Made by DATEXIS (Data Science and Text-based Information Systems) at Beuth University of Applied Sciences Berlin
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)
Funded by The Federal Ministry for Economic Affairs and Energy; Grant: 01MD19013D, Smart-MD Project, Digital Technologies
Treating auditory verbal agnosia with intravenous immunoglobulin (IVIG) is controversial because of its inconsistency as a treatment method. Although IVIG is normally used to treat immune diseases, some individuals with auditory verbal agnosia have responded positively to the use of IVIG. Additionally, patients are more likely to relapse when treated with IVIG than other pharmacological treatments. IVIG is, thus, a controversial treatment as its efficacy in treating auditory verbal agnosia is dependent upon each individual and varies from case to case.
There are clinical trials being done to further research for treatments. At the National Institute of Neurological Disorders and Stroke (NINDS) they support research for rare diseases like agnosia. Some organizations that are recruiting for trials are using clincaltrials.gov and give status updates on the trials.
Given the unknown nature of MES, treatments have been largely dependent on an individual basis. Treatments can vary from being as little as self-reassurance to pharmaceutical medications.
Medications can be helpful, such as antipsychotics, benzodiazepines or antiepileptics, but there is very limited evidence for this. Some case studies have found that switching to a prednisolone steroid after a betamethasone steroid which caused MES helped alleviate hallucinations or the use of the acetylcholinesterase inhibitor, Donepezil, have also found that it successfully treated an individual's MES. However, because of the heterogeneous etiology, these methods cannot be applied as general treatment.
Other than treatment by medicinal means, individuals have also successfully alleviated musical hallucinations by cochlear implants, listening to different songs via an external source, or by attempting to block them through mental effort, depending on how severe their condition is.
In incidents where tumors and their pressure effects are the cause of pure word deafness, removal of the tumor has been shown to allow for the return of most auditory verbal comprehension.
Psychopharmacological treatments include anti-psychotic medications. Psychology research shows that first step in treatment is for the patient to realize that the voices they hear are creation of their own mind. This realization is argued to allow patients to reclaim a measure of control over their lives. Some additional psychological interventions might allow for the process of controlling these phenomena of auditory hallucinations but more research is needed.
These strategies elicit the use of an unaffected modality. For example, visual agnosics can use tactile information in replacement of visual information. Alternatively, an individual with prosopagnosia can use auditory information in order to replace visual information. For example, an individual with prosopagnosia can wait for someone to speak, and will usually recognize the individual from their speech.
The primary means of treating auditory hallucinations is antipsychotic medications which affect dopamine metabolism. If the primary diagnosis is a mood disorder (with psychotic features), adjunctive medications are often used (e.g., antidepressants or mood stabilizers). These medical approaches may allow the person to function normally but are not a cure as they do not eradicate the underlying thought disorder.
Speech and language therapy is typically the primary treatment for individuals with aphasia. The goal of speech and language therapy is to increase the person’s communication abilities to a level functional for daily life. Goals are chosen based on collaboration between speech language pathologists, patients, and their family/caregivers. Goals should be individualized based on the person’s aphasia symptoms and communicative needs. In 2016, Wallace et al. found the following outcomes were commonly prioritized in therapy: communication, life participation, physical and emotional well-being, normalcy, and health and support services. However, available research is inconclusive about which specific approach to speech and language therapy is most effective in treating global aphasia.
Therapy can be either group or individual. Group therapies that integrate the use of visual aids allow for enhanced social and communication-skill development. Group therapy sessions typically revolve around simple, preplanned activities or games, and aim to facilitate social communication.
One particular therapy designed specifically for treatment of aphasia is Visual Action Therapy (VAT). VAT is a non-verbal gestural output program with 3 phases and 30 total steps. The program teaches unilateral gestures as symbolic representations of real life objects. Research on the effectiveness of VAT is limited and inconclusive.
One important therapy technique includes teaching family members and caregivers strategies for more effectively communicating with their loved ones. Research offers such strategies including, simplifying sentences and using common words, gaining the person's attention before speaking, using pointing and visual cues, allowing for adequate response time, and creating a quiet environment free of distractions.
Another approach to speech and language treatment is constraint-induced language therapy (CILT). CILT involves teaching the patient to use speech in small segments but avoid using gestures and familiar words . The speech language pathologist provides positive feedback throughout and ignores any mistakes made by the patient. The intensity with which this treatment is provided has been debated in the literature. One study, performed in 2015, compared the outcomes of patients with aphasia who received CILT for either 30 hours total over 2 weeks or 30 hours distributed over 10 weeks. Results showed that both groups made significant speech and language improvements. Overall, CILT is an effective treatment at a variety of intensities.
Research supporting the efficacy of pharmacological treatments for aphasia is limited. To date, no large scale clinical trials have proven benefits of pharmacological treatment.
To date, there is no successful method of treatment that "cures" musical hallucinations. There have been successful therapies in single cases that have ameliorated the hallucinations. Some of these successes include drugs such as neuroleptics, antidepressants, and certain anticonvulsive drugs. A musical hallucination was alleviated, for example, by antidepressant medications given to patients with depression. Sanchez reported that some authors have suggested that the use of hearing aids may improve musical hallucination symptoms. They believed that the external environment influences the auditory hallucinations, showing worsening of symptoms in quieter environments than in noisier ones. Oliver Sacks' patient, Mrs. O'C, reported being in an "ocean of sound" despite being in a quiet room due to a small thrombosis or infarction in her right temporal lobe. After treatment, Mrs. O'C was relinquished of her musical experience but said that, "I do miss the old songs. Now, with lots of them, I can't even recall them. It was like being given back a forgotten bit of my childhood again." Sacks also reported another elderly woman, Mrs. O'M, who had a mild case of deafness and reported hearing musical pieces. When she was treated with anticonvulsive medications, her musical hallucinations ceased but when asked if she missed them, she said "Not on your life."
Treatment of APD typically focuses on three primary areas: changing learning environment, developing higher-order skills to compensate for the disorder, and remediation of the auditory deficit itself. However, there is a lack of well-conducted evaluations of intervention using randomized controlled trial methodology. Most evidence for effectiveness adopts weaker standards of evidence, such as showing that performance improves after training. This does not control for possible influences of practice, maturation, or placebo effects. Recent research has shown that practice with basic auditory processing tasks (i.e. auditory training) may improve performance on auditory processing measures and phonemic awareness measures. Changes after auditory training have also been recorded at the physiological level. Many of these tasks are incorporated into computer-based auditory training programs such as Earobics and Fast ForWord, an adaptive software available at home and in clinics worldwide, but overall, evidence for effectiveness of these computerised interventions in improving language and literacy is not impressive. One small-scale uncontrolled study reported successful outcomes for children with APD using auditory training software.
Treating additional issues related to APD can result in success. For example, treatment for phonological disorders (difficulty in speech) can result in success in terms of both the phonological disorder as well as APD. In one study, speech therapy improved auditory evoked potentials (a measure of brain activity in the auditory portions of the brain).
While there is evidence that language training is effective for improving APD, there is no current research supporting the following APD treatments:
- Auditory Integration Training typically involves a child attending two 30-minute sessions per day for ten days.
- Lindamood-Bell Learning Processes (particularly, the Visualizing and Verbalizing program)
- Physical activities that require frequent crossing of the midline (e.g., occupational therapy)
- Sound Field Amplification
- Neuro-Sensory Educational Therapy
- Neurofeedback
However, use of a FM transmitter has been shown to produce significant improvements over time with children.
One possible treatment for hyperacusis is retraining therapy which uses broadband noise. Tinnitus retraining therapy, a treatment originally used to treat tinnitus, uses broadband noise to treat hyperacusis. Pink noise can also be used to treat hyperacusis. By listening to broadband noise at soft levels for a disciplined period of time each day, patients can rebuild (i.e., re-establish) their tolerances to sound.
Another possible treatment is cognitive behavioral therapy (CBT), which may also be combined with retraining therapy.
Treatment options that offer “cures” for NIHL are under research and development. Currently there are no commonly used cures, but rather assistive devices and therapies to try and manage the symptoms of NIHL.
Several clinical trials have been conducted to treat temporary NIHL occurring after a traumatic noise event, such as a gunshot or firework. In 2007, individuals with acute acoustic trauma after firecracker exposure were injected intratympanically with a cell permeable ligand, AM-111. The trial found AM-111 to have a therapeutic effect on at least 2 cases of those with acute trauma. Treatment with a combination of prednisolone and piracetam appeared to rescue patients with acute trauma after exposure to gunshots. However, those who received the treatment within an hour of exposure had higher rates of recovery and significantly lower threshold shifts compared to those who received treatment after 1 hour.
Additionally, clinical trials using antioxidants after a traumatic noise event to reduce reactive oxygen species have displayed promising results. Antibiotic injections with allopurinol, lazaroids, α-D-tocopherol, and mannitol were found to reduce the threshold shift after noise exposure. Another antioxidant, Ebselen, has been shown to have promising results for both TTS and PTS. Ebselen mimics gluthathione peroxide, an enzyme that has many functions, including scavenging hydrogen peroxide and reactive oxygen species. After noise exposure, gluthathione peroxide decreases in the ear. An oral administration of ebselen in both preclinical tests on guinea pigs and human trials indicate that noise induced TTS and PTS was reduced.
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.
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.
Treatment modalities fall into three categories: pharmacological, surgical, and management. As SNHL is a physiologic degradation and considered permanent, there are as of this time, no approved or recommended treatments.
There have been significant advances in identification of human deafness genes and elucidation of their cellular mechanisms as well as their physiological function in mice. Nevertheless, pharmacological treatment options are very limited and clinically unproven. Such pharmaceutical treatments as are employed are palliative rather than curative, and addressed to the underlying cause if one can be identified, in order to avert progressive damage.
Profound or total hearing loss may be amenable to management by cochlear implants, which stimulate cochlear nerve endings directly. A cochlear implant is surgical implantation of a battery powered electronic medical device in the inner ear. Unlike hearing aids, which make sounds louder, cochlear implants do the work of damaged parts of the inner ear (cochlea) to provide sound signals to the brain. These consist of both internal implanted electrodes and magnets and external components. The quality of sound is different than natural hearing but may enable the recipient to better recognize speech and environmental sounds.
Because of risk and expense, such surgery is reserved for cases of severe and disabling hearing impairment
Management of sensorineural hearing loss involves employing strategies to support existing hearing such as lip-reading, enhanced communication etc. and amplification using hearing aids. Hearing aids are specifically tuned to the individual hearing loss to give maximum benefit.
About half of people with SSNHL will recover some or all of their hearing spontaneously, usually within one to two weeks from onset. Eighty-five percent of those who receive treatment from an otolaryngologist (sometimes called an ENT) will recover some of their hearing.
- vitamins and antioxidants
- vasodilators
- betahistine (Betaserc), an anti-vertigo drug
- hyperbaric oxygen
- anti-inflammatory agents, primarily oral corticosteroids such as prednisone, methylprednisone
- Intratympanic administration - Gel formulations are under investigation to provide more consistent drug delivery to the inner ear. Local drug delivery can be accomplished through intratympanic administration, a minimally invasive procedure where the ear drum is anesthetized and a drug is administered into the middle ear. From the middle ear, a drug can diffuse across the round window membrane into the inner ear. Intratympanic administration of steroids may be effective for sudden sensorineural hearing loss for some patients, but high quality clinical data has not been generated. Intratympanic administration of an anti-apoptotic peptide (JNK inhibitor) is currently being evaluated in late-stage clinical development.
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.
The main form of sensory integration therapy is a type of occupational therapy that places a child in a room specifically designed to stimulate and challenge all of the senses.
During the session, the therapist works closely with the child to provide a level of sensory stimulation that the child can cope with, and encourage movement within the room. Sensory integration therapy is driven by four main principles:
- Just right challenge (the child must be able to successfully meet the challenges that are presented through playful activities)
- Adaptive response (the child adapts his behavior with new and useful strategies in response to the challenges presented)
- Active engagement (the child will want to participate because the activities are fun)
- Child directed (the child's preferences are used to initiate therapeutic experiences within the session)
This therapy retains all of the above-mentioned four principles and adds:
- Intensity (person attends therapy daily for a prolonged period of time)
- Developmental approach (therapist adapts to the developmental age of the person, against actual age)
- Test-retest systematic evaluation (all clients are evaluated before and after)
- Process driven vs. activity driven (therapist focuses on the "Just right" emotional connection and the process that reinforces the relationship)
- Parent education (parent education sessions are scheduled into the therapy process)
- "joie de vivre" (happiness of life is therapy's main goal, attained through social participation, self-regulation, and self-esteem)
- Combination of best practice interventions (is often accompanied by integrated listening system therapy, floor time, and electronic media such as Xbox Kinect, Nintendo Wii, Makoto II machine training and others)
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.
Furosemide injections prior to noise exposure have been shown to decrease the endocochlear potential. This decrease results in a reduction of active cochlear displacements and it is believed that the protection by furosemide stems from the limitation of excessive vibrations while the cochlear amplifier is depressed.
When evaluating the prognosis of a patient, the main contributing participant factors that influence the extent of neuroplasticity, or the brain's ability to change are: age, lesion location, pre-existing cognitive status, motivation, age, overall health, and interaction amongst these. After brain damage, initial signs of global aphasia may appear within the first two days due to brain swelling (cerebral edema). With some time and natural recovery, impairment presentation may progress into expressive aphasia (most commonly) or receptive aphasia. Due to the size and location of the lesion associated with global aphasia, the prognosis for language abilities is poor. Research has shown that the prognosis of long-term language abilities is determined by the initial severity level of aphasia within the first four weeks after a stroke. As a result, there is a poor prognosis for persons who retain a diagnosis of aphasia after one month due to limited initial language abilities. Nonetheless, in the first year post-stroke, patients with global aphasia showed improvement in their Western Aphasia Battery (WAB) scores from baseline. When compared to individuals with Broca’s, Wernicke’s, anomic, and conduction types of aphasia, those with Broca’s aphasia showed the best rate and extent of improvement followed by global aphasia. The rate of improvement in language function was highest in the first four weeks after stroke.
Although the prognosis for persons diagnosed with global aphasia is poor, improvement in varying aspects of language is possible. For example, in 1992, Ferro performed research in which he studied the recovery of individuals with acute global aphasia, resulting from the five different lesion sites. The first lesion site was in the fronto-tempo-parietal region of the brain; patients with lesions in this location saw the least amount of gains out of all of the participants in the study, and they often never recovered from global aphasia. However, the second lesion site was the anterior, suprasylvian, frontal part of the brain; the third lesion site was the subcortical infarcts; and the fourth lesion site was the posterior, suprasylvian, parietal infarcts. Participants with lesions two, three, and four often recovered to a less severe form of aphasia, such as Broca's or transcortical. The fifth lesion site was a double lesion in both the frontal and temporal infarcts; patients with lesions at this site showed slight improvement. However, studies show that spontaneous improvement, if it happens, occurs within six months, but complete recovery is rare.
Studies have shown that persons with global aphasia have improved their verbal and nonverbal speech and language skills through speech and language therapy. One study examined the recovery of a group of individuals who were classified as having global aphasia at 3 months poststroke. The individuals received intensive speech and language intervention. The results of the study illustrated that all of the patients showed improvement. The greatest area of improvement was in auditory comprehension, and the least in the use of propositional speech. After 6 months poststroke, the individuals showed an increased use of gestures to communicate, as their communication skills remained severely impaired.
During therapy, most progress is seen within the first 3 years, but it is possible for language abilities to continuously improve at a steady rate due to long-term intensive language intervention. While improvement in language abilities is possible with intervention, only 20 percent of persons diagnosed with global aphasia achieve functional use of language. Communication of basic needs and the comprehension of simple conversations on highly familiar topics, are examples of common functional language use for this population.
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)
Vitamins A, C and E have been shown to be 'free radical scavengers' by studies looking for protective tendencies of antioxidants. In addition, NAC, or N-acetyl-L-cysteine (acetylcysteine), has been shown to reduce ROS formation associated with the excessive vibrations induced by the noise exposure.