Tinnitus, Hyperacusis, and Auditory Hallucinations
The topics covered in this chapter (tinnitus, hyperacusis, and auditory hallucinations) easily could have been included as segments in other chapters. However, it was difficult to determine in which chapter to include these topics as these common auditory disorders can have a number of different etiologies and a variety of sites of lesion and origins. Given this challenge, as well as the keen general interest in these disorders, the decision was made to include a separate chapter for coverage of these three entities. Although these three entities are often described as disorders, they are actually “symptoms” that are associated with a variety of underlying disorders. Both terms are used interchangeably in this chapter as they are in the literature, but the reader should keep in mind when reading through this chapter that the three conditions being discussed are actually symptoms and not disorders.
Tinnitus has been written about for many years in the fields of otology, psychology, and audiology, and it has been the focus of considerable research; however, in spite of these efforts, a cure for this disorder remains elusive. An awareness of this problem and a better understanding of the nature and origins of this auditory symptom have been enhanced by the many contributions of the late Jack Vernon. His efforts in this area have also led to the development of interventions that can help moderate the patient’s tinnitus and/or the individual’s ability to cope with this challenging symptom. In addition to these efforts, the fine work of the American Tinnitus Association has done much to inform the public about this common and bothersome symptom. In this regard, public information and education has played, and should continue to play, a major role in reducing one of the main causes of tinnitus (i.e., excessive noise exposure). Without a doubt, there have been advances in the understanding of tinnitus and certain treatments have been shown to help some individuals. Interestingly, most treatment approaches have been nonmedical in nature with their roots anchored in the early approaches introduced by Jack Vernon.
Hyperacusis has not been studied as long or as extensively as tinnitus. However, in many patients, tinnitus and hyperacusis coexist and are treated together. As was the case with tinnitus, no definitive cure for hyperacusis has been identified, but through counseling and various treatment approaches (e.g., desensitization therapy), this disorder can be managed successfully in many individuals. Procedures such as these can be a critical factor in restoring or establishing the ability of patients to function normally in their everyday activities despite the continued presence of aversions to many common, everyday sounds.
Auditory hallucinations are new to audiology here in the United States. Although new to audiology, this disorder has been studied by psychologists and psychiatrists for some time as many individuals with auditory hallucinations present with psychiatric conditions, such as schizophrenia. Recent research has implicated changes in the anatomy of the central auditory system associated with hallucinations and has shown that many people without comorbid diagnoses of psychological and/or psychiatric conditions also experience auditory hallucinations. These findings have stirred the interest and attention of audiologists. The elderly with histories of long-standing severe hearing loss are one group of patients who seem to be prone to auditory hallucinations. Another group that experiences auditory hallucinations includes patients with neurologic damage involving the central auditory structures. As you will read later in this chapter, the definitions of subjective tinnitus and auditory hallucinations are quite similar, with both involving the perception of sound in the absence of an external auditory stimulus.
The research surrounding tinnitus, hyperacusis, and auditory hallucinations is indeed interesting and will be pursued more in the future. Similarities as well as differences in these three hearing disorders will likely herald advances in both the understanding and treatment of these problems. However, it is important to keep in mind that not all patients who experience one or more of these “hearing” symptoms will have an auditory basis for their disorder. Some will have a nonauditory basis (e.g., a psychological or psychiatric condition), and others may have a comorbid auditory and nonauditory basis. Referrals to other professionals will be critical for those cases where psychological or psychiatric problems are suspected either as a primary cause or as a comorbid condition.
Tinnitus can be defined as the perception of sound in the absence of an environmental stimulus (Chan, 2009). It is derived from the Latin word tinnire meaning “ringing.” Many people are bothered by tinnitus, with some experiencing debilitating tinnitus. Despite being a common disorder and the focus of considerable research, a cure for this disorder remains elusive. However, some treatments do provide relief from tinnitus for at least some patients.
Tinnitus generally is classified into two main categories, subjective and objective. Subjective tinnitus can be heard only by the person who experiences it. It is often described as a ringing, hissing, humming, chirping (as in the sounds made by crickets and/or cicadas), whistling, blowing, or roaring sound. Objective tinnitus, on the other hand, can be measured if a probe microphone or stethoscope (even a hearing aid stethoscope) is placed in the ear canal or on the pinna. It also may be heard by others who are in close proximity to the individual if the tinnitus is loud enough to be perceived. This type of tinnitus is commonly described by sufferers as being a pulsatile, clicking, or rushing type of sound (Marion & Cevette, 1991).
As discussed previously, tinnitus is actually a symptom. Hence, this section title is redundant, but it provides an opportunity to discuss the various characteristics of tinnitus. The perceptual experience of tinnitus can be quite diverse, as was mentioned earlier, and in some individuals, more than one type of sound can be experienced. The tinnitus can be constant, intermittent, fluctuating, triggered by external and/or internal stimuli, and unilateral or bilateral. In addition, it may be accompanied by hearing loss or normal hearing, vestibular symptoms, and a variety of other ear symptoms. The tinnitus and other aural symptoms may vary in severity from essentially unnoticeable to intolerable (Chan, 2009; Marion & Cevette, 1991).
Tinnitus is a common disorder of the auditory system with some reports relating that approximately 10% of the U.S. adult population, or more than 25 million Americans, experience tinnitus lasting at least 5 min (Centers for Disease Control and Prevention, 2018; National Institute on Deafness and Other Communication Disorders, 2016). The incidence does increase with age, and 1 in 200 cases with tinnitus is considered to be severely bothered by it (Tyler & Erlandsson, 2003).
Objective tinnitus usually is pulsatile in nature and often has a vascular basis. This vascular involvement is usually located around the temporal bone. Arteriovenous shunts, venous hum, paragangliomas, neoplasms, hypertension, and elevated intercranial pressure are some of the etiologic bases for objective tinnitus (Chan, 2009; Møller, 2000).
Subjective tinnitus is often a result of noise exposure (18%), trauma (8%), otologic infections or illness (8%), and drugs (2%) (Henry, Dennis, & Schechter, 2005). It can be associated with general sensorineural hearing loss, Ménière’s disease, strokes of the central nervous system, vascular loops, and aging (Henry et al., 2005; Møller, 2000). In the remainder of the cases (64%), the subjective experiences of tinnitus being reported could possibly be attributed to psychological or nonauditory factors, such as negative counseling or other undetermined factors or events.
Due to the likely presence of multiple pathophysiologic factors in many tinnitus sufferers, it is difficult to determine which one actually triggers the tinnitus. It may be possible that a constellation of factors needs to exist to create the perception of tinnitus. Although the previous discussion has focused on pathophysiologic alterations or compromise in the cochlea and/or the auditory nerve, it also is important to realize that involvement of the auditory pathways in the brain can additionally result in tinnitus (Lockwood, Salvi, & Burkard, 2002; Møller, 2000).
Objective tinnitus is most often related to dysfunction of the middle ear or its immediate area (Chan, 2009). Muscular or vascular problems in this region of the head often give rise to pulsatile or clicking-type sounds that are heard by the patient. Seldom does this type of tinnitus originate from the cochlea or more central auditory structures.
This type of finding would suggest that the central auditory pathways may be responsible for the generation and/or persistence of tinnitus in some cases. This is not to say that cochlear damage cannot cause tinnitus. It is highly likely that damage to the cochlea from insults such as high intensity noise or Ménière’s disease will result in the cochlea generating subjective tinnitus. However, a study by Lockwood et al. (2002) indicates that neural activity within the brain also can generate tinnitus (at least some of the time). Functional imaging studies have demonstrated that tinnitus activates the auditory cortex on only one side of the brain, whereas an external tonal stimulus activates both cortices. In addition, changes in functional imaging measures have been documented for individuals who can increase the loudness of their tinnitus by gazing in a certain direction or by clenching their teeth. In these cases, the changes in functional imaging measures revealed increased cortical activity that correlated with the tinnitus provoking maneuvers (see Lockwood et al., 2002, and Møller, 2000, for reviews).
An additional observation that is worth mentioning relates to what appears to be a disconnect between the apparent anatomic site of abnormality and the site of physiologic abnormality. This “disconnect” could add to the difficulty of interpreting the triggers and nature of tinnitus in many patients. Additional research is needed to delineate the nature and exact site of physiologic abnormalities in individuals for whom there may be a discrepancy between the apparent anatomic site of pathology and the actual physiologic site of abnormality.
At this point in time, it is probably best to keep an open mind and entertain the possibility that subjective tinnitus can be localized in the cochlea, the auditory nerve, and/or the central pathways. Currently, there is little in terms of test procedures that allow the accurate localization of tinnitus within the peripheral and/or central auditory system. Tinnitus often can be localized by the patient to one ear, both ears, or to an area more centrally located in the head. If localized to one or both ears, it is commonly assumed that the tinnitus arises from a peripheral auditory structure. On the other hand, it is inviting to think that tinnitus located in the head or midline may have a central origin; however, currently there is a paucity of evidence to support a precise locus of this symptom.
Medical and Audiologic Evaluation
One of the most important steps in the medical evaluation of tinnitus is obtaining a thorough history from the patient in order to begin the process of making a correct diagnosis of the etiology (Perry & Gantz, 2000). However, it should be noted that the etiology cannot always be determined, but the medical evaluation can help rule out a more serious medical involvement (i.e., a retrocochlear lesion). Closely listening to the patient’s reported symptoms is key. It is important to determine if the reported tinnitus is the chief complaint, or if this symptom is secondary to other issues (hearing loss, vertigo, etc.). In addition, the characterization of the tinnitus, such as whether it is symmetric (often reported to be “in the head”) or lateralized to one ear or the other, is of the utmost importance.
Following a thorough case history, a full head and neck examination is necessary. Included in this examination should be a comprehensive otologic examination, including otomicroscopy. Additionally, each cranial nerve should be examined as some otologic disorders (such as a mass lesion) may affect primarily the auditory nerve, resulting in tinnitus, but they also may affect one or more of the adjacent nerves. An examination of all of the cranial nerves can provide insight as to the basis of the underlying problem (Fortune, Haynes, & Hall, 1999). An audiologic evaluation is also warranted to determine if a hearing loss is present and to rule out significant retrocochlear involvement as a contributory factor to the symptom if a hearing loss is identified. Although nonpulsatile, bilateral tinnitus often does not require any further medical examination, individuals who present with unilateral tinnitus or pulsatile tinnitus, as well as those with asymmetric hearing loss, should be seen for a magnetic resonance imaging (MRI) with gadolinium contrast procedure (Schwaber, 2003). In particular, the internal auditory canals should be examined. For those patients who cannot undergo an MRI, an auditory brainstem response (ABR) evaluation or computed tomography (CT) scan should be considered. In addition to traditional imaging, patients with objective pulsatile tinnitus should be evaluated for a variety of disorders including neoplasms, vascular lesions, benign intracranial hypertension, great vessel bruits, and high cardiac output (Perry & Gantz, 2000). These individuals should undergo additional examinations as appropriate, which may include magnetic resonance angiography (MRA) or arteriograms. The benefit of MRA is that it is essentially noninvasive, and it is additionally helpful in determining both arterial and venous involvement. Laboratory testing also may be indicated for some patients. Patients may be evaluated for a variety of disorders, which may include endocrinopathies, metabolic disorders, autoimmune diseases, and syphilis (House & Derebery, 1995).
A complete audiologic evaluation is an important early step in the diagnosis and treatment of tinnitus. Classic puretone and speech audiometric procedures along with immittance testing and otoacoustic emissions should be completed. If indicated, a workup for auditory nerve or central involvement should be carried out (Marion & Cevette, 1991). For puretone thresholds, the use of pulsed tones may make it easier for the patient to identify the stimuli accurately. It also is helpful to have the patient indicate how much the tinnitus bothers him or her by determining what percentage of the time the patient is aware of the tinnitus versus what percentage of the time they are actually disturbed by it. Møller (2000) advocates using three categories for this purpose. These categories include (1) mild—does not interfere with daily living; (2) moderate—annoying and unpleasant; and (3) severe—interferes with daily living in major ways. A number of self-assessment scales have been developed for the purpose of documenting the effects of tinnitus on the patient’s daily activities. A listing of some of the more common self-assessment scales is provided later.
An audiologic, or sometimes termed psychoacoustic, assessment of tinnitus can prove valuable in a number of ways and should be included in the evaluation of the patient who presents with tinnitus. Tyler and Erlandsson (2003) mention five ways that the evaluation of tinnitus can be used. These include: (1) to confirm that the patient has tinnitus, (2) to monitor changes in the tinnitus over time, (3) to provide insights as to underlying mechanisms, (4) to help in the fitting of devices for tinnitus treatment, and (5) to render a determination of the reliability of the patient’s report of tinnitus, which may be important, especially in legal cases. Although such testing is often desirable, many patients will find tinnitus assessments to be challenging and often difficult to complete. Therefore, it is important that the audiologist’s rationale for conducting the psychoacoustic testing be well thought out and made clear to the patient. The evaluation of tinnitus is indeed difficult and not as accurate or precise as most would like it to be. This fact should be understood by those undertaking this challenging task. Despite various psychoacoustic strategies that have been invoked to complete tinnitus matching procedures in the clinical setting, the results remain quite variable as tinnitus has many components and it often changes quickly in pitch and loudness, rendering it difficult to measure.
The key aspects of tinnitus assessment center around pitch and loudness matching (see Marion & Cevette, 1991; Tyler & Erlandsson, 2003). In tinnitus matching procedures, the patient is asked to match the pitch and loudness of his or her tinnitus to external sounds presented under earphones. Multiple replications are usually required to reach stable values. In cases of unilateral tinnitus, matching can be performed with the external sound presented to the ipsilateral ear (ear with the tinnitus) or the contralateral ear (ear without the tinnitus). If bilateral tinnitus is present, each ear must be evaluated separately, which can be challenging. In addition to the matching procedure, a masking procedure can be used. In the masking procedure, the sound pressure level (SPL) of a broadband noise needed to mask out perception of the tinnitus is determined. This procedure, as well as the loudness matching procedure, can give some indication of the perceived loudness of the tinnitus. It has been reported that most people match their tinnitus to an external stimulus that is in the 10 to 30 dB sensation level (SL) range (Møller, 2000). However, a more recent study has shown that the average intensity match for tinnitus was under 10 dB SL for patients with histories of noise exposure (Nageris, Attius, & Raveh, 2010), and clinical experience has also shown that the intensity match for the majority of patients is below 10 dB SL (Vernon & Meikle, 2000). It is important to note that the measurement of tinnitus loudness is affected considerably by the frequency at which the measure is obtained. The SL values typically are lower when loudness is measured at the pitch (i.e., the frequency) of the tinnitus than when it is measured at a lower frequency (see Vernon & Meikle, 2000, for additional information on loudness matching procedures and findings).
Sometimes the frequency of the tinnitus is related to particular otologic problems. For example, the tinnitus experienced by patients with Ménière’s disease is often matched to a low-frequency stimulus and is occasionally pulsatile in nature, whereas the tinnitus experienced by patients with noise-induced loss is more commonly matched to a high-frequency stimulus (Douek & Reid, 1968). Even the more recent report from Nageris et al. (2010) reflected this trend in individuals with noise-induced hearing losses. However, from the present authors’ view, the strength of these relationships has not always been highly reliable or specific. Therefore, it is important that one does not limit testing to a particular frequency range when assessing patients, even if one anticipates a likely match within a specific frequency range based on the patient’s presenting symptoms and/or otologic diagnosis.
The masking of a patient’s tinnitus has been discussed for many years, both as a diagnostic procedure and as a management tool. In most cases, tinnitus can be effectively masked using a broadband noise stimulus. In fact, 91% of the patients in an investigation conducted by Vernon and Meikle (1988) achieved complete masking of the tinnitus. If a patient’s tinnitus can be easily masked with a broadband stimulus, it may indicate that a masking device may work well as a management option for that patient. In a large number of subjects with tinnitus, Savastano (2008) reported that slightly more than 50% of the patients had their tinnitus masked by a broadband noise in the 31 to 60 dB range, whereas slightly more than 30% required levels in excess of 60 dB (although not specified in this study, these levels were assumed to be effective masking levels and not SL measures). Savastano (2008) also related that individuals with hearing loss required higher levels of noise to mask their tinnitus than those with normal hearing (see qualifying comment offered earlier).
In assessing tinnitus, perhaps one of the most interesting measures is that of residual inhibition (RI). This measure is accomplished by using a masking noise (usually a broadband noise, but narrow-band noise or tones can also be used) that is presented above the intensity level needed to mask the tinnitus (usually 10 dB higher) for a period of time (usually 1 min). After the exposure time has lapsed, patients are asked if they still hear the tinnitus, and if they do, they are asked if the tinnitus sounds as loud as it did before the presentation of the masker, or whether it appears that the intensity of the tinnitus has been reduced. If the patients report hearing no tinnitus, it is considered positive or complete RI, and the period of time required for the tinnitus to return is measured. If the tinnitus is the same after the presentation of the masker, it is considered a negative result; and if the tinnitus is present but reduced in loudness, it is classified as a partial RI (Meikle et al., 2004).
Residual inhibition (the time period with complete or partial tinnitus relief) can last from seconds to several minutes. Most people with tinnitus have some degree of RI (Meikle et al., 2004), but some (10% to 15%) note complete abolition of the tinnitus signal (Savastano, 2008). In a large data set, between 2% and 3% of individuals who experienced RI had durations of greater than 10 min (Meikle et al., 2004). Residual inhibition does not have strong implications for routine clinical application at this time, but it is a phenomenon of great interest and should be studied more. Therefore, the authors believe that measures of RI should be included in the evaluation of tinnitus.
Medical and Audiologic Treatment
As outlined previously, numerous disorders are associated with tinnitus and treatment of these associated disorders varies significantly. Perhaps one of the most common medical approaches to tinnitus is pharmacologic management. This is partially related to the strong psychological component associated with tinnitus, which is discussed in the following section.
Psychological Treatment
When a nonotologic or nonaudiologic etiology for tinnitus is uncovered, referrals to other specialists, such as dentists, neurologists, and/or psychologists may be necessary. Psychological treatment plays a critical role for many patients with tinnitus as there is a high comorbidity between tinnitus and associated psychological disorders. These often include anxiety, depression, sleep disturbance, and general social impairment. As a result, tinnitus is often diagnosed as a psychological disorder with psychological consequences (Wilson & Henry, 2000). In conjunction with audiologic management, cognitive behavioral therapy may prove beneficial for patients with significant tinnitus. Tinnitus is often compared to and treated in a manner similar to chronic pain (Tonndorf, 1987). Patients with tinnitus, not unlike patients with chronic pain, have extreme difficulty coping with the symptom and often feel as though they have no control over the tinnitus itself. As a result, several treatment approaches have been suggested to help alleviate the psychological impact of tinnitus on the patient’s life. In addition to pharmacologic agents, these include approaches such as biofeedback, cognitive behavioral therapy, and relaxation training.
There are numerous assessments related to the psychological impact of tinnitus. Examples of these include the Tinnitus Functional Index (Henry et al., 2016), the Tinnitus Reaction Questionnaire (Wilson, Henry, Bowen, & Haralambous, 1991), the Tinnitus Handicap Inventory (Newman, Jacobson, & Spitzer, 1996), the Tinnitus Effects Questionnaire (Hallam, 1996), the Tinnitus Severity Scale (Halford & Anderson, 1991), the Tinnitus Handicap Questionnaire (Kuk, Tyler, Russell, & Jordan, 1990), and the Tinnitus Coping Style Questionnaire (Budd & Pugh, 1996). These types of questionnaires have proven to be very beneficial to clinicians as they can be used to determine the psychological impact of tinnitus on a patient’s everyday functioning. In addition to providing an initial assessment of the impact of the tinnitus on the patient’s functioning, these questionnaires can be used to monitor the efficacy of treatment as well.
Relaxation methods are a form of biofeedback that have been employed with patients who have chronic tinnitus. Some of the first psychological approaches to the treatment or management of tinnitus used similar techniques. The most common form of training is progressive muscular relaxation as described by Bernstein and Borkovec (1973). Through a series of exercises, the patient learns to tense and relax muscle groups. Although this approach alone may not demonstrate significant benefit, it may prove beneficial when used in conjunction with other therapeutic techniques. It also may be effective in treating or managing some of the other disorders/symptoms that are often experienced by the tinnitus sufferer. For example, many patients with tinnitus suffer from severe sleep disturbance, and this particular approach has proven to be helpful in assisting patients with general sleep disorders (Morin, Culbert, & Schwartz, 1994).
Due to the strong psychological component associated with tinnitus, cognitive behavioral therapy (CBT) for use with tinnitus patients was first recommended by Sweetow in the 1980s (see Sweetow, 2000). More recently, the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) published their clinical practice guidelines on tinnitus in which they recommend CBT as a treatment approach for persistent, bothersome tinnitus (see Tunkel et al., 2014). The CBT approach aims to provide patients with the skills needed to change negative associations and behaviors by restructuring their thoughts to be more accurate and positive (Tunkel et al., 2014). The following systematic 10-step approach is the recommended procedure for implementing CBT with tinnitus patients (Sweetow, 2000).
1. Define the problem in terms of a framework that allows for amenable solutions.
2. Identify the behaviors and thoughts affected by the tinnitus.
3. List the maladaptive strategies and cognitive distortions currently employed.
4. Distinguish between the tinnitus experience and the maladaptive behavior.
5. Identify alternative thoughts, behaviors, and strategies.
6. Encourage the patient to formulate and prioritize attainable target goals.
7. Collaboratively devise and rehearse strategies that can be measured.
8. Regularly assess success or failure of coping strategies.
9. Question and challenge unsubstantiated statements.
10. Lay a framework for maintenance of positive change.
Research findings have documented that CBT is an effective approach to tinnitus management (see Aazh & Moore, 2018). In addition, a recent study showed that CBT can be an efficacious management approach even through the use of an Internet delivery system (Beukes, Andersson, Allen, Manchaiah, & Baguley, 2018). This management approach (whether audiologist-guided or Internet provided) also has been shown to have long-term efficacy with respect to tinnitus relief (Beukes, Allen, Baguley, Manchaiah, & Andersson, 2018). Although CBT has been found to be a helpful management approach for the tinnitus patient, care must be taken to counsel the patient that tinnitus generally is an incurable condition. As is the case in many of the current therapies and/or interventions for tinnitus, CBT is designed to help in the management of the condition but will not result in the elimination of the tinnitus.
Pharmacologic Treatment
While not the recommended first line of treatment in the management of tinnitus, numerous drugs have been investigated for the treatment of tinnitus (Elgoyhen & Langguth, 2010). These drugs include antiarrhythmics, anticonvulsants, anxiolytics, glutamate receptor antagonists, antidepressants, and other miscellaneous pharmaceuticals or homeopathic agents, including both controlled and over-the-counter medications. However, it should be noted that there are no standardized protocols for the use of these medications to treat tinnitus at this time.
Perhaps the most routinely prescribed drugs are antidepressants (Darlington & Smith, 2007). This is likely a result of the high comorbidity between tinnitus and psychological involvement. Additionally, anxiolytics have been prescribed with success in managing tinnitus in some patients. Both antidepressants and anxiolytics have been shown to result in statistically significant improvements in tinnitus patients when evaluated using a double-blind, placebo-controlled investigational approach (Johnson, Brummett, & Schleuning, 1993; Sullivan, Katon, Russo, Dobie, & Sakai, 1992). Intravenous lidocaine also has proven to be somewhat effective; however, the effect is short-lived, and there can be notable side effects (Dodson & Sismanis, 2004). Hence, the use of intravenous lidocaine is not a practical approach to the treatment of tinnitus.
Although a number of pharmacologic treatments are available, no one drug has proven to be effective in treating all tinnitus patients. In most cases, the drugs prescribed do not treat the tinnitus itself, but rather are used to manage many of symptoms that accompany tinnitus (i.e., depression, stress, and anxiety). It should also be noted that there currently are no medications that the Food and Drug Administration (FDA) has approved specifically for the treatment of tinnitus. The use of pharmacologic treatments, if employed, should be closely monitored by a physician and will likely prove most beneficial if used in conjunction with other nonpharmaceutical management strategies.
Sound Generators and Maskers
One of the earliest forms of treatment for tinnitus was the use of sound generators and maskers. This form of management can be dated to the early 1820s when the famous French physician Jean Itard described trying to “cover up the internal noise” by using various environmental noises. The first attempt to use ear-level devices to mask the tinnitus was initiated in the early to mid-1970s (see Vernon, 1975). These devices have been termed tinnitus maskers and tinnitus instruments and are officially classified by the Food and Drug Administration as therapeutic devices. Tinnitus maskers can be worn as both behind-the-ear and in-the-ear devices.
Nonwearable devices are often used to assist in masking tinnitus, particularly in an effort to improve sleep disturbance. Reports indicate that nearly 70% of patients with tinnitus suffer from sleep disturbance (Meikle et al., 2004). As a result, many patients use items such as fans, televisions, radios, commercially available sound generators, and cell phone applications to “mask” their tinnitus and promote sleep.
Traditional hearing aids also have been used in the management of tinnitus patients. In general, the literature indicates that the majority of patients with tinnitus receive relief of this symptom from hearing aids as well as sound generators (Berberian et al., 2017; Park et al., 2018). In fact, the AAO-HNS recommends a hearing aid evaluation as one of the primary approaches for patients with persistent, bothersome tinnitus who have comorbid hearing loss (Tunkel et al., 2014). Amplification devices such as hearing aids have proven successful because when these devices are worn by the tinnitus sufferer, ambient noise in the environment is amplified, and the patient’s tinnitus is masked. Kochkin and Tyler (2008) have reported that approximately 60% of individuals with tinnitus who were fitted with hearing aids have reported improvement in their tinnitus symptoms.
There have been many significant developments in signal processing since the time that tinnitus maskers were first introduced. At the present time, devices that combine both a traditional hearing aid and a tinnitus masker in one unit are commercially available. These instruments are appropriate for patients who may need additional tinnitus masking beyond that provided by a hearing aid alone (see Folmer, Martin, Shi, & Edlefsen, 2006, and Searchfield, 2006, for additional discussion).
Although ear-level maskers continue to be used, they are limited as a management approach in that, when they are not being worn, the tinnitus typically continues to be problematic for the patient. It is important for the clinician fitting such devices to be keenly aware of the importance of appropriate fitting techniques. This includes assessments of the patient’s tinnitus—including pitch and loudness matching, minimum masking level, and residual inhibition as outlined previously. Additionally, one must understand that there is a distinct difference between an effective versus an acceptable level of masking (Vernon & Meikle, 2000). Effective masking or “complete masking” refers to the ability to successfully cover the tinnitus so that the patient can no longer hear it, whereas an acceptable level of masking or “partial masking” refers to a situation in which the patient is provided a masking sound which offers some relief from the tinnitus. In the latter situation, the tinnitus is still present but is perceived by the patient to be of lower intensity and less of an annoyance. There are a number of factors that determine whether complete masking can be achieved for a given patient. These include such variables as the level of the tinnitus, the frequency of the tinnitus (e.g., if the tinnitus is matched to a speech frequency, it may be difficult to provide sufficient masking without affecting speech understanding), and the presence of multiple tinnitus sounds (for additional information, see Vernon & Meikle, 2000).
Neurophysiologic Rehabilitation
Neurophysiologic rehabilitation relies on the plasticity of the brain to create neural changes, which can alleviate the tinnitus. Plasticity can be defined as the alteration of nerve cell pathways to better conform to immediate environmental influences, with this alteration often associated with behavioral change (Musiek & Berge, 1998). It has been theorized that tinnitus is driven not only by a dysfunction of the auditory system, but also by involvement of the limbic and autonomic nervous systems (Jastreboff, 2000). The strong emotional and often physical response exhibited by many tinnitus sufferers would suggest that there are nonauditory areas within the central nervous system that contribute to tinnitus. The limbic system is responsible for behavioral responses including mood state and emotion. The autonomic nervous system, on the other hand, provides for motor innervations. As many patients report issues associated with sleep, anxiety, and tension, this would suggest that both systems may somehow play a role in tinnitus. The theory behind the neurophysiologic rehabilitative approach is that if both the audiologic (central auditory nervous system) and the psychological (limbic system) aspects can be addressed, improvement in symptoms will be observed. In the most simplistic terms, it is theorized that the “central gain” produced within the brain is enhanced because of lack of inhibitory neural control, which leads to enhanced excitatory activity. In essence, the loss of sensory input results in enhanced central auditory activity (Auerbach, Rodriques, & Salvi, 2014). Tinnitus (as well as hyperacusis) is believed to be a result of such activity. Neurophysiologic rehabilitation theoretically aims to decrease the central gain by reorganizing the auditory inhibitory and excitatory balance within the brain (Hanley, Davis, Paki, Quinn, & Bellekom, 2008). One such approach has been Tinnitus Habituation Therapy (THT), which was introduced on a theoretical basis by Hallum and colleagues (Hallam, Rachman, & Hinchcliffe, 1984). The goal of this therapy is not to cure tinnitus but rather to filter and block tinnitus-related activity within the brain, thus reducing awareness and disturbance (Jastreboff, 2000). In recent years, several THT approaches have been relatively successful in achieving this goal.
Tinnitus Retraining Therapy (TRT) is a multicomponent program that utilizes sound therapy along with counseling to achieve habituation of tinnitus. Tinnitus patients are categorized along a 4-point scale, with 0 suggesting a low impact on life and 4 suggesting a high impact on life with significant hyperacusis and prolonged sound-induced exacerbation (Jastreboff, 2000). The intent of TRT is to remove the negative association attached to tinnitus perception and to help the patient to habituate to the tinnitus so that it becomes less aversive (Jastreboff, 2007). This is achieved by presenting low-level, broadband acoustic stimulation, which is used to initiate and encourage tinnitus habituation. The effectiveness of TRT has been reported to be significant, with improvement in symptoms reported for up to 80% of patients (Jastreboff, Gray, & Gold, 1996). A large clinical trial of more than 800 veterans demonstrated benefit from TRT, particularly for those individuals with significant tinnitus (Henry et al., 2006).
Another therapeutic technique is one first described by Davis in the 1990s. Davis developed a device (Neuromonics Oasis) and a program (Neuromonics Tinnitus Treatment, NTT) intended to address the audiologic, psychological, and neurologic aspects of tinnitus (see Davis, Wilde, Steed, & Hanley, 2008). With the recent advances in technology, the original device has been replaced by an application (Neuromonics OasisPro), which when prescribed by an audiologist, can be downloaded to an Apple iPhone, iPad, or iPod (Neuromonics, n.d.). In this treatment approach, the patient is presented with precisely designed music that has been tailored spectrally to account for the patient’s auditory thresholds and loudness tolerance. The theory behind the NTT program is that, over time, plasticity will occur through which the negative conscious association with tinnitus will be reduced and its disturbance decreased. This is a multistage treatment program that takes approximately six to nine months for the patient to complete. Several reports have demonstrated the efficacy of treatment with this approach (Davis et al., 2008; Hanley et al., 2008; Jang, Johnson, & Chandrasekhar, 2010).
A variety of therapeutic tools are available to assist clinicians with today’s tinnitus patients. The previous expression, “you’ll just have to live with it” rarely applies to today’s patients. What is important to understand is that, although treatment approaches are available that may help alleviate the “symptom,” there are still no cures for the disorder.
Hyperacusis is a disorder related to loudness perception. Specifically, it is defined as the “consistently exaggerated or inappropriate responses to sounds that are neither threatening nor uncomfortably loud to a typical person” (Klein, Armstrong, Greer, & Brown, 1990). It is not to be confused with phonophobia (i.e., a fear of sounds) or misophonia (i.e., a dislike for particular sounds). Phonophobia and misophonia differ from hyperacusis in that they typically have strong emotional links related to particular sounds, whereas hyperacusis, which also may have emotional links, tends to be generalized to nearly all loud sounds (Baguley & Andersson, 2007). Hyperacusis also varies from recruitment, which is associated with an abnormally rapid growth of loudness perception as intensity increases (a symptom typically found in individuals with cochlear impairment). For example, a patient with recruitment typically would have some degree of sensorineural hearing loss and would perceive a moderately loud sound as uncomfortable (i.e., sounds that are not perceived as uncomfortable by normal hearers are perceived by patients with recruitment as being uncomfortably loud), whereas a patient with hyperacusis often presents with normal hearing and is disturbed even by low-intensity sounds.
Hyperacusis, like tinnitus, is a symptom and not a disorder. Patients who experience hyperacusis will often demonstrate some overt behaviors that reflect their aversion to sounds that are perceived as being “too loud.” Such behaviors include the avoidance of sounds that are perceived to be too loud, covering one’s ears with the hands when anticipating an aversive sound may occur, and/or grimacing when exposed to offending sounds.
There is a paucity of data related to the incidence and prevalence of hyperacusis. European reports suggest incidence rates in the adult population of anywhere from 8% (Andersson, Lindvall, Hursti, & Carlbring, 2002) to 15% (Fabijań ska, Rogowski, Bartnik, & Skarżyński, 1999), whereas rates among children have been reported to be around 3.2%, with 9% of children experiencing phonophobia (Coelho, Sanchez, & Tyler, 2007) and 17.2% in adolescents (Olsen Widén & Erlandsson, 2004). Of note is that there is a high comorbidity of hyperacusis (approximately 40%) in patients whose primary complaint is tinnitus (Jastreboff & Jastreboff, 2000; Sood & Coles, 1988). However, according to Anari, Axelsson, Eliasson, and Magnusson (1999), the comorbidity of these two conditions is reported to be even higher, with 86% of the patients in their sample who presented with hyperacusis as their primary complaint also reporting experiencing some degree of tinnitus.
Given the commonality between hyperacusis and tinnitus, one would be led to speculate that there is a shared mechanism(s) underlying the etiology and pathology of the two disorders. Although in some cases of hyperacusis an underlying etiology can be found, in the majority of cases, no specific etiology can be identified (Baguley, 2003). Several peripheral and central conditions often result in hyperacusis. Table 8–1 lists some of the common peripheral and central conditions that may be associated with hyperacusis.
Although the exact mechanism(s) underlying hyperacusis is unknown, there are several hypotheses which attempt to explain this phenomenon. It is believed that there is a strong link between hyperacusis and the neurotransmitter 5-HT. This is a serotonin receptor that regulates the modulation of many neurotransmitters, including those responsible for aggression, anxiety, appetite, cognition, learning, memory, mood, nausea, and sleep. Marriage and Barnes (1995) have suggested that when 5-HT becomes disordered, the result is not only hyperacusis but also other disorders as outlined in Table 8–1. It also has been hypothesized that there are enhanced cortical responses secondary to poor regulation of GABAergic neurons. GABAergic neurons are responsible for inhibition of central activity. Recent studies of hyperacusis have demonstrated a reduction in neuronal activity in the auditory cortex (see Wang, Luo, Huang, Zhou, & Chen, 2008). However, a more recent report has demonstrated that individuals with hyperacusis have increased amplitude for late auditory evoked potentials compared to control subjects (Norris & Ceranic, 2011). Although there are many theories related to the mechanism(s) underlying hyperacusis, there is no consensus regarding its etiology or its specific site of origin at this time.
Table 8–1. Examples of Peripheral and Central Conditions as well as Other Hormonal and Infectious Diseases That Can Result in Hyperacusis
Source: Based on Katzenell & Segal (2001).