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Sudden hearing loss is defined as a loss of hearing that begins with no warning and for no apparent reason, developing over 24 hours or less. Despite the fact that sudden hearing loss is a relatively common disease that has been the subject of considerable clinical research, sudden hearing loss continues to be vexing for doctor and patient because the cause often cannot be determined, the treatment isna€™t always effective, and the prognosis is usually uncertain. Many patients with sudden hearing loss are initially given another diagnosis, such as ear infection or wax impaction, and so the true nature of their problem may not be recognized until later, delaying appropriate treatment.
Sudden hearing loss can be an indication of an underlying medical condition, such as Meniere's disease (a disruption of the fluid balance in the inner ear), autoimmune inner ear disease (when the bodya€™s immune system mistakenly attacks the tissues of your inner ear), or a tumor of the hearing nerve (acoustic neuroma). It is generally accepted that steroids (cortisone), taken orally over 1 to 2 weeks, are the single most beneficial treatment for sudden hearing loss, although they do not lead to improvement in every case. Other treatments are used selectively, depending on the findings and suspected cause of the hearing loss.
Most doctors agree that treatments are most effective when started as early as possible after the onset of the loss. Sudden hearing loss is associated with vertigo in between 20 to 60% of patients (Rambold et al, 2005). There is presently no convincing evidence that any oral or intravenous treatment for idiopathic SHL is better than placebo. According to Zhang et al (2015), in China, in a study of about 250 people, SHL is commonly seen in middle aged people. In our medical practice in Chicago, of the 54 SHL patients in our database, the median age was 60.
Some authors maintain that vascular disease is the most likely cause (Rambold et al, 2005). Also supporting the idea that vascular causes are important, is the observation that SHL following surgery outside the inner ear, is most commonly associated with cardiopulmonary bypass surgery (Page and Peters, 2015). It is better to have a minor hearing loss, no vertigo, and rapid treatment (within 1 week).
If you have experienced a SHL, you might be able to follow your hearing loss using a cell-phone hearing app. Occasionally, unfortunate persons experience hearing loss on the opposite ear, a year or more later. Bottom line: At this writing (2016) almost everything looks either ineffective or slightly effective. We presently suggest that most people start on oral steroids quickly (preferably same day), check and follow their hearing with a cell-phone hearing app, and if the hearing loss is severe, consider the more aggressive steroid regimens outlined below.
Ng et al (2014) performed a metaanalysis of intratympanic steroids and reviewed 187 randomized trials, but found only 5 that met their inclusion criteria. A slightly better recent double-blinded study of only 60 patients split into 3 groups (Battaglia et al, 2008) indicated that patients treated with a combination of intratympanic dexamethasone and high dose steroids are more likely to recover hearing than those treated with high dose steroids alone.
Dallan et al (2011) reported that intratympanic steroids had identical results to a mixture of oral steroids, pentoxifylinne, low molecular weight heparin and vitamin E. Kim et al (2015) compared use of both IT and oral steroids to patients with either one alone -- this was an unblinded retrospective study -- thus the level of evidence is low. Steroid injections through the ear drum are more costly (in the USA) than the alternatives of no-treatment or oral steroid treatment.
Steroid injections through the ear drum are prone to produce long lasting perforations of the ear drum. SHL is a very disturbing experience and there have been many unusual protocols and drugs advocated.
There are several protocols involving increasing blood flow or oxygenation: We think that the oxygen part of these treatments is a placebo.
These studies are implausible -- , is is difficult to see why this treatment should work and we would not suggest anyone go forward with this until it confirmed with a placebo-controlled and adequately powered study. Haberkamp and Tanyeri (1999) noted that while numerous treatments have been studied aiming to improve blood flow, such as carbogen inhalation or stellate ganglion block, all remain controversial or simply lack convincing evidence of efficacy. Recently, Kim et al (2011) compared steroids +carbogen+lipoprostaglandin E1 to several other variants including steroids alone, but not including placebo, They found a small advantage for the combination group. At the University of Freiburg, exploration of the middle ear and patching is recommended for patients with SHL. Similar results are reported in another German Otolaryngology Dept, in Cologne (Kampfner et al, 2013).
Lipo-prostaglandin E1 in combination with steroid therapy is effective for treatment of sudden sensorineural hearing loss in Korean patients with Type 2 diabetes. Coelho DH, Thacker LR, Hsu DW.Variability in the Management of Idiopathic Sudden Sensorineural Hearing Loss.
Ng JH1, Ho RC, Cheong CS, Ng A, Yuen HW, Ngo RY.Intratympanic steroids as a salvage treatment for sudden sensorineural hearing loss? Kampfner D, Anagiotos A, Luers JC, Hüttenbrink KB, Preuss SF Analysis of 101 patients with severe to profound sudden unilateral hearing loss treated with explorative tympanotomy and sealing of the round window membrane. Khan AS, Sheikh Z, Khan S, Dwivedi R, Benjamin E.Viagra deafness-Sensorineural hearing loss and phosphodiesterase-5 inhibitors. Linthicum FH Jr, Doherty J, Berliner KI.Idiopathic Sudden Sensorineural Hearing Loss: Vascular or Viral? Niu X and many others.The relationship between hearing loss and vestibular dysfunction in patients with sudden sensorineural hearing loss. Samuelsson AK, Hyden D, Roberg M, Skogh T.Evaluation of Anti-hsp70 Antibody Screening in Sudden Deafness. Wang Y1, Zhang L1, Zhang J1, Zhang X1, Zhang W1, Chen X1, Tang S2.The clinical analysis of bilateral successive sudden sensorineural hearing loss. Zhang X, Xu X, Ma W, Zhang Q, Tong B, Yu H, Xu M, Ren T, Rosenhall U, Anniko M, Duan M.Acta Otolaryngol.
According to US government statistics from the 60's (National Center for Health Statistics), only 8% of the entire US population has any difficulty with hearing at all. Examples of conditions that cause sensorineural hearing loss are noise and just living a long time. If you have a sensorineural hearing loss, your goal should be to find out why, if you can prevent it from getting worse, and if a hearing aid could help you in overcoming the impairments associated with hearing loss. If you have to have a hearing loss, it is best to have a conductive one, as these disorders are often fixable. If you have a conductive hearing loss, your goal should be to find out why, if you can prevent it from getting worse, and if a hearing aid, surgery, or perhaps just wax removal might help you in overcoming the impairments associated with hearing loss. MRI scan of person with central hearing loss, showing high-signal (white spots) in inferior colliculi. Figure 3a: Audiogram (hearing test) typical of early Meniere's disease on the right side (x=left, o=right).
Figure 3b: Peaked Audiogram typical of middle-stage Meniere's disease, again on the right side.
Figure 3c: Flat audiogram typical of late-stage Meniere's disease, again on the right side.


Dawes P, Fortnum H, Moore DR, Emsley R, Norman P, Cruickshanks K, Davis A, Edmondson-Jones M, McCormack A, Lutman M, Munro K A Population Snapshot of 40- to 69-Year Olds in the United Kingdom. Leskowitz MJ1, Caruana FF2, Siedlecki B2, Qian ZJ1, Spitzer JB2, Lalwani AK2.Asymmetric hearing loss is common and benign in patients aged 95 years and older. Figure: The outer ear consists of the auricle (unlabelled), the external auditory canal, and the lateral surface of the tympanic membrane (TM). Surprisingly perhaps, hypertension, obesity and diabetes is not associated with an increased risk of hearing loss(Shargorodsky et al 2010). Dance clubs are often very loud and may be the main source of loud noise exposure outside of occupational exposure.
Musician's earplugs are available in ER-9, ER-15, and ER-25, according to ones need for noise reduction.
Although diabetes per se is not associated with an overall greater risk of hearing loss (Shargorodsky et al 2010), hearing loss starts at an early age than the normal population.
Diabetics are also more prone to get external and middle ear infections, as well as more prone to develop cranial nerve palsies and stroke. As diabetes is commonly an autoimmune disorder, autoimmune inner ear disease may also contribute.
The hearing loss arises in the hearing organ of the inner ear (cochlea) or in the hearing nerve, not in the middle or outer ear.
Even with prompt treatment, however, patients may not improve, and hearing loss may be permanent.
Sometimes an additional (a€?boostera€?) dose of steroids is injected directly into the ear. Although not all patients regain hearing, options exist even for patients left with permanent hearing loss.
It is very rare that hearing loss occurs in both ears within 72 hours, so generally the timing definition of SHL alone leaves this situation out.
The wide spread probably depends on what you define as vertigo -- a little bit of dizziness, or a profound sensation of rotation. Thus people who already have ear damage -- are more likely to have more hearing loss documented going forward. Migraine is very common (15% of the population), far more so than SHL, and even occasional SHL due to migraine could be very significant. This is surprising as pregnancy is a risk factor for another cranial nerve syndrome -- Bell's palsy. MRI testing is not always ordered -- in fact, only about half the time (Coelho et al, 2011).
Two meta-analysis studies of steroid treatment (Conlin and Parnes, 2007; Labus et al, 2010) suggested there was no benefit. Hearing outcome is not altered differentially by IV steroids administered in the first day vs.
This doesn't happen very often, and there is an obvious fallacy in that if hearing tends to improve anyway, improvements cannot necessarily be attributed to steroids. This involved 10, 90-minute sessions of breathing pure oxygen at 2.2 atmospheric pressure in a chamber.
Very few placebo controlled studies have performed of treatment of SHL and for this reason, there is presently a limited ability to determine what is the optimal treatment of SHL. Rather, there are a few unusual institutions that perform surgery in spite of the general opinion that surgery is not indicated.
First, it is difficult to follow the logic described by the authors that led them to designated fistula or no-fistula. Even if there is an additional effect of adding prostaglandin, this is a very narrow population and it seems doubtful that this experience generalizes to others.
Uramoto, et al, in a paper entitled Vitamin E and vitamin C in the treatment of idiopathic sudden sensorineural hearing loss.
This study from China suggests that intravenous administration of a mushroom extract improves hearing in SHL. Combinatin therapy (intratympanic dexamethosone + high-dose predisone taper) for the treatment of idiopathic sudden sensorineural hearing loss. Prognostic model for predicting hearing recoery in idiopathic sudden sensorineural hearing lossl.
Experimental otoacoustic emission and auditory brainstem response changes by stellate ganglion blockage in rat. Intratympanic dexamethasone for sudden sensorineural hearing loss after failure of systemic therapy. Potential Benefits of Combination Therapy as Primary Treatment for Sudden Sensorineural Hearing Loss.
Results of exploratory tympanotomy following sudden unilateral deafness and its effects on hearing restoration.
Association between the V Leiden G1691A mutation and sudden sensorineural hearing loss in Italian population: a meta-analysis. Furthermore, only 3% of the population had difficulty hearing anything other than faint speech. Adults with hearing loss are more likely to be unemployed and earn less wages (Jung and Bhattacharyya, 2012).
If we do the math, 90% of 8% of the population with a hearing loss works out to about 7% of the population. Persons with cochlear deficits fail OAE testing, while persons with 8th nerve deficits fail ABR testing. If we do the math again, as only 8% of the population has a hearing loss at all, and 90% of them are sensorineural, this means that only 0.8% of the population has a conductive hearing loss. Central hearing loss generally isn't helped to a great extent by medication or surgery, but preventing progression is important. Sudden hearing loss as well as Congenital hearing loss may be due to sensorineural, conductive or central mechanisms.
Association of hearing loss with decreased employment and income among adults in the United States .Ann Otol Rhinol Laryngol.
The middle ear includes the medial surface of the eardrum, the ossicular chain, the eustachian tube, and the tympanic segment of the facial nerve.
Postmeningitic hearing loss can be due to lesions of the cochlea, brainstem and higher auditory pathways, but usually is related to suppurative labyrinthitis (cochlear).
It is a subtype of sensorineural hearing loss, and because it is due to cochlear damage, it is more specifically a cochlear problem.
We have encountered several patients in our practice with transient hearing loss after going to a loud club.
These ear plugs require a visit to a clinic to get an impression made, so that they are specific for the ear being fitted. The effects of type I diabetes mellitus on the cochlear structure and vasculature in human temporal bones.


A long-term study on hearing status in patients with nasopharyngeal carcinoma after radiotherapy. Unsuccessful cochlear implantation in two patients with superficial siderosis of the central nervous system. Injection achieves higher doses in the inner ear fluids, but it does not always lead to recovery of hearing. However, sometimes it is difficult to know -- such as someone with a preexisting unilateral hearing loss, who loses hearing in the other ear. And probably people who care enough about their hearing to get it tested in the past, are more likely to keep caring about it when they have a change.
An additional study of 11 more temporal bones in 2013 (Linthicum et al), was suggestive of a viral etiology. The author's opinion is that migraine is a much more common cause of SHL than is generally appreciated in the otolaryngologic community. However, perhaps it relates to the fact that pregnancy is often reduces Migraine substantially. These apps might also be useful for following ones response to treatment (or no treatment as it may be). In our opinion, MRI is not always necessary as one must consider availability, severity of the situation, and followup. They suggested that the hearing impairment in this select group is more severe and outcome of treatment is worse. In a more recent evidence based review, Lawrence and Thevasagayam (2015) suggested that either oral or intratympanic steroids should generally be offered.
The steroid regimen involved prescribing 66 tablets of prednsone (10 mg) given in a dose of 6 tablets for 7 days, then 5 capsules for 2 days, then 1 less capsule per day until finished. It seems to us that the injections themselves might have an adverse effect on hearing (compared to oral steroids given without a placebo injection).
We do not recommend IT steroids otherwise -- for example, in persons with minor hearing loss. In a more recent evidenced based review, Lawrence and Thevasagayam (2015) suggested that antivirals should not generally be offered routinely.
Horn et al (2005) also reported some good responses to hyperbaric treatment in an uncontrolled study of 9 patients. In a recent evidenced based review, Lawrence and Thevasagayam (2015) suggested that "Due to the variability in the vasodilator and vasoactive agents used, there is insufficient evidence to support the routine use of these agents. At this writing we do not feel that there is enough evidence for either treatment to advocate for its use. According to their article on their experience in 97 patients, surgery can be beneficial if performed within 7 days (Maier et al, 2008). In US data between 1988 and 2006, mild or worse unilateral hearing loss (25 db or worse), is reported in 1.8% of adolescents. If you are older, statistics say that while many of your older associates may have the same problem, it will probably get worse, so you should prepare yourself and do your best to slow down the rate that it gets worse. The inner ear includes the auditory-vestibular nerve, the cochlea and the vestibular system (semicircular canals).
The piccolo generates sound levels up to 112db…roughly equivalent to a jackhammer at 30 feet. Pathological studies suggest that it is due to microangiopathic involvement of inner ear blood vessels and subsequent stria vascularis atrophy and hair cell loss (Fukushima et al, 2004). Considering this unlikely but devastating possibility, we think it is prudent to take the more aggressive approach in treating SHL (see below). There is an obvious trend for an increasing number of positive reports, albeit nearly all uncontrolled, over time. Or perhaps this is because controlled studies show no effect, and studies that show no effect are hard to publish. We are not sure ourselves whether oral or IT steroids are superior in terms of hearing results to doing nothing, but we are certain that large amounts of oral steroids can cause significant side effects. We are very unenthused about this treatment suggestion deriving from this uncontrolled trial.
Second, the natural history of SHL is such that improvement cannot be necessarily attributed to surgical exploration and patching. It is puzzling that these patients were operated, and even more puzzling that they got better. There was greater risk in individuals with work exposure to loud noises, lower socioeconomic background and in ethnic minorities. Other things that cause conductive hearing losses are fluid in the middle ear, and disorders of the small bones (ossicles) in the middle ear.
High levels of cholesteral and low levels of Coenzyme Q are associated with SHL (Cadoni et al, 2007). A recent trial comparing oral steroids to intratympanic injections suggested that there was no difference (Nakache et al, 2015).
This study suggested a powerful treatment effect where the combination group did far better than groups with either IT dexamethasone alone or high dose steroids + placebo injections. Concerning intravenous steroids, they are likely superior to oral steroids, but they are far more difficult to administer. Their method of judging whether or not a fistula was present was direct observation of the round window while displacing the stapes footplate. Inner-ear membrane rupture is treated with bedrest; in certain cases, surgical exploration of the ear may be recommended to patch the inner ear membranes (round and oval windows) with fat from the earlobe (a minor procedure with low risk but uncertain benefit). Of course, most of the processes that they noticed were either disorders that affected spinal fluid (such as meningitis or superficial siderosis), the body as a whole (a variety of autoimmune inner ear disease), and bilateral forms of unilateral ear disorders. They reported a statistically better effect in the IT patients, with no change in the controls.
This protocol also would be expected to have numerous steroid side effects due to the prolonged use of high-dose prednisone. Specific causes have specific treatments: AIED is treated with steroids or immune suppressants, Menierea€™s disease with diuretics and low-salt diet, acoustic neuroma with surgery or radiotherapy. The problem with this study is that it is not a protocol likely to be helpful clinically, as it is not presently common to give 10 days of intravenous steroids in the treatment of SHL. Another concern with this study is that it is implausible in that conventional thought is that treatments provided after 10 days of illness are intrinsically unlikely to work. The obvious problem here that makes interpretation impossible, is the lack of definition of what was done as well as the lack of controls.



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