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
Once diagnosed, the gap in the temporal bone can be repaired by surgical resurfacing of the affected bone or plugging of the superior semicircular canal. These techniques are performed by accessing the site of the dehiscence either via a middle fossa craniotomy or via a canal drilled through the transmastoid bone behind the affected ear. Bone cement has been the material most often used, in spite of its tendency to slippage and resorption, and a consequent high failure rate; recently, soft tissue grafts have been substituted.
Patients are advised to treat with bed rest and avoiding activities that increase intracranial pressure (i.e. weightlifting, valsalva, scuba diving, flying in airplanes) with the hopes of the membrane healing on their own. Appropriate Physical therapy / vestibular rehabilitation techniques can be helpful in managing symptoms of movement sensitivity.
When diagnosing, PLF should be differentiated from Ménière's disease. Tympanostomy has been reported to be a way to diagnose and cure PLF.
Superior canal dehiscence syndrome (SCDS) is a set of hearing and balance symptoms, related to a rare medical condition of the inner ear, known as "superior canal dehiscence". The symptoms are caused by a thinning or complete absence of the part of the temporal bone overlying the superior semicircular canal of the vestibular system. There is evidence that this rare defect, or susceptibility, is congenital. There are also numerous cases of symptoms arising after physical trauma to the head. It was first described in 1998 by Lloyd B. Minor of Johns Hopkins University in Baltimore.
Historically, to temporarily alleviate symptoms, patients have tried positional maneuvers, such as tilting their head to one side or upside down, lie down on their backs, or sit in a chair with their head between their knees. Similarly, a routine of lying down four times per day with legs elevated to around 20 inches for at least two weeks has been attempted as well. Depending on the underlying cause of the disorder, the individual may need to remove caffeine from their diet, reduce exercise, or gain weight. It may be the case that the symptoms are induced by anxiety; anxiolytic drugs or supplements (e.g., GABA) combined with the removal of caffeine from the diet could offer a simple strategy to determine if anxiety is the root cause.
Estrogen (Premarin) nasal drops or saturated potassium iodide have been used to induce edema of the eustachian tube opening. Nasal medications containing diluted hydrochloric acid, chlorobutanol, and benzyl alcohol have been reported to be effective in some patients, with few side effects. Food and Drug Administration approval is still pending, however.
In extreme cases surgical intervention may attempt to restore the Eustachian tube tissues with fat, gel foam, or cartilage or scar it closed with cautery. These methods are not always successful.
The widespread use of wetsuits has allowed people to surf in much colder waters, which has increased the incidence and severity of surfer's ear for people who do not properly protect their ears.
- Avoid activity during extremely cold or windy conditions.
- Keep the ear canal as warm and dry as possible.
- Ear plugs
- Wetsuit hood
- Swim cap
- Diving helmet
Cholesteatoma is a persistent disease. Once the diagnosis of cholesteatoma is made in a patient who can tolerate a general anesthetic, the standard treatment is to surgically remove the growth.
The challenge of cholesteatoma surgery is to permanently remove the cholesteatoma whilst retaining or reconstructing the normal functions of the structures housed within the temporal bone.
The general objective of cholesteatoma surgery has two parts. It is both directed against the underlying pathology and directed towards maintaining the normal functions of the temporal bone. These aims are conflicting and this makes cholesteatoma surgery extremely challenging.
Sometimes, the situation results in a clash of surgical aims. The need to fully remove a progressive disease like cholesteatoma is the surgeon's first priority. Preservation of hearing is secondary to this primary aim. If the disease can be removed easily so that there is no increased risk of residual disease, then the ossicles may be preserved. If the disease is difficult to remove, so that there is an increased risk of residual disease, then removal of involved ossicles in order to fully clear cholesteatoma has generally been regarded as necessary and reasonable.
In other words, the aims of cholesteatoma treatment form a hierarchy. The paramount objective is the complete removal of cholesteatoma. The remaining objectives, such as hearing preservation, are subordinate to the need for complete removal of cholesteatoma. This hierarchy of aims has led to the development of a wide range of strategies for the treatment of cholesteatoma.
Management falls into three modalities: surgical treatment, pharmaceutical treatment, and supportive, depending on the nature and location of the specific cause.
In cases of infection, antibiotics or antifungal medications are an option. Some conditions are amenable to surgical intervention such as middle ear fluid, cholesteatoma, otosclerosis. If conductive hearing loss is due to head trauma, surgical repair is an option. If absence or deformation of ear structures cannot be corrected, or if the patient declines surgery, hearing aids which amplify sounds are a possible treatment option. Bone conduction hearing aids are useful as these deliver sound directly, through bone, to the cochlea or organ of hearing bypassing the pathology. These can be on a soft or hard headband or can be inserted surgically, a bone anchored hearing aid, of which there are several types. Conventional air conduction hearing aids can also be used.
The treatment will vary with the different grades, but the most common is a surgical repair. The surgical option is cosmetic reconstruction of the external ear's normal shape and repair of the ear canal. In less severe cases the reconstruction will be sufficient to restore hearing. In grades of anotia/microtia that affect the middle ear the surgery with the use of a Bone Anchored Hearing Aid (BAHA) will likely restore the hearing. The BAHA may be surgically implanted onto the skull which would allow for some hearing repair by conduction through the skull bone. "This allows sound vibrations to travel through bones in the head to the inner ear."
BAHA: An implantable hearing device. It is the only hearing aid device that works via direct bone conduction.
The variation in technique in cholesteatoma surgery results from each surgeon's judgment whether to retain or remove certain structures housed within the temporal bone in order to facilitate the removal of cholesteatoma. This typically involves some form of mastoidectomy which may or may not involve removing the posterior ear canal wall and the ossicles.
Removal of the canal wall facilitates the complete clearance of cholesteatoma from the temporal bone in three ways:
1. it removes a large surface onto which cholesteatoma may be adherent;
2. it removes a barrier behind which the cholesteatoma may be hidden;
3. it removes an impediment to the introduction of instruments used for the removal of cholesteatoma.
Thus removal of the canal wall provides one of the most effective strategies for achieving the primary aim of cholesteatoma surgery, the complete removal of cholesteatoma. However, there is a trade-off, since the functional impact of canal wall removal is also important.
The removal of the ear canal wall results in:
- a space, the "mastoid cavity", which is less likely than the original ear canal to resist infection;
- exposure of the ossicles, which may allow the subsequent formation of a new cholesteatoma deep to the ossicles. To prevent this, these ossicles must be removed, which may diminish the patient's hearing.
The formation of a mastoid cavity by removal of the canal wall is the simplest and most effective procedure for facilitating the removal of cholesteatoma, but may bestow the most lasting infirmity due to loss of ear function upon the patient treated in this way.
The following strategies are employed to mitigate the effects of canal wall removal:
1. careful design and construction of the mastoid cavity. This is essential for the health and integrity of the protective sheet of migrating, keratising epithelium which lines the distorted ear canal. This requires the surgeon to saucerise the cavity. A high facial ridge and an inappropriately small cartilaginous meatus are obstructions to epithelial migration and are particularly high risk factors for failure of the self-cleaning mechanism of the external ear.
2. partial obliteration of the mastoid cavity. This can be performed using a wide range of materials. Many of these resorb in time, which means that the long-term results of such surgery are poorer than the short-term results.
3. reconstruction of the ear canal wall. Canal wall reconstruction has been performed using ear canal skin alone, fascia, cartilage, titanium as well as by replacing the original intact wall. If the reconstruction is poorly performed, it may result in a high rate of recurrent cholesteatoma.
4. preservation of the ear canal wall. If poorly performed, it may result in a high rate of both residual and recurrent cholesteatoma.
5. reconstruction of the chain of hearing bones.
Clearly, preservation and restoration of ear function at the same time as total removal of cholesteatoma requires a high level of surgical expertise.
Medical treatment with anti-vertigo medications may be considered in acute, severe exacerbation of BPPV, but in most cases are not indicated. These primarily include drugs of the anti-histamine and anti-cholinergic class, such as meclizine and hyoscine butylbromide (scopolamine) respectively. The medical management of vestibular syndromes has become increasingly popular over the last decade, and numerous novel drug therapies (including existing drugs with new indications) have emerged for the treatment of vertigo/dizziness syndromes. These drugs vary considerably in their mechanisms of action, with many of them being receptor- or ion channel-specific. Among them are betahistine or dexamethasone/gentamicin for the treatment of Ménière's disease, carbamazepine/oxcarbazepine for the treatment of paroxysmal dysarthria and ataxia in multiple sclerosis, metoprolol/topiramate or valproic acid/tricyclic antidepressant for the treatment of vestibular migraine, and 4-aminopyridine for the treatment of episodic ataxia type 2 and both downbeat and upbeat nystagmus. These drug therapies offer symptomatic treatment, and do not affect the disease process or resolution rate. Medications may be used to suppress symptoms during the positioning maneuvers if the patient's symptoms are severe and intolerable. More dose-specific studies are required, however, in order to determine the most effective drug(s) for both acute symptom relief and long-term remission of the condition.
Surgical treatments, such as a semi-circular canal occlusion, do exist for BPPV, but carry the same risk as any neurosurgical procedure. Surgery is reserved as a last resort option for severe and persistent cases which fail vestibular rehabilitation (including particle repositioning and habituation therapy).
The majority of patients present in their mid-30s to late 40s. This is likely due to a combination of the slow growth of the bone and the decreased participation in activities associated with surfer's ear past the 30's. However surfer's ear is possible at any age and is directly proportional to the amount of time spent in cold, wet, windy weather without adequate protection.
The normal ear canal is approximately 7mm in diameter and has a volume of approximately 0.8 ml (approximately one-sixth of a teaspoon). As the condition progresses the diameter narrows and can even close completely if untreated, although sufferers generally seek help once the passage has constricted to 0.5-2mm due to the noticeable hearing impairment. While not necessarily harmful in and of itself, constriction of the ear canal from these growths can trap debris, leading to painful and difficult to treat infections.
Typically, testing is first done to determine the quality of hearing. This can be done as early as in the first two weeks with a BAER test (Brain Stem Auditory Response Test). At age 5–6, CT or CAT scans of the middle ear can be done to elucidate its development and clarify which patients are appropriate candidates for surgery to improve hearing. For younger individuals, this is done under sedation.
The hearing loss associated with congenital aural atresia is a conductive hearing loss—hearing loss caused by inefficient conduction of sound to the inner ear. Essentially, children with aural atresia have hearing loss because the sound cannot travel into the (usually) healthy inner ear—there is no ear canal, no eardrum, and the small ear bones (malleus/hammer, incus/anvil, and stapes/stirrup) are underdeveloped. "Usually" is in parentheses because rarely, a child with atresia also has a malformation of the inner ear leading to a sensorineural hearing loss (as many as 19% in one study). Sensorineural hearing loss is caused by a problem in the inner ear, the cochlea. Sensorineural hearing loss is not correctable by surgery, but properly fitted and adjusted hearing amplification (hearing aids) generally provide excellent rehabilitation for this hearing loss. If the hearing loss is severe to profound in both ears, the child may be a candidate for a cochlear implant (beyond the scope of this discussion).
Unilateral sensorineural hearing loss was not generally considered a serious disability by the medical establishment before the nineties; it was thought that the afflicted person was able to adjust to it from birth. In general, there are exceptional advantages to gain from an intervention to enable hearing in the microtic ear, especially in bilateral microtia. Children with untreated unilateral sensorineural hearing loss are more likely to have to repeat a grade in school and/or need supplemental services (e.g., FM system – see below) than their peers.
Children with unilateral sensorineural hearing loss often require years of speech therapy in order to learn how to enunciate and understand spoken language. What is truly unclear, and the subject of an ongoing research study, is the effect of unilateral conductive hearing loss (in children with unilateral aural atresia) on scholastic performance. If atresia surgery or some form of amplification is not used, special steps should be taken to ensure that the child is accessing and understanding all of the verbal information presented in school settings. Recommendations for improving a child's hearing in the academic setting include preferential seating in class, an FM system (the teacher wears a microphone, and the sound is transmitted to a speaker at the child's desk or to an ear bud or hearing aid the child wears), a bone-anchored hearing aid (BAHA), or conventional hearing aids. Age for BAHA implantation depends on whether the child is in Europe (18 months) or the US (age 5). Until then it is possible to fit a BAHA on a softband
It is important to note that not all children with aural atresia are candidates for atresia repair. Candidacy for atresia surgery is based on the hearing test (audiogram) and CT scan imaging. If a canal is built where one does not exist, minor complications can arise from the body's natural tendency to heal an open wound closed. Repairing aural atresia is a very detailed and complicated surgical procedure which requires an expert in atresia repair. While complications from this surgery can arise, the risk of complications is greatly reduced when using a highly experienced otologist. Atresia patients who opt for surgery will temporarily have the canal packed with gelatin sponge and silicone sheeting to prevent closure. The timing of ear canal reconstruction (canalplasty) depends on the type of external ear (Microtia) repair desired by the patient and family. Two surgical teams in the USA are currently able to reconstruct the canal at the same time as the external ear in a single surgical stage (one stage ear reconstruction).
In cases where a later surgical reconstruction of the external ear of the child might be possible, positioning of the BAHA implant is critical. It may be necessary to position the implant further back than usual to enable successful reconstructive surgery – but not so far as to compromise hearing performance. If the reconstruction is ultimately successful, it is easy to remove the percutaneous BAHA abutment. If the surgery is unsuccessful, the abutment can be replaced and the implant re-activated to restore hearing.
While there is no cure, most people with tinnitus get used to it over time; for a minority, it remains a significant problem.
If there is an underlying cause, treating it may lead to improvements. Otherwise, the primary treatment for tinnitus is talk therapy and sound therapy; there are no effective medications.
The age when outer ear surgery can be attempted depends upon the technique chosen. The earliest is 7 for Rib Cartilage Grafts. However, some surgeons recommend waiting until a later age, such as 8–10 when the ear is closer to adult size. External ear prostheses have been made for children as young as 5.
For auricular reconstruction, there are several different options:
1. "Rib Cartilage Graft Reconstruction:" This surgery may be performed by specialists in the technique. It involves sculpting the patient's own rib cartilage into the form of an ear. Because the cartilage is the patient's own living tissue, the reconstructed ear continues to grow as the child does. In order to be sure that the rib cage is large enough to provide the necessary donor tissue, some surgeons wait until the patient is 8 years of age; however, some surgeons with more experience with this technique may begin the surgery on a child aged six. The major advantage of this surgery is that the patient's own tissue is used for the reconstruction. This surgery varies from two to four stages depending on the surgeon's preferred method. A novel one stage ear reconstruction technique is performed by a few select surgeons. One team is able to reconstruct the entire external ear and ear canal in one operation.
2. "Reconstruct the ear using a polyethylene plastic implant (also called Medpor):" This is a 1–2 stage surgery that can start at age 3 and can be done as an outpatient without hospitalization. Using the porous framework, which allows the patient's tissue to grow into the material and the patient's own tissue flap, a new ear is constructed in a single surgery. A small second surgery is performed in 3–6 months if needed for minor adjustments. This surgery should only be performed by experts in the techniques involved. The use of porous polyethylene implants for ear reconstruction was initiated in the 1980s by Alexander Berghaus.
3. "Ear Prosthesis:" An auricular (ear) prosthesis is custom made by an anaplastologist to mirror the other ear. Prosthetic ears can appear very realistic. They require a few minutes of daily care. They are typically made of silicone, which is colored to match the surrounding skin and can be attached using either adhesive or with titanium screws inserted into the skull to which the prosthetic is attached with a magnetic or bar/clip type system. These screws are the same as the BAHA (bone anchored hearing aid) screws and can be placed simultaneously. The biggest advantage over any surgery is having a prosthetic ear that allows the affected ear to appear as normal as possible to the natural ear. The biggest disadvantage is the daily care involved and knowing that the prosthesis is not real.
Initial line of treatment is with anti-inflammatory drugs or cortisone injections. There have been trials with gloves which help protect the ulnar nerve from compression. The most radical treatment option is surgery to relieve tension in the volar carpal ligament which forms the roof of Guyon's canal, thereby reducing compression on the ulnar nerve.
The place of chiropractic-, physical-, occupational-, massage- and osteopathic therapy was not confirmed in scientific studies. These treatments can be both expensive as well as dangerous (causing permanent damage when performed wrongly).
It is advised to consult a physician beforehand starting any therapy, albeit an alternative approach, to avoid any permanent nerve damage.
Anotia ("no ear") describes a rare congenital deformity that involves the complete absence of the pinna, the outer projected portion of the ear, and narrowing or absence of the ear canal. This contrasts with microtia, in which a small part of the pinna is present. Anotia and microtia may occur unilaterally (only one ear affected) or bilaterally (both ears affected). This deformity results in conductive hearing loss, deafness.
Surgical treatments may be used to treat the condition, and include retro-rectal levatorplasty, post-anal repair, retro-anal levator plate myorrhaphy.
Shingles is prevented by immunizing against the causal virus, varicella zoster, for example through Zostavax, a stronger version of chickenpox vaccine.
Patulous Eustachian tube is a physical disorder. The exact causes may vary depending on the person. Weight loss is a commonly cited cause of the disorder due to the nature of the Eustachian tube itself. Fatty tissues hold the tube closed most of the time in healthy individuals. When circumstances cause overall body fat to diminish, the tissue surrounding the Eustachian tube shrinks and this function is disrupted.
Activities and substances which dehydrate the body have the same effect and are also possible causes of patulous Eustachian tube. Examples are stimulants (including caffeine) and exercise. Exercise may have a more short-term effect than caffeine or weight loss in this regard.
Pregnancy can also be a cause of patulous Eustachian tube due to the effects of pregnancy hormones on surface tension and mucus in the respiratory system.
Granulomatosis with polyangiitis can also be a cause of this disorder. It is yet unknown why.
The disorder can be associated with a number of psychological symptoms, anxiety, depression, social phobia, body image disorders, and patients may be subjected to discrimination, bullying and name calling especially when young. A multi-disciplinary team and parental support should include these issues.
Sedative drugs are often prescribed for vertigo and dizziness, but these usually treat the symptoms rather than the underlying cause. Lorazepam (Ativan) is often used and is a sedative which has no effect on the disease process, but rather helps patients cope with the sensation.
Anti-nauseants, like those prescribed for motion sickness, are also often prescribed but do not affect the prognosis of the disorder.
Specifically for Meniere's disease a medication called Serc (Beta-histine) is available. There is some evidence to support its effectiveness in reducing the frequency of attacks. Also Diuretics, like Diazide (HCTZ/triamterene), are effective in many patients. Finally, ototoxic medications delivered either systemically or through the eardrum can eliminate the vertigo associated with Meniere's in many cases, although there is about a 10% risk of further hearing loss when using ototoxic medications.
Treatment is specific for underlying disorder of balance disorder:
- anticholinergics
- antihistamines
- benzodiazepines
- calcium channel antagonists, specifically Verapamil and Nimodipine
- GABA modulators, specifically gabapentin and baclofen
- Neurotransmitter reuptake inhibitors such as SSRIs, SNRIs and Tricyclics
Hearing loss with craniofacial syndromes is a common occurrence. Many of these multianomaly disorders involve structural malformations of the outer or middle ear, making a significant hearing loss highly likely.