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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)
Funded by The Federal Ministry for Economic Affairs and Energy; Grant: 01MD19013D, Smart-MD Project, Digital Technologies
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.
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.
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.
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.
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.
Currently, no forms of treatment have proven effective in treating amusia. One study has shown tone differentiation techniques to have some success, however future research on treatment of this disorder will be necessary to verify this technique as an appropriate treatment.
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.
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.
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.
Those who lose their hearing later in life, such as in late adolescence or adulthood, face their own challenges. For example, they must adjust to living with the adaptations that make it possible for them to live independently. They may have to adapt to using hearing aids or a cochlear implant, develop speech-reading skills, and/or learn sign language. The affected person may need to use a TTY, a videophone, an interpreter, or relay service to communicate over the telephone. Loneliness and depression can arise as a result of isolation (from the inability to communicate with friends and loved ones) and difficulty in accepting their disability. The challenge is made greater by the need for those around them to adapt to the person's hearing loss.
Treatment of Foix–Chavany–Marie syndrome depends on the onset of symptoms and involves a multidisciplinary approach. Drugs are used in neurological recovery depending on the etiological classification of FCMS. FCMS caused by epilepsy, specifically resulting in the development of lesions in the bilateral and subcortical regions of the brain can be treated using antiepileptic drugs to reverse abnormal EEG changes and induce complete neurological recovery. In addition, a hemispherectomy can be performed to reverse neurological deficits and control the seizures. This procedure can result in a complete recovery from epileptic seizures. Physical therapy is also used to manage symptoms and improve quality of life. Classical FCMS resulting in the decline of ones ability to speak and swallow can be treated using neuromuscular electrical stimulation and traditional dysphagia therapy. Speech therapy further targeting dysphagia can strengthen oral musculature using modified feeding techniques and postures. Therapeutic feedings include practicing oral and lingual movements using ice chips. In addition, different procedures can be performed by a neurosurgeon to alleviate some symptoms.
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.
Degrees of vision loss vary dramatically, although the ICD-9 released in 1979 categorized them into three tiers: normal vision, low vision, and blindness. Two significant causes of vision loss due to sensory failures include media opacity and optic nerve diseases, although hypoxia and retinal disease can also lead to blindness. Most causes of vision loss can cause varying degrees of damage, from total blindness to a negligible effect. Media opacity occurs in the presence of opacities in the eye tissues or fluid, distorting and/or blocking the image prior to contact with the photoreceptor cells. Vision loss often results despite correctly functioning retinal receptors. Optic nerve diseases such as optic neuritis or retrobulbar neuritis lead to dysfunction in the afferent nerve pathway once the signal has been correctly transmitted from retinal photoreceptors.
Partial or total vision loss may affect every single area of a person's life. Though loss of eyesight may occur naturally as we age, trauma to the eye or exposure to hazardous conditions may also cause this serious condition. Workers in virtually any field may be at risk of sustaining eye injuries through trauma or exposure. A traumatic eye injury occurs when the eye itself sustains some form of trauma, whether a penetrating injury such as a laceration or a non-penetrating injury such as an impact. Because the eye is a delicate and complex organ, even a slight injury may have a temporary or permanent effect on eyesight.
There are three treatment options available to a patient. These options are observation, microsurgical removal and radiation (radiosurgery or radiotherapy). Determining which treatment to choose involves consideration of many factors including the size of the tumor, its location, the patient's age, physical health and current symptoms. About 25% of all acoustic neuromas are treated with medical management consisting of a periodic monitoring of the patient's neurological status, serial imaging studies, and the use of hearing aids when appropriate.
One of the last great obstacles in the management of acoustic neuromas is hearing preservation and/or rehabilitation after hearing loss. Hearing loss is both a symptom and concommitant risk, regardless of the treatment option chosen.
Treatment does not restore hearing already lost, though there are a few rare cases of hearing recovery reported.
A diagnosis of NF2 related bilateral acoustic neuromas creates the possibility of complete deafness if the tumors are left to grow unchecked. Preventing or treating the complete deafness that may befall individuals with NF2 requires complex decision making. The trend at most academic U.S. medical centers is to recommend treatment for the smallest tumor which has the best chance of preserving hearing. If this goal is successful, then treatment can also be offered for the remaining tumor. If hearing is not preserved at the initial treatment, then usually the second tumor, in the only-hearing ear, is just observed. If it shows continued growth and becomes life-threatening, or if the hearing is lost over time as the tumor grows, then treatment is undertaken. This strategy has the highest chance of preserving hearing for the longest time possible.
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.
There is no known cure to BVVL however a Dutch group have reported the first promising attempt at treatment of the disorder with high doses of riboflavin. This Riboflavin protocol seems to be beneficial in almost all cases. Specialist medical advice is of course essential to ensure the protocol is understood and followed correctly.
Patients will almost certainly require additional symptomatic treatment and supportive care. This must be specifically customized to the needs of the individual but could include mobility aids, hearing aids or cochlear implants, vision aids, gastrostomy feeding and assisted ventilation, while steroids may or may not help patients.
The first report of BVVL syndrome in Japanese literature was of a woman that had BVVL and showed improvement after such treatments. The patient was a sixty-year-old woman who had symptoms such as sensorineural deafness, weakness, and atrophy since she was 15 years old. Around the age of 49 the patient was officially diagnosed with BVVL, incubated, and then attached to a respirator to improve her CO2 narcosis. After the treatments, the patient still required respiratory assistance during sleep; however, the patient no longer needed assistance by a respirator during the daytime.
Many types of sense loss occur due to a dysfunctional sensation process, whether it be ineffective receptors, nerve damage, or cerebral impairment. Unlike agnosia, these impairments are due to damages prior to the perception process.
There is no known direct treatment. Current treatment efforts focus on managing the complications of Wolfram syndrome, such as diabetes mellitus and diabetes insipidus.
Another treatment option for an acoustic neuroma is radiation. Stereotactic radiation can be delivered as single fraction stereotactic radiosurgery (SRS) or as multi-session fractionated stereotactic radiotherapy (FSR). Both techniques are performed in the outpatient setting, not requiring general anesthesia or a hospital stay. The purpose of these techniques is to arrest the growth of the tumor. This treatment has not been well studied and thus it is unclear if it is better than observation or surgery.
All types of radiation therapy for acoustic neuromas may result in "tumor control" in which the tumor cells die and necrosis occurs. Tumor control means that the tumor growth may slow or stop and, in some cases, the tumor may shrink in size. Acoustic neuroma tumors have been completely eliminated by radiation treatments in almost no cases. In other words, radiation cannot remove the tumor like microsurgery would. Tumors under 2.5 - 3.0 cm, without significant involvement of the brainstem, are more favorable for radiation treatment. Side effects can occur when the brainstem is irradiated and in some cases of large tumors, radiation is suggested against.
In single dose treatments, hundreds of small beams of radiation are aimed at the tumor. This results in a concentrated dose of radiation to the tumor and avoids exposure of surrounding brain tissues to the radiation. Many patients have been successfully treated this way. Facial weakness or numbness, in the hands of experienced radiation physicians, occurs in only a small percent of cases. Hearing can be preserved in some cases.
The multi-dose treatment, FSR, delivers smaller doses of radiation over a period of time, requiring the patient to return to the treatment location on a daily basis, from 3 to 30 times, generally over several weeks. Each visit lasts a few minutes and most patients are free to go about their daily business before and after each treatment session. Early data indicates that FSR may result in better hearing preservation when compared to single-session SRS.
Radiated patients require lifetime follow-up with MRI scans. Follow-up after SRS and FSR typically involves an MRI scan and audiogram at six months, one year, then yearly for several years, then every second or third year indefinitely to make sure the tumor does not start to grow again. Patients should understand there have been rare reports of malignant degeneration (a benign tumor becoming malignant) after radiotherapy. In some cases the tumor does not die and continues to grow. In those instances, another treatment is necessary - either microsurgery or sometimes another dose of radiation.
Studies are beginning to appear for the other modalities. All of the techniques use computers to create three dimensional models of the tumor and surrounding neural structures. Radiation physicists then create dosimetry maps showing the level of radiation to be received by the tumor and the normal tissues. Surgeons, radiation therapists and physicists then modify the dosimetry to maximize tumor doses and minimize radiation toxicity to surrounding normal tissues. Treatments generally last 30–60 minutes. Just like for surgery, the experience of the team in treating acoustic neuromas with all modalities (surgery and radiation) can affect outcomes.
There are a multitude of studies supporting short-term (<5 yrs.) and longer-term (over 10 yrs.) tumor control with radiation. Unfortunately, as is the case with microsurgical studies, most have inconsistent follow-up to draw definitive conclusions.
Nonsyndromic deafness is hearing loss that is not associated with other signs and symptoms. In contrast, syndromic deafness involves hearing loss that occurs with abnormalities in other parts of the body. Genetic changes are related to the following types of nonsyndromic deafness.
- DFNA: nonsyndromic deafness, autosomal dominant
- DFNB: nonsyndromic deafness, autosomal recessive
- DFNX: nonsyndromic deafness, X-linked
- nonsyndromic deafness, mitochondrial
Each type is numbered in the order in which it was described. For example, DFNA1 was the first described autosomal dominant type of nonsyndromic deafness. Mitochondrial nonsyndromic deafness involves changes to the small amount of DNA found in mitochondria, the energy-producing centers within cells.
Most forms of nonsyndromic deafness are associated with permanent hearing loss caused by damage to structures in the inner ear. The inner ear consists of three parts: a snail-shaped structure called the cochlea that helps process sound, nerves that send information from the cochlea to the brain, and structures involved with balance. Loss of hearing caused by changes in the inner ear is called sensorineural deafness. Hearing loss that results from changes in the middle ear is called conductive hearing loss. The middle ear contains three tiny bones that help transfer sound from the eardrum to the inner ear. Some forms of nonsyndromic deafness involve changes in both the inner ear and the middle ear; this combination is called mixed hearing loss.
The severity of hearing loss varies and can change over time. It can affect one ear (unilateral) or both ears (bilateral). Degrees of hearing loss range from mild (difficulty understanding soft speech) to profound (inability to hear even very loud noises). The loss may be stable, or it may progress as a person gets older. Particular types of nonsyndromic deafness often show distinctive patterns of hearing loss. For example, the loss may be more pronounced at high, middle, or low tones.
Nonsyndromic deafness can occur at any age. Hearing loss that is present before a child learns to speak is classified as prelingual or congenital. Hearing loss that occurs after the development of speech is classified as postlingual.
Unfortunately, there is not one specific treatment option that can rid a person of this syndrome. However, there are many routes one can take to make living with this disease a lot easier. For example, there are many treatment programs that doctors can specialize for patients and their needs. Meeting with a doctor is very crucial and these specializations can be very useful. Also, one can seek help from pediatricians, EENT doctors, audiologists, and orthopedists. Brace fittings, hearing aids, and physical therapy can also be pushed by one's doctor, so that a patient can live normally. Additionally, anticonvulsant drugs can be used to stop seizures.
Over the past decade, much has been discovered about amusia. However, there remains a great deal more to learn. While a method of treatment for people with amusia has not been defined, tone differentiation techniques have been used on amusic patients with some success. It was found with this research that children reacted positively to these tone differentiation techniques, while adults found the training annoying. However, further research in this direction would aid in determining if this would be a viable treatment option for people with amusia. Additional research can also serve to indicate which processing component in the brain is essential for normal music development. Also, it would be extremely beneficial to investigate musical learning in relation to amusia since this could provide valuable insights into other forms of learning disabilities such as dysphasia and dyslexia.