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Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
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Universal Newborn Hearing Screenings (UNHS) is mandated in a majority of the United States. Auditory neuropathy is sometimes difficult to catch right away, even with these precautions in place. Parental suspicion of a hearing loss is a trustworthy screening tool for hearing loss, too; if it is suspected, that is sufficient reason to seek a hearing evaluation from an audiologist.
In most parts of Australia, hearing screening via AABR testing is mandated, meaning that essentially all congenital (i.e., not those related to later onset degenerative disorders) auditory neuropathy cases should be diagnosed at birth.
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). A focused perceptual training protocol for children with amblyaudia called Auditory Rehabilitation for Interaural Asymmetry (ARIA) was developed in 2001 which has been found to improve dichotic listening performance in the non-dominant ear and enhance general listening skills. ARIA is now available in a number of clinical sites in the U.S., Canada, Australia and New Zealand. It is also undergoing clinical research trials involving electrophysiologic measures and activation patterns acquired through functional magnetic resonance imaging (fMRI) techniques to further establish its efficacy to remediate amblyaudia.
When testing the auditory system, there really is no characteristic presentation on the audiogram.
When diagnosing someone with auditory neuropathy, there is no characteristic level of functioning either. People can present relatively little dysfunction other than problems of hearing speech in noise, or can present as completely deaf and gaining no useful information from auditory signals.
Hearing aids are sometimes prescribed, with mixed success.
Some people with auditory neuropathy obtain cochlear implants, also with mixed success.
1. SCAN is the most common tool for diagnosing APD, and it also standardized. It is composed for four subsets: discrimination of monaurally presented single words against background noise, acoustically degraded single words, dichotically presented single words, sentence stimuli. Different versions of the test are used depending on the age of the patient.
2. Random Gap Detection Test (RGDT) is also a standardized test. It assesses an individual’s gap detection threshold of tones and white noise. The exam includes stimuli at four different frequencies (500, 1000, 2000, and 4000 Hz) and white noise clicks of 50 ms duration. It is a useful test because it provides an index of auditory temporal resolution. In children, an overall gap detection threshold greater than 20 ms means they have failed.
3. Gaps in Noise Test (GIN) also measures temporal resolution by testing the patient's gap detection threshold in white noise.
4. Pitch Patterns Sequence Test (PPT) and Duration Patterns Sequence Test (DPT) measure auditory pattern identification. The PPS has s series of three tones presented at either of two pitches (high or low). Meanwhile, the DPS has a series of three tones that vary in duration rather than pitch (long or short). Patients are then asked to describe the pattern of pitches presented.
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 and the Dichotic Words Test. 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 (). 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. The diagnosis can also be made if performance in both ears is below normal but performance in the non-dominant ear is significantly poorer, thereby resulting in an abnormally large asymmetry between the two ears. Amblyaudia is emerging as a distinct subtype of auditory processing disorder (APD).
It has been discovered that APD and ADHD present overlapping symptoms. Below is a ranked order of behavioral symptoms that are most frequently observed in each disorder. Professionals evaluated the overlap of symptoms between the two disorders. The order below is of symptoms that are almost always observed. This chart proves that although the symptoms listed are different, it is easy to get confused between many of them.
There is a high rate of co-occurrence between AD/HD and CAPD. Research shows that 84% of children with APD have confirmed or suspected ADHD. Co-occurrence between ADHD and APD is 41% for children with confirmed diagnosis of ADHD, and 43% for children suspected of having ADHD.
Auditory perception can improve with time.There seems to be a level of neuroplasticity that allows patients to recover the ability to perceive environmental and certain musical sounds. Patients presenting with cortical hearing loss and no other associated symptoms recover to a variable degree, depending on the size and type of the cerebral lesion. Patients whose symptoms include both motor deficits and aphasias often have larger lesions with an associated poorer prognosis in regard to functional status and recovery.
Cochlear or auditory brainstem implantation could also be treatment options. Electrical stimulation of the peripheral auditory system may result in improved sound perception or cortical remapping in patients with cortical deafness. However, hearing aids are an inappropriate answer for cases like these. Any auditory signal, regardless if has been amplified to normal or high intensities, is useless to a system unable to complete its processing. Ideally, patients should be directed toward resources to aid them in lip-reading, learning American Sign Language, as well as speech and occupational therapy. Patients should follow-up regularly to evaluate for any long-term recovery.
This may include a blood or other sera test for inflammatory markers such as those for autoinflammatory diseases.
Assessment will usually include an interview with the child’s caregiver, observation of the child in an unstructured setting, a hearing test, and standardized tests of language and nonverbal ability. There is a wide range of language assessments in English. Some are restricted for use by speech and language professionals (therapists or SALTs in the UK, speech-language pathologists, SLPs, in the US and Australia).
A commonly used test battery for diagnosis of SLI is the Clinical Evaluation of Language Fundamentals (CELF).
Assessments that can be completed by a parent or teacher can be useful to identify children who may require more in-depth evaluation.
The Grammar and Phonology Screening (GAPS) test is a quick (ten minute) simple and accurate screening test developed and standardized in the UK. It is suitable for children from 3;4 to 6;8 years;months and can be administered by professionals and non-professionals (including parents) alike, and has been demonstrated to be highly accurate (98% accuracy) in identifying impaired children who need specialist help vs non-impaired children. This makes it potentially a feasible test for widespread screening.
The Children’s Communication Checklist (CCC–2) is a parent questionnaire suitable for testing language skills in school-aged children.
Informal assessments, such as language samples, may also be used. This procedure is useful when the normative sample of a given test is inappropriate for a given child, for instance, if the child is bilingual and the sample was of monolingual children. It is also an ecologically valid measure of all aspects of language (e.g. semantics, syntax, pragmatics, etc.).
To complete a language sample, the SLP will spend about 15 minutes talking with the child. The sample may be of a conversation (Hadley, 1998), or narrative retell. In a narrative language sample, the SLP will tell the child a story using a wordless picture book (e.g. "Frog Where Are You?", Mayer, 1969), then ask the child to use the pictures and tell the story back.
Language samples are typically transcribed using computer software such as the Systematic Analysis of Language Software (SALT, Miller et al. 2012), and then analyzed. For example, the SLP might look for whether the child introduces characters to their story or jumps right in, whether the events follow a logical order, and whether the narrative includes a main idea or theme and supporting details.
As part of differential diagnosis, an MRI scan may be done to check for vascular anomalies, tumors, and structural problems like enlarged mastoids. MRI and other types of scan cannot directly detect or measure age-related hearing loss.
Research has shown that PC based spatial hearing training software can help some of the children identified as failing to develop their spatial hearing skills (perhaps because of frequent bouts of otitis media with effusion). Further research is needed to discover if a similar approach would help those over 60 to recover the loss of their spatial hearing. One such study showed that dichotic test scores for the left ear improved with daily training. Related research into the plasticity of white-matter (see Lövdén et al. for example) suggests some recovery may be possible.
Music training leads to superior understanding of speech in noise across age groups and musical experience protects against age-related degradation in neural timing. Unlike speech (fast temporal information), music (pitch information) is primarily processed by areas of the brain in the right hemisphere. Given that it seems likely that the right ear advantage (REA) for speech is present from birth, it would follow that a left ear advantage for music is also present from birth and that MOC efferent inhibition (of the right ear) plays a similar role in creating this advantage. Does greater exposure to music increase conscious control of cochlear gain and inhibition? Further research is needed to explore the apparent ability of music to promote an enhanced capability of speech in noise recognition.
Bilateral digital hearing aids do not preserve localization cues (see, for example, Van den Bogaert et al., 2006) This means that audiologists when fitting hearing aids to patients (with a mild to moderate age related loss) risk negatively impacting their spatial hearing capability. With those patients who feel that their lack of understanding of speech in background noise is their primary hearing difficulty then hearing aids may simply make their problem even worse - their spatial hearing gain will be reduced by in the region of 10 dB. Although further research is needed, there is a growing number of studies which have shown that open-fit hearing aids are better able to preserve localisation cues (see, for example, Alworth 2011)
Assessment will usually include an interview with the child’s caregiver, observation of the child in an unstructured setting, a hearing test, and standardized tests of language. There is a wide range of language assessments in English. Some are restricted for use by experts in speech-language pathology: speech and language therapists (SaLTs/SLTs) in the UK, speech-language pathologists (SLPs) in the US and Australia. A commonly used test battery for diagnosis of DLD is the Clinical Evaluation of Language Fundamentals (CELF).
Assessments that can be completed by a parent or teacher can be useful to identify children who may require more in-depth evaluation. The Children’s Communication Checklist (CCC–2) is a parent questionnaire suitable for assessing everyday use of language in children aged 4 years and above who can speak in sentences.
Informal assessments, such as language samples, are often used by speech-language therapists/pathologists to complement formal testing and give an indication of the child's language in a more naturalistic context. A language sample may be of a conversation or narrative retell. In a narrative language sample, an adult may tell the child a story using a wordless picture book (e.g. Frog Where Are You?, Mayer, 1969), then ask the child to use the pictures and tell the story back. Language samples can be transcribed using computer software such as the Systematic Analysis of Language Software, and then analyzed for a range of features: e.g., the grammatical complexity of the child's utterances, whether the child introduces characters to their story or jumps right in, whether the events follow a logical order, and whether the narrative includes a main idea or theme and supporting details.
It seems that somatic anxiety and situations of stress may be determinants of speech-hearing disability.
Some studies indicated an increased prevalence of a family history of hearing impairment in these patients. The pattern of results is suggestive that King-Kopetzky patients may be related to conditions of autosomal dominant inheritance.
Auditory neuropathy spectrum disorder (ANSD) is a specific form of hearing loss defined by the presence of normal or near-normal otoacoustic emissions (OAEs) but the absence of normal middle ear reflexes and severely abnormal or completely absent auditory brainstem response (ABRs).
Individuals presenting with this recently recognised hearing loss appear to display sporadic windows of hearing and not. Very few (1 in 14) will go on to develop normal speech and language but with poor speech perception in background noise and in others, no speech perception and therefore language development is possible.
The condition was originally termed auditory neuropathy (AN) and in 2001 as Auditory Neuropathy / Auditory Dys-synchrony (AN/AD) (to include those cases where no true neuropathy was apparent). In 2008 at a meeting convened at Lake Como in Italy (Guidelines Development
Conference on the Identification and Management
of Infants with Auditory Neuropathy, International
Newborn Hearing Screening Conference, Como, Italy,
June 19–21, 2008), a group of leading authorities on the condition reached a consensus and renamed it as auditory neuropathy spectrum disorder.
Individuals with conduction aphasia are able to express themselves fairly well, with some word finding and functional comprehension difficulty. Although people with aphasia may be able to express themselves fairly well, they tend to have issues repeating phrases, especially phrases that are long and complex. When asked to repeat something, the patient will be unable to do so without significant difficulty, repeatedly attempting to self-correct ("conduite d'approche"). When asked a question, however, patients can answer spontaneously and fluently.
Several standardized test batteries exist for diagnosing and classifying aphasias. These tests are capable of identifying conduction aphasia with relative accuracy. The Boston Diagnostic Aphasia Examination (BDAE) and the Western Aphasia Battery (WAB) are two commonly used test batteries for diagnosing conduction aphasia. These examinations involve a set of tests, which include asking patients to name pictures, read printed words, count aloud, and repeat words and non-words (such as "shwazel").
Sensory processing disorder since 1994 is accepted in the Diagnostic Classification of Mental Health and Developmental Disorders of Infancy and Early Childhood (DC:0-3R) and is not recognized as a mental disorder in medical manuals such as the ICD-10 or the DSM-5.
Diagnosis is primarily arrived at by the use of standardized tests, standardized questionnaires, expert observational scales, and free play observation at an occupational therapy gym. Observation of functional activities might be carried at school and home as well. Some scales that are not exclusively used in SPD evaluations are used to measure visual perception, function, neurology and motor skills.
Depending on the country, diagnosis is made by different professionals, such as occupational therapists, psychologists, learning specialists, physiotherapists and/or speech and language therapists. In some countries it is recommended to have a full psychological and neurological evaluation if symptoms are too severe.
The basic diagnostic test is similar to a normal audiogram. The difference is that additionally to the hearing threshold at each test frequency also the lowest uncomfortable sound level is measured. This level is called "loudness discomfort level" (LDL) or "uncomfortable loudness level" (ULL). In patients with hyperacusis this level is considerably lower than in normal subjects, and usually across most parts of the auditory spectrum.
It is estimated that up to 16.5% of elementary school aged children present elevated SOR behaviors in the tactile or auditory modalities. However, this figure might represent an underestimation of Sensory Over Responsivity prevalence, since this study did not include children with developmental disorders or those delivered preterm, who are more likely to present it.
This figure is, nonetheless, larger than what previous studies with smaller samples had shown: an estimate of 5–13% of elementary school aged children.
Incidence for the remaining subtypes is currently unknown.
It was found that based on sensitized measures of auditory dysfunction and on psychological assessment, Subjects could be subdivided into seven subcategories:
1. middle ear dysfunction
2. mild cochlear pathology
3. central/medial olivocochlear efferent system (MOCS) auditory dysfunction
4. purely psychological problems
5. multiple auditory pathologies
6. combined auditory dysfunction and psychological problems
7. unknown
Different subgroups may represent different pathogenic and aetiological factors. Thus, subcategorization provides further understanding of the basis of King–Kopetzky syndrome, and hence may guide the rehabilitative management of these patients.This was suggested by Professor Dafydd Stephens and F Zhao at the Welsh Hearing Institute, Cardiff University.
Epidemiological surveys, in the US and Canada, estimated the prevalence of SLI in 5-year-olds at around 7 percent. However, neither study adopted the stringent 'discrepancy' criteria of the Diagnostic and Statistical Manual of Mental Disorders or ICD-10; SLI was diagnosed if the child scored below cut-off on standardized language tests, but had a nonverbal IQ of 90 or above and no other exclusionary criteria.
Cortical deafness is a rare form of sensorineural hearing loss caused by damage to the primary auditory cortex. Cortical deafness is an auditory disorder where the patient is unable to hear sounds but has no apparent damage to the anatomy of the ear (see auditory system), which can be thought of as the combination of auditory verbal agnosia and auditory agnosia. Patients with cortical deafness cannot hear any sounds, that is, they are not aware of sounds including non-speech, voices, and speech sounds. Although patients appear and feel completely deaf, they can still exhibit some reflex responses such as turning their head towards a loud sound.
Cortical deafness is caused by bilateral cortical lesions in the primary auditory cortex located in the temporal lobes of the brain. The ascending auditory pathways are damaged, causing a loss of perception of sound. Inner ear functions, however, remains intact. Cortical deafness is most often cause by stroke, but can also result from brain injury or birth defects. More specifically, a common cause is bilateral embolic stroke to the area of Heschl's gyri. Cortical deafness is extremely rare, with only twelve reported cases. Each case has a distinct context and different rates of recovery.
It is thought that cortical deafness could be a part of a spectrum of an overall cortical hearing disorder. In some cases, patients with cortical deafness have had recovery of some hearing function, resulting in partial auditory deficits such as auditory verbal agnosia. This syndrome might be difficult to distinguish from a bilateral temporal lesion such as described above.
Sign language therapy has been identified as one of the top five most common treatments for auditory verbal agnosia. This type of therapy is most useful because, unlike other treatment methods, it does not rely on fixing the damaged areas of the brain. This is particularly important with AVA cases because it has been so hard to identify the causes of the agnosia in the first place, much less treat those areas directly. Sign language therapy, then, allows the person to cope and work around the disability, much in the same way it helps deaf people. In the beginning of therapy, most will work on identifying key objects and establishing an initial core vocabulary of signs. After this, the patient graduates to expand the vocabulary to intangible items or items that are not in view or present. Later, the patient learns single signs and then sentences consisting of two or more signs. In different cases, the sentences are first written down and then the patient is asked to sign them and speak them simultaneously. Because different AVA patients vary in the level of speech or comprehension they have, sign language therapy learning order and techniques are very specific to the individual's needs.
Spatial hearing loss can be diagnosed using the Listening in Spatialized Noise – Sentences test (LiSN-S), which was designed to assess the ability of children with central auditory processing disorder (CAPD) to understand speech in background noise. The LiSN-S allows audiologists to measure how well a person uses spatial (and pitch information) to understand speech in noise. Inability to use spatial information has been found to be a leading cause of CAPD in children.
Test participants repeat a series of target sentences which are presented simultaneously with competing speech. The listener's speech reception threshold (SRT) for target sentences is calculated using an adaptive procedure. The targets are perceived as coming from in front of the listener whereas the distracters vary according to where they are perceived spatially (either directly in front or either side of the listener). The vocal identity of the distracters also varies (either the same as, or different from, the speaker of the target sentences).
Performance on the LISN-S is evaluated by comparing listeners' performances across four listening conditions, generating two SRT measures and three "advantage" measures. The advantage measures represent the benefit in dB gained when either talker, spatial, or both talker and spatial cues are available to the listener. The use of advantage measures minimizes the influence of higher order skills on test performance. This serves to control for the inevitable differences that exist between individuals in functions such as language or memory.
Dichotic listening tests can be used to measure the efficacy of the attentional control of cochlear inhibition and the inter-hemispheric transfer of auditory information. Dichotic listening performance typically increases (and the right-ear advantage decreases) with the development of the Corpus Callosum (CC), peaking before the fourth decade. During middle age and older the auditory system ages, the CC reduces in size, and dichotic listening becomes worse, primarily in the left ear. Dichotic listening tests typically involve two different auditory stimuli (usually speech) presented simultaneously, one to each ear, using a set of headphones. Participants are asked to attend to one or (in a divided-attention test) both of the messages.
The activity of the medial olivocochlear bundle (MOC) and its inhibition of cochlear gain can be measured using a Distortion Product Otoacoustic Emission (DPOE) recording method. This involves the contralateral presentation of broadband noise and the measurement of both DPOAE amplitudes and the latency of onset of DPOAE suppression. DPOAE suppression is significantly affected by age and becomes difficult to detect by approximately 50 years of age.
Treatment for aphasias is generally individualized, focusing on specific language and communication improvements, and regular exercise with communication tasks. Regular therapy for conduction aphasics has been shown to result in steady improvement on the Western Aphasia Battery. However, conduction aphasia is a mild aphasia, and conduction aphasics score highly on the WAB at baseline.
DLD is defined purely in behavioural terms: there is no biological test. There are three points that need to be met for a diagnosis of DLD:
1. The child has language difficulties that create obstacles to communication or learning in everyday life,
2. The child's language problems are unlikely to resolve by five years of age, and
3. The problems are not associated with a known biomedical condition such as brain injury, neurodegenerative conditions, genetic conditions or chromosome disorders such as Down Syndrome, sensorineural hearing loss, or Autism Spectrum Disorder or Intellectual Disability.
For research and epidemiological purposes, specific cutoffs on language assessments have been used to document the first criterion. Tomblin et al. proposed the EpiSLI criterion, based on five composite scores representing performance in three domains of language (vocabulary, grammar, and narration) and two modalities (comprehension and production). Children scoring in the lowest 10% on two or more composite scores are identified as having language disorder.
The second criterion, persistence of language problems, can be difficult to judge in a young child, but longitudinal studies have shown that difficulties are less likely to resolve for children who have poor language comprehension, rather than difficulties confined to expressive language. In addition, children with isolated difficulties in just one of the areas noted under 'subtypes' tend to make better progress than those whose language is impaired in several areas.
The third criterion specifies that DLD is used for children whose language disorder is not part of another biomedical condition, such as a genetic syndrome, a sensorineural hearing loss, neurological disease, Autism Spectrum Disorder or Intellectual Disability – these were termed 'differentiating conditions' by the CATALISE panel. Language disorders occurring with these conditions need to be assessed and children offered appropriate intervention, but a terminological distinction is made so that these cases would be diagnosed as Language Disorder associated with ___, with the main diagnosis being specified: e.g. "Language Disorder associated with Autism Spectrum Disorder." The reasoning behind these diagnostic distinctions is discussed further by Bishop (2017).