By Danish Shahab; David A. Klodd, PhD; and Miriam I. Saadia-Redleaf, MD, FACS
A case study and discussion about strategies for mitigating musical tinnitus
Musical tinnitus is the awareness of an autonomous, self-perpetuating musical piece or fragment that the patient clearly recognizes as internal, and that progresses to obnoxious repetitiveness.1-6 Most of these patients are musical people2,5,6 with worsening sensorineural hearing loss.1-8 Musical tinnitus secondary to sensorineural deprivation2,5 has been reported, as have two cases of musical tinnitus that were relieved by cochlear implant activation,2,4 but to our knowledge, there have been no reports of musical tinnitus caused by cochlear implant activation.
A 56-year-old woman with adult onset progressive sensorineural hearing loss was cleared for bilateral cochlear implantation. She had a 40-year history of complex partial seizures with secondary generalization, occurring 2 times per year after fatigue or stress. She had been on 200 milligrams of lamotrigine twice daily for years.
Her musical history included singing in church and school choirs. While she could still hear, she listened to at least 2 hours of music per day. One major goal of implantation, she told the psychologist, was to hear music again.
Implantation with Cochlear CI512 devices was uneventful; all impedances in the common ground, monopolar 1, monopolar 2, and monopolar 1+2 were normal. There was no evidence of short or open circuits. Neural response telemetry was obtained on all electrodes tested (1, 3, 6, 8, 11, 13, 16, 19, and 22).
The patient’s initial stimulation was of the left ear first, followed 3 days later by the right. After each initial stimulation, she reported that sound quality was unnatural, and she was able to identify simple numbers 0-20. Nine days later, the patient first reported constant tinnitus with and without the external device in place. By that time, she was able to hear her husband while conversing at a street intersection, and her comfort levels had been lowered.
The tinnitus began as rhythmic chanting—the repetitious progression of a few notes—in her right ear. Within weeks, it progressed to both ears, and then to her entire head. She found that the noise would lessen if she was very distracted. She also found a familiar tune would soon begin to resonate in her head, replacing the constant chanting. The musical tinnitus would be gone upon initially awakening in the morning, but would return within a few minutes. The tinnitus was present whether she had her external device physically on or off, and whether the external device was turned on or off.
Initial attempts at intervention were through reprogramming of the implants. Throughout the month after initial stimulation, the patient continued to complain of constant chanting. Initially, both right and left processors were reprogrammed, disabling electrodes 1 to 3. Another program was created 14 days later for the right ear using Whisper and ADRO strategy. Nine days later, she further reported that listening to white noise in the background (eg, water running in the kitchen sink, the TV between channels) did not reduce her musical tinnitus. A #6 strength magnet was tried temporarily. On her next visit, electrodes 21 and 22 were disabled. Four months after initial stimulation, electrodes 15 to 21 were deactivated one at a time, but in the end only electrodes 20 and 21 were deactivated. At 6 months, a loaner external processor was tried on the right ear. At 10 months, electrodes 1 and 2 were reintroduced since there was no effect on her ever-present chanting. At 11 months, the implant manufacturer’s representative met with the patient, reviewed her file, and agreed that nothing else could be tried to minimize her musical tinnitus.
During the months after implantation, the patient tried various other remedies. Temporal bone CT scan showed good placement of both electrode arrays. Cochlear implant integrity testing was normal for each device. Neurologic, psychiatric, and psychological evaluations were performed. A 72-hour EEG found mild slow wave abnormalities without epileptiform discharges in the left temporal area, indicating a slight neurophysiological disturbance in that region. (The patient was right handed.) These were new compared to earlier EEGs. Neurologic evaluation was otherwise unchanged from preoperative exams.
Psychiatric evaluation found no evidence that the musical tinnitus was psychotic in nature, but that she did have mild depression and anxiety. Psychological evaluation was unchanged from her preoperative evaluation.
Medications that failed to suppress this patient’s tinnitus were an increased dose of lamotrigine, as well as serial trials of prednisone, risperidone, haloperidol, and quetiapine. Low-dose gabapentin gave transient relief, but a trial of increasing dosage found no significant relief.
Modifications of the patient’s auditory input were attempted. A trial with the show tunes that she had listened to as a child found that they would replace the chanting within a few minutes of hearing them. Subsequent to this modest improvement, she arranged a song list of popular hits she had enjoyed as a young adult, and she found that playing these through the t-coil for her right implant provided some relief.
A tinnitus handicap scale questionnaire9 was administered before and after the intervention with popular tunes, while the patient was only on her baseline lamotrigine dose. It showed no improvement in her tinnitus handicap score, despite her new technique of suppressing the noise.
Musical tinnitus has been reported after deterioration of sensorineural hearing, but not after cochlear implantation. Since the implantation reduces or destroys residual hearing, implanted patients would logically be susceptible to the onset of musical tinnitus. Indeed, the typical cochlear implant patient fits the profile of the musical tinnitus patient perfectly: someone with sensorineural hearing loss who has a deterioration of their remaining hearing. This correspondence leads us to question whether musical tinnitus in the post-implant patient might be underreported.
Medications. There is no clear relationship between musical tinnitus and medications. There have been some reports of new onset musical tinnitus occurring after medication use,3 including cyclopentolate hydrochloride eyedrops and pentoxifylline.
There have been many attempts to use medications to lessen the symptoms of which quetiapine and gabapentin have had modest success.2 Clonazepam, Stelazine, and risperidone seem not to help.2 Diphenylhydantoin has been reported to help in one patient.6 Donepezil, meclobemide, and carbamazepine may have helped in the past.3 In our patient, gabapentin was of only minimal help.
Masking and music. In many of the earlier reports of musical tinnitus, the most reliable methods of suppressing the tinnitus have been to sing or subvocalize a familiar song or musical piece.2,6,7,8 Two earlier reports have described patients whose worsening sensorineural loss led to musical tinnitus, but whose subsequent cochlear implants suppressed their tinnitus upon activation.2,4 Unfortunately for our patient, reprogramming her implant did not help suppress her musical tinnitus. Gabapentin gave an unacceptable degree of relief. Controlling the musical input through her t-coil helped her subjectively, but her assessment via the tinnitus handicap scale9 was unchanged.
Other considerations. Musical tinnitus can have a central component to the disorder, such as lesions in the auditory association cortex,6 the pons and mesencephalon,3 or the basal ganglia.3 However, the most consistent central area of involvement has been the right superior temporal cortex—showing EEG changes in some patients,2 causing musical tinnitus with direct surgical stimulation,3 and showing increased metabolic activity on PET and SPECT scans.2,3,6 Our patient also showed EEG changes; however, these were in her left, not her right, temporal region.
Most patients, however, show no abnormalities on EEG or MRI.2 Instead, a peripheral hearing loss appears to deprive the central auditory pathways of stimulation.2,3,5,6 Similarly, our patient suffered a sudden drop in her already minimal auditory stimulation when she underwent bilateral implantation. Interestingly, of those patients who suffer from musical tinnitus (like our patient), almost all had very active musical lives, being musicians, singers, and composers.
Like our patient, they too found the most successful remediation of their musical tinnitus by replacing the obnoxious tune with another of their choosing.
This report describes onset of musical tinnitus after cochlear implant activation. We expect that this phenomenon will become more widely reported. With the increased access to musical recordings, the average adult listens to far more music today than in any time in history,10 creating the largest population of music lovers ever in history. As a result, these people may be susceptible to musical tinnitus as their hearing decreases for any reason. In looking at the literature, the only consistently effective intervention appears to be replacement of the obnoxious tune with another musical selection.
|Danish Shahab, BS, is an MD candidate (Class of 2013) at the University of Illinois at Chicago College of Medicine. David A. Klodd, PhD, is Director of Audiology and Miriam I. Saadia-Redleaf, MD, FACS, is Director of Otology/Neurotology at the University of Illinois Health Sciences-Chicago. CORRESPONDENCE can be addressed to Dr Redleaf at: [email protected]|
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