BACKGROUNDMeniere's disease is a unique, progressive disease of the inner ear. The complex manifestation presents diagnostic challenges. The cochlear symptoms often present before vertigo and tend to be ignored. This study aimed to analyze the characteristics of cochlear symptoms and functions associated with Meniere's disease to investigate the regularity of the development of this disorder.

METHODSOne-hundred fifteen patients who were diagnosed with definite unilateral Meniere's disease at the Hearing and Vestibular Clinic of the Department of Otorhinolaryngology of Beijing Tongren Hospital from August 2013 to November 2015 were recruited in this retrospective study. Initial symptoms, duration from initial symptoms to the diagnosis, hearing thresholds, audiogram patterns, and caloric test results were collected and analyzed for each patient. Data were analyzed using SPSS 13.0 statistical software by Spearman's correlation, Kruskal-Wallis H test, Chi-square test, and Fisher's exact test.

RESULTSThe average hearing threshold of these patients was 45.24 ± 18.40 dB HL. A majority of the patients (55.65%) were in Stage 3. The initial presentation of the disorder in 58 cases (50.43%) comprised only cochlear symptoms without vertigo. A weak, positive correlation was found between the degree of hearing loss and duration of the disease from initial symptoms to the diagnosis (rs = 0.288, P = 0.002). Upward-sloping, inverted "V," downward-sloping, and flat pattern were the main audiometric patterns observed with a distinctive distribution between stages (P < 0.001). Based on the configurations of audiograms, the audiometric patterns had a weak correlation to the duration (rs = 0.269, P = 0.004), and there was a tendency of duration to rising from upward-sloping, inverted "V", downward-sloping to flat pattern. (H = 10.024, P = 0.018). Frequencies of tinnitus in 56 patients (64.4%) were at the lowest points of the audiograms, i.e., the frequencies of the poorest hearing threshold. The patients at an advanced stage (Stage 3 [56] and Stage 4 [73]) exhibited a significantly higher abnormality of canal paresis than those at the earlier stages (Stage 1 [23] and Stage 2 [42]) (χ2 = 5.973, P = 0.015).

CONCLUSIONSPatients with definite Meniere's disease always have a moderate to severe sensorineural hearing loss before diagnosis. Cochlear symptoms are the most common initial presentation. With the progression of the duration, the hearing impairment becomes more severe and the distribution of the audiometric pattern is distinctive between stages.

Adolescent ; Adult ; Aged ; Caloric Tests ; Cochlea ; physiopathology ; Female ; Hearing Loss ; diagnosis ; physiopathology ; Humans ; Male ; Meniere Disease ; diagnosis ; physiopathology ; Middle Aged ; Retrospective Studies ; Tinnitus ; diagnosis ; physiopathology ; Vertigo ; diagnosis ; physiopathology ; Young Adult

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

BACKGROUNDMitochondrial diseases are a group of energy metabolic disorders with multisystem involvements. Variable clinical features present a major challenge in pediatric diagnoses. We summarized the clinical spectrum of m.3243A>G mutation in Chinese pediatric patients, to define the common clinical manifestations and study the correlation between heteroplasmic degree of the mutation and clinical severity of the disease.

METHODSClinical data of one-hundred pediatric patients with symptomatic mitochondrial disease harboring m.3243A>G mutation from 2007 to 2013 were retrospectively reviewed. Detection of m.3243A>G mutation ratio was performed by polymerase chain reaction (PCR)-restriction fragment length polymorphism. Correlation between m.3243A>G mutation ratio and age was evaluated. The differences in clinical symptom frequency of patients with low, middle, and high levels of mutation ratio were analyzed by Chi-square test.

RESULTSSixty-six patients (66%) had suffered a delayed diagnosis for an average of 2 years. The most frequent symptoms were seizures (76%), short stature (73%), elevated plasma lactate (70%), abnormal magnetic resonance imaging/computed tomography (MRI/CT) changes (68%), vomiting (55%), decreased vision (52%), headache (50%), and muscle weakness (48%). The mutation ratio was correlated negatively with onset age (r = -0.470, P < 0.001). Myopathy was more frequent in patients with a high level of mutation ratio. However, patients with a low or middle level of m.3243A>G mutation ratio were more likely to suffer hearing loss, decreased vision, and gastrointestinal disturbance than patients with a high level of mutation ratio.

CONCLUSIONSOur study showed that half of Chinese pediatric patients with m.3243A>G mutation presented seizures, short stature, abnormal MRI/CT changes, elevated plasma lactate, vomiting, and headache. Pediatric patients with these recurrent symptoms should be considered for screening m.3243A>G mutation. Clinical manifestations and laboratory abnormalities should be carefully monitored in patients with this point mutation.

Adolescent ; Age of Onset ; Asian Continental Ancestry Group ; Chi-Square Distribution ; Child ; Child, Preschool ; DNA, Mitochondrial ; genetics ; Female ; Hearing Loss ; pathology ; physiopathology ; Humans ; Infant ; Lactic Acid ; blood ; Magnetic Resonance Imaging ; Male ; Mitochondrial Diseases ; blood ; genetics ; pathology ; physiopathology ; Mutation ; Point Mutation ; genetics ; Polymerase Chain Reaction ; Retrospective Studies ; Seizures ; pathology ; physiopathology

OBJECTIVE: To analyze the abnormal conditions of the affected ear and the contralateral ear of patients with unilateral Meniere's disease and the prevalence of bilateral abnormalities among these unilateral Meniere disease population, providing reference for the clinical treatment strategies for Meniére disease. METHOD: A retrospective analysis of 106 Meni6re disease cases was performed, the abnormal incidence of the affected ears, the contralateral ears and the bilateral abnormalities were calculated, and the disease characteristics were analyzed. RESULT: The bilateral ears abnormal incidence of unilateral Meniére disease was 35. 85% (38/106); the cochlear symptoms of the contralateral ears often occurred 2. 25 years later of the symptoms of Meni6re disease; contralateral cochlear symptoms included at least two symptoms of tinnitus, deafness and ear fullness; 39. 47%(15/38) patients with bilateral abnormalities would appear binaural hearing impairment. CONCLUSION This study showed that about one-third of unilateral Meniére diseases have binaural symptoms, among which about one-third would occur bilateral hearing loss. Therefore, it is necessary to consider the course of disease and the symptoms of the contralateral ear before taking damage or destructive method for treating Meniére's disease clinically.
Cochlea ; physiopathology ; Deafness ; Ear ; abnormalities ; Hearing Loss, Bilateral ; epidemiology ; Humans ; Incidence ; Meniere Disease ; epidemiology ; Prevalence ; Retrospective Studies ; Tinnitus

OBJECTIVE: To evaluate the characteristics and prognosis of sudden sensorineural hearing loss through vestibular evoked myogenic potentials. METHOD: Fifty patients with unilateral sudden hearing loss underwent an ear test battery, including audiometry, cervical vestibular evoked myogenic potential (cVEMP) and ocular vestibular evoked myogenic potential(oVEMP). The cVEMP and oVEMP in patients with sudden sensorineural hearing loss were investigated. Their associations with initial hearing threshold, the type of audiogram, the results of coloric test and hearing recovery were also accessed. RESULT: After one-month treatment, the average threshold declined significantly in affected ears, with normal VEMP rates improved significantly (P < 0.05). Initial hearing threshold and hearing recovery were significantly associated with the results of coloric test and the results of VEMP test (P < 0.05). CONCLUSION Our study indicated that vestibular evoked myogenic potential examination was not only a useful additional diagnostic tool in the neurotological evaluation of patients suffering sudden hearing loss, but also very valuable in the prognosis of sudden sensorineural hearing loss.
Hearing Loss, Sensorineural ; diagnosis ; Hearing Loss, Sudden ; diagnosis ; Hearing Loss, Unilateral ; diagnosis ; Hearing Tests ; Humans ; Otolithic Membrane ; physiopathology ; Prognosis ; Vestibular Evoked Myogenic Potentials

BACKGROUNDTo establish the prevalence of hearing deficit in children with Down syndrome (DS) in Hong Kong as measured by brainstem auditory evoked potentials (BAEP). The secondary objective is to examine the agreement between BAEP and clinical questioning in detecting hearing deficit in DS.

METHODSConsecutive DS patients attending the Down's Clinic in a regional pediatric referral center were recruited into this cross-sectional study. BAEP data performed within 12 months were retrieved. The care-taker was interviewed with a structured questionnaire to detect any symptom of hearing impairment. BAEP findings and clinical questionings were compared in an agreement analysis using quadratic weighted kappa statistics.

RESULTSFifty DS patients (35 male, 15 female, mean age 11.70 years ± 5.74 standard deviation) were recruited. Eighteen patients (36.0%) were identified having hearing deficit by BAEP. Among patients with hearing impairment, 13 patients (72.2%) had a conductive deficit, and most have mild to moderate hearing loss. Five patients (27.8%) had sensorineural deficit and most have moderate to severe degree. Eight (44.4%) had bilateral hearing deficit. Care-takers of 13 patients (26.0%) reported symptoms of hearing impairment, with 9 (69.2%) having mild symptoms, 3 (23.1%) had moderate symptoms and 1 (7.7%) had severe symptoms. The weighted kappa was 0.045 (95.0% confidence interval - 0.138-0.229), indicating very poor strength of agreement between BAEP and clinical questioning. For patients with conductive hearing impairment, only 1 patients (7.7%) recalled history of otitis media.

CONCLUSIONSThe estimated point prevalence of hearing impairment in Chinese DS children in Hong Kong is 36%. Our finding of poor strength of agreement between objective testing and symptom questioning reflects significant underestimation of hearing impairment by history taking alone. In view of the high prevalence and low parental awareness, continuous surveillance of hearing is mandatory for DS patients throughout childhood and adolescence.

Adolescent ; Child ; Cross-Sectional Studies ; Down Syndrome ; epidemiology ; physiopathology ; Evoked Potentials, Auditory ; physiology ; Female ; Hearing Loss ; epidemiology ; etiology ; Humans ; Male ; Prevalence

Female ; Hearing Loss, Central ; physiopathology ; Humans ; Male ; Mitochondrial Encephalomyopathies ; physiopathology

The eosinophilic otitis media(EOM) is an intractable disease characterized by the presence of a highly viscous yellow effusion with extensive accumulation of eosinophils in the middle ear; granulation tissue can been discovered in the middle ear cavity; most of patients have association with bronchial asthma; resist to conventional treatment for otitis media; EOM patients show gradual deterioration of hearing and sometimes become deaf suddenly; effective treatment involves use of topical and oral steroids. This article summarizes the progress of the EOM's diagnosis and treatment.
Asthma ; complications ; Ear, Middle ; physiopathology ; Eosinophilia ; pathology ; Eosinophils ; Hearing Loss ; complications ; Humans ; Otitis Media ; pathology

OBJECTIVE: This study was aimed at investigating the ability of lexical tone perception in listeners with sensorineural hearing impairment and auditory. neuropathy spectrum disorder. METHOD: Three groups of subjects were recruited in this study, including 11 subjects with normal hearing, 14 subjects with sensorineural hearing impairment, and 25 subjects with auditory neuropathy spectrum disorder. Ten monosyllabic syllables were selected, and combined with four lexical tones which were made up of 40 tone tokens as the original test materials. Then, these original words were recorded using one adult male and one adult female native Beijing Mandarin speaker. The speakers were asked to record these 40 monosyllabic words multiple times, and the 80 tokens in which the durations of four tones in each monosyllabic word were within 5ms precision were chosen as the test tone tokens. The subjects were asked to perform a four-alternative forced-choice study and select which tone they had heard. RESULT: The mean and standard deviation of the tone perception correct scores for normal-hearing subjects, subjects with sensorineural hearing impairment, and subjects with auditory neuropathy spectrum disorder were (97.3 ± 2.8)%, (88.0 ± 9.9)%, and (65.7 ± 17.1)%, respectively. Significant differences in tone perception scores were found to be between subjects with normal hearing and subjects with sensorineural hearing impairment (P < 0.01) and between subjects with auditory neuropathy spectrum disorder (P < 0.01). In addition, a significant difference was found to be between subjects with sensorineural hearing impairment and auditory neuropathy spectrum disorder (P < 0.05). A significantly negative correlation was observed between tone perception score and pure tone hearing thresholds for both subjects with sensorineural hearing loss (r = -0.756, P < 0.01) and subjects with auditory neuropathy spectrum disorder (r = -0.546, P < 0.01). CONCLUSION As the hearing loss became more severe, the ability to perceive lexical tone for both subjects with sensorineural hearing loss and auditory neuropathy spectrum disorder reduced. Subjects with auditory neuropathy spectrum disorder had more degraded ability to perceive lexical tone and a larger individual difference, in comparison with subjects with sensorineural hearing loss.
Adult ; Audiometry, Pure-Tone ; Case-Control Studies ; Female ; Hearing ; Hearing Loss, Central ; physiopathology ; Hearing Loss, Sensorineural ; physiopathology ; Humans ; Language ; Male ; Speech Perception

OBJECTIVE: To investigate the result of vestibular evoked myogenic potentials (VEMP) of primary benign paroxysmal positional vertigo(BPPV)and to identify the characteristics in VEMP examination of the primary BPPV and to observe the relevance of patients with primary BPPV and abnormal VEMP with hearing loss. METHOD: Patients with primary BPPV were tested with pure tone audiometry, videonystagmograph and VEMPs test. We analyzed the difference in the two groups with normal hearing and hearing loss, discussed the etiology and pathogenesis. RESULT: Primary BPPV comprised 23.0% with hearing lost, 77.0% hearing normal. The results of oVEMP were abnormal in 79. 7% (59/74) of the cases; and the results of cVEMP were abnormal in 66. 2% (49/74) of the cases; oVEMP and cVEMP differences to the diagnosis of primary BPPV (P<0. 05); oVEMP and cVEMP differences to the diagnosis primary BPPV with hearing lost (P<0. 05). CONCLUSION oVEMP detection positive rate of primary BPPV is higher than cVEMP,which may be due to otolithic particles falling from the utricle; positive rate of cVEMP in primary BPPV with hearing loss is higher than that of oVEMP, which may related to the cochlear and sacculus occured in the same embryonic tissue structure.
Audiometry, Pure-Tone ; Benign Paroxysmal Positional Vertigo ; physiopathology ; Cochlea ; Hearing Loss ; etiology ; Hearing Tests ; Humans ; Otolithic Membrane ; Saccule and Utricle ; Vestibular Evoked Myogenic Potentials