I've suffered from tinnitus since May 11, 1996. I've had to accept the fact that these awful noises will never go away for the rest of my life, but what I can't accept is the (sometimes intense) soreness, tingling, etc., in my ears as well as slight pains in my jaws and headaches which have all cropped up in recent years. Aspirin, Tylenol, etc., have little or no effect. Obviously tinnitus is not self-correcting which is why I haven't noticed any improvement since I swore off headphones and loud music a couple of years ago (all of you I-Pod users out there, be warned).
A bit of background: I'm a 32 year-old male in excellent physical health with no history of head trauma or infectious disease (e.g. Meniere's); my tinnitus was caused entirely by use of headphones. Amazingly, an ENT has tested me and told me that my hearing is still perfect despite the tinnitus. And, I don't have any other tinnitus-related symptoms such as insomnia or vertigo.
Any suggestions? Wow! God bless you. I am only slightly familiar with this terrible condition as I only suffer from this at night when I'm about to fall asleep. My husband also has a mild case because when in Iraq he suffered an IED attack that damaged his earring. He went through extensive surgery, but this is the final outcome for him. Your case sounds alot more intense than any other I've heard of. I can't imagine living with that for 10 years. The only thing I could suggest for you would be to research clinics or hospitals around the country that specialize in this type of condition. Maybe they can offer you some hope. Well good luck to you and God Bless! :) Just for the record I actually wrote the resource report that I "cut and pasted". Report It
Everyone in the world will tell you there is no cure. . . except one, who actually is successful in treating it for over 25 years. Go to www.tinnitus-center.com. After specific blood work has been taken - then the doctor will determine whether or not you can be help. Report It
INTRODUCTION 鈥?Tinnitus is a perception of sound in proximity to the head in the absence of an external source. It can be perceived as being within one or both ears, within or around the head, or as an outside distant noise. The sound is often a buzzing, ringing, or hissing, although it can also sound like other noises.
Tinnitus can be continuous (a never ending sound) or occur intermittently. Although both may have a significant impact on the patient, the latter is not usually related to a serious underlying medical problem. The sound may be pulsatile or non-pulsatile. Pulsatile tinnitus raises more concern for underlying significant pathology, though non-pulsatile tinnitus may also be associated with underlying disease.
The epidemiology, pathogenesis, and diagnosis of tinnitus will be discussed here. The treatment of tinnitus is discussed separately. (See "Treatment of tinnitus").
EPIDEMIOLOGY 鈥?According to the American Tinnitus Association, an estimated 50 million people in the United States have chronic tinnitus, persisting for greater than six months [1]. For 12 million, it is severe enough to interfere with daily activities. These people are effectively disabled by their tinnitus to varying degrees.
Tinnitus is more common in men than women and the prevalence increases with age [2,3].
IMPACT OF TINNITUS 鈥?The impact of tinnitus on an individual can be significant. Some individuals "experience" tinnitus, while others "suffer from it." Overall, about 25 percent of tinnitus sufferers report an increase in tinnitus severity over time [4]. Chronic tinnitus is unlikely to remit completely, but often becomes less bothersome over time, especially in the setting of hearing loss.
The impact of tinnitus can be consistently measured by two outcome instruments: the Tinnitus Handicap Inventory (show figure 1) [5] and the Tinnitus Reaction Questionnaire [6]. Studies of these instruments indicate that the degree of disability perceived by tinnitus patients, and its impact on the patient's quality of life, does not correlate with loudness, type of tinnitus, or length of time with tinnitus.
Concurrent mood disorders can increase the perception of disability. Increased tinnitus disability also has been demonstrated in patients with insomnia [7].
ETIOLOGY/PATHOGENESIS 鈥?Tinnitus can be triggered anywhere along the auditory pathway (show figure 2). It is believed to be encoded in neurons within the auditory cortex. The majority of patients have "sensorineural" tinnitus, due to hearing loss at the cochlea or cochlear nerve level.
Somatic sounds may be perceived as tinnitus, and originate in structures with proximity to the cochlea. These sounds are often generated in vascular structures, but may also be produced by musculoskeletal structures. Somatic sounds are most often associated with pulsatile tinnitus and continuous tonal tinnitus (single pure-tone) is usually not somatic in origin.
In a retrospective review of 84 patients with pulsatile tinnitus seen in a neurology department, 42 percent were found to have a significant vascular disorder (most commonly a dural arteriovenous fistula [AVF] or a carotid-cavernous sinus fistula) [8]. In 12 patients (14 percent), nonvascular disorders such as paraganglioma or intracranial hypertension (due to a variety of causes) explained the tinnitus. Thus, patients with pulsatile tinnitus should be thoroughly evaluated. (See "Diagnosis" below).
Vascular disorders 鈥?Pulsatile tinnitus is most commonly, though not exclusively, vascular in etiology. Some vascular tinnitus, such as venous hums and tinnitus due to atherosclerotic plaque narrowing of vessels, can be non-pulsatile.
Arterial bruits 鈥?Arterial vessels near the temporal bone may transmit sounds associated with turbulent blood flow, especially if the loudness of the sound exceeds the hearing threshold in that ear. The petrous carotid system is the most common source, although other arteries may also be involved [9]. An arterial bruit is not itself a serious condition, although the patient may require an evaluation for underlying atherosclerotic disease.
These patients usually do not have other otologic complaints (eg, hearing loss, vertigo, aural fullness). As with many other causes of tinnitus, their tinnitus is greatest in quiet environments (eg at night).
Arteriovenous shunts 鈥?Congenital arteriovenous malformations (AVMs) are rarely associated with hearing loss or tinnitus. Acquired arteriovenous fistulas (AVFs) are more likely to be symptomatic. Dural AVFs are often associated with dural venous sinus thrombosis, which may occur spontaneously or be associated with infection, tumor, trauma, or surgery. Large dural AVFs can result in intracranial hemorrhage; early detection and treatment (surgery and/or vascular embolization) can be life-saving for high grade lesions.
Paraganglioma 鈥?This is a vascular neoplasm arising from the paraganglia cells found around the carotid bifurcation, within the jugular bulb, or along the tympanic arteries in the middle ear. Also known as glomus tumors, they commonly cause a loud pulsing tinnitus that may interfere with hearing. The lesion may be visible through the tympanic membrane as a reddish or blue mass, or may be palpable in the neck. As the tumor enlarges, it may cause hearing loss because of impingement on the ossicular chain (conductive loss) or the labyrinth or cochlea (sensorineural loss). Other cranial nerves may also be affected (eg, facial nerve or lower cranial nerve palsies).
Venous hums 鈥?These may be heard in patients with systemic hypertension, increased intracranial pressure (often due to pseudotumor cerebri), or in patients with a dehiscent or dominant jugular bulb (abnormally high placement of the jugular bulb). The latter may also cause a conductive hearing loss. Tinnitus in patients with a venous hum is often described as a soft, low-pitched hum that may decrease or stop with pressure over the jugular vein, with a change in head position, or with activity [9].
Neurologic disorders 鈥?Pulsatile tinnitus of muscular origin can result from spasm of one or both of the muscles within the middle ear (the tensor tympani and the stapedius muscle). These muscles are enervated by cranial nerves V and VII respectively. Such muscle spasms can occur spontaneously, because of local otologic disease, and also in the presence of neurologic disease such as multiple sclerosis. Patients may also complain of hearing loss or aural fullness associated with these muscle spasms. Tympanometry and otoscopy can be particularly useful in diagnosing middle ear spasmodic activity.
Clicking noises or irregular or rapid pulsations may also result from myoclonus of the palatal muscles that attach to the Eustachian tube orifice. Myoclonus of the palatal muscles most often is caused by an underlying neurologic abnormality, such as multiple sclerosis, microvascular disease affecting the brainstem, or neuropathy related to metabolic or toxic etiology; the history and physical examination should include a search for other neurologic disease.
Eustachian tube dysfunction 鈥?A patulous eustachian tube can cause tinnitus with sounds similar to an ocean roar that may be synchronous with respiration [9]. It most commonly occurs after significant weight loss or after external beam radiation to or near the nasopharynx. The symptoms may disappear when the patient lies down. Patients can also complain of an unusual awareness of their own voice (autophony) and of ear discomfort. The cause of these symptoms is a eustachian tube that remains abnormally patent, allowing too much and then too little aeration of the middle ear space with respiration.
Other somatic disorders 鈥?Somatic non-pulsatile tinnitus is commonly caused by temporomandibular joint (TMJ) dysfunction [10]. It has also been associated with whiplash injuries [11] and other cervical-spinal disorders [12]. Tinnitus may improve when patients respond favorably to treatment for symptoms of TMJ dysfunction and craniocervical disease. The exact neurophysiologic mechanism for the generation of tinnitus from either the TMJ or the cervical spine is not known, but may involve disinhibition of the dorsal cochlear nucleus [11].
Tinnitus with a machine-like or pulsing character is sometimes associated with intracranial lesions, such as chondrosarcoma, aberrant carotid artery, and endolymphatic sac tumors.
Tinnitus originating from the auditory system 鈥?Most tinnitus is due to a sensorineural hearing loss with resulting dysfunction within the auditory system. The auditory system includes the cochlear end-organ, the cochlear nerve (with its projections to and from the cochlea), the brainstem (site of the cochlear nuclei), and the primary and secondary auditory cortical projections (show figure 2).
Etiologies of tinnitus generated from within the auditory system are as varied as the types of noises that patients report (show table 1). The presence of tinnitus often is an early indicator of cochlear hair cell dysrunction or loss, as in the case of prolonged noise exposure [13], Meniere's syndrome (also characterized by aural fullness and vertigo), or ototoxicity.
Ototoxic medications 鈥?Tinnitus is commonly caused by ototoxic medications (show table 2). Ototoxicity affects the various components of the cochleovestibular end-organ. When such structures are damaged, a change in neural firing between the end-organ and the remainder of the auditory system can be exhibited by hearing loss, distortions in hearing, or tinnitus.
Presbycusis 鈥?Presbycusis (sensorineural hearing loss with aging) or any acquired high frequency hearing loss is commonly associated with tinnitus (often described as a high-pitched ringing sound, crickets, or bells in the ear) along with the hearing loss.
Otosclerosis 鈥?This is a condition of abnormal bone repair of the stapes footplate bone (third bone in the ossicular chain) and of the otic capsule. Tinnitus can result when otosclerosis damages cochlear structures. Progressive otosclerosis can result in fixation of the stapes footplate and worsening conductive hearing loss (see "Etiology of hearing loss in adults", section on otosclerosis).
Acoustic neuroma 鈥?Tumors compressing or stretching the cochlear nerve can cause tinnitus; tinnitus can be the presenting sign of a schwannoma of the vestibular nerve within the cerebellar-pontine angle or the internal auditory canal (acoustic neuroma). (See "Acoustic neuroma").
Chiari malformations 鈥?Tinnitus is one of the auditory signs associated with a symptomatic Chiari malformation and occurs when low lying cerebellar tonsils causes tension on the auditory nerve [14].
Other etiologies 鈥?Hearing loss due to a variety of causes, including vascular ischemic events, infection, nerve compression, genetic predisposition, congenital hearing loss, endocrine or metabolic damage to the auditory system, can produce tinnitus to a variable degree. (See "Etiology of hearing loss in adults").
Tinnitus may occur with barotrauma to the middle or inner ear (often associated with vertigo and hearing loss) and with fluid in the middle ear (eg, with otitis media).
Pathogenesis 鈥?Recent pathogenetic theories target the central nervous system as the source or "generator" of all tinnitus that does not have a somatic origin, even in patients whose associated hearing losses are due to cochlear injury [15]. PET scanning and functional MRI studies indicate that the loss of cochlear input to neurons in the central auditory pathways (such as occurs with cochlear hair cell damage due to ototoxicity, noise trauma, or a lesion of the cochlear nerve) can result in abnormal neural activity in the auditory cortex. Such activity has been linked to the perception of tinnitus [16,17].
A current theory likens tinnitus to phantom pain perception that is thought to arise from a loss of suppression of neural activity [18-20]. Known neural feedback loops act to help tune and reinforce auditory memory in the central auditory cortex. Disruption of auditory input or the feedback loop may lead to the creation of alternative neural synapses and to loss of inhibition of normal synapses.
Tinnitus has also been likened to a type of auditory seizure [21], and antiseizure medications have had limited success in some patients [22]. Abnormal auditory-evoked magnetic field potentials associated with tinnitus can be suppressed in selected patients with intravenous lidocaine [23], confirming a central tinnitus phenomenon and potentially indicating a physiologic mechanism for lidocaine sensitive tinnitus (see below).
Electrical stimulation, with round window electrodes or promontory stimulation, can suppress tinnitus in patients with profound hearing loss [24,25]. Electrical promontory stimulation may suppress abnormal spontaneous activity of cochlear nerve fibers; alternatively, it may provide background activity that acts to suppress abnormal central neural connections in patients with baseline sensorineural hearing loss and tinnitus. Cochlear implantation for hearing loss has had a secondary result of relieving tinnitus, with reported success for tinnitus improvement varying from 34 to 93 percent [26,27].
Many patients with tinnitus exhibit signs of anxiety and/or depression [28], and elevated serum serotonin levels have been found in some tinnitus patients [29]. Serotonin and GABA receptors are found throughout the auditory system, and neurotransmitter abnormalities may play a role in some patients with tinnitus [29-31].
These theories encompass disruption of normal neural firing patterns along the entire auditory pathway, from the end organ to the auditory cortex. They may explain tinnitus in patients who do not exhibit hearing loss, as well as in patients who recover from temporary hearing loss (eg, noise-induced hearing loss) but develop tinnitus that is persistent.
DIAGNOSIS 鈥?It is important to keep in mind that tinnitus is frequently a sign of hearing loss or other cochlear injury, and may be the only complaint in a patient with a central nervous system lesion. History and physical examination are the first steps in establishing the etiology of tinnitus. All patients with continuous tinnitus, regardles of the character of the tinnitus, should have an audiological evaluation. Other tests are warranted in specific circumstances.
History 鈥?The history in patients with tinnitus should include a description of the tinnitus (episodic or constant, pulsatile or non-pulsatile, rhythmicity, pitch, quality of the sound), as well as inciting or alleviating factors. Patients should be asked about previous ear disease, noise exposure, hearing status, head injury, and symptoms suggesting TMJ syndrome. All medications and supplements should be reviewed. The history should review other medical conditions, including hypertension, atherosclerosis, neurologic illness, and prior surgery. Patients should be specifically asked about depression, anxiety, and insomnia which can both exacerbate tinnitus and magnify its impact on quality of life.
Tinnitus that is distinctly pulsing or is described as rushing, flowing, or humming is usually vascular in origin. Patients often describe an increase in frequency and intensity with exercise, and some may recognize a connection with their pulse. Changes in intensity or pitch with head motion or body position (lying down versus sitting or standing) also strongly suggest a vascular tinnitus.
Clicking tinnitus almost always has a physiologic explanation. Myoclonus of the palatal muscles or middle ear structures can occur spontaneously, but may also suggest significant neurologic disease. Some patients report a mechanical sounding tinnitus that is not tonal in nature. A diligent investigation searching for vascular or somatic causes is warranted for this rare complaint.
Tonal descriptions of tinnitus can help in the evaluation of a patient for a specific diagnosis or treatment.
A high-pitched continuous tone is by far the most commonly described type of tinnitus. High-pitched tinnitus is frequently a result of a sensorineural hearing loss or may suggest cochlear injury.
Low-pitched tinnitus is often seen in patients with Meniere's disease, although it also can be idiopathic.
Physical examination 鈥?A complete head and neck examination, including cranial nerve examination and evaluation of the tympanic membrane should be performed in all patients. Palatal myoclonus may be suppressed upon wide jaw opening; thus, its absence on oral examination does not rule out the diagnosis (nasopharyngoscopy may be indicated when suspicion is high).
In patients with suspected vascular tinnitus, auscultation over the neck, periauricular area, temple, orbit, and mastoid should be performed in various positions. The effects of positioning and vascular compression of the neck on the involved side should be noted. Tinnitus of venous origin can often be suppressed by careful pressure on the jugular vein.
Specialized testing
Suspected vascular tinnitus 鈥?Patients with infrequent episodes of pulsatile tinnitus or those with short duration, mild tinnitus can be initially observed.
However, because frequent or constant pulsatile tinnitus can herald a potentially life-threatening illness, all of these patients require evaluation by an otolaryngologist or neurotologist. When physical examination does not reveal a specific vascular or musculoskeletal source in these patients, further investigation to rule out a central nervous system (CNS) lesion such as a dural arteriovenous fistula (AVF), arteriovenous malformation (AVM) or aneurysm, or a skull base tumor should be carried out.
The gold standard for AVF diagnosing intracranial vascular lesions is angiography. These lesions often can also be diagnosed noninvasively with MR angiography [32] or CT angiography [33]. High resolution CT scanning is required to delineate the extent of involvement of the skull-base if a paraganlgiolma is suspected and may be sufficient to evaluate other CNS lesions in selected patients. MRI can diagnose a Chiari malformation, vasculitis, CNS tumors, multiple sclerosis, and may indicate the presence of increased intracranial pressure (such as that seen in pseudotumor cerebri), or tumors. Many patients require both contrast MRI and contrast CT because of the varied nature of disorders that cause pulsatile tinnitus. If both of these studies are normal, and suspicion for a vascular lesion remains high, angiography or MR angiography is warranted.
Our current protocol involves audiometric testing followed by an extnesive history and physical exam, which guides additional diagnostic testing. When an intracranial vascular lesion is suspected, we obtain an MRI with contrast initially, followed by CT/CT angiography and subsequent interventional angiography in appropriate circumstances.
Suspected auditory system tinnitus 鈥?For patients with tinnitus that is suspected to originate within the auditory system an initial battery of audiometric tests is essential in the evaluation. This battery includes a pure-tone audiogram, tympanometry, auditory reflex testing, determination of speech discrimination abilities, and otoacoustic emissions testing. These tests identify asymmetries between the two ears and indicate abnormalities in the middle ear, cochlea, and brainstem; as well, they can define the site of abnormality within the auditory system or confirm normal functioning. Such testing is performed in an audiologist's office, ideally one affiliated with an otolaryngology department or practice.
Asymmetry in hearing function, reflex testing, or otoacoustic emissions, in patients with no identified otologic abnormality, should be followed-up with auditory brainstem resonse testing (ABR or BAER) and imaging studies (eg, MRI) to rule out inner ear anomalies, CNS lesions and neurologic disease. Further workup may involve neurologic or neurosurgical consultation, endocrine evaluation, or angiography.
SUMMARY AND RECOMMENDATIONS
Tinnitus affects 50 million people in the US, and interferes with daily activity in a quarter of those affected. Prevalence is greater in men and increases with age. (See "Epidemiology" above).
Pulsatile tinnitus is most commonly vascular in origin and requires thorough evaluation. Differential diagnosis includes intracranial arteriovenous malformation, arteriovenous fistula, arterial bruit, and paraganglioma. Clicking pulsatile tinnitus may indicate a neurologic disorder causing myoclonus of palatal muscles or of the muscles in the inner ear. (See "Vascular disorders" above and see "Neurologic disorders" above).
Tinnitus is most commonly due to abnormalities within the auditory system, often with unexplained etiology, but may be associated with sensorineural hearing loss, ototoxic medications, infection, vascular ischemia or acoustic neuroma. (See "Tinnitus originating from the auditory system" above).
All sensorineural tinnitus is believed to be generated within the central nervous system, with cochlear abnormalities leading to loss of inhibition of cortical auditory neurons. Serotonergic neurotransmitters may play a role. (See "Pathogenesis" above).
Evaluation of tinnitus should include a thorough history, an examination including auscultation for bruits in patients with possible vascular tinnitus, and a complete head and neck examination in all patients. (See "Diagnosis" above).
Patients with pulsatile tinnitus, other than those with infrequent intermittent symptoms, should have otolaryngologic investigation and may require contrast CT scanning, contrast MR scanning and/or angiography. (See "Suspected vascular tinnitus" above). Audiometric testing is indicated for patients with tinnitus suspected of arising within the auditory system. (See "Suspected auditory system tinnitus" above). |
