Musical ear syndrome (MES) is a condition seen in people who have hearing loss and subsequently develop auditory hallucinations. "MES" has also been associated with musical hallucinations, which is a complex form of auditory hallucinations where an individual may experience music or sounds that are heard without an external source. [1] It is comparable to Charles Bonnet syndrome (visual hallucinations in visually impaired people) [2] and some have suggested this phenomenon could be included under this diagnosis. [3]
It is postulated that by the "release phenomenon" MES is caused by hypersensitivity in the auditory cortex caused by sensory deprivation, secondary to their hearing loss. [4] This "hole" in the hearing range is "plugged" by the brain confabulating a piece of information – in this case a piece of music. A similar occurrence is seen with strokes of the visual cortex where a visual field defect occurs and the brain conjures a piece of visual data to fill the spot. This is described by sufferers as an image in the visual field.
The hallucinations are usually not unpleasant but may irritate due to their persistent nature. It is common for sufferers to have a history of tinnitus. [5]
Investigations such as magnetic resonance imaging or CT scanning and electroencephalograms (EEGs) may be worthwhile, but will rarely show any serious pathology. It is believed that because this kind of phenomenon is usually heterogenous in causation, a wide variety of factors need to be considered, which could give a possible explanation for why MES is seen as underdiagnosed. [6] Some of these factors may include significant trauma to the head or any side-effects from substances such as antidepressants, marijuana, alcohol, procaine, or general anesthesia. [7]
MES may occur even when there are little to no symptoms derived from medical testing. [8]
Given the unknown nature of MES, treatments have been largely dependent on an individual basis. Treatments can vary from being as little as self-reassurance to pharmaceutical medications. [1]
Medications can be helpful, such as antipsychotics, benzodiazepines, or antiepileptics, but there is very limited evidence for this. Some case studies have found that switching to a prednisolone steroid after a betamethasone steroid which caused MES helped alleviate hallucinations or the use of the acetylcholinesterase inhibitor, donepezil, has also found that it successfully treated an individual's MES. [6] [7] However, because of the heterogeneous etiology, these methods cannot be applied as general treatment. [6]
Other than treatment by medicinal means, individuals have also successfully alleviated musical hallucinations by cochlear implants, listening to different songs via an external source, or attempting to block them through mental effort, depending on how severe their condition is. [9]
Hallucinations can be reduced by providing the brain with a percentage of the lost input from hearing loss, and patients can maximize their hearing capability by utilizing hearing aids. Hearing aids can make up some of the patients' hearing loss, and potentially alleviate these musical hallucinations. [8] However, this is not effective for all patients. [10]
It is believed that non-drug treatment options are better than drop options for the elderly population that may suffer from MES. [8]
The occurrence of MES has been suggested to be very high among the hearing impaired through acquired deafness or the ear condition known as tinnitus. [4] Though exact causation is uncertain, it has been theorized that the "release phenomenon" is taken into effect. The "release phenomenon" says that individuals with acquired deafness may experience musical hallucinations because of the lack of stimulation, which can give room for the brain to interpret internal sounds as being external. [11]
Sufferers typically hear music or singing and the condition is more common in women. [12] The hallucinatory experiences differ from that commonly experienced in psychotic disorders although there may be some overlap. The most important distinction is the realization that the hallucinations are not real. Delusional beliefs associated with the hallucinations may occur, but some degree of insight should be preserved. There should not be any other psychotic symptoms present, especially hallucinations in other modalities.
Due to the high correlation with hearing loss, MES is common in the elderly due to their often depreciating hearing abilities.
Musical hallucinations and MES have only become widely recognizable in the last few decades of research, but there are indications throughout history that have described symptoms of musical hallucinations. The Romantic composer Robert Schumann was said to have heard entire symphonies in his head from which he drew as inspiration for his music, but later in his life this phenomenon had diminished to just a note that played ceaselessly within his head. [9] An alternative explanation is that his symptoms were caused by syphilis or mercury poisoning used for its treatment. The Russian composer Dmitri Shostakovich was also recorded as experiencing music hallucinations after some shrapnel was removed from his skull. [9]
MES is typically a harmless disease, but can be somewhat disturbing to patients, and can disrupt their quality of life. MES is not considered a mental illness, but rather a side effect of hearing loss. [8]
Ramsay Hunt syndrome type 2, commonly referred to simply as Ramsay Hunt syndrome (RHS) and also known as herpes zoster oticus, is inflammation of the geniculate ganglion of the facial nerve as a late consequence of varicella zoster virus (VZV). In regard to the frequency, less than 1% of varicella zoster infections involve the facial nerve and result in RHS. It is traditionally defined as a triad of ipsilateral facial paralysis, otalgia, and vesicles close to the ear and auditory canal. Due to its proximity to the vestibulocochlear nerve, the virus can spread and cause hearing loss, tinnitus, and vertigo. It is common for diagnoses to be overlooked or delayed, which can raise the likelihood of long-term consequences. It is more complicated than Bell's palsy. Therapy aims to shorten its overall length, while also providing pain relief and averting any consequences.
A hallucination is a perception in the absence of an external stimulus that has the compelling sense of reality. They are distinguishable from several related phenomena, such as dreaming, which does not involve wakefulness; pseudohallucination, which does not mimic real perception, and is accurately perceived as unreal; illusion, which involves distorted or misinterpreted real perception; and mental imagery, which does not mimic real perception, and is under voluntary control. Hallucinations also differ from "delusional perceptions", in which a correctly sensed and interpreted stimulus is given some additional significance.
Hearing loss is a partial or total inability to hear. Hearing loss may be present at birth or acquired at any time afterwards. Hearing loss may occur in one or both ears. In children, hearing problems can affect the ability to acquire spoken language, and in adults it can create difficulties with social interaction and at work. Hearing loss can be temporary or permanent. Hearing loss related to age usually affects both ears and is due to cochlear hair cell loss. In some people, particularly older people, hearing loss can result in loneliness.
Ménière's disease (MD) is a disease of the inner ear that is characterized by potentially severe and incapacitating episodes of vertigo, tinnitus, hearing loss, and a feeling of fullness in the ear. Typically, only one ear is affected initially, but over time, both ears may become involved. Episodes generally last from 20 minutes to a few hours. The time between episodes varies. The hearing loss and ringing in the ears can become constant over time.
Cholesteatoma is a destructive and expanding growth consisting of keratinizing squamous epithelium in the middle ear and/or mastoid process. Cholesteatomas are not cancerous as the name may suggest, but can cause significant problems because of their erosive and expansile properties. This can result in the destruction of the bones of the middle ear (ossicles), as well as growth through the base of the skull into the brain. They often become infected and can result in chronically draining ears. Treatment almost always consists of surgical removal.
Tinnitus is a condition when a person hears a ringing sound or a different variety of sound when no corresponding external sound is present and other people cannot hear it. Nearly everyone experiences faint "normal tinnitus" in a completely quiet room; but this is of concern only if it is bothersome, interferes with normal hearing, or is associated with other problems. The word tinnitus comes from the Latin tinnire, "to ring". In some people, it interferes with concentration, and can be associated with anxiety and depression.
Otosclerosis is a condition of the middle ear where portions of the dense enchondral layer of the bony labyrinth remodel into one or more lesions of irregularly-laid spongy bone. As the lesions reach the stapes the bone is resorbed, then hardened (sclerotized), which limits its movement and results in hearing loss, tinnitus, vertigo or a combination of these. The term otosclerosis is something of a misnomer: much of the clinical course is characterized by lucent rather than sclerotic bony changes, so the disease is also known as otospongiosis.
The temporal lobe is one of the four major lobes of the cerebral cortex in the brain of mammals. The temporal lobe is located beneath the lateral fissure on both cerebral hemispheres of the mammalian brain.
Visual snow syndrome (VSS) is an uncommon neurological condition in which the primary symptom is that affected individuals see persistent flickering white, black, transparent, or colored dots across the whole visual field.
Sensorineural hearing loss (SNHL) is a type of hearing loss in which the root cause lies in the inner ear, sensory organ, or the vestibulocochlear nerve. SNHL accounts for about 90% of reported hearing loss. SNHL is usually permanent and can be mild, moderate, severe, profound, or total. Various other descriptors can be used depending on the shape of the audiogram, such as high frequency, low frequency, U-shaped, notched, peaked, or flat.
Hyperacusis is an increased sensitivity to sound and a low tolerance for environmental noise. Definitions of hyperacusis can vary significantly; it often revolves around damage to or dysfunction of the stapes bone, stapedius muscle or tensor tympani (eardrum). It is often categorized into four subtypes: loudness, pain, annoyance, and fear. It can be a highly debilitating hearing disorder.
Cogan syndrome is a rare disorder characterized by recurrent inflammation of the front of the eye and often fever, fatigue, and weight loss, episodes of vertigo (dizziness), tinnitus and hearing loss. It can lead to deafness or blindness if untreated. The classic form of the disease was first described by D. G. Cogan in 1945.
An auditory hallucination, or paracusia, is a form of hallucination that involves perceiving sounds without auditory stimulus. While experiencing an auditory hallucination, the affected person hears a sound or sounds that did not come from the natural environment.
Geniculate ganglionitis or geniculate neuralgia (GN), also called nervus intermedius neuralgia, Ramsay Hunt syndrome, or Hunt's neuralgia, is a rare disorder characterized by severe paroxysmal neuralgic pain deep in the ear, that may spread to the ear canal, outer ear, mastoid or eye regions. GN may also occur in combination with trigeminal or glossopharyngeal neuralgia.
Cortical deafness is a rare form of sensorineural hearing loss caused by damage to the primary auditory cortex. Cortical deafness is an auditory disorder where the patient is unable to hear sounds but has no apparent damage to the structures of the ear. It has been argued to be as the combination of auditory verbal agnosia and auditory agnosia. Patients with cortical deafness cannot hear any sounds, that is, they are not aware of sounds including non-speech, voices, and speech sounds. Although patients appear and feel completely deaf, they can still exhibit some reflex responses such as turning their head towards a loud sound.
The semicircular canal dehiscence (SCD) is a category of rare neurotological diseases/disorders affecting the inner ears, which gathers the superior SCD, lateral SCD and posterior SCD. These SCDs induce SCD syndromes (SCDSs), which define specific sets of hearing and balance symptoms. This entry mainly deals with the superior SCDS.
Mohr–Tranebjærg syndrome (MTS) is a rare X-linked recessive syndrome also known as deafness–dystonia syndrome and caused by mutation in the TIMM8A gene. It is characterized by clinical manifestations commencing with early childhood onset hearing loss, followed by adolescent onset progressive dystonia or ataxia, visual impairment from early adulthood onwards and dementia from the 4th decade onwards. The severity of the symptoms may vary, but they progress usually to severe deafness and dystonia and sometimes are accompanied by cortical deterioration of vision and mental deterioration.
Musical hallucinations describes a neurological disorder in which the patient will hallucinate songs, tunes, instruments and melodies. These hallucinations are not correlated with psychotic illness. A majority of patients who have symptoms of musical hallucinations are older and have onset conditions predisposing them to the disease. While there is no set form of treatment, research has discovered medications and alternative therapies to be successful in alleviating the hallucinations.
Causes of hearing loss include ageing, genetics, perinatal problems, loud sounds, and diseases. For some kinds of hearing loss the cause may be classified as of unknown cause.
Cochlear hydrops is a condition of the inner ear involving a pathological increase of fluid affecting the cochlea. This results in swelling that can lead to hearing loss or changes in hearing perception. It is a form of endolymphatic hydrops and related to Ménière's disease. Cochlear hydrops refers to a case of inner-ear hydrops that only involves auditory symptoms and does not cause vestibular issues.