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When TTTS occurs, the middle ear is contracting involuntarily, which produces an audible sensation called the middle ear myoclonus. When the tensor tympani muscle experiences a spasm without the provocation of loud sounds, it's called the acoustic reflex , which is part of a normal auditory system.
Middle ear myoclonus occurs in the muscles of the middle ear. These muscles may include the tensor tympani and stapedius muscles. It can involve the muscles surrounding the Eustachian tube, which include the tensor veli palatini, levator veli palatini, and salpingopharyngeus. Those affected describe it as a thumping sound or sensation in the ear.
In many people with hyperacusis, an increased activity develops in the tensor tympani muscle in the middle ear as part of the startle response to some sounds. This lowered reflex threshold for tensor tympani contraction is activated by the perception/anticipation of loud sound, and is called tonic tensor tympani syndrome (TTTS).
Inner ear malformations, however, are not typically seen in this population. Middle ear disease is common among individuals with Nager syndrome. Chronic otitis media and Eustachian tube deformity can result in conductive hearing loss. For this reason, early detection and treatment for middle ear disease is crucial in this population.
The acoustic reflex (also known as the stapedius reflex, [1] stapedial reflex, [2] auditory reflex, [3] middle-ear-muscle reflex (MEM reflex, MEMR), [4] attenuation reflex, [5] cochleostapedial reflex [6] or intra-aural reflex [6]) is an involuntary muscle contraction that occurs in the middle ear in response to loud sound stimuli or when the person starts to vocalize.
Middle ear causes of pulsatile tinnitus include patulous eustachian tube, otosclerosis, or middle ear myoclonus (e.g., stapedial or tensor tympani myoclonus). The most common inner ear cause of pulsatile tinnitus is superior semicircular canal dehiscence. [58] Pulsatile tinnitus may also indicate idiopathic intracranial hypertension. [59]
If the ear is dry and not infected, an air conduction aid could be tried; if the ear is draining, a direct bone conduction hearing aid is often the best solution. If the conductive part of the hearing loss is more than 30–35 dB , an air conduction device could have problems overcoming this gap.
The middle ear is the portion of the ear medial to the eardrum, and distal to the oval window of the cochlea (of the inner ear). The mammalian middle ear contains three ossicles (malleus, incus, and stapes), which transfer the vibrations of the eardrum into waves in the fluid and membranes of the inner ear .