Objective:This study aims to analyze the threshold changes in distortion product otoacoustic emissions（DPOAE） and auditory brainstem response（ABR） in adult Otof-/- mice before and after gene therapy, evaluating its effectiveness and exploring methods for assessing hearing recovery post-treatment. Methods:At the age of 4 weeks, adult Otof-/- mice received an inner ear injection of a therapeutic agent containing intein-mediated recombination of the OTOF gene, delivered via dual AAV vectors through the round window membrane（RWM）. Immunofluorescence staining assessed the proportion of inner ear hair cells with restored otoferlin expression and the number of synapses.Statistical analysis was performed to compare the DPOAE and ABR thresholds before and after the treatment. Results:AAV-PHP. eB demonstrates high transduction efficiency in inner ear hair cells. The therapeutic regimen corrected hearing loss in adult Otof-/- mice without impacting auditory function in wild-type mice. The changes in DPOAE and ABR thresholds after gene therapy are significantly correlated at 16 kHz. Post-treatment,a slight increase in DPOAE was observeds,followed by a recovery trend at 2 months post-treatment. Conclusion:Gene therapy significantly restored hearing in adult Otof-/- mice, though the surgical delivery may cause transient hearing damage. Precise and gentle surgical techniques are essential to maximize gene therapy's efficacy.
Mice ; Animals ; Otoacoustic Emissions, Spontaneous/physiology* ; Hearing/physiology* ; Ear, Inner ; Hearing Loss/therapy* ; Genetic Therapy ; Auditory Threshold/physiology* ; Evoked Potentials, Auditory, Brain Stem/physiology* ; Membrane Proteins

Objective: The objective of this study is to evaluate the newborn hearing screening program in The Medical City based on the Joint Committee on Infant Hearing (JCIH) 2007 Position Statement Quality Indicators. Methods: Study Design: Cross - Sectional Survey. Setting: Tertiary Private Hospital. Participants: All newborns who underwent newborn hearing screening in The Medical City for the year 2015. Results: Of 2,010 patients delivered in the hospital in year 2015, 1,986 (98.8%) were screened. Among the 59 babies with initial “refer” results, 15 (25.42%) “referred” a second time while 24 (40.68%) “passed” the rescreening. Twenty (33.89%) did not undergo rescreening (10 were classified as dropouts, while another 10 did not undergo rescreening for various reasons. Of those who “referred” during rescreening, only 9 (60%) had further evaluation done with ABR/ASSR. Among these, 4 (26.66%) had hearing loss and proceeded with the appropriate monitoring and management while 5 (33.33%) had normal hearing. Conclusion The current newborn hearing screening program in the Medical City was able to reach JCIH 2007 quality indicators for screening but not for confirmation of hearing Loss. All patients with hearing loss were managed with early rehabilitation.
Otoacoustic Emissions, Spontaneous ; Evoked Potentials, Auditory, Brain Stem

OBJECTIVETo study the audiological and otological status of cleft palate infants with the application of distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), tympanometry with 1000 Hz and 226 Hz probe tones.

METHODSTotally 45 cleft palate infants aged 8-24 months were included in the study. Most of them were examined for DPOAE, ABR and two frequency tympanometry.

RESULTSMost infants failed the three tests,among whom 6.7% ears passed DPOAE and 33.3% of ears had normal ABR hearing threshold. In addition, 8.9% of ears turned out normal in the 1000 Hz probe-tone tympanometry, and 13.3% were type A in the 226 Hz probe-tone tympanometry. Finally, 1000 Hz tympanometry had more agreement with DPOAE and latency of ABR wave I than 226 Hz tympanometry.

CONCLUSIONMost cleft palate infants have audiological and otological problems, which should be evaluated in a more comprehensive manner.

Acoustic Impedance Tests ; Cleft Palate ; physiopathology ; Evoked Potentials, Auditory, Brain Stem ; Humans ; Infant ; Infant, Newborn ; Neonatal Screening ; Otoacoustic Emissions, Spontaneous

BACKGROUNDThe prevalence of sudden sensorineural hearing loss in children (CSSNHL) is consistently increasing. However, the pathology and prognosis of CSSNHL are still poorly understood. This retrospective study evaluated clinical characteristics and possible associated factors of CSSNHL.

METHODSOne hundred and thirty-six CSSNHL patients treated in Department of Otolaryngology-Head and Neck Surgery and Institute of Otolaryngology at Chinese PLA General Hospital between July 2008 and August 2015 were included in this study. These patients were analyzed for clinical characteristics, audiological characteristics, laboratory examinations, and prognostic factors.

RESULTSAmong the 136 patients (151 ears), 121 patients (121 ears, 80.1%) were diagnosed with unilaterally CSSNHL, and 15 patients (30 ears, 19.9%) with bilateral CSSNHL. The complete recovery rate of CSSNHL was 9.3%, and the overall recovery rate was 37.7%. We found that initial degree of hearing loss, onset of treatment, tinnitus, the ascending type audiogram, gender, side of hearing loss, the recorded auditory brainstem response (ABR), and distortion product otoacoustic emissions (DPOAEs) had prognostic significance. Age, ear fullness, and vertigo had no significant correlation with recovery. Furthermore, the relevant blood tests showed 30.8% of the children had abnormal white blood cell (WBC) counts, 22.1% had elevated homocysteine levels, 65.8% had high alkaline phosphatase (ALP), 33.8% had high IgE antibody levels, and 86.1% had positive cytomegalovirus (CMV) IgG antibodies.

CONCLUSIONSCSSNHL commonly occurs unilaterally and results in severe hearing loss. Initial severe hearing loss and bilateral hearing loss are negative prognostic factors for hearing recovery, while positive prognostic factors include tinnitus, gender, the ascending type audiogram, early treatment, identifiable ABR waves, and DPOAEs. Age, vertigo, and ear fullness are not correlated with the recovery. Some serologic indicators, including the level of WBC, platelet, homocysteine, ALP, positive CMV IgG antibody, fibrinogen, and some immunologic indicators, are closely related to CSSNHL.

Adolescent ; Child ; Child, Preschool ; Evoked Potentials, Auditory, Brain Stem ; Female ; Hearing Loss, Sensorineural ; blood ; etiology ; physiopathology ; Humans ; Male ; Otoacoustic Emissions, Spontaneous ; Retrospective Studies

OBJECTIVES: The present study investigates the usefulness of transiently evoked otoacoustic emissions (TEOAEs) and distortion product OAEs (DPOAEs) in detecting small changes in the hearing of young smoking adults. METHODS: Otoacoustic emissions were acquired from the ears of 48 young adults (age, 20 to 27 years). The dataset was divided into two groups, smoking (24 persons/48 ears) and nonsmoking (24 persons/48 ears). The level of smoking was relatively small in comparison to previous studies, an average of 3.8 years and 8.7 cigarettes per day. In each ear three OAE measurements were made: TEOAEs, DPOAEs, and spontaneous OAEs (SOAEs). Pure tone audiometry and tympanometry were also conducted. Audiometric thresholds did not differ significantly between the datasets. Half-octave-band values of OAE signal to noise ratios and response levels were used to assess statistical differences. RESULTS: Averaged data initially revealed that differences between the two study groups occurred only for TEOAEs at 1 kHz. However when the datasets were divided into ears with and without SOAEs more differences became apparent, both for TEOAEs and DPOAEs. In ears that exhibited SOAEs, both smokers and nonsmokers, there were no statistically significant differences between evoked OAEs; however in all ears without SOAEs, evoked OAEs were higher in the ears of nonsmokers, by as much as 5 dB. These differences were most prominent in the 1-2 kHz range. CONCLUSION: A general decrease in OAE levels was found in the group of smokers. However, in ears which exhibited SOAEs, there was no difference between the evoked OAEs of smokers and nonsmokers. We conclude that smoking had not yet measurably affected the ears of those with acute hearing (i.e., those who exhibit SOAEs). However, in ears without SOAEs, smokers exhibited smaller evoked OAE amplitudes than nonsmokers, even though their audiometric thresholds were within the norm.
Acoustic Impedance Tests ; Adult ; Audiometry ; Audiometry, Pure-Tone ; Cochlea ; Dataset ; Ear ; Hearing ; Humans ; Otoacoustic Emissions, Spontaneous ; Signal-To-Noise Ratio ; Smoke* ; Smoking* ; Tobacco Products ; Young Adult*

OBJECTIVES: The aim of this study was to investigate hearing and outer cells function in patients with psoriatic arthritis. Our investigation was a prospective case control study. METHODS: A total of 31 psoriatic arthritis patients (62 ears) and 31 healthy control subjects (62 ears) were enrolled in the study. We investigated hearing changes of patients and controls via pure tone audiometry, speech discrimination scores, tympanometry, acoustic reflex, and transient product otoacoustic emission. RESULTS: The mean age of psoriatic arthritis patients was 36.1+/-8.5 years (range, 14 to 62 years). The average age of the control group was 37.9+/-8.1 years (range, 16 to 62 years). There were statistically significant differences between pure tone audiometry in all frequencies and right and left emission at the 4.0 and 1.0 in psoriatic arthritis patients versus controls (P<0.05). This difference was evident, especially at high frequencies. There was no statistically significant difference between the ages and genders of the patient and control groups (P>0.05). Both audiological and otoacoustic emissions were not significantly different between right and left ear (P>0.05). CONCLUSION: Based on the audiological and otoacoustic findings; it is likely that the cochlear outer hair cells become subtly damaged in psoriatic arthritis patients, consequently leading to changes in hearing thresholds. These data suggest that it is important to screen psoriatic arthritis patients for hearing changes with otoacoustic emissions and audiologic tests regularly.
Acoustic Impedance Tests ; Arthritis, Psoriatic* ; Audiometry ; Audiometry, Pure-Tone ; Audiometry, Speech ; Case-Control Studies ; Cochlea* ; Discrimination (Psychology) ; Ear ; Hair* ; Hearing* ; Humans ; Otoacoustic Emissions, Spontaneous ; Prospective Studies ; Reflex, Acoustic

To explore the audiological features in children who were sever sensorineural hearing loss infected with rubella virus. There were two cases of rubella virus infection in children who were deaf, they conducted the distortion product otoacoustic emission, ABR and auditory steady-state evoked response (ASSR) examination, then analyzed the results comprehensively. Two patients' mothers were prompted to have infected rubella virus during the early three months pregnant period by history and laboratory tests. The two patients were not detected deafness gene mutation. Audiology results implied the two patients were very severe binaural sensorineural deafness, so they were recommended to equipped with hearing aids and cochlear implant surgery. Early pregnancy women infected with rubella virus can cause very severe offspring sensorineural deafness. The crowd whose mother were suspected to infect with rubella virus in early pregnancy, that should be tracked and detected hearing in order to achieve early detection, early intervention and early treatment.
Child ; Cochlear Implantation ; Cochlear Implants ; Evoked Potentials, Auditory ; Female ; Hearing Aids ; Hearing Loss, Sensorineural ; etiology ; virology ; Humans ; Otoacoustic Emissions, Spontaneous ; Pregnancy ; Rubella ; complications ; Rubella virus ; pathogenicity

OBJECTIVE: To explore the risk factors of the newborns who failed initial hearing screening by analysing the distortion production otoacoustic emission (DPOAE) results of 1021 newborns with potential risk factors of hearing loss. METHOD: All newborns, who were born in obstetrical department and admitted in the neonatal department of the Nanfang Hospital during June 2009 to January 2012 and underwent initial hearing screening, were included in this study. Their clinical data and DPOAE results were analyzed retrospectively in order to identify the risk factors for failure of initial hearing screening in infants; cases who failed the DPOAE test were followed up by telephone interviews. RESULT: (1) One hundred and thirty-seven cases (13.42%) of the 1021 newborns did not pass the hearing screening. 51 cases (5.00%) did not pass the test in both ears. Meanwhile, left ear in 47 cases (4.60%) and right ear in another 39 cases (3.82%) failed the test respectively. (2) Univariate analysis showed that 14 factors had significant influence on the hearing screening results, such as birth weight, small for gestational age, multiple pregnancy, gestational age, delivery mode, oligohydramnion, oxytocin, blood sugar level of newborn, Apgar scores at 1 min, exposed prenatally to glucocorticoid, maxillofacial deformity, hypoxic-ischemic encephalopathy, neonatal respiratory distress syndrome and neonatal asphyxia (P < 0.01). (3) Multivariate Logistic regression analysis suggested that birthweight less than 1500 g, multiple pregnancy, Apgar scores of 0-4 at 1 min, exposed prenatally to glucocorticoid and maxillofacial deformity were risk factors for failure of initial hearing screening (OR were 3.132, 1.808, 2.615, 1.827 and 12.174 respectively; 95% CI were 1.466-6.691, 1.120-2.917, 1.317-5.336, 1.130-2.953 and 1.986-74.632 respectively). (4) Results of telephone interviews revealed that Apgar scores of 0-4 at 1 min would be a risk factor of language development. CONCLUSION Birthweight less than 1500 g, multiple pregnancy, Apgar scores of 0-4 at 1 min, exposed prenatally to glucocorticoid and maxillofacial deformity are risk factors of failure of initial hearing screening among newborns with potential hearing loss. Monitoring of the hearing condition of the infants at risk should be strengthened.
Deafness ; diagnosis ; Female ; Hearing Disorders ; diagnosis ; Hearing Tests ; Humans ; Infant, Newborn ; Neonatal Screening ; Otoacoustic Emissions, Spontaneous ; Pregnancy ; Risk Factors

OBJECTIVE: To investigate the relationship between passing rate of otoacoustic emission (OAE) and pure tone thresholds. METHOD: OAE including both transient evoked otoacoustic emission (TEOAE) and distortion products otoacoustic emission (DPOAE) as well as pure tone thresholds were measured in subjects with normal hearing and sensory hearing loss. The passing rates of OAE were calculated in subjects with different age groups and different pure tone thresholds. RESULT: TEOAE can identify hearing loss at frequencies of 2 kHz and below. For TEOAE and 2 kHz DPOAE, passing rate has no difference between different age groups. For 4 kHz DPOAE, the passing rate in age over 50 years group is lower than other groups. The passing rate decreased as the pure tone threshold increased. When TEOAE and DPOAE suggest " pass", 70%, 81%, 79% subjects had pure tone threshold lower than 25 dB HL and 94%, 96%, 93% lower than 40 dB HL respectively. When they suggest "refer", 93%, 84%, 87% subjects had pure tone threshold higher than 25 dB HL and 71%, 58%, 68% higher than 40 dB HL respectively. CONCLUSION TEOAE is sensitive to hearing loss at middle and low frequencies but not at high frequencies. Measuring both TEOAE and DPOAE might provide more information of hearing loss at 0.5, 1.0, 2.0 and 4.0 kHz. It is worth noting that even OAE suggest "pass" or "refer", because there is still exist the possibility of hearing loss.
Audiometry, Pure-Tone ; Auditory Threshold ; Hearing Loss, Sensorineural ; diagnosis ; Humans ; Otoacoustic Emissions, Spontaneous

OBJECTIVES: To evaluate the frequency of risk factors and their influence on the evoked otoacoustic emission (OAE) of infants. METHODS: All newborns between November 2009 and June 2012 in Haydarpasa Numune Education and Research Hospital were tested on distortion evoked OAE screening test. Total of 2,284 infants were examined. Sex, maternal infectious disease, birth type (vaginal birth or caesarean sectio), birth weight, familial hearing loss, intermarriage of parents, hyperbilirubinemia, intensive care were analyzed as risk factors. RESULTS: Total of 2,284 neonates were screened (1,220 males and 1,064 females) for the presence of OAE in both ears. Vaginal delivery, maternal infections during pregnancy, intermarriage of parents relative, low birth weight(<1,500 g) are related risk factors to failure of screening with OAE in our study. There was no statistically significant difference in sex ratios, birth weight, familial hearing loss, hyperbilirubinemia, and intensive care stay. CONCLUSION: Risk factors are only as useful as their predictive power. Not enough is known about which risk factors are relevant, which babies have the risk factors, or which babies will fail to attend follow-up, the effectiveness of targeted hearing loss testing is questionable at this point in time. A system needs to be developed to clarify which risk factors are discoverable, predictive and useful.
Birth Weight ; Communicable Diseases ; Ear ; Education ; Follow-Up Studies ; Hearing Loss* ; Humans ; Hyperbilirubinemia ; Infant* ; Infant, Newborn ; Male ; Marriage ; Mass Screening ; Otoacoustic Emissions, Spontaneous ; Parents ; Parturition ; Pregnancy ; Risk Factors* ; Sex Ratio ; Critical Care