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

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

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 explore the clinical characteristics and prognosis of patients with idiopathic sudden sensorineural hearing loss (ISSHL) with vertigo. METHOD: By analyzing the clinical data of 271 ISSHL patients, they were divided into without vertigo group (n = 169) and vertigo group (n = 102). In vertigo group, 34 cases were patients with benign paroxysmal positional vertigo (BPPV) secondary to the ISSHL. All patients received conventional treatment. According to the types of BPPV, patients with secondary BPPV received Epley maneuver or Barbecue roll maneuver. By analyzing the results of the pure tone audiometry test and treatment outcomes of the patients, we summarized the clinical characteristics of ISSHL patients with vertigo. RESULT: The audiometric curves of ISSHL with vertigo group were mainly at high frequency. The degrees of hearing loss of these patients were severe and profound. After treatment, the improvement of hearing threshold for ISSHL with vertigo group was lower than that for ISSHL without vertigo group. What's more, the rate of recovery, success and total effective of audition for ISSHL with vertigo group was also obviously lower than that for ISSHL without vertigo group. Of all the patients with BPPV, 27 cases of posterior semicircular canal and 7 cases of lateral semicircular canal were identified. All patients with BPPV were diagnosed as the same ears as the ISSHL. CONCLUSION ISSHL with vertigo group lost hearing more severely than ISSHL without vertigo group. Also, the improvement of hearing and the effective after treatment were really poor. The symptoms of ISSHL with BPPV group improved and eased significantly than that of ISSHL without BPPV group. The major of BPPV secondary to the ISSHL occurs in the posterior semicircular canal. The canalith repositioning is an effective therapy to the secondary BPPV.
Audiometry, Pure-Tone ; Auditory Perception ; Benign Paroxysmal Positional Vertigo ; complications ; therapy ; Hearing Loss, Sensorineural ; complications ; therapy ; Hearing Loss, Sudden ; complications ; therapy ; Humans ; Patient Positioning ; Prognosis ; Semicircular Canals ; physiopathology ; Treatment Outcome

OBJECTIVE: To investigate the characteristics of air-conducted ocular vestibular-evoked myogenic potential(oVEMP) in patients with sudden sensorineural hearing loss (SSHL). So as to understanding the range and the degree of the vestibular system damage in patients with SSHL. METHOD: Thirty-five cases of unilateral SSHL were enrolled as the observing group from December in 2013 to December in 2014. 500 Hz air-conducted tone burst was employed as stimulation. Fifity-five healthy young subjects were recruited as the control group. The results were compared between the affected ears, the contralateral ears and the normal controls. RESULT: Thirty-five patients with SSHL, 31 of them in both ears lead to oVEMP Waveform, 4 sick ears did not elicit oVEMP waveform, extraction rate of 88%. 95 dBHL air-conducted get the latencies of P1 and N1, P1-N1 interval, peak-to-peak P1-N1 amplitude in healthy ear were (11.92 ± 0.85)ms; (17.07 ± 1.04)ms, (5.15 ± 0.69)ms, (5.44 ± 2.53) µv. 95 dBHL air-conducted get the latencies of P1 and N1, P1-N1 interval, peak-to-peak P1-N1 amplitude in sick ear were (12.16 ± 0.76) ms; (16.94 ± 2.57)ms, (5.16 ± 0.73)ms, (2.89 ± 1.66) v. The 55 cases (110 ears) were elicited P1-N1 complex typical, extraction rate was 100%. The threshold of oVEMP examination was (82.23 ± 2.92) dBHL, 95 dBHL air-conducted get the latencies of P1 and N1, P1-N1 interval, peak-to-peak P1-N1 amplitude in these healthy young people were (11.53 ± 0.80)ms, (16.61 ± 0.87) ms, (5.18 ± 1.04) ms, (5.96 ± 2.59) µv, there were no significant differences between the affected ears, the contralateral ears and the normal controls in the latencies P1 and N1, P1-N1 interval. The wave amplitude in SSHL were lower than the healthy ear and the healthy control group. CONCLUSION Through the detection of oVEMP in patients with SSHL,we observed that P1-N1 wave amplitude decreased, and it may be related to the damage of the utricle and vestibular nerve.
Case-Control Studies ; Hearing Loss, Sensorineural ; physiopathology ; Humans ; Saccule and Utricle ; pathology ; Vestibular Evoked Myogenic Potentials ; Vestibular Nerve ; pathology ; Vestibule, Labyrinth ; physiopathology

The purpose of this article is to report on the first known Korean case of Susac syndrome. An 18-year-old female came to our clinic reporting blurred vision of the left eye for 2 days. She also complained of decreased hearing with tinnitus of the right ear and mild headache. She was previously healthy and had no remarkable medical history. Best-corrected visual acuity was 20 / 50 in the left eye and 20 / 20 in the right eye. An axiomatic triad of ocular, cochlear, and neurologic involvement was observed in the patient. Fluorescein angiography showed branched retinal arterial occlusions in the left eye. A sudden right sensorineural hearing loss was observed on audimetry. Magnetic resonance images showed a hyperintense lesion in the white matter around the corpus callosum. The patient was treated with high doses of systemic corticosteroids, and no neuropsychological sequelae were observed. This is the first case report of Susac syndrome in Korea. In cases of retinal arterial occlusion with hearing loss or neuropsychological symptoms, Susac syndrome should be suspected.
Adolescent ; Diagnosis, Differential ; Female ; Fluorescein Angiography ; Fundus Oculi ; Hearing ; Hearing Loss, Sensorineural/*diagnosis/physiopathology ; Humans ; Magnetic Resonance Imaging ; Retinal Artery Occlusion/*diagnosis/physiopathology ; Susac Syndrome/*diagnosis/physiopathology ; Visual Acuity

OBJECTIVE: To study the vestibular function of patients with acute low-tone sensorineural hearing loss (ALHL). So we can know whether their vestibular function was influence. METHOD: Vestibular function tests were performed on fifty-two ALHL patients by recording electronystagmography (ENG) and static posturography (SPG) before and after their therapy. Then made a comparison with normal. RESULT: Length of locus and velocity of postural sway of the ALHL subjects were significantly increased in comparison with normal (P < 0.05) when eyes closed. After therapy, two (3.8%) ALHL subjects were still recorded abnormal ENG results. CONCLUSION Most ALHL subjects without vertigo symptom, but their vestibular function was abnormal, so vestibular function tests should be performed on patients with ALHL.
Adolescent ; Adult ; Case-Control Studies ; Female ; Hearing Loss, Sensorineural ; physiopathology ; Humans ; Male ; Middle Aged ; Prognosis ; Vestibular Function Tests ; Young Adult

The cochlear dead regions are common exist in the case of moderate to profound sensorineural hearing loss. Patients with dead regions have demonstrated poor understanding of speech in noise and report less satisfaction with hearing aids than patients with no dead regions. Dead regions are assumed to occur if a hearing-impaired listener can be shown to be using off-place listening. A correctly diagnosed dead regions facilitates accurate and informed aural rehabilitation.
Cochlea ; physiopathology ; Hearing Loss, Sensorineural ; diagnosis ; rehabilitation ; Humans

OBJECTIVE: To investigate the law of auditory remodeling by studying the Cortical Auditory Evoked Potential in a group of post-lingually deaf adults in the early stage of the cochlear implant use. METHOD: Ten post-lingually deaf adults implanted with PULSAR ci 100 of Medel device ranged from 19 to 52 years old with duration of deafness from 3 months to 23 years.were involved. Their P1-N1-P2 responses to /ba/ at around 60-70 dB SPi and open-set speech recognition scores of mono-syllabic words were recorded at 1. 3 and 6 months after the switch-on of cochlear implants. RESULT: Six out of 10 typical waveforms of Pl-N1-P2 were obtained. The other I were with atypical waveforms. The group with atypical waveform was characterized by long duration and early onset of deafness. There was no significant difference regarding either the amplitude or the latency of each peak at the 3 data collection time points. There was significant difference among the 3 data collection time points about the speech recognition scores with the highest score at the 6th month of switch on. There was no significant correlation between the peak of the CAEP and speech recognition score. CONCLUSION In the first 6 months of cochlear implant use in the post-lingually deaf adults, the duration and the onset age of deafness played important role in respect of the presence and waveform morphology of the CAEPs. It needs at least 6 months for the central auditory system to make use of the audio input from the cochlear implant.
Adult ; Cochlear Implantation ; Cochlear Implants ; Evoked Potentials, Auditory ; Female ; Hearing Loss, Sensorineural ; physiopathology ; rehabilitation ; Humans ; Middle Aged ; Treatment Outcome ; Young Adult

Although the basic principles for the function of peripheral auditory system have been known for many years, the molecular mechanisms which affect deafness are not clear. In recent years, the study of hereditary deafness associated mouse models has revealed the molecular basis which is related with the formation and function of the hair bundle and the mechanosensory organelle of hair cell. This review focused on the role of protein network, which is formed by the proteins encoded by the Usher syndrome type 1 genes, in hair-bundle development and mechanotransducer channel gating. And the review also showed how the stereocilia rootlets contribute to the hair bundle's mechanical properties and how the hair bundle produces suppressive masking. Finally, the review revealed multiple roles of the tectorial membrane and extracellular matrix in the hair bundles stimulating in the cochlea.
Animals ; Cochlea ; physiopathology ; Disease Models, Animal ; Extracellular Matrix ; physiology ; Hair Cells, Auditory ; pathology ; Hearing Loss, Sensorineural ; genetics ; Humans ; Mechanotransduction, Cellular ; Mice ; Usher Syndromes ; genetics