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Classification for cochleovestibular malformations.

The CT results were reviewed for malformations of bony otic capsule under the following subgroups: Cochlear malformations Vestibular malformations Semicircular canal malformations Internal auditory canal (IAC) malformations Vestibular and cochlear aqueduct malformations.

Cochlear malformations were classified as Michel deformity, Common cavity deformity, Cochlear aplasia, Hypoplastic cochlea, Incomplete partition types I (IP-I) Incomplete partition types II (IP-II) (Mondini deformity).

Incomplete partition type I (cystic cochleovestibular malformation) is defined as a malformation in which the cochlea lacks the entire modiolus and cribriform area, resulting in a cystic appearance, and there is an accompanying large cystic vestibule. has an empty, cystic cochlea and vestibule without an enlarged vestibular aqueduct.

In IP-II (the Mondini deformity), there is a cochlea consisting of 1.5 turns (in which the middle and apical turns coalesce to form a cystic apex) accompanied by a dilated vestibule and enlarged vestibular aqueduct.

for clinical purposes to classify these malformations (in descending order of severity) as follows: 1. Michel deformity, 2. cochlear aplasia, 3. common cavity, 4. IP-I (cystic cochleovestibular malformation), 5. cochlear hypoplasia, 6. IP-II (Mondini deformity).

Failure of otic placode formation during the third gestational week results in complete labyrinthine aplasia (Michel anomaly, Fig 1), whereas insults occurring later (during the seventh week) would only cause mild abnormalities (IP-II or Mondini dysplasia)

In 2002, Sennaroglu and Saatci roposed a classification for cochleovestibular malformations that included, in order of decreasing severity: Labyrinthine aplasia Cochlear aplasia Common cavity deformities Cystic cochleovestibular malformations (IP-I) cochleovestibular hypoplasia IP-II

3rd week : complete labyrinthine aplasia (or Michel deformity) 4th week : common cavity to the cochlea and vestibule 5th week : cochlear aplasia 6th week : cochlear hypoplasia 7th week : incomplete partition and dilatational defects (including Mondini dysplasia)

Michel deformity 6% Cochlear aplasia 5% Common cavity 8% Cochlear hypoplasia 12% Incomplete Partition 41% IP-I (Cystic cochleovestibular malformation) 20% IP-II (Mondini deformity) 19% IP-III (X-linked Deafness) 2% LVAS 15%

Complete labyrinthine aplasia


Complete labyrinthine aplasia (CLA), also referred to as Michel aplasia, is a severe congenital anomaly of the inner ear, Defined by the complete absence of inner ear structures.

6% of the cochlear malformations The most severe malformation Either there is no response or profound hearing loss at 125, 250 and 500 Hz at maximum audimetric limits

CI contraindicated Auditory Brainstem Implant (ABI) can be done

A, Axial CT scan of the temporal bones of patient 1 shows bilateral absence of total inner ear structures with aplasia of the otic capsules bilaterally.

B, CT scan of patient 4 reveals unilateral CLA on the left with a type 1 incomplete cochlear partition on the right. The petrous bone and the otic capsule are hypoplastic on the left.

A, Flattening of the cochlear promontory (arrow) in CLA. B, Normal appearance of the cochlear promontory (arrow) and inner ear in a healthy patient.

Coronal reformatted image of a patient with unilateral (left-sided) CLA reveals severe narrowing of the IAC on the left.

Case 1: 9-year-old girl with profound bilateral congenital sensorineural hearing loss. A, Axial CT scan shows right petrous bone aplasia with absence of inner ear structures. The medial wall of the middle ear is flattened (arrow), being in close contact with the infratentorial nervous structures. Note normal differentiation of the malleus,

Coronal CT scan shows a normally developed external and middle right ear. The long process of the incus (arrow) leans against the medial middle ear wall. The oval window, the stapes, and the second portion of the facial nerve are absent. A small dehiscence of the medial wall of the middle ear (arrowhead), located at the IAM, probably corresponds to the entrance of the facial nerve.

Case 1: 9-year-old girl with profound bilateral congenital sensorineural hearing loss.

Cochlear aplasia

[Clinical features: absent cochlea with intact but often deformed vestibular labyrinth; sensorineural hearing loss

5% of the cochlear malformations Either there is no response or profound hearing loss at 125, 250 and 500 Hz at maximum audimetric limits

CI contraindicated ABI is the only option

Cochlear hypoplasia.

12% of cochlear malformations Cochlear aperture may be aplastic Cochlear nerve may be absent Three different forms are present

If they have absent cochlear aperture or absent cochlear nerve CI is contraindicated, ABI should be done. Patients with hypoplastic nerve is very difficult to decide between CI and ABI

Some patients had mild SNHL who make use of hearing aids with normal language development

This is another example of moderate SNHL who is rehabilitated with hearing aids They may show airbone gaps without middle ear pathology

Sometimes cochlear apex is missing and they have only the base of the cochlea They have better hearing level at high frequencies than the low frequencies

Profound hearing loss at 125, 250 and 500 Hz at maximum audimetric limits They are candidates for CI or ABI

If they have absent cochlear aperture or absent cochlear nerve CI is contraindicated, ABI should be done. Patients with hypoplastic nerve is very difficult to decide between CI and ABI

A, Coronal CT reformatted image through the cochlea demonstrates lack of modiolus and a basal turn of the cochlea.

15% of cochleovestibular malformations Midpoint between the posterior labyrinth and operculum > 1.5 mm

Hearing is usually not stable and usually shows progressive loss over time The hearing level also shows fluctuations and sometimes sudden SNHL. The other common form of presentation is profound SNHL

Air-bone gap in LVA is not due to disease in the middle ear. LVA introduces a third mobile window into the inner ear, which can produce an air-bone gap.

It cause shunting the air-conducted sound away from the cochlea, thus elevating air conduction thresholds It increases the difference in impedance between the scala vestibuli and scala tympani side of the cochlear partition during bone conduction testing, thus improving thresholds for bone conducted sound.