What is Mondini aplasia?

What is Mondini aplasia?

Mondini dysplasia, also known as Mondini malformation and Mondini defect, is an abnormality of the inner ear that is associated with sensorineural hearing loss. This deformity was first described in 1791 by Mondini after examining the inner ear of a deaf boy.

How rare is Mondini dysplasia?

The Mondini is the most common malformation, and accounts for about 55% of all inner ear malformations. The cochlear develops only 1 1/2 turns, and lacks the interscalar septum. Often there is also a large vestibular aqueduct. By definition, basilar turn development is intact.

What is Scheibe dysplasia?

Scheibe dysplasia was first described in humans by Scheibe (1). It is considered to be the most common cause of profound congenital hearing impairment (2). In this type of aplasia, the membranous semicircular canals and utricle are fully formed.

What is cochlear hypoplasia?

Cochlear hypoplasia is a group of inner ear malformations defined by a smaller than normal cochlea with various internal architectural abnormalities such as having <2 turns.

What is presbycusis caused by?

Presbycusis is usually a sensorineural hearing disorder. It is most commonly caused by gradual changes in the inner ear. The cumulative effects of repeated exposure to daily traffic sounds or construction work, noisy offices, equip- ment that produces noise, and loud music can cause sensorineural hearing loss.

How common is Pendred syndrome?

How common is Pendred syndrome? The SLC26A4 gene, which causes Pendred syndrome, accounts for about 5 to 10 percent of hereditary hearing loss. As researchers gain more knowledge about the syndrome and its features, they hope to improve doctors’ ability to detect and diagnose the disorder.

Is Mondini Syndrome genetic?

Being that Mondini dysplasia has been associated with a variety of conditions, inheritance patterns, and both genetic and non-genetic causes, it appears to be inherited in some cases, with the inheritance pattern being dependent upon the underlying cause of the condition in each individual or family.

How do you pronounce Scheibe?


  1. IPA: /ˈʃaɪ̯bə/
  2. Audio. (file)
  3. Hyphenation: Schei‧be.

What is stria Vascularis?

The stria vascularis is a specialized epithelial structure of the mammalian cochlea that produces endolymph, the potassium-rich fluid responsible for the positive endocochlear potential of the cochlear lumen.

Where is a cochlear implant placed?

The internal part is placed under the skin behind the ear during an outpatient surgery. A thin wire and small electrodes lead to the cochlea, which is part of the inner ear. The wire sends signals to the cochlear nerve, which sends sound information to the brain to produce a hearing sensation.

What causes conductive hearing loss?

Common reasons for conductive hearing loss include blockage of your ear canal, a hole in your ear drum, problems with three small bones in your ear, or fluid in the space between your ear drum and cochlea. Fortunately, most cases of conductive hearing loss can be improved.

What is the most common form of presbycusis?

The most common types of presbycusis are sensory (cilia or hair cell loss), neural (spiral ganglion cell loss), metabolic (stria vascularis), and cochlear “Presbycusis has a serious impact on the elderly because it diminishes their ability to communicate and thus their functional independence”conductive (spiral …

What kind of malformation is Mondini dysplasia?

Unfortunately, “Mondini dysplasia” has been used as an all-encompassing term to describe multiple variants of inner-ear anatomy. Malformations including cochlear hypoplasia, cochlear aplasia, and complete labyrinthine aplasia have at times been incorrectly placed into this larger category. [ 2]

How did Carlo Mondini describe the Mondini deformity?

Mondini deformity refers to the absence of the apical modiolus and interscalar septum, resulting in an incomplete partitioning of the cochlea together with an enlarged vestibular aqueduct (EVA) and dilated vestibule. It was first described in 1791 by Carlo Mondini after dissecting the inner ear of an 8-year-old deaf boy.

Can a person with Mondini dysplasia have both ears?

Symptoms Symptoms. Mondini dysplasia is a congenital malformation (present at birth). It may occur either unilaterally (in one ear) or bilaterally (in both ears). Most affected individuals have profound sensorineural hearing loss, but some individuals do have residual hearing.

What happens to the cochlea in Mondini dysplasia?

Individuals with Mondini dysplasia have one and a half coils of the cochlea instead of the normal two coils. [1] It may occur in one ear (unilateral) or both ears (bilateral) and can cause varying degrees of sensorineural hearing loss, although most individuals have profound hearing loss. [1]

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