What is Mondini dysplasia?
What is Mondini dysplasia?
Mondini syndrome, also known as Mondini dysplasia or a Mondini malformation, describes a condition where the cochlea is incomplete, with only one and a half turns instead of the normal two and a half turns.
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.
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.
Is Mondini dysplasia genetic?
The Mondini dysplasia also has been reported as an isolated finding in nonsyndromic cases,10,21 and families with congenital sensorineural hearing loss with autosomal dominant inheritance22 and presumed autosomal recessive inheritance23 have been described, but in none of these cases was the genetic defect identified.
What is a Mondini?
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.
What is cochlear nerve dysplasia?
The terms cochlear nerve (CN) hypoplasia or aplasia describe either a small or absent CN. These malformations have been estimated to occur radiologically in 0.8 and 1.8% of profoundly deaf children [1]. The collective term cochlear nerve deficiency (CND) is commonly used.
What are the contraindications to cochlear implant surgery?
Contraindications to cochlear implantation may include deafness due to lesions of the eighth cranial nerve or brain stem. In addition, chronic infections of the middle ear and mastoid cavity or tympanic membrane perforation can be contraindications.
How do you pronounce Scheibe?
Pronunciation
- IPA: /ˈʃaɪ̯bə/
- Audio. 0:02. (file)
- Hyphenation: Schei‧be.
What type of hearing loss is seen in otosclerosis?
Otosclerosis is a form of conductive hearing loss. In some cases, as the ear loses its ability to transmit sound, people may first notice low-frequency hearing loss, meaning that low-pitched sounds are harder to hear.
How does an auditory brainstem implant work?
A cochlear implant works by bypassing the damaged areas of the inner ear. It uses an array of electrodes to stimulate the auditory nerve directly. The auditory nerve sends signals generated by the implant to the brain, which recognizes the signals as sound.
