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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. To study the imaging findings of the inner ear abnormalities on Computed tomography (CT) and magnetic resonance imaging (MRI). 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. 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.
The hearing industry is constantly striving to develop hearing instruments that improve listening for people with hearing impairment. Though patient comfort has definitely been enhanced with some of the techniques listed above, the jury is still out on whether speech understanding is consistently improved across all listening conditions with these techniques (FM being the exception). Hearing instruments manipulate the amplitude of a signal, making sound louder across frequencies. One signal processing strategy that addresses the limitations of amplification, and unusable hearing, is proportional frequency compression. The use of proportional frequency compression as a method to tap into useable hearing has been mentioned in several publications over the past few years.7-8 Though various proportional frequency compression algorithms exist7-9, to date, AVR Communications is the only company producing ear-level proportional frequency compression hearing instruments with their ImpaCt and Logicom-20 hearing instruments. The proportional frequency compression technology described above functions in a selective manner. For this reason, the frequency compression component in ImpaCt and Logicom-20 hearing instruments is programmable.
The programmable and digital hearing instrument provided detection, but the proportional frequency compression instrument provided the high frequency cues required for discrimination.
Case 2: 24-year-old with moderately severe to profound bilateral sensorineural hearing loss. Though the aided thresholds with the WDRC instrument were good, aided thresholds with the proportional frequency compression aid were significantly better from 1-6 kHz.
Conclusion The above case studies underline the need for hearing care professionals to consider carefully all available options when treating high frequency hearing loss.
Acknowledgements The author thanks Danelle Knowlton, MA, Oakland Children’s Hospital, Oakland, CA, and Claudia Hawley, MA, West Metro Audiological Services, Minnetonka, MN, for providing the case studies in this article, and Barak Dar, Victoria Miskowiec and Joel Skoog for their input. Are the consequences of neonatal hearing loss serious enough that we need to be concerned about them in the first place?
Slide #47 shows data from an annual survey done each year by Gallaudet University showing that children with severe profound bilateral losses suffer from substantial deficits in reading comprehension. Although virtually everyone agrees that severe profound bilateral hearing loss has substantial negative consequences for all aspects of academic performance, the consequences of mild bilateral or unilateral hearing loss are less well known. The data in slide #50 come from the Greater Boston Otitis Media Study Group, where 194 children were followed prospectively for 7 years.
Classic stigmata of congenital syphilis SNHL, interstitial keratitis, Hutchinson teeth (notched incisors), mulberry molars, Clutton joints (bilateral painless knee effusions), nasal septal perforation & saddle deformity& frontal bossing. The technological advances confronting dispensing professionals and consumers are mind-boggling: digital signal processing hearing instruments, hearing instruments with multiple microphones, FM receivers integrated into hearing instruments, disposable hearing aids and much more. With all the sophisticated technology being introduced, why is improving speech understanding still such a challenge and why is measuring client benefit so difficult? A process called Dynamic Speech Re-Coding first identifies specific characteristics in speech to determine if that speech sound needs to be frequency compressed.
If we draw a line at 2 kHz, the information below this line is what the patient hears to discriminate the sentence. 2b shows the original recording played through a hearing instrument with proportional frequency compression enabled.
The child is being mainstreamed in an oral program and receives auditory-oral therapy weekly. The client achieved 68% discrimination with the WDRC hearing instrument and 92% with the frequency compression hearing instrument.
The results of these cases support changing conventional amplification to amplification with proportional frequency compression. Fortunately, there is a wealth of data on this question, and only three very brief examples will be given related to children with severe profound bilateral losses, children with mild bilateral and unilateral losses, and children with fluctuating conductive losses (slide #46). There are, however, a number of studies demonstrating that even mild or unilateral sensorineural hearing loss has substantial negative consequences. Although virtually all newborn hearing screening programs focus only on identifying children with permanent congenital hearing loss, some children with fluctuating conductive losses are often identified. Each time the child visited his or her primary- care physician, data were collected about episodes of otitis media (there were an average of 7 visits per year during the birth to 3-year-old period). Skeletal findings osteochondritis& periostitis of long bones SNHL, interstitial keratitis, Hutchinson teeth (notched incisors), mulberry molars, Clutton joints (bilateral painless knee effusions), nasal septal perforation & saddle deformity& frontal bossing.

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. And, given the time constraints of a busy practice, how can one quickly assess the benefits of hearing instruments in light of the diverse populations served?
The selective proportional frequency compression process is designed to ensure that only desired sounds are moved lower in frequency while other sounds are not modified. The test results associated with each case were obtained during the same session a frequency compression hearing aid was fit.
Three hearing instruments were considered: a power programmable hearing aid, a power digital hearing aid, and the ImpaCt DSR675 power hearing aid (Fig. At 4 kHz, a significant separation in performance between the hearing instruments can be observed. The correlation between the aided thresholds, Ling Test and NU-6 results once again support the use of the Ling Test to predict speech discrimination ability. That gap continues to widen over time, with the average deaf child or youth never exceeding a grade equivalent of 3 years, in spite of the fact that most of them are enrolled in educational programs specifically designed for deaf students.
Slide #48 summarizes information about five different students in which a sample of children with unilateral hearing loss were compared to similar children with normal hearing, and measures were taken about their performance in math, language, and social functioning. The reason for presenting this information is to emphasize the point that even mild fluctuating hearing losses have an important negative effect on developmental outcomes. Data were also collected periodically on a wide variety of measures related to intellectual ability, cognitive functioning, and language competency by trained diagnosticians who were uninformed about the purposes of the study.
1: Image showing process of embryogenesis of inner ear from 4th week to 8th week of gestation.
The proportional frequency compression aid provided 15 dB better thresholds (sound is approximately five times louder) than the programmable and the digital hearing instruments. The clients will now be able to hear plurals, possessives, third person singular, contractions, etc. Proportional frequency compression is designed to provide dispensing professionals with a tool to tap into the high frequency speech cues clients need to improve speech recognition, and the Ling Test can serve as a quick, accurate test to assess hearing instrument benefit.
As shown in slide #49, children with unilateral hearing loss lagged substantially behind their peers regardless of the measure used, whether math, language, or social functioning. Results at 7 years of age for two groups of children are reported here: those with less than 30 days of otitis media over the 3-year period compared to those with 130 or more days of otitis media over the same period.
1b, proportional Frequency Compression for Voiceless Sounds (FCVL) is applied at a factor of 2.25.
The client achieved 64% wearing the programmable hearing instrument, 52% with the DSP instrument and 80% wearing the frequency compression instrument. The addition of this single phoneme is significant for speech and language development, speech understanding and speech production. Even though the sample sizes are quite small in each of the studies, matching was generally done quite well and the results are very consistent. After statistically adjusting the results for a variety of demographic and socio-economic status variables, children with fewer episodes of otitis media did substantially better than their peers on all measures.
The correlation between the aided thresholds, Ling Test and WIPI results support the use of the Ling Test to predict speech discrimination ability.
The difference averaged about one-half standard deviation, which is equivalent to more than a year's worth of development in reading or math achievement. Audibility and discrimination of high frequency voiceless sounds are made possible because relationships of the energy peaks within a sound and between sounds has been maintained. Because the degree of useable hearing will vary from patient to patient, the prescribed amount of frequency compression will vary accordingly. The amplified signal is compressed to the frequency band where the acoustic response of the hearing instrument (shaded area) provides effective gain and the client has better residual hearing.

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15.09.2013 admin

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