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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.
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.
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.
Earlier workers suggested the use of calcium fluoride; now sodium fluoride is the preferred compound. Fluoride ions inhibit the rapid progression of disease. In the otosclerotic ear, there occurs formation of hydroxylapatite crystals which lead to stapes (or other) fixation. The administration of fluoride replaces the hydroxyl radical with fluoride leading to the formation of fluorapatite crystals. Hence, the progression of disease is considerably slowed down and active disease process is arrested.
This treatment cannot reverse conductive hearing loss, but may slow the progression of both the conductive and sensorineural components of the disease process. Otofluor, containing sodium fluoride, is one treatment. Recently, some success has been claimed with a second such treatment, bisphosphonate medications that inhibit bone destruction. However, these early reports are based on non-randomized case studies that do not meet standards of clinical trials. There are numerous side-effects to both pharmaceutical treatments, including occasional stomach upset, allergic itching, and increased joint pains which can lead to arthritis. In the worst case, bisphosphonates may lead to osteonecrosis of the auditory canal itself. Finally, neither approach has been proven to be beneficial after the commonly preferred method of surgery has been undertaken.
A child with a congenital hearing loss should begin receiving treatment before 6 months of age. Studies suggest that children treated this early are usually able to develop communication skills (using spoken or sign language) that are as good as those of hearing peers.
In the United States of America, because of a Federal law (the Individuals with Disabilities Education Act), children with a hearing loss between birth and 3 years of age have the right to receive interdisciplinary assessment and early intervention services at little or no cost. After age 3, early intervention and special education programs are provided through the public school system.
There are a number of treatment options available, and parents will need to decide which are most appropriate for their child. They will need to consider the child’s age, developmental level and personality, the severity of the hearing loss, as well as their own preferences. Ideally a team of experts including the child’s primary care provider, an otolaryngologist, a speech-language pathologist, audiologist and an educator will work closely with the parents to create an Individualized Family Service Plan. Treatment plans can be changed as the child gets older.
Children as young as 4 weeks of age can benefit from a hearing aid. These devices amplify sound, making it possible for many children to hear spoken words and develop language. However, some children with severe to profound hearing loss may not be able to hear enough sound, even with a hearing aid, to make speech audible. A behind-the-ear hearing aid is often recommended for young children because it is safer and more easily fitted and adjusted as the child grows as compared to one that fits within the ear.
Parents also will need to decide how their family and child are going to communicate. If the child is going to communicate orally (speech), s/he may need assistance learning listening skills and lip reading skills to help her/him understand what others are saying. Many children with hearing loss also need speech or language therapy.
A child also can learn to communicate using a form of sign language. In the United States of America, the type preferred by most deaf adults is American Sign Language (ASL), which has rules and grammar that is distinct from English. There are also several variations of sign language that can be used along with spoken English which are standard in English-speaking countries outside the United States.
There is also a visual model of spoken language called cued speech. Learning to lip read is very difficult because many sounds look the same on the lips. Cued speech enables young children with hearing loss to clearly see what is being said, and learn spoken languages with normal grammar and vocabulary. It clarifies lip reading using 8 hand shapes in 4 positions and usually takes less than 20 hours to learn the entire system.
Surgery may be recommended if a child has a permanent conductive hearing loss caused by malformations of the outer or middle ear, or by repeated ear infections. Although fluid in the middle ear usually results in only temporary hearing loss, chronic ear infection can cause a child to fall behind in language skills. In some cases, a doctor may suggest inserting a tube through the eardrum to allow the middle ear to drain. This procedure generally does not require an overnight hospital stay.
Surgery also may be an option for some children with severe to profound sensorineural hearing loss. A device called a cochlear implant can be surgically inserted in the inner ear of children as young as 12 months of age to stimulate hearing. The surgery requires a hospital stay of one to several days. With additional language and speech therapy, children with cochlear implants may learn to understand speech and speak reasonably well, but the amount of improvement is variable.
Once a child is diagnosed, the immediate and anticipated reaction of the parents and immediate family is one of the denial. Doctors or the audiologists need to counsel the family, help them cope with the situation and encourage them to look forward to solutions to overcome the problem. Often when the family is told about the excellent options available for a hearing impaired child, the chances of acceptance are much better. Once the family accepts the handicap, half the battle is over and rehabilitation can begin.
The type of intervention required depends on several factors. Chief among these is the degree of impairment. When a child has a fair degree of residual hearing, the correct intervention would be fitting "optimised" hearing aids. "Optimisation" means fitting the child with a hearing aid appropriate to its degree of deafness.
Today a variety of good quality hearing aids are available – analog or digital body worn (for small children) or ear level for older children. When fitting a hearing aid, a competent audiologist has to assess the child's residual hearing, look at the hearing aid's performance and fit the child with an appropriate instrument. Equally important is the ear mould, which has to be custom made to suit the shape of the child's ear.
If a child has profound or total deafness, the benefits of hearing aids are limited. Depending upon the level and type of hearing loss, cochlear implants may be used instead of hearing aids.
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.
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 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.
There are various methods to treat otosclerosis. However the method of choice is a procedure known as Stapedectomy.
Early attempts at hearing restoration via the simple freeing the stapes from its sclerotic attachments to the oval window were met with temporary improvement in hearing, but the conductive hearing loss would almost always recur. A stapedectomy consists of removing a portion of the sclerotic stapes footplate and replacing it with an implant that is secured to the incus. This procedure restores continuity of ossicular movement and allows transmission of sound waves from the eardrum to the inner ear.
A modern variant of this surgery called a stapedotomy, is performed by drilling a small hole in the stapes footplate with a micro-drill or a laser, and the insertion of a piston-like prothesis. The success rate of either surgery depends greatly on the skill and the familiarity with the procedure of the surgeon. However, comparisons have shown stapedotomy to yield results at least as good as stapedectomy, with fewer complications, and thus stapedotomy is preferred under normal circumstances.
There is no treatment to correct an enlarged vestibular aqueduct. Any hearing loss will need management with amplification and support in education and at work. If the hearing loss becomes severe to profound cochlear implants can be of significant value. Vestibular disturbance is usually short-lived and associated with head trauma but significant vestibular hypofunction may require rehabilitation.
People with enlarged vestibular aqueducts are advised to avoid head trauma where possible. This usually means avoiding contact sports such as boxing and rugby, but also horse riding, trampolining and other sports where head injury may occur. Some have symptoms when flying and should limit these activities if affected.
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.
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.
"Ginkgo biloba" does not appear to be effective. The American Academy of Otolaryngology recommends against taking melatonin or zinc supplements to relieve symptoms of tinnitus. In addition, a 2016 Cochrane Review concluded that evidence is not sufficient to support taking zinc supplements to reduce symptoms associated with tinnitus.
At present, presbycusis, being primarily sensorineural in nature, cannot be prevented, ameliorated or cured. Treatment options fall into three categories: pharmacological, surgical and management.
- There are no approved or recommended pharmaceutical treatments for presbycusis.
there were no medications effective for tinnitus. There is not enough evidence to determine if antidepressants or acamprosate are useful. While there is tentative evidence for benzodiazepines, it is insufficient to support usage. Anticonvulsants have not been found to be useful. Steroid injections into the middle ear also do not seem to be effective.
Botulinum toxin injection has been tried with some success in some of the rare cases of objective tinnitus from a palatal tremor.
These are surgically implanted hearing aids inserted onto the middle ear. These aids work by directly vibrating the ossicles, and are cosmetically favorable due to their hidden nature.
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.
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.
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.
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.
AIED treatment is a rapidly changing field. Several medical therapies have been proposed in the treatment of AIED, with corticosteroid therapy being the most effective. However, corticosteroid therapy (specifically with prednisone and dexamethasone) has demonstrated limited effectiveness in some patients, suggesting the need for novel treatment methods. The goal of most AIED treatments is to administer corticosteroids over a certain period of time, re-evaluate hearing at each appointment, and eventually taper off corticosteroid administration. Ideally, patients can be tapered off with hearing fully recovered, though this is the least likely outcome. Often, tumor necrosis factor-α (TNF-α) inhibitors must be administered alongside corticosteroids to achieve a favorable outcome and eventual end to corticosteroid treatment. Recent papers have indicated that the TNF-α inhibitor, infliximab, has the potential to allow for sustained patient improvement and alleviation of symptoms.
Cytotoxic agents such as cyclophosphamide and methotrexate have been used in AIED treatment in the past; however, findings have suggested limited symptom alleviation from these drugs.
Treatment differs depending on the cause. Each cause has a different treatment, and may involve either medical treatment, surgery, or therapy. If serious damage has already been done, then the focus of treatment is upon avoidance of vestibular suppressants and ototoxins. It is recommended that you tell your physicians to avoid drugs that end in mycin ( Azithromycin, Erythromycin ) because of possible reactions which could lead to setbacks. Vestibular rehabilitation is important. Your physician will try to keep the administering of drugs to a minimum.
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
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.
Dysequilibrium arising from bilateral loss of vestibular function – such as can occur from ototoxic drugs such as gentamicin – can also be treated with balance retraining exercises (vestibular rehabilitation) although the improvement is not likely to be full recovery.