Norma R. Mraz, M.A.
Mraz Audiology Consulting, Alpharetta, GA
https://www.tinnitus-ears.com
Robert L. Folmer, Ph.D.
OHSU Tinnitus Clinic
Oregon Health & Science University, Portland, OR
https://www.ohsu.edu/ohrc/tinnitusclinic
INTRODUCTION:
The term "hyperacusis" is sometimes used to denote painful sensitivity to sounds, and is not necessarily correlated to audiometric thresholds.1 Katzenell & Segal2 stated hyperacusis is an "increased sensitivity to sound ... that would not trouble a normal individual." Jastreboff & Jastreboff3 defined hyperacusis as an "abnormally strong reaction occurring within the auditory pathways resulting from exposure to moderate sound; as a consequence, patients express reduced tolerance to suprathreshold sounds. This phenomenon may be, but typically is not, related to recruitment."
Known causes of hyperacusis include sensorineural or conductive hearing loss; perilymphatic fistula, head trauma, acoustic trauma, cerebrovascular accident (CVA), neoplasm, autism, epilepsy, Williams Syndrome, schizophrenia, Lyme Disease and Ramsay Hunt Syndrome. Hyperacusis has been reported in patients with Bell's Palsy, and has been reported in patients who previously underwent stapedectomy.2 Hypersensitivity to sound can also be triggered by certain medications such as antidepressants, antipsychotics, anesthetics, or recreational drugs. It is estimated that 25 to 40% of hyperacusis patients experience chronic tinnitus.
OVERPROTECTION - HYPERACUSIS - PHONOPHOBIA:
Hypersensitivity to everyday sounds leads some patients to develop phonophobia - an overwhelming fear of sound or noise. Patients with hyperacusis and phonophobia often spend an inordinate amount of time monitoring sound levels in their immediate environment. Some of these people mistakenly believe that exposure to low-level sounds can cause additional damage to their hearing. A majority of patients with severe hyperacusis overprotect their ears by wearing earplugs or earmuffs (or both) much of the time. Overuse of earplugs or earmuffs (that is, daily use even when the patient is not exposed to hazardous sound levels) can lead to further hypersensitization of the patient's auditory system. A vicious cycle of overprotection- hyperacusis-phonophobia (OHP) develops. Some patients stop going to movies, restaurants, religious events and other functions because sound exposures in these environments are perceived as painful or potentially harmful. Patients have quit working because anxiety about possible sound exposure at work was overwhelming. In extreme cases, patients become recluses, rarely leaving their homes. The vicious OHP cycle can have devastating consequences for a patient's personal relationships, self-image, lifestyle and quality of life.
OHP and TRT:
It is possible for patients to break the OHP cycle. Improvement can be achieved if clinicians are willing and able to spend significant amounts of time with each patient utilizing Tinnitus Retraining Therapy (TRT) protocols. TRT protocols can be customized to address hyperacusis and the particular circumstances of each patient.3
Following is a case report of one patient with extreme hyperacusis and phonophobia who was treated successfully with TRT.
A CASE STUDY:
A 52-year-old male presented with tinnitus, hearing loss and extreme hyperacusis. He reported waking up one morning at age 33 and discovering - for the first time - that the sound of running water seemed uncomfortably loud to him. Mild bilateral tinnitus began two days after this increase in "sound sensitivity." His tinnitus and sound sensitivity worsened over time, the latter evolving into hyperacusis. He developed insomnia and increased anxiety. He wore ear plugs with increasing regularity to avoid exposure to uncomfortable sounds. Over the next seven years the patient was evaluated by 5 otolaryngologists and 5 audiologists. In 1983, only one of the physicians diagnosed hyperacusis. Unfortunately this physician, like all of the others, told the patient there was nothing that could be done to improve his situation. The patient received evaluations and treatment from a psychiatrist, two psychologists, a chiropractor, an osteopath, and three different spiritual/faith healers. He took Xanax, pursued biofeedback, received transtympanic lidocaine injections, and took megadoses of vitamin supplements. The patient's condition did not improve. At age 43 he went on short-term disability leave from his once successful career in business. Six months later, his otolaryngologist certified him as 100% disabled and the patient began to receive Social Security benefits. He reluctantly but steadily retreated from life as he had lived it. The patient moved from a large city to a remote rural area in order to reduce the risk of sound exposure. He built and spent most of his time in a sound-attenuated room in the back corner of his basement. He bricked over the basement windows and boarded up the rest of the windows in his house. All floor space was carpeted. The refrigerator was moved to an outside shed. The patient stopped talking on the telephone because it seemed too loud to him. Instead, he communicated with the outside world via a TDD (telecommunications device for the deaf) and relay operators. Eventually, even his soft tapping on the TDD keys seemed too loud to him, so he wore headphones to muffle the sound of the keyboard. He still watched television, but muted its audio output and turned on the closed caption feature. There was no radio or computer in the house. The patient wore earmuffs inside the home when his activities (such as stirring powder into a glass of water) involved even minimal sound exposure. Over time he eliminated crunchy and solid foods from his diet because he could not tolerate the sound of his own chewing. He stopped shaving his face, washing his hair, and taking showers because these sounds were perceived as painful. He bathed himself using sanitary wipes. He gave his guitar to his sister because he was no longer able to tolerate the sound of the instrument he once loved to play. He stopped driving his car and rarely left his home, except to collect mail from his mailbox. He wore earplugs and ear muffs whenever he walked outside of his house because even the thought of being exposed to sounds such as birds chirping, wind blowing, or a car horn caused intense feelings of anxiety and fear. He relied on his mother and sister to do his laundry and buy groceries for him during their weekly visits. Otherwise, he was isolated from society.
The patient learned about the clinic where author NM worked by reading an article in Tinnitus Today, the quarterly publication of the American Tinnitus Association. He contacted the clinic by letter and requested a home visit by clinicians. Due to the severity of his situation, his request was granted. Conversations -- including introductions, patient history, education and counseling -- were conducted in whispered voices. Prior to initiating exams or tests, clear and concise instructions were given to the patient. He was provided with regular reassurance. His efforts and cooperation were encouraged and praised throughout the appointment.
AUDIOMETRIC RESULTS:
Otoscopic examination - accomplished slowly and carefully - revealed clean ear canals and normal tympanic membranes bilaterally. Audiometric testing was conducted with extreme caution. Headphones were placed carefully over the patient's ears. Pure tone threshold testing was done in 1 dB increments in ascending order. This procedure eliminated the risk of exposing the patient to sounds he would perceive as uncomfortably loud. Audiometric results revealed mild to moderate sensorineural hearing loss from 1000-8000 Hz in the right ear, and mild to moderately severe sensorineural hearing loss in the left ear (see Figure 1 audiogram). Speech reception thresholds and speech discrimination scores could not be evaluated due to the patient's sound sensitivity. His most comfortable loudness level was 50 dB HL. However, at 55 dB HL, the patient reported being very uncomfortable. Loudness discomfort levels (repeated twice) suggested phonophobia because the patient would not or could not tolerate any sound greater than 10 dB SL (sensation level). For this reason, the patient's tinnitus could not be matched for pitch or loudness. At the conclusion of audiometric testing, the patient showed signs of fatigue.
Figure 1. Pure tone air conduction thresholds recorded during the initial appointment
Most Comfortable Level: 50 dB HL
Uncomfortable Level: 55 dB HL
Even though the patient had significant hearing loss, amplification was not recommended because of the severity of his hyperacusis. A customized TRT program was initiated. To desensitize his auditory system, the patient was fitted with two Silent Star behind-the-ear (BTE) sound generators (Starkey Laboratories, Inc., Eden Prairie, MN) and free-field open ear molds. These devices generate low-level, continuous broad-band sound. To get used to the sensation of having something in his ears, the patient first wore the devices with the sound turned off. After wearing the devices this way for one week, he then turned them on with the volume control set at minimum. He was instructed to turn up the volume on the devices until their sound was "just audible" and to wear them at least two hours every day for two weeks. The next step was to wear the devices with their sound turned on at least four hours per day for two more weeks. After four weeks, the patient progressed to wearing the devices more than four hours per day and gradually increased the volume to "clearly audible." In addition to behind-the-ear sound generators, he also used tabletop sound machines and CDs to add a variety of low-level ambient sounds to his home. The patient received detailed information, education, and directive counseling regarding his hyperacusis and tinnitus. Follow-up counseling and encouragement were provided by telephone using the TDD and telephone relay initially.
As time passed, the patient started to notice improvements in his sound sensitivity and phonophobia. He reported that his concerns about walking outside were beginning to wane, but he continued to wear earmuffs when he left his home. Eventually, he decided to drive his car for the first time in six years and to do his own grocery shopping. The patient accomplished these tasks while wearing earmuffs. He began to listen to music at low volumes. He progressed from communicating through TDD and telephone relay service to using a speaker phone. The patient's diet gradually changed from liquids to soft foods and then to more solid, crunchy foods. Even though the sound of running water in the shower or bathtub still bothered him, he was again able to shave and bathe using soap and water with a cloth. One year after his initial appointment, the patient was participating in more activities inside and outside of his home. His use of earplugs and earmuffs was less frequent. He continued to exhibit phonobia, but the severity of his hyperacusis and tinnitus had decreased. He attended a few counseling sessions with a psychologist to address his phonobia. He took Remeron to treat his depression and insomnia. Twenty-seven months into the program, the patient reported he was doing very well. He had removed the bricks and boards from the windows of his house. He purchased a computer and became active on the internet helping other people who suffer from hyperacusis. He was again able to enjoy playing guitar. The patient enrolled at a local university and is pursuing a new career. As his sound sensitivity continues to improve, the patient might consider pursuing a trial period with hearing aids or combination instruments (combination of hearing aids + sound generators) in the future.
DISCUSSION:
It is difficult -- if not impossible -- to determine the particular contributions of physical vs. psychological components to this patient's condition. Physical symptoms included sensorineural hearing loss, recruitment and tinnitus. Hyperacusis evolved from increased sound sensitivity and was combined with phonophobia.
Patients with extreme hyperacusis and tinnitus often experience insomnia, anxiety, depression, and isolation.4 All of these factors were present in this case study, and must be addressed for patients to improve.
A team of health care professionals including a physician, an audiologist and a psychologist worked together to help this patient recover from desperate circumstances. Open lines of communication, professional availability and flexibility among the clinicians were vital to the success of the management program.
TRT remained the primary treatment protocol in this case. TRT is a time-intensive therapeutic process that combines patient education, directive counseling, reassurance and sound therapy. In this case, these procedures improved the quality of life for this individual who suffered tremendously from OHP.
TRT was not a quick fix. Because the patient's symptoms were complex and developed over 19 years, a considerable time commitment was necessary to facilitate improvement.
TRT does not usually provide a "cure" for tinnitus or hyperacusis, but its principles can be used to design and implement individualized management programs that provide help and relief for patients suffering from these symptoms.
References:
- Dorland's Illustrated Medical Dictionary
- Philadelphia: W.B. Saunders Company,
1985. - Katzenell U, Segal S. Hyperacusis: review and clinical guidelines. Otol Neurotol 2001;22:321-327.
- Jastreboff PJ, Jastreboff MM. Tinnitus retraining therapy (TRT) as a method for treatment of tinnitus and hyperacusis patients. J Am Acad Audiol 2000;11(3):162-177.
- Folmer RL, Griest SE, Martin WH. Chronic tinnitus as phantom auditory pain. Otolaryngology-Head and Neck Surgery 2001;124(4):394-400.
Recommended Audiology Online Readings:
Managing Chronic Tinnitus As Phantom Auditory Pain, by Robert L. Folmer Ph.D. /audiology/newroot/articles/arc_disp.asp?catid=6&id=245
Tinnitus Retraining Therapy: An Update, Pawel J. Jastreboff, Ph. D.,Sc. D., Professor, and Margaret M. Jastreboff, Ph. D., Associate Professor, Department of Otolaryngology, Emory University /audiology/newroot/articles/arc_disp.asp?catid=6&id=227
