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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.
Audiology, immunology. 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.
Usher syndrome is characterized by hearing loss and a gradual visual impairment. The hearing loss is caused by a defective inner ear, whereas the vision loss results from retinitis pigmentosa (RP), a degeneration of the retinal cells. Usually, the rod cells of the retina are affected first, leading to early night blindness ( nyctalopia) and the ...
The superior semicircular canal dehiscence syndrome (SSCDS) is a set of hearing and balance symptoms that a rare disease/disorder of the inner ear's superior semicircular canal/duct induces. [ 3 ] [ 4 ] [ 5 ] The symptoms are caused by a thinning or complete absence of the part of the temporal bone overlying the superior semicircular canal of ...
Sensorineural hearing loss ( SNHL) is a type of hearing loss in which the root cause lies in the inner ear, sensory organ ( cochlea and associated structures), or the vestibulocochlear nerve ( cranial nerve VIII). SNHL accounts for about 90% of reported hearing loss. [citation needed] SNHL is usually permanent and can be mild, moderate, severe ...
One is that Mass Eye and Ear is a specialized eye institute that sees a high number of people with rare eye diseases, so the population may have been a little skewed.