OBJECTIVETo study the relationship between the degrees of peripheral auditory dysfunction and clinical dementia rating (CDR) in the patients with mild cognitive impairment (MCI) and Alzheimer's disease (AD).

METHODSPure-tone thresholds (PT), word recognition scores (WRS), acoustic immittance and auditory brain-stem responses (ABR) were done to evaluate the auditory function in 24 cases of the patients with MCI and in 31 cases of the patients with AD and in 50 subjects of the control group. Clinical dementia rating (CDR) questionnaire was used to define the dementia degree of the subjects.

RESULTSTwenty-four MCI patients and 31 AD patients were selected, with average age of 72.0 +/- 6. 5 and 73.1+/-7. 5 of whom 70.8% and 67.7% were female separately. There was no significant difference in PTT and WRS between the MCI and AD groups (P > 0.05). In order to ascertain the relationship between hearing level and degree of dementia, all subjects were divided into 4 groups according their hearing loss (PTA <25 dB:0, 25-30 dB:1, 31-35 dB:2, >35 dB:3) to compare their CDR scores (the control:0, MCI:0. 5, mild AD:1). The more the CDR scores have, the more hearing impairment after controlling the confounder factors (Kendalls tau b = - 0.285, P = 0.018). No significant difference was found between the two groups in audiometry reliability, acoustic immittance and ABR (P > 0.05).

CONCLUSIONThe positive relationship was founded the peripheral hearing impairment and the score of CDR questionnaire in less than 0.5 score of CDR groups and mild AD patients.

Aged ; Alzheimer Disease ; physiopathology ; Case-Control Studies ; Cognition Disorders ; physiopathology ; Female ; Hearing Loss ; Humans ; Male

The prevalence of auditory neuropathy is not known, although the majority of cases are felt to lie within the population of neonatal intensive care unit graduates. We report three cases of auditory neuropathy, out of 211 children with sensorineural hearing loss, seen at our audiology clinic from April 1, 1999 to December 31, 2003. Two patients did not have a risk factor for hearing impairment. Screening policies based solely on transient evoked otoacoustic emissions testing will not detect auditory neuropathy effectively, and may falsely reassure parents and professionals unaware of this condition.
Auditory Pathways ; physiopathology ; Child ; Child, Preschool ; Cochlear Nerve ; physiopathology ; Hearing Disorders ; diagnosis ; Hearing Loss, Sensorineural ; diagnosis ; etiology ; Hearing Tests ; Humans ; Male ; Otoacoustic Emissions, Spontaneous ; Prevalence ; Risk Factors

Cerebral Palsy ; diagnosis ; etiology ; physiopathology ; Hearing Disorders ; etiology ; Humans ; Interleukin-6 ; analysis ; Prognosis ; Research Report

OBJECTIVE: To explore the clinical and audiological characteristics of children with congenital malformations of the external ear. METHOD: One hundred and ten patients with congenital malformations of the external ear ranged from 2002 to 2006 were involved in this study. The mean age was one year and four months. The children were divided into three groups according to the appearance of the external ear. The first group consisted of 94 patients with auricle malformation and atresia of the external auditory meatus. The second group consisted of 8 patients with abnormal external auditory meatus (no atresia) and auricle malformation. The other 8 patients only with auricle malformation were included in the third group. All children underwent ABR tests, while some of these children accepted DPOAE and Acoustic-immittance measurements. RESULT: (1) Severe abnormal results of ABR were observed in 79 abnormal ears (78.22%) in children of first group, while moderate or severe abnormal results of ABR were observed only in 8 opposite side ears (normal ears). (2) Sixty-two and a half percent (5 ears) of ears of the second group (8 ears) had severely abnormal ABR results. (3) Severe abnormal ABR results occurred in 44.44 percent (4 ears) of ears of the third group (9 ears). Moderate or severe abnormal results of ABR were not observed in the opposite side ears (normal ears) in second and third groups. CONCLUSION Congenital malformations of the external ear is an important factor affecting children's hearing. Auditory nerve impairment can be observed in children with congenital malformations of the external ear.
Adolescent ; Child ; Child, Preschool ; Ear, External ; abnormalities ; physiopathology ; Evoked Potentials, Auditory, Brain Stem ; Female ; Hearing Disorders ; congenital ; Hearing Tests ; Humans ; Infant ; Infant, Newborn ; Male

OBJECTIVE: To explore characteristics and causes of equilibrium function deficits after traumatic brain injury(TBI). METHODS: Ninety-five patients after TBI in traffic accidents were tested using computerized dynamic posturography (CDP). The CDP findings of patients were compared with normal value. The patients were grouped based on TBI and audition disorders degrees. The results were compared within the patients groups. RESULTS: The equilibrium scores of the TBI group were significantly lower than the normal value. The utilization rate decreased for vision and vestibular sensation information in the TBI group, especially for vestibular sensation. The primary TBI degree and audition decrease had no significant affect on posture stability. CONCLUSION The poor posture stability was observed in TBI patients without limbs disturbance. The response and adaptation abilities decrease in these patients. This could be explained by the damage to peripheral vestibular and central balance structures.
Accidents, Traffic ; Adult ; Auditory Threshold ; Brain Injuries/complications* ; Dizziness/etiology* ; Female ; Hearing Disorders/physiopathology* ; Humans ; Middle Aged ; Postural Balance ; Posture ; Sensation Disorders/physiopathology* ; Severity of Illness Index ; Vestibular Diseases/physiopathology* ; Vestibular Function Tests ; Young Adult

OBJECTIVETo investigate the clinical and genetic characteristics of three Chinese Meniere's disease (MD) families and decipher the mechanism of MD further.

METHODSPersonal and family medical evidence of hearing loss, vestibular symptoms, and other clinical abnormalities of the participants were identified, clinical and genetic features were analyzed. Targeted 307 genes capture and high-throughput sequencing were performed on the two ascertained members of family 1007184.

RESULTSEight patients from these three families showed post-lingual sensorineural hearing loss, six women and two men were involved. Age of onset in these affected members concentrated in the middle age, with the average age of 39.3 years old. Among them, patients from 1407278 were accompanied by migraine. All of the three probands presented as recurrent vertigo firstly, and then fluctuated hearing loss showed up, accompanying by tinnitus and ear fullness feeling. The hearing loss manifested as late-onset, low frequency-involved pattern, with subsequent gradual progression from moderate to severe level. Some of the patients progressed to severe level involving all frequencies at higher ages. In addition, most of the cases showed revitalization. Four cases received vestibular function tests, three of which had varying dysfunction of vestibular function, while the other one had normal vestibular function. Patients who had abnormal vestibular function showed much more severe hearing impairment. The three-generation family 1007193 had an autosomal recessive genetic characteristics, family 1007184 showed autosomal dominant inheritance of characteristics, family 1407278 were either autosomal dominant or X-linked dominant pattern. Through target genes capture high-throughput sequencing technology, we identified two candidate variants in the two members of family 1007184, named c. 2057G>A in EGFLAM and c. 1961C>T in ITGA8.

CONCLUSIONMeniere's disease has some genetic and familial aggregation in Chinese population, but its complex genetic pathogenic mechanisms need further study.

Adult ; Deafness ; Family Health ; Female ; Hearing Loss, Sensorineural ; etiology ; physiopathology ; Humans ; Inheritance Patterns ; Male ; Meniere Disease ; complications ; genetics ; physiopathology ; Middle Aged ; Migraine Disorders ; etiology ; Tinnitus ; etiology ; Vestibular Function Tests ; Vestibule, Labyrinth ; physiopathology

OBJECTIVETo study the correlation between brainstem auditory evoked potential (BAEP) and serum neuron-specific enolase (NSE) in neonates with asphyxia and explore the role of NSE in the evaluation of hearing impairment following asphyxia.

METHODSFifty-two term neonates with asphyxia, including 38 cases of simple asphyxia (mild: 23 cases; severe: 15 cases) and 14 cases of asphyxia complicated by hypoxic-ischemic encephalopathy (HIE), were enrolled. In the double-blind trial, BAEP and NSE were simultaneously detected 7 days after birth. The patients who did not pass BAEP test received another BAEP and NSE examinations 3 months after birth. Thirty healthy term neonates served as normal control group.

RESULTSOf the 52 neonates with asphyxia, 50.0% and 21.2% of patients failed the initial and the second BAEP tests, respectively. The detection rates of BAEP anomalies in the simple severe asphyxia group in the initial and the second tests (63.3% and 26.3%, respectively) were significantly higher than those in the simple mild asphyxia group (36.9% and 5.9%, respectively)(P<0.05). The neonates with asphyxia complicated by HIE showed a higher detection rate of BAEP anomalies in the second test compared with the asphyxiated neonates without HIE (31.3% vs 16.7%; P<0.05). Mean serum NSE levels in asphyxiated neonates were significantly higher than those in normal controls (<0.01). There were significant differences in serum NSE levels between the neonates with mild and severe asphyxia (26.70+/-2.34 microg/L vs 17.18+/-3.16 microg/L; P<0.01). The asphyxiated neonates with HIE had serum NSE levels similar to the simple severely asphyxiated neonates. Serum NSE levels in patients who failed the initial BAEP test were significantly higher than those who passed the test (25.69+/-4.12 microg/L vs 17.15+/-3.09 microg/L; <0.01). Serum NSE levels had a positive correlation with wave V reaction threshold detected in the BAFP test (<0.05).

CONCLUSIONSThe serum level of NSE is closely correlated with BAEP, and it may be useful to the evaluation of the hearing impairment and the outcome in neonates with asphyxia.

Asphyxia Neonatorum ; blood ; complications ; physiopathology ; Double-Blind Method ; Evoked Potentials, Auditory, Brain Stem ; Hearing Disorders ; etiology ; Humans ; Hypoxia-Ischemia, Brain ; etiology ; Infant ; Infant, Newborn ; Phosphopyruvate Hydratase ; blood

OBJECTIVE: To analyze normal pure tone hearing thresholds patients with aural fullness using evoked otoacoustic emission in order to detect the early cochlear impairment. METHOD: Forty-three normal pure tone hearing thresholds patients (72 ears, aural fullness group) with aural fullness were served as subjects and 30 normal volunteers (60 ears, control group) as controls. Transiently evoked otoacoustic emission (TEQAF) and distortion product evoked otoacoustic emission (DPOAE) were tested with Capella otoacoustic emission machine. The DPOAE detection rate and amplitudes at all frequencies,the passing rate and wave signal noise ratio (SNR) ,wave reproducibility, band SNR and band reproducibility of TEOAE were recorded and analyzed. RESULT: (1) Only on the frequency points of 0.50 kHz and 0.75 kHz, the detection rate of DPOAE in aural fullness group was lower than that in control group (P < 0.05). There was no significant difference in the rate among other frequency points (P > 0.05). (2) The passing rate of TEOAE was 100w in control group and 90. in aural fullness group. There were statistical differences between two groups (chi2 = 6.16, P < 0.05). (3) Compared with the control group, the DPOAE amplitudes at all frequencies, the wave signal noise ratio (SNR), wave reproducibility, band SNR and band reproducibility of TEOAE in patients with aural fullness were significantly decreased. There were statistical differences (P < 0.05 or P < 0.01). CONCLUSION Some patients of normal hearing thresholds with aural fullness have had early harm of Outer hair cell in cochlear. TEOAE and DPOAE may he used to detect these lesions early before the hearing impairment occurred.
Adolescent ; Adult ; Audiometry, Pure-Tone ; Auditory Threshold ; Case-Control Studies ; Female ; Hearing Disorders ; physiopathology ; Humans ; Male ; Middle Aged ; Otoacoustic Emissions, Spontaneous ; Young Adult

OBJECTIVE: To analysis the characteristics of audiology and clinical genetics of a Chinese family with the DFNA5 genetic hearing loss in detail. METHOD: A detailed family history and clinical data were collected. The Chinese pedigree is an autosomal-dominant inherited hearing loss. The data of audiological examination about genetic characteristics was analysed. The relationship between the hearing-impaired of this family and age was contrasted. RESULT: This Chinese family spanned five generations and comprised 42 members. The mode of inheritance of the families should be autosomal dominant according to the pedigree. Pure-tone audiograms showed a so-called Z shape curve. The hearing loss is sensorineural, progressive and beginning at the high frequencies. The audiograms were fairly symmetric. Whole frequencies became involved with increasing age. CONCLUSION The Chinese family with the DFNA5 mutation was an autosomal dominant pedigree. In this family, non-syndromic symmetric hearing impairment was severest at the high frequencies early, and gradually accumulated all frequencies of hearing. A mutation in DFNA5 leads to a type of hearing loss that closely resembles the frequently observed age-related hearing impairment. It should take into account DFNA5 mutation which the audiogram of a genetic hearing impaired has the same feature.
Adolescent ; Adult ; Asian Continental Ancestry Group ; genetics ; Audiology ; Child ; Chromosome Disorders ; genetics ; Female ; Hearing Loss ; genetics ; physiopathology ; Hearing Tests ; Humans ; Male ; Middle Aged ; Pedigree ; Receptors, Estrogen ; genetics ; Young Adult

OBJECTIVE: To analyze the difference of subjective hearing threshold and objective hearing threshold, and to discuss the importance of standard for hearing evaluation in forensic medicine. METHODS: Three hundred and fifty-five cases (387 ears) of forensic medical identification with hearing impairment were retrospectively analyzed including the items entrusted and hearing test results. All cases were collected from 2004 to 2012 in the forensic science center. RESULTS: In the 387 ears, 218 ears (56.3%) were evaluated the degree of disability and 106 ears (27.4%) were identified the degree of damage. In the disability degree evaluation, the subjective hearing threshold and the objective hearing threshold were significant different in 120 ears (55.0%), while in damage degree evaluation, the subjective hearing threshold and the objective hearing threshold were significant different in 69 ears (65.1%). CONCLUSION Because of camouflaging or exaggerating the hearing impairment by the wounded, the subjective hearing threshold can't accurately assess the existence and the degree of hearing impairment. In the forensic identification, auditory brainstem response, 40 Hz auditory event related potential and auditory steady-state response should be combined in the application to evaluate the hearing impairment for the wounded in order to ensure the reliability of the evaluation of hearing impairment.
Acoustic Stimulation/methods* ; Adolescent ; Adult ; Aged ; Audiometry, Evoked Response/methods* ; Audiometry, Pure-Tone ; Auditory Threshold ; Child ; Child, Preschool ; Disability Evaluation ; Evoked Potentials, Auditory ; Evoked Potentials, Auditory, Brain Stem/physiology* ; Expert Testimony/methods* ; Female ; Forensic Medicine/methods* ; Hearing Disorders/physiopathology* ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Severity of Illness Index ; Young Adult