Clinical Study on 136 Children with Sudden Sensorineural Hearing Loss.

BACKGROUND: The 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. METHODS: One 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. RESULTS: Among 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. CONCLUSIONS: CSSNHL 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.

Development of hair cells in inner ear is associated with expression and promoter methylation of Notch-1 in postnatal mice.

The present study was designed to investigate the correlation among the number of hair cells in inner ear, Notch-1 gene expression levels and its methylation status of the promoter region in the postnatal mice. The hair cells in inner ear were collected from postnatal mice at day 0, 4, 8 and 16 and counted by immunofluorescence. Notch-1 mRNA expression were measured by real-time quantitative polymerize chain reaction (PCR). Methylation levels of CpG islands in Notch-1 promoters were analyzed by matrix-assisted laser desorption/ionization-time of flight mass spectrometry. The results showed that the number of hair cells in the inner ear increased gradually after birth, which were positively correlated to Notch-1 mRNA expression. However, analysis on methylation of CpG sites in Notch-1 promoter showed that the methylation rates increased gradually after births, which were correlated with the decreased expression of Notch-1. Drug lesion induced the loss of hair cells, and stimulated the expression of Notch-1 mRNA expression, but didn't influence the methylation rates of Notch-1 promoter. We concluded that the Notch-1 mRNA expression level in inner ear tissues is correlated with the development of hair cells. CpG islands in Notch-1 promoter region manifest hypermethylation status when hair cells in inner ear are mature.

[Study of clinical characteristics and curative effects of sudden hearing loss patients with vertigo].

OBJECTIVE: To analyze the clinical characteristics, prognosis and therapeutic effects of sudden sensorineural hearing loss (SSHL) patients associated with vertigo, and to investigate the strategy of diagnosis and treatment. METHODS: We retrospectively analyzed the clinical characteristics of 240 patients diagnosed as SSHL with vertigo, who were treated in the Chinese PLA General Hospital from July 2008 to August 2012. Various factors affecting the therapeutic effects were analyzed, such as audiological features, vestibular function tests, genders, audiograms, lasting before seeing a doctor, courses of vertigo and vascular factors. RESULT: Among the contemporaneous SSHL patients (873 cases), the cases with vertigo accounted for 27.49% (240/873). Among the 240 patients with vertigo, the cases with different hearing impaired degree of mild, moderate, severe and profound were 30, 13, 28 and 34, respectively, primarily by the profound cases. Detailed vestibular function tests were performed in 97 patients, with 54 cases having unilateral vestibular disfunction and 43 patients having normal vestibular function, among which 23 cases were diagnosed as benign paroxymal positional vertigo (BBPV). The relationship between vestibular function and different hearing impaired degrees or various audiogram types had no statistically significant difference. 219 cases had detailed records of the onset time of cochlear and vestibular symptoms, including 122 patients with cochlear symptoms and dizziness occurring simultaneously. After standardized drug treatment, the total effective rate was 46.67%, with recovery in 17 cases, excellent in 34 cases, better in 61 cases and poor in 128 cases, respectively. Statistical analysis showed that different genders, audiogram types, vertigo courses of time, the results of vestibular function and neck vascular ultrasounds were not related to the curative effects, while, the treatment time after onset was significantly associated with treatment effects. CONCLUSIONS: SSHL with vertigo has a high incidence, primarily single side affected, with relatively severe hearing impairment, and total deafness and downslope hearing curve mainly. Vestibular function can be normal or low in SSHL patients with vertigo, with a higher incidence of BPPV. Vestibular and cochlear symptoms occur simultaneously in more than half of the patients. The detection rate of vestibular dysfunction gradually increased, as the degree of hearing loss increased, without statistical significance although. The therapeutic effects of sudden hearing loss with vertigo cases have no relationship with dizziness duration or vestibular function, while the disease course plays an important role in treatment.

[The study of clinical characteristics of sudden sensorineural hearing loss patients with tinnitus].

OBJECTIVE: To analysis the characteristics of sudden sensorineural hearing loss (SSHL) patients with tinnitus, and explore the relationship of characteristics of tinnitus and audiology. METHOD: Patients diagnosed as SSHL with tinnitus were studied in the research. All patients' clinical features were analyzed, such as tinnitus frequency, pure tone audiometry, tinnitus, hearing loss degree, results of residual inhibition test. RESULT: Thirty cases were identified as mild degree hearing loss, 13 cases as moderate degree, 28 cases as severe degree and 34 cases as profound degree. And hearing impaired frequency of 13 cases was ascertained at low-frequency, 39 cases at middle-high-frequency, and 53 cases at full-range-frequency. The incidence of patients with low-frequency was about 41. 9% (44/105), and it was about 21. 9% (23/105) in those with middle-frequency. And it was 36. 2% (38/105) in cases of high-frequency tinnitus. The chi-square test show statistically significant differences between patients with the low-frequency, middle-frequency and high-frequency of the hearing loss (P<0. 05). In tinnitus residual inhibition test, positive rate of convergence type masking curve was about 72.0%, tinnitus separated type masking curve 20.0%, overlapping type was 57.9%, and the spacing type was 43.5%. There was a statistically significant difference among cases with different type masking curve (P<0. 05)with the spacing residual inhibition test positive rate. CONCLUSION: There are individual differences of clinical characteristics among SSHL patients with tinnitus. Tinnitus frequency is consistent with the frequency of hearing loss. Patients had the more serious the degree of hearing loss, who had more serious tinnitus grading. Cases with the converged type curve will be fit for tinnitus masking. Therefore, combining the tinnitus detection with the audiological tests, we could obtain the clinical characteristics of SSHL patients with tinnitus.

[Prognostic factors of sudden sensorineural hearing loss in children].

OBJECTIVE: The aim of this retrospective study was to analyze the recovery rate of sudden sensorineural hearing loss in children, and explore the prognostic factors in order to guide the clinical diagnosis and treatment. METHOD: A retrospective review was conducted for the prognosis of children with sudden sensorineural hearing loss during the past 5 years (from November 2010 to May 2015) in Chinese PLA General Hospital. This paper have a complete clinical data of 101 patients (113 ears)with sudden hearing loss, ranging from 0 to 18 years old Patients were divided into four groups according to hearing recovery and eight putative prognostic factors were analyzed. RESULT: Among 101 patients (113 ears), the ratio of male and female was 60:53. Treatment was initiated from 1 to 183 days after disease onset, with an average of (18.5 ± 22.1) d. Bilateral and unilateral hearing loss were 24 ears and 89 ears, respectively. The proportion of mild hearing loss, moderate hearing loss, severe hearing loss and profound hearing loss were 7.1%, 6.2%, 23.9% and 62.8%, respectively. Vertigo and tinnitus occurred in 54.9% and 77.9% of the patients, respectively. After the treatment, the complete recovery rate was 9.7% and the overall recovery rate was 36.3%. The degree of hearing loss, earlier treatment onset, sex and bilateral involvement were significantly associated with hearing recovery (P < 0.05). CONCLUSION: Sudden sensorineural hearing loss in children was generally identified as severe and profound hearing loss, but after positive and timely treatment, it can be improved or even cured. The mild hearing loss, earlier treatment onset, unilateral hearing loss and female were positive prognostic factors. The concurrence of tinnitus or vertigo, the results of ABR and DPOAE had no significant influence on prognosis.

[Epidemiological studies on mtDNA 12S rRNA A1555G mutation of 10 non syndromic hearing loss families in Yunnan province].

OBJECTIVE: To investigate the meaning of the mutation screening, prevalence, inheritance and the intervention or the prevention for the specific drugs in 10 families with non-syndrome hearing loss in Yunnan Province, China. METHOD: To do a questionnaire about the cases of ten families with non-syndrome hearing loss and to draw a detailed matriarchal family tree detailed. Following that, the A1555G mutation-positive individuals were detected and confirmed using DNA extracting, PCR amplification and sequencing for family volunteer. RESULT: There are 96 members have attended the blood collection in these ten families. Thirty-six of them had the normal hearing and 60 of them had the sensory neural hearing loss. However, 4 out of those had no A1555G point mutation, and 92 had A1555G point mutation (95.8%). While 7 of those were Heterogeneity, the rest were all homogeneous mutation. There were also 73 patients who had amino glycoside antibiotic medication history. However all the rest cases had a history of amino glycoside antibiotic medication were not clear yet. CONCLUSION: The proportion of patients with drug-induced deafness is high in Yunnan province and the mutation rate of mitochondrial DNA A1555G is also high. It is worthy to do DNA 12SrRNA A1555G mutation screening for drug intervention and prevention.

[Research on mitochondrial DNA T1095C gene variation in military noise-induced hearing loss].

OBJECTIVE: To study the clinical and sequence character of the entire mitochondrial genome in five subjects with mitochondrial 12SrRNA T1095C mutation, and to analyze its relationship with the military noise-induced hearing loss (NIHL). METHOD: Three hundreds and four soldiers exposed to military noise were selected in Yunan and Beijing, including susceptible (experimental) and tolerance (control) groups. Mitochondrial 12SrRNA T1095C mutation were found in 5 subjects. Then the complete nucleotide sequence of five subjects were sequenced and its clinical character were analyzed. RESULT: m12SrRNA T1095C mutation were identified in 5 subjects of experimental group,and none were found in control group. There was significant difference between them (P < 0.05). All five soldiers had the history of military noise exposure and showed sensorineural deafness of different degrees. Sequence analysis of the complete mitochondrial genomes showed the distinct sets of mtDNA polymorphism besides T1095C mutation in five subjects. CONCLUSION: The T1095C mutation in hearing loss subjects with various genetic background and history of military noise exposure, is involved in the pathogenesis of hearing impairment. It indicates that the T1095C mutation do relate well with military noise induced-hearing loss.

[The expressions of TP, MK and CD105 in laryngeal squamous cell carcinoma and their clinical significance].

OBJECTIVE: To explore the expressions of thymidine phosphorylase (TP), midkine (MK) and MVD marked with CD105 antibody in laryngeal squamous cell carcinoma (LSCC) and their clinical significance. METHOD: The expressions of TP, MK and CD105 in LSCC tissues of 43 cases were studied by immunohistochemical staining. RESULT: The positive expression rates of TP and MK in LSCC were 67.4% and 60.5% respectively, the mean value of MVD was 6.01 +/- 1.78. MVD was significantly higher in tumor tissue with both positive TP and MK than in that with both negative TP and MK (7.07 +/- 3.26 vs. 4.03 +/- 1.90, P < 0.05). The expression of TP, MK and CD105 were all correlated with T-stage and lymph node metastasis. Positive TP, MK expression and high MVD were all associated with a poor survival, and positive expression of both TP and MK in tumors conferred a poorer prognosis than negative expression of those factors in tumors, but only the lymph node metastasis and MVD were independent prognostic factors on multivariate analysis. CONCLUSION: Both TP and MK are important for angiogenesis in LSCC. TP, MK and angiogenesis are all closely correlated with the progress of LSCC and the lymph node metastasis. The lymph node metastasis and MVD marked with CD105 antibody were independent prognostic factors. TP and MK may affect the progression and prognosis of tumor by promotion of angiogenesis. A combinative detection of TP, MK and CD105 can be as valuable tumor marker and prognostic factor for LSCC.

[A study on the correlation between distortion product otoacoustic emissions and transient evoked otoacoustic emissions].

OBJECTIVE: To study the characteristic and correlation of distortion product otoacoustic emissions and transient evoked otoacoustic emissions. METHOD: Contrast Band SNR and Band response amplitude of TEOAE with 2f1-f2 amplitude and SNR of DPOAE were investigated in states before or after noise exposure with or without contralateral suppression(CS) in 20 (40 ears) normal otologic youth volunteer subjects. RESULT: Although evoked by different acoustic stimulus and showed various characteristics of patterns respectively, DPOAE and TEOAE had a good correlation between their measuring values at most adjacent frequencies thus formed certain value relation. CONCLUSION: TEOAE measurement is save time and superior in middle frequency range while DPOAE measurement is better frequency specific and superior in the higher frequency. Between their amplitude and SNR there were good correlations so significant linear regression parameters can be obtained. According to these mentioned above they could be selected and used, moreover, make up a deficiency each other in scientific research and clinical practice.

[A study on the effects of contralateral acoustic suppression to transient evoked otoacoustic emissions after noise exposure].

OBJECTIVE: To investigate the effects of contralateral acoustic suppression(CAS) on transient evoked otoacoustic emissions(TEOAE) in Noise Exposure Condition, in order to explore the effects of efferent positive feedback regulation on the cochlear mechanisms and the effects of CAS on the sensitivity of TEOAE measure for finding changes of cochlear function condition induced by noise exposure. METHOD: Observing the effects of CAS (60 dB SPL WN) on the changes of TEOAE parameters, Response Amplitude, Reproducibility, Band SNR and Band Reproducibility, before and after noise exposure. RESULT: The values of every index of TEOAE were decreased respectively so that the effects of noise exposure on cochlear function can be reflected sensitively. A small decrease of values can be induced by CAS before noise exposure, of some decrease of indices were significant. After noise exposure, the changes of TEOAE parameters become much more with CAS than without. CONCLUSION: Noise exposure can effect obviously cochlear function condition and decrease OAE measurement values. CAS had suppressing action on cochlear activity by a negative feedback and can increase obviously the sensitivity of TEOAE measurement in finding the changes induced by noise exposure.