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 is no treatment, surgical or otherwise, for hearing loss due to the most common causes (age, noise, and genetic defects). For a few specific conditions, surgical intervention can provide a remedy:
- surgical correction of superior canal dehiscence
- myringotomy, surgical insertion of drainage ventilation tubes in the tympanic membrane. Such placement is usually temporary until the underlying pathology (infection or other inflammation) can be resolved.
- radiotherapy or surgical excision of vestibular schwannoma or acoustic neuroma, though, in most cases, it is unlikely that hearing will be preserved
- Stapedectomy and stapedotomy for otosclerosis - replacement or reshaping of the stapes bone of the middle ear can restore hearing in cases of conductive hearing loss
Surgical and implantable hearing aids are an alternative to conventional external hearing aids.
If the ear is dry and not infected, an air conduction aid could be tried; if the ear is draining, a direct bone condition hearing aid is often the best solution. If the conductive part of the hearing loss is more than 30–35 dB, an air conduction device could have problems overcoming this gap. A bone-anchored hearing aid could, in this situation, be a good option.
The active bone conduction hearing implant Bonebridge is also an option. This implant is invisible under the intact skin and therefore minimises the risk of skin irritations.
Cochlear implants improve outcomes in people with hearing loss in either one or both ears. They work by artificial stimulation of the cochlear nerve by providing an electric impulse substitution for the firing of hair cells. They are expensive, and require programming along with extensive training for effectiveness.
Cochlear implants as well as bone conduction implants can help with single sided deafness.
Middle ear implants or bone conduction implants can help with conductive hearing loss.
People with cochlear implants are at a higher risk for bacterial meningitis. Thus, meningitis vaccination is recommended. People who have hearing loss, especially those who develop a hearing problem in childhood or old age, may need support and technical adaptations as part of the rehabilitation process. Recent research shows variations in efficacy but some studies show that if implanted at a very young age, some profoundly impaired children can acquire effective hearing and speech, particularly if supported by appropriate rehabilitation.
Treatment depends on the specific cause if known as well as the extent, type and configuration of the hearing loss. Most hearing loss, that resulting from age and noise, is progressive and irreversible, and there are currently no approved or recommended treatments; management is by hearing aid. A few specific kinds of hearing loss are amenable to surgical treatment. In other cases, treatment is addressed to underlying pathologies, but any hearing loss incurred may be permanent.
There are a number of devices that can improve hearing in those who are deaf or hard of hearing or allow people with these conditions to manage better in their lives.
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.
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.
As of 2012 there has only been one small-scale study comparing CROS systems.
One study of the BAHA system showed a benefit depending on the patient's transcranial attenuation. Another study showed that sound localisation was not improved, but the effect of the head shadow was reduced.
In cases where the causes are environmental, the treatment is to eliminate or reduce these causes first of all, and then to fit patients with a hearing aid, especially if they are elderly. When the loss is due to heredity, total deafness is often the end result. On the one hand, persons who experience gradual deterioration of their hearing are fortunate in that they have learned to speak. Ultimately the affected person may bridge communication problems by becoming skilled in sign language, speech-reading, using a hearing aid, or accepting elective surgery to use a prosthetic devices such as a cochlear implant.
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.
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.
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.
Treatment is supportive and consists of management of manifestations. User of hearing aids and/or cochlear implant, suitable educational programs can be offered. Periodic surveillance is also important.
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.
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.
Hearing aids and cochlear implants may make the child able to hear sounds in their hearing range—but they don't restore normal hearing. Cochlear implants can stimulate the auditory nerve directly to restore some hearing, but the sound quality isn't that of a normal hearing ear, suggesting that deafness cannot be fully overcome by medical devices. Some say that the benefits and safety of cochlear implants continues to grow, especially when children with implants receive a lot of oral educational support. It is a goal for some audiologists to test and fit a deaf child with a cochlear implant by six months of age, so that they don't get behind in learning language. In fact, there are expectations that if children get fit for implants early enough, they can acquire verbal language skills to the same level as their peers with normal hearing.
School-age children with unilateral hearing loss tend to have poorer grades and require educational assistance. This is not the case with everyone, however. They can also be perceived to have behavioral issues.
People afflicted with UHL have great difficulty locating the source of any sound. They may be unable to locate an alarm or a ringing telephone. The swimming game Marco Polo is generally impossible for them.
When wearing stereo headphones, people with unilateral hearing loss can hear only one channel, hence the panning information (volume and time differences between channels) is lost; some instruments may be heard better than others if they are mixed predominantly to one channel, and in extreme cases of sound production, such as complete stereo separation or stereo-switching, only part of the composition can be heard; in games using 3D audio effects, sound may not be perceived appropriately due to coming to the disabled ear. This can be corrected by using settings in the software or hardware—audio player, OS, amplifier or sound source—to adjust balance to one channel (only if the setting downmixes sound from both channels to one), or there may be an option to outright downmix both channels to mono. Such settings may be available via the device or software's accessibility features. As hardware solutions, stereo-to-mono adapters may be available to receive mono sound in stereo headphones from a stereo sound source, or some monaural headsets for cellphones and VOIP communication may combine stereo sound to mono (though headphones for voice communication typically offer lower audio quality than headphones targeted for listening to music). From the standpoint of sound fidelity, sound information in downmixed mono channel will, in any case, differ from that in either of the source channels or what is perceived by a normal-hearing person, thus technically some audio quality is lost (for example, the same or slightly different sound occurrences in two channels, with time delay between them, will be merged to a sound in the mono channel that unavoidably cannot correspond to the intent of the sound producer); however, such loss is most probably unnoticeable, especially compared to other distortions inherent in sound reproduction, and to the person's problems from hearing loss.
Prelingual hearing loss can be either acquired, meaning it occurred after birth due to illness or injury, or it can be congenital, meaning it was present at birth. Congenital hearing loss can be caused by genetic or nongenetic factors. The nongenetic factors account for about one fourth of the congenital hearing losses in infants. These factors could include: Maternal infections, such as rubella, cytomegalovirus, or herpes simplex virus, lack of oxygen, maternal diabetes, toxemia during pregnancy, low birth weight, prematurity, birth injuries, toxins including drugs and alcohol consumed by the mother during pregnancy, and complications associated with the Rh factor in the blood/jaundice. Genetic factors account for over half of the infants with congenital hearing loss. Most of these are caused by an autosomal recessive hearing loss or an autosomal dominant hearing loss. Autosomal recessive hearing loss is when both parents carry the recessive gene, and pass it on to their child. The autosomal dominant hearing loss is when an abnormal gene from one parent is able to cause hearing loss even though the matching gene from the other parent is normal.
In some cases, the loss is extremely sudden and can be traced to specific diseases, such as meningitis, or to ototoxic medications, such as Gentamicin. In both cases, the final degree of loss varies. Some experience only partial loss, while others become profoundly deaf. Hearing aids and cochlear implants may be used to regain a sense of hearing, with different people experiencing differing degrees of success. It is possible that the affected person may need to rely on speech-reading and/or sign language for communication.
In most cases the loss is a long term degradation in hearing loss. Discrediting earlier notions of presbycusis, Rosen demonstrated that long term hearing loss is usually the product of chronic exposure to environmental noise in industrialized countries (Rosen, 1965). The U.S. Environmental Protection Agency has asserted the same sentiment and testified before the U.S. Congress that approximately 34 million Americans are exposed to noise pollution levels (mostly from roadway and aircraft noise) that expose humans to noise health effects including the risk of hearing loss (EPA, 1972).
Certain genetic conditions can also lead to post-lingual deafness. In contrast to genetic causes of pre-lingual deafness, which are frequently autosomal recessive, genetic causes of post-lingual deafness tend to be autosomal dominant.
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."
Beat deafness is a form of congenital amusia characterized by a person's inability to distinguish musical rhythm or move in time to it.
No specific treatment exists for Pendred syndrome. Speech and language support and hearing aids are important. Cochlear implants may be needed if the hearing loss drops to severe to profound levels and can improve language skills. If thyroid hormone levels are decreased, thyroid hormone supplements may be required. Patients are advised to take precautions against head injury.
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.
Generally, humans have the ability to hear musical beat and rhythm beginning in infancy. Some people, however, are unable to identify beat and rhythm of music, suffering from what is known as beat deafness. Beat deafness is a newly discovered form of congenital amusia, in which people lack the ability to identify or “hear” the beat in a piece of music. Unlike most hearing impairments in which an individual is unable to hear any sort of sound stimuli, those with beat deafness are generally able to hear normally, but unable to identify beat and rhythm in music. Those with beat deafness are also unable to dance in step to any type of music. Even people who do not dance well can at least coordinate their movements to the song they are listening to, because they can easily keep time to the beat.
Auditory arrhythmia is the inability to rhythmically perform music, to keep time, and to replicate musical or rhythmic patterns. It has been caused by damage to the cerebrum or rewiring of the brain.
Neuroscientists have learned a lot about the role of the brain in numerous cognitive mechanisms by understanding corresponding disorders. Similarly, neuroscientists have come to learn a lot about music cognition by studying music-specific disorders. Even though music is most often viewed from a "historical perspective rather than a biological one" music has significantly gained the attention of neuroscientists all around the world. For many centuries music has been strongly associated with art and culture. The reason for this increased interest in music is because it "provides a tool to study numerous aspects of neuroscience, from motor skill learning to emotion".
An individual with this condition has an especially difficult time maintaining a steady beat, and even has difficulty following along to a steady rhythm. Before it was a known disorder, it was thought that these individuals were just severely uncoordinated, and therefore were unable to follow along with the music. It has been discovered recently that problems with rhythm in Schizophrenia, Parkinson's, and Attention Deficit Hyperactive Disorder are also found to have a correlation to rhythm deficiencies.