessed. Since echoic memories are heard once, they are stored for slightly longer periods of time than iconic memories. Auditory stimuli are received by the ear one at a time before they can be processed and understood.

Auditory processing disorder (APD), rarely known as King-Kopetzky syndrome or auditory disability with normal hearing (ADN), is a neurodevelopmental disorder affecting the way the brain processes sounds. Individuals with APD usually have normal structure and function of the ear, but cannot process the information they hear in the same way as others do, which leads to difficulties in recognizing and interpreting sounds, especially the sounds composing speech. It is thought that these difficulties arise from dysfunction in the central nervous system. This is, in part, essentially a failure of the cocktail party effect found in most people.

Dichotic pitch is a pitch heard due to binaural processing, when the brain combines two noises presented simultaneously to the ears. In other words, it cannot be heard when the sound stimulus is presented monaurally but, when it is presented binaurally a sensation of a pitch can be heard. The binaural stimulus is presented to both ears through headphones simultaneously, and is the same in several respects except for a narrow frequency band that is manipulated. The most common variation is the Huggins Pitch, which presents white-noise that only differ in the interaural phase relation over a narrow range of frequencies. For humans, this phenomenon is restricted to fundamental frequencies lower than 330 Hz and extremely low sound pressure levels. Experts investigate the effects of the dichotic pitch on the brain. For instance, there are studies that suggested it evokes activation at the lateral end of Heschl's gyrus.

Dichotic listening is a psychological test commonly used to investigate selective attention and the lateralization of brain function within the auditory system. It is used within the fields of cognitive psychology and neuroscience.

Spatial hearing loss refers to a form of deafness that is an inability to use spatial cues about where a sound originates from in space. Poor sound localization in turn affects the ability to understand speech in the presence of background noise.

The frequency following response (FFR), also referred to as frequency following potential (FFP) or envelope following response (EFR), is an evoked potential generated by periodic or nearly-periodic auditory stimuli. Part of the auditory brainstem response (ABR), the FFR reflects sustained neural activity integrated over a population of neural elements: "the brainstem response...can be divided into transient and sustained portions, namely the onset response and the frequency-following response (FFR)". It is often phase-locked to the individual cycles of the stimulus waveform and/or the envelope of the periodic stimuli. It has not been well studied with respect to its clinical utility, although it can be used as part of a test battery for helping to diagnose auditory neuropathy. This may be in conjunction with, or as a replacement for, otoacoustic emissions.

Selective auditory attention, or selective hearing, is a process of the auditory system where an individual selects or focuses on certain stimuli for auditory information processing while other stimuli are disregarded. This selection is very important as the processing and memory capabilities for humans have a limited capacity. When people use selective hearing, noise from the surrounding environment is heard by the auditory system but only certain parts of the auditory information are chosen to be processed by the brain.

Edwin Rubel is an American academic and Developmental Neurobiologist holding the position of emeritus professor at the University of Washington. He was the Founding Director and first Virginia Merrill Bloedel Chair in Basic Hearing Research from 1989 to 2017.

Temporal envelope (ENV) and temporal fine structure (TFS) are changes in the amplitude and frequency of sound perceived by humans over time. These temporal changes are responsible for several aspects of auditory perception, including loudness, pitch and timbre perception and spatial hearing.

Binaural unmasking is phenomenon of auditory perception discovered by Ira Hirsh. In binaural unmasking, the brain combines information from the two ears in order to improve signal detection and identification in noise. The phenomenon is most commonly observed when there is a difference between the interaural phase of the signal and the interaural phase of the noise. When such a difference is present there is an improvement in masking threshold compared to a reference situation in which the interaural phases are the same, or when the stimulus has been presented monaurally. Those two cases usually give very similar thresholds. The size of the improvement is known as the "binaural masking level difference" (BMLD), or simply as the "masking level difference".

References

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