Unilateral hearing loss
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Unilateral hearing loss (UHL) is a type of
hearing impairment 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 la ...
where there is normal hearing in one ear and impaired hearing in the other ear.


Signs and symptoms

Patients with unilateral hearing loss have difficulty: * Hearing conversation on their impaired side * Localizing sound * Understanding speech in the presence of background noise * In interpersonal interaction in social settings * Focusing on individual sound sources in large, open environments * Heavy impairment of the auditory Figure–ground perception In quiet conditions, speech discrimination is no worse than normal hearing in those with partial deafness; however, in noisy environments speech discrimination is almost always severe. The prevalence is 3–8.3% of the population. Individuals who are diagnosed with Single Sided Deafness have difficulties with sound localization and speech in noise discrimination. Children with SSD are more likely to experience developmental delays- school, speech, behavioral problems.


Causes

Known causes include genetics, maternal illness and injury. Examples of these causes are physical trauma,
acoustic neuroma A vestibular schwannoma (VS), also called acoustic neuroma, is a benign tumor that develops on the vestibulocochlear nerve that passes from the inner ear to the brain. The tumor originates when Schwann cells that form the insulating myelin sheath ...
, maternal prenatal illness such as measles, labyrinthitis,
microtia Microtia is a congenital deformity where the auricle (external ear) is underdeveloped. A completely undeveloped pinna is referred to as anotia. Because microtia and anotia have the same origin, it can be referred to as microtia-anotia. Microti ...
,
meningitis Meningitis is acute or chronic inflammation of the protective membranes covering the brain and spinal cord, collectively called the meninges. The most common symptoms are fever, headache, and neck stiffness. Other symptoms include confusion or ...
,
Ménière's disease 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 initi ...
,
Waardenburg syndrome Waardenburg syndrome is a group of rare genetic conditions characterised by at least some degree of congenital hearing loss and pigmentation deficiencies, which can include bright blue eyes (or one blue eye and one brown eye), a white foreloc ...
,
mumps MUMPS ("Massachusetts General Hospital Utility Multi-Programming System"), or M, is an imperative, high-level programming language with an integrated transaction processing key–value database. It was originally developed at Massachusetts Gene ...
(epidemic parotitis), and
mastoiditis Mastoiditis is the result of an infection that extends to the air cells of the skull behind the ear. Specifically, it is an inflammation of the mucosal lining of the mastoid antrum and mastoid air cell system inside the mastoid process. The ma ...
. SSD's most severe form of unilateral hearing loss is caused by: sudden sensorineural hearing loss (SSNHL), acoustic neuroma, anomalies inner ear abnormalities, cochlear nerve deficiency (CND), mumps, congenital cytomegalovirus (CMV) infection, meningitis and auditory neuropathy spectrum disorder (ANSD) treatment is based on the cause of the hearing loss. Limited treatment when the cause is the Central auditory system or Auditory nerve.


Prevalence

A 1998 study of schoolchildren found that per thousand, 6–12 had some form of unilateral hearing loss and 0–5 had moderate to profound unilateral hearing loss. It was estimated that in 1998 some 391,000 school-aged children in the United States had unilateral hearing loss.


Profound unilateral hearing loss

Profound unilateral hearing loss is a specific type of
hearing loss 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 l ...
when one ear has no functional hearing ability (91 dB or greater hearing loss). People with profound unilateral hearing loss can only hear in
monaural Monaural or monophonic sound reproduction (often shortened to mono) is sound intended to be heard as if it were emanating from one position. This contrasts with stereophonic sound or ''stereo'', which uses two separate audio channels to reproduc ...
(mono). Profound unilateral hearing loss or single-sided deafness, SSD, makes hearing comprehension very difficult. With speech and background noise presented at the same level, persons with unilateral deafness were found to listen only about 30–35% of the conversation. A person with SSD needs to make more effort when communicating with others. When a patient can hear from only one ear, and there are limited possibilities to compensate for the disability, e.g., changing listening position, group discussions and dynamic listening situations become difficult. Individuals with profound unilateral hearing loss are often perceived as socially awkward due to constant attempts to maximize hearing leading to socially unique body language and mannerisms. UHL also negatively affects hearing and comprehension by making it impossible for the patient to determine the direction, distance and movement of sound sources. In an evaluation using the Speech, Spatial and Qualities of Hearing Scale (SSQ) questionnaire, SSD results in a greater handicap than subjects with a severe hearing loss in both ears. Profound SSD is often confused with sensory discrimination disorder (SDD), a type of
sensory processing disorder Sensory processing disorder (SPD, formerly known as sensory integration dysfunction) is a condition in which multisensory input is not adequately processed in order to provide appropriate responses to the demands of the environment. Sensory proces ...
, and can lead to incorrect processing of sensory information or auditory input during interpersonal communications. Profound unilateral hearing loss is known to cause: * Irritability * Sound aversion: any presence of noise, no matter how low * Body language and mannerisms which appear socially awkward or unusual, like staring at others mouths or tilt the head frequently * Frequent headaches,
stress Stress may refer to: Science and medicine * Stress (biology), an organism's response to a stressor such as an environmental condition * Stress (linguistics), relative emphasis or prominence given to a syllable in a word, or to a word in a phrase ...
* Social isolation * Chronic interpersonal communication difficulties due to inability of brain to isolate or beam form sounds and voices of other individuals * Appearance of anxiousness even in low noise situations * Jumpiness * Trouble figuring out where sounds are coming from. * Variable light dizziness * Trouble paying attention to what people are saying: "evasive" behaviour. * Misdiagnoses as
ADHD Attention deficit hyperactivity disorder (ADHD) is a neurodevelopmental disorder characterised by excessive amounts of inattention, hyperactivity, and impulsivity that are pervasive, impairing in multiple contexts, and otherwise age-inap ...
* Seeming lack of awareness of other people's personal space and moods since brain is hyper-focused on deciphering auditory information in lieu of non-verbal social cues. * Lack of sound depth: any background noise (in the room, in the car) is flat and wrongly interpreted by the brain. The effect is similar to what happens when trying to hear someone speaking in a noisy crowd on a
mono Mono may refer to: Common meanings * Infectious mononucleosis, "the kissing disease" * Monaural, monophonic sound reproduction, often shortened to mono * Mono-, a numerical prefix representing anything single Music Performers * Mono (Japanes ...
recording. The effect is also similar to talking on the phone to someone who is in a noisy environment (see also: King-Kopetzky syndrome) * Inability to filter out background noise or selectively listen to only the important portion of the noise in the environment. * For
sensorineural hearing loss Sensorineural hearing loss (SNHL) is a type of hearing loss in which the root cause lies in the inner ear or sensory organ (cochlea and associated structures) or the vestibulocochlear nerve (cranial nerve VIII). SNHL accounts for about 90% of re ...
, the lack of input coming from the damaged sensory apparatus can cause "ghost beeps" or ringing/
tinnitus Tinnitus is the perception of sound when no corresponding external sound is present. Nearly everyone experiences a faint "normal tinnitus" in a completely quiet room; but it is of concern only if it is bothersome, interferes with normal hearin ...
as the brain attempts to interpret the now missing sensory data. The frequency and the volume of the noise can increase according to one's physical condition (stress, fatigue, etc.). This can aggravate social problems and increase the difficulty of speech comprehension. * Talking loudly or "broadcasting": the affected person cannot perceive the volume of his or her voice relative to other people in the same room or close company, resulting in being characterized by others (who may be located beyond normal auditory range) as domineering or boorish


Treatment

Several hearing devices have been shown to benefit individuals with unilateral hearing loss. Contralateral Routing of Signals (CROS) hearing aids are hearing aids that take sound from the ear with poorer hearing and transmit to the ear with better hearing. They consist of a microphone placed near the impaired ear and an amplifier (hearing aid) near the normal ear. The two units are connected either by a wire behind the neck or by wireless transmission. The aid appears as two behind-the-ear
hearing aid A hearing aid is a device designed to improve hearing by making sound audible to a person with hearing loss. Hearing aids are classified as medical devices in most countries, and regulated by the respective regulations. Small audio amplifiers s ...
s and is sometimes incorporated into eyeglasses. Bone Anchored Hearing Aids (BAHAs) are bone conduction devices that are surgically implanted into the mastoid bone, with an
abutment An abutment is the substructure at the ends of a bridge span or dam supporting its superstructure. Single-span bridges have abutments at each end which provide vertical and lateral support for the span, as well as acting as retaining wal ...
protruding through the skin. The external hearing aid clicks onto the abutment and uses bone conduction to transmit sound to the cochlea of the normal hearing ear. The external component can also be attached to a
headband A headband is a clothing accessory worn in the hair or around the forehead, usually to hold hair away from the face or eyes. Headbands generally consist of a loop of elastic material or a horseshoe-shaped piece of flexible plastic or metal. T ...
or softband and used as a non-surgical device. The BONEBRIDGE bone conduction implant is surgically implanted into the mastoid bone completely underneath the skin. The audio processor is worn externally and held in place by magnets. It sends sound as electrical signals through the skin to the implant. The implant then sends sound vibrations to the
cochlea The cochlea is the part of the inner ear involved in hearing. It is a spiral-shaped cavity in the bony labyrinth, in humans making 2.75 turns around its axis, the modiolus. A core component of the cochlea is the Organ of Corti, the sensory o ...
of the normal hearing ear through the skull via bone conduction. The ADHEAR bone conduction system is a non-surgical device that sticks to the skin behind the ear. It vibrates to send sound vibrations through the skin and the skull bone to the cochlea of the normal hearing ear. The
SoundBite A sound bite or soundbite is a short clip of speech or music extracted from a longer piece of audio, often used to promote or exemplify the full length piece. In the context of journalism, a sound bite is characterized by a short phrase or sentence ...
intraoral bone conduction system used bone conduction via the
teeth A tooth ( : teeth) is a hard, calcified structure found in the jaws (or mouths) of many vertebrates and used to break down food. Some animals, particularly carnivores and omnivores, also use teeth to help with capturing or wounding prey, ...
. One component resembled a conventional behind-the-ear hearing aid that wirelessly connects to a second component worn in the mouth that resembles a conventional dental appliance. The device was discontinued in 2015 and is no longer available. A
cochlear implant A cochlear implant (CI) is a surgically implanted neuroprosthesis that provides a person who has moderate-to-profound sensorineural hearing loss with sound perception. With the help of therapy, cochlear implants may allow for improved speech unde ...
can also be used to treat unilateral hearing loss in many countries.  The device is surgically implanted in the cochlea of non-hearing ear, with a sound processor worn externally.  Using electrical stimulation of the cochlea, sound is sent to the hearing nerve and onto the brain.


Evaluation

According to Snapp 2019, CROS technology solutions provide a noninvasive, aesthetically appealing, low-cost option for individuals with single sided deafness (SSD). The primary benefits of a CROS hearing devices are improved sound awareness from the impaired side and better hearing in noise when speech is located on the impaired side. However, CROS solutions do not provide restoration of binaural hearing and cannot improve tasks requiring binaural input, such as localization. One study of the BAHA system showed a benefit depending on the patient's transcranial attenuation. Another study showed that sound localization was not improved, but the effect of the 
head shadow A head shadow (or acoustic shadow) is a region of reduced amplitude of a sound because it is obstructed by the head. It is an example of diffraction. Sound may have to travel through and around the head in order to reach an ear. The obstruction c ...
 was reduced. The BAHA system has been shown to have higher patient satisfaction and greater perceived benefit by users than the CROS system. The BONEBRIDGE system has been shown to provide good hearing outcomes, and patients also reported high subjective benefit. The device has been shown to decrease the head shadow effect. Sprinzl et al. (2016) also found that the BONEBRIDGE system had a lower complication rate when compared to other implanted bone conduction devices. Studies of the ADHEAR system have shown that patients report higher comfort satisfaction and longer daily wearing times compared to the BAHA system with a headband or softband, due to the lack of pressure on the skin. Better outcomes compared to CROS hearing aids have also been reported. Studies on the SoundBite system showed it to have strong hearing benefit and high patient satisfaction, particularly due to its non-surgical design. Some users reported acoustic feedback from the device. Cochlear implants (CIs) have been shown to benefit patients with unilateral hearing loss in terms of tinnitus reduction, localization, speech understanding, and quality of life (QoL). The increased ability to locate sounds is explained by the fact that, unlike with the other treatments available, treating unilateral hearing loss with a cochlear implant results in both cochleae being stimulated, which helps the brain to better decipher the location of the sound. However, patients may still have difficulties at frontal locations and on the CI side.


Other Hearing issues

School-age children with unilateral hearing loss tend to have poorer grades and require educational assistance. This is not the case with everyone, however. They can also be perceived to have behavioral issues. People affected by UHL have great difficulty locating the source of any sound. They may be unable to locate an alarm or a ringing telephone. The swimming game
Marco Polo Marco Polo (, , ; 8 January 1324) was a Venetian merchant, explorer and writer who travelled through Asia along the Silk Road between 1271 and 1295. His travels are recorded in '' The Travels of Marco Polo'' (also known as ''Book of the Marv ...
is generally impossible for them. When wearing
stereo Stereophonic sound, or more commonly stereo, is a method of sound reproduction that recreates a multi-directional, 3-dimensional audible perspective. This is usually achieved by using two independent audio channels through a configuration ...
headphones, people with unilateral hearing loss can hear only one channel, hence the panning information (volume and time differences between channels) is lost; some instruments may be heard better than others if they are mixed predominantly to one channel, and in extreme cases of sound production, such as complete stereo separation or stereo-switching, only part of the composition can be heard; in games using
3D audio effect 3D audio effects are a group of sound effects that manipulate the sound produced by stereo speakers, surround-sound speakers, speaker-arrays, or headphones. This frequently involves the virtual placement of sound sources anywhere in three-dimensio ...
s, sound may not be perceived appropriately due to coming to the disabled ear. This can be corrected by using settings in the software or hardware—audio player, OS, amplifier or sound source—to adjust
balance Balance or balancing may refer to: Common meanings * Balance (ability) in biomechanics * Balance (accounting) * Balance or weighing scale * Balance as in equality or equilibrium Arts and entertainment Film * ''Balance'' (1983 film), a Bulgaria ...
to one channel (only if the setting downmixes sound from both channels to one), or there may be an option to outright downmix both channels to mono. Such settings may be available via the device or software's accessibility features.Apple - Accessibility - iOS
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See also

*
Cocktail party effect The cocktail party effect is the phenomenon of the brain's ability to focus one's auditory attention on a particular stimulus while filtering out a range of other stimuli, such as when a partygoer can focus on a single conversation in a noisy room ...
* SoundBite Hearing System


References


External links


Profound Unilateral Hearing Loss FAQ's and Flash Animation

BoysTownHospital.org

Unilateral Hearing Loss - Notes
{{DEFAULTSORT:Unilateral Hearing Loss Hearing loss