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Autoimmune inner ear disease (AIED) was first defined by Dr. Brian McCabe in a landmark paper describing an autoimmune loss of hearing. [2] The disease results in progressive sensorineural hearing loss (SNHL) that acts bilaterally and asymmetrically, and sometimes affects an individual's vestibular system.
Hearing loss that worsens with age but is caused by factors other than normal aging, such as noise-induced hearing loss, is not presbycusis, although differentiating the individual effects of multiple causes of hearing loss can be difficult. One in three persons have significant hearing loss by age 65; by age 75, one in two.
Profound unilateral hearing loss is a specific type of hearing loss 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 (mono). Profound unilateral hearing loss or single-sided deafness, SSD, makes hearing comprehension very difficult.
Hearing loss is a partial or total inability to hear. [5] Hearing loss may be present at birth or acquired at any time afterwards. [6] [7] Hearing loss may occur in one or both ears. [2] 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. [8]
Hearing loss due to noise has been described as primarily a condition of modern society. [17] In preindustrial times, humans had far less exposure to loud sounds. Studies of primitive peoples indicate that much of what has been attributed to age-related hearing loss may be long-term cumulative damage from all sources, especially noise.
A negative Stenger result indicates that the patient responded to the signal presented to the better ear, indicating an organic asymmetric hearing loss. A positive Stenger result indicates that the patient did not respond even though the signal was 10 to 20 dB above the better ear, indicating a non-organic hearing loss. [4]
Indications include unilateral hearing loss (85%), speech impediments, disequilibrium, tremors or other loss of motor control. The cerebellopontine angle cistern is a subarachnoid cistern formed by the cerebellopontine angle that lies between the cerebellum and the pons .
It is typically experienced as a secondary symptom of sensorineural hearing loss, although not all patients with sensorineural hearing loss experience diplacusis or tinnitus. [ 1 ] [ 2 ] The onset is usually spontaneous and can occur following an acoustic trauma , for example an explosive noise, or in the presence of an ear infection . [ 3 ]