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Objective tests used for the evaluation of hearing sensitivity 2.

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Impedance audiometry 2. Tympanometry A measurement of compliance change in the middle ear apparatus to transmit sounds to the inner ear as air pressure is varied in the external auditory canal [ 4950 ]. Acoustic reflex test Following high intensity acoustic stimulation, a sudden decrease in compliance occurs with an approximate 10 ms delay as a consequence of the contraction of the stapedius muscle.

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Otoacoustic emission OAE Spontaneous and evoked sound waves that originate from the inner ear are transmitted through the ossicles and the eardrum into the external auditory meatus can be measured.

Auditory brain stem responses ABR Auditory brain stem responses are far-field potentials that measure all ranges of the early auditory evoked potentials AEP using signal averaging [ 5253 ].

It consists of a series of seven waves I-VII. Click stimulus: used to test the critical high frequency region approximately 2—4 kHz.

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Chirp stimulus: applied to activate the entire cochlea instantaneously. Auditory steady state responses ASSR Auditory steady state responses are AEPs that are used to objectively estimate the hearing sensitivity in individuals with normal hearing sensitivity and with various degrees and configurations of sensorineural hearing loss SNHL [ 55 ]. Immunofluorescence Blood samples were collected in parallel for protein analyses in tubes containing EDTA as anticoagulant.

Blood cells of non-diseased and hearing loss patients were washed with phosphate buffer saline PBS, pH 7. Results 3.

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Examination of patients expecting cochlear implantation The outer ear was inspected by physical examination and found to be anatomically and functionally intact in all subjects prior to any hearing assessment Figure 1A — H. Hearing sensitivity was subsequently evaluated with objective procedures, applying the crosscheck principle to achieve reliable results, confirmed by multiple analyses [ 49 ]. The middle ear is essentially an impedance matching system.

Altered Potassium Ion Homeostasis in Hearing Loss

Impedance is simply a measure of acceptance or rejection of the energy per unit time. While transmitted through the tympanic membrane and other structures of the middle ear, acoustic signals can be modified, depending on the mass, the elasticity and the resistance of the system that would determine the amount of energy accepted or reflected.

The overall configuration of tympanometry and acoustic reflex tests provide greater authority and assurance in the evaluation of middle ear pathologies and conductive hearing loss. Middle ear analysis was performed prior to any other tests to unquestionably exclude any pathology that might alter subsequent results [ 4950 ].

Otoacoustic emissions OAEs indicate the functional integrity of the outer hair cells in the inner ear [ 4951 ]. The source that generates retrograde sound transmission is the frequency following cell-vibration of the outer hair cells, representing their active, non-linear characteristics.

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Several modes of stimulation and registration i. All curves are color-coded: red lines represent the right ear while blue lines represent the left ear. Figure 1A and B clearly shows that the active, non-linear vibrations of the outer hair cells to an external sound stimulus are sensitively recordable with DPOAE exhibiting the normal function of the endocochlear cellular amplification mechanisms Figure 1A.

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In hearing-impaired patients, DPOAE can be registered up to a moderate degree of sensorineural hearing loss; however, the signal tapers off beyond a certain extent Figure 1B. Neural responses during an auditory brainstem response test ABR are generated subcortically by the auditory nerve and subsequent brain stem fiber tracts and nuclei. Using either click or chirp stimuli is still the gold standard for electro-physiologically assessing the integrity of the auditory nerve and the brain stem pathways as they lead the electrical stimuli towards the cortex [ 52535455 ] Figure 1C and D.

Neuronal mix 106 5 anti aging points in the brainstem as generator relay structures are identified in the form of waves on the electrophysiological recordings in normal subjects Figure 1C ; while these points are not registered in patients with a profound hearing loss Figure 1D.

Auditory steady state responses on amplitude-modulated stimuli are highly avusy suisse anti aging, since it takes many years to objectively estimate hearing sensitivity in individuals with normal hearing and with various degrees and configurations of SNHL.

In a non-diseased subject, normal thresholds were registered on all the measured frequencies Figure 1G ; while a profound hearing loss is demonstrated in a patient with hereditary sensorineural deafness Figure 1H.

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Figure 1. Objective clinical measures of the auditory function in patients with hearing loss.

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All curves are color-coded. Red represents the right ear, and blue represents the left ear. A-B: The active, non-linear vibrations of the outer hair cells to external sound stimuli are sensitively recordable with the distortion product otoacoustic emission test DPOAE exhibiting the normal function of the endocochlear cellular amplification mechanisms A.

In hearing impaired patients, DPOAE can be registered up to a moderate degree of sensorineural hearing loss.

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However, the signal tapers off beyond a certain extent B. C-D: Auditory brainstem response tests ABR precisely reveals the functional integrity of the vestibulocochlear nerve leading the electrical stimuli towards the cortex. Neuronal junction points in the brainstem as generator relay structures are identified in the form of waves in the electrophysiological recordings in normal subjects C ; however, they are not present in patients with a profound hearing loss D.

In a non-diseased subject, normal thresholds were registered on all the measured frequencies Ewhile a profound hearing loss is shown in a patient with hereditary sensorineural deafness F. In patients with profound hearing loss, ABRs are missing H. Mutation of connexin genes in blood cells in patients with hearing loss In all patients the most frequently occurring GJ genes in the cochlea were tested for mutations of GJB genes from DNA of blood cells as described above.

Figure 2 shows the distribution in patients from Hungary involving 13 non-diseased patients and 80 patients selected for cochlear implantation. Mutations in GJB6 genes were not detected mix 106 5 anti aging this study Table 1.

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Met34Thr rsGJB2: c. Glu47Ter rs The distribution of these ratios for homozygotes and heterozygotes may change with time, but the rate of this change cannot be estimated at this moment.

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Figure 2. Distribution of connexins and Kir2. In all cells, the nuclei were counterstained with DAPI blue and scale bars are shown on each image.

Thirteen patients with hearing loss were tested for ion channels and compared to controls with same age and sex in each experiment.

The protein pattern of both Cx26 and Cx43 changed on the surface of blood cells Figures 3 and 4appearing as large patches in the control cells and reorganized in smaller groups of connexins in diseased cells. Furthermore, the shape of the area around the nucleus was round in diseased cells and the cytosol was disintegrated.

The protein level of Cx26 was variable on the surface of blood cells from patients with hearing loss. We could not identify the GJB2 gene mutation in Patient 1, but Patient 2 and 3 showed mutations with decreased protein level of Cx We suppose that Patient 1 has a mutation in another type of gene.

Figure 3.

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Pattern of connexin26 ion channels on blood mix 106 5 anti aging of patients expecting cochlear implantation. Figure 4. Alteration of protein levels of connexin43 ion channels in blood cells in patients with deafness.