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High-field MRI versus high-resolution CT of temporal bone in inner ear pathologies of children with bilateral profound sensorineural hearing loss: A pictorial essay.
For better image resolution of nerves oblique parasagittal view which was perpendicular to the plane of the internal auditory canal (IAC) is taken on both sides.
The semicircular canals start to develop from the utricle segments of the otocysts at 7–8 gestational weeks. Apposition of a shell of ossification proceeds rapidly between 18 and 24 weeks of gestation.
The vestibule is a relatively large ovoid perilymphatic space measuring approximately 4 mm in diameter.
The vestibule is continuous anteriorly with the cochlea and posteriorly with the semicircular canals.
Each of the canals makes about two thirds of a circle and measures about 1 mm in cross-sectional diameter. The nonampulated ends of the superior and posterior semicircular canals join to form the bony common crus.
The perilymphatic space of each semicircular canal opens into and communicates freely with the vestibule at both ends. The cochlea consists of a bony canal wound around a conical central core called the modiolus. The interconnecting spaces actually within the membranous labyrinth constitute the endolymphatic cavity.
The cochlear duct is a spiral tube lying within the cochlea and attached to its outer wall. The remainder of the sac protrudes from the inferior aperture of the aqueduct and lies between the periosteum of the petrous bone and the dura mater. Vestibulocochlear nerve carries special afferent fibers for hearing (the cochlear component) and balance (the vestibular component). Branches of the cochlear nerve pass through the lamina of modiolus to innervate the receptors in the spiral ganglion. 2002 (5) proposed a new classification system that divided incomplete partition (IP) into subcategories: IP type I (cystic cochlear malformation) and IP type II (classic Mondini dysplasia).
Other anomalies cannot be explained by a premature arrest in development alone and appear to arise from an aberrant embryologic process.
An example of this type of anomaly is a cochlea of normal length but abnormal size or coiling geometry.
This condition is defined as complete absence of inner ear structures and is caused by developmental arrest of otic placode early during the third week of gestational age. Failure of cochlea development late in the third week of gestation results in this condition. Labyrinthitis ossificans represents pathologic ossification of the membranous labyrinth as a response to an insult to the inner ear. The most commonly recognised semicircular canal abnormality is a short lateral semicircular canal being confluent with the vestibule. Ears with malformations limited to the vestibular system often have normal or near-normal hearing. The LSCC malformation is associated with SNHL and conductive hearing loss (CHL) varied from mild to profound but did not correlate with the severity of LSCC malformation.
Enlargement of the VA is the most common radiographically detectable malformation of the inner ear (3,5).

It also may be the sole radiographically detectable abnormality of the inner ear in a child with hearing loss.
The advent of high-resolution CT in the axial plane has made assessment of the VA much easier. The vestibular aqueduct was considered enlarged if the external pore was greater than 2 mm. Primary importance of detecting enlargement of the IAC is its association with spontaneous CSF leak and the occurrence of gusher during stapes surgery.
A narrow IAC may accompany inner ear malformations or may be the sole radiographically detectable anomaly in a deaf child. Following are the list of imaging findings what the cochlear implant surgeon wants to know from the reporting radiologist. 2 clicks for more privacy: On the first click the button will be activated and you can then share the poster with a second click. Temporal bone micro-anatomy on high resolution CT: Comparison of multi-planar 2-dimentional and volume-rendered 3-dimentional evaluation of middle ear and inner ear structures.
The purpose of audiological evaluation is to quantify and qualify hearing in terms of the degree of hearing loss, the type of hearing loss and the configuration of the hearing loss. Audiological evaluation is also carried out for purposes of monitoring an already identified hearing loss. Once a treatment and management plan is in place, it is still important for an individual’s hearing loss to be checked periodically to determine its stability.
The audiologist may also take measurements that will provide information about the status of the outer and middle ear. After the test battery is completed, the audiologist will review each component of the audiologic evaluation to obtain a profile of hearing abilities and needs. Otolaryngologists (also known Ear, Nose & Throat doctors) specialize in disorders of the head and neck ranging from hearing loss to cancer. The audiologic evaluation consists of a battery of tests each providing specific standalone information.
Once a particular hearing loss has been identified, a treatment and management plan is put into place. The audiologist will use an otoscope, an instrument that contains a light and a magnifying lens, to examine the ear canal and eardrum. Additional specialized testing may be indicated and recommended on the initial test results. Harrison Lin is an ENT-otolaryngologist in Orange, California and is affiliated with University of California, Irvine Medical Center. 8: Left oblique para-sagittal (3D FIESTA sequence) view showing vestibulo-cochlear and facial nerve in internal auditory canal.
9: Axial 3D FIESTA showing Michel’s deformity on right side (red arrow) in the form of complete absence of inner ear structures. 11: Axial 3D FIESTA with superimposed reconstructed 3D image showing Mondini’s deformity (red arrows) and small dysplastic vestibule (yellow arrows) on both sides. The ear canal is examined for the presence of excessive wax (cerumen), or foreign objects (food, toys, pieces of cotton swabs, etc.).
Tympanometry, one aspect of immittance testing, can assist in the detection of fluid in the middle ear, perforation of the eardrum, or wax blocking the ear canal.
He received his medical degree from Keck School of Medicine of USC and has been in practice between 6-10 years.

1: Image showing process of embryogenesis of inner ear from 4th week to 8th week of gestation.
The eardrum (tympanic membrane) is examined for any perforation and signs of fluid or infection. The audiologist will also determine speech reception thresholdor the faintest speech that can be heard half the time.
Acoustic reflex measurement, another aspect of immittance testing, can add diagnostic information about middle ear function and hearing loss. Then the audiologist will determine word recognition or ability to recognize words at a comfortable loudness level. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.AbstractThe bone-conduction (BC) tone ABR has been used clinically for over 20 years. He is one of 18 doctors at University of California, Irvine Medical Center who specialize in Otolaryngology. The current study formally evaluated the test performance of the BC tone-evoked ABR in infants with hearing loss. Thornton, “Universal newborn screening for permanent childhood hearing impairment: an 8-year follow-up of a controlled trial,” Lancet, vol. Gravel, “The evidence for universal newborn hearing screening,” American Journal of Audiology, vol. IntroductionHearing loss is a relatively common sensory impairment that may have a wide variety of harmful effects on the affected child and family if not recognized and managed. Thus, early hearing screening and intervention programs, such as the British Columbia Early Hearing Program (BCEHP), have the goal of screening all newborns for hearing loss by one month of age, comprehensive diagnostic assessment by three months of age, and implementation of intervention services and amplification no later than six months of age [4, 5]. The tone-evoked auditory brainstem response (ABR) is an important part of the diagnostic test battery and is currently the gold-standard technique for diagnosing type and degree of hearing loss in infants who do not pass hearing screening [5–7]. A review of past and recent literature indicates that the tone-evoked ABR shows good correspondence with subsequent behavioural thresholds for AC stimuli. Mehl, “Language of early- and later-identified children with hearing loss,” Pediatrics, vol. Hyde, “Auditory brainstem response in audiometric threshold prediction,” in Handbook of Clinical Audiology, J. Stapells, “Frequency-specific evoked potential audiometry in infants,” in A Sound Foundation Through Early Amplification, R.
Stapells, “Frequency-specific threshold assessment in young infants using the transient ABR and the brainstem ASSR,” in Comprehensive Handbook of Pediatric Audiology, R.
Eggermont, “The inadequacy of click-evoked auditory brainstem responses in audiological applications,” Annals of the New York Academy of Sciences, vol. Picton, “The strategy of evoked potential audiometry,” in Early Diagnosis of Hearing Loss, S. Oates, “Estimation of the pore-tone audiogram by the auditory brainstem response: a review,” Audiology and Neuro-Otology, vol. Stapells, “Auditory brainstem response assessment of infants and children,” Seminars in Hearing, vol. Cho Lieu, “Initial audiologic assessment of infants referred from well baby, special care, and neonatal intensive care unit nurseries,” American Journal of Audiology, vol.

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