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 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

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

OBJECTIVETo undergo tinnitus evaluation test and masking therapy of subject tinnitus associated with sensorineural hearing loss as a reference for diagnosis and guiding masking therapy.

METHODSThe 66 patients with subject tinnitus were diagnosed as sensorineural hearing loss. Sixty-six patients divide into three groups according to the results of pure tone audiometry, including steep drop type in 28 patients, slow drop type in 20 patients, and flat type in 18 patients. All the patients underwent tinnitus evaluation tests (pitch matching, intensity matching, tinnitus masking curves, residual inhibition) and masking therapy.

RESULTSTinnitus with steep drop type manifest was as low intensity [average intensity (5.7 +/- 2.9) dB (x(-) +/- s)] and high frequency (median with 4750 Hz). Residual inhibition was almost positive, but was usually consistent with convergence and congruence tinnitus masking curves. Masking therapy had better effect in the treatment of this type of tinnitus (effective rate 89.3%). Tinnitus with slow drop type manifest was as low intensity [average intensity: (6.2 +/- 4.8) dB] and high frequency (median: 4050 Hz). The distribution of residual inhibition and tinnitus masking curves had no obviously characteristics. The effective rate of masking therapy of this type of tinnitus (55.0%) was higher than tinnitus with flat type but low than that of tinnitus with steep drop type. The intensity of tinnitus with flat type [average intensity: (9.2 +/- 5.0) dB] was higher than that of the previous groups. The distribution of frequency of this type had no obviously characteristics. The residual inhibition was almost negative, and was usually consistent with divergence and persistence tinnitus masking curves. Masking therapy had unsatisfactory curative effect in the treatment of this type of tinnitus (effective rate 11.1%). The effective rate of masking therapy was significant differences among the three groups (chi(2) = 9.127, P < 0.05).

CONCLUSIONSFor the tinnitus patients with steep drop type audiometric curve, they are recommended masking therapy. For the tinnitus patients with slow drop type audiometric curve, masking therapy may be attempted to perform. For the tinnitus patients with flat type audiometric curve, they do not recommend the masking therapy.

Adolescent ; Adult ; Aged ; Audiometry, Pure-Tone ; Female ; Hearing Loss, Sensorineural ; diagnosis ; physiopathology ; therapy ; Humans ; Male ; Middle Aged ; Perceptual Masking ; Tinnitus ; diagnosis ; physiopathology ; therapy ; Young Adult

OBJECTIVE: To analyze the constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss(SNHL). METHOD: Imaging and auditory data of 125 cases (225 ears) in 860 pediatric patients with congenital SNHL who referred to ENT department of Shanghai Children Medical Center from February 2005 to January 2010 were retrospectively studied. RESULT: Congenital malformations of inner ear accounted for 14.5% in 860 pediatric patients with congenital SNHL. Bilateral ear was involved in 98 cases (78.4%), unilateral in 27 cases (21.6%). One hundred and sixty-seven ears (74.2%) were identified as profound deafness, 36 ears (16%) severe deafness and 22 ears (9.8%) moderate deafness in 225 ears, respectively. In present group, large vestibular aqueduct (75.6%) was the most common and next was vestibular deformity (32%), then cochleovestibular deformity (23.1%). Mondini deformity (55.8%) was the most common and common cavity deformity (28.9%) next in cochleovestibular deformity. Profound deafness in cochlea involved congenital malformations of inner ear was significantly more than those in no cochlea involved malformations. CONCLUSION The results are of importance for understanding the constitute of congenital malformations of inner ear in pediatric patients with congenital SNHL in China, for etiological diagnosis of congenital SNHL, for intervention including hearing aids or cochlear implant and prognosis.
Child ; Child, Preschool ; Ear, Inner ; abnormalities ; Female ; Hearing Loss, Sensorineural ; diagnosis ; pathology ; physiopathology ; Hearing Tests ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Retrospective Studies ; Tomography, X-Ray Computed

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

OBJECTIVETo assess the prognostic factors associated with the sudden idiopathic sensorineural hearing loss, to predict the prognosis of patient with idiopathic sensorineural hearing loss precisely.

METHODSEight hundreds and eighty two patients with idiopathic sudden sensorineural hearing loss were retrospectively reviewed during January 2006 to March 2007. Patients whose initial hearing threshold < or =40 dB were excluded. The patients with initial hearing threshold >40 dB were recruited, which was divided into six subgroups based on the patterns of audiogram: downgrade audiogram subgroup, upgrade audiogram subgroup, flat audiogram subgroup, concave audiogram subgroup, profound audiogram subgroup and total deafness subgroup.

RESULTSRegarding to the relationship between the time point for initial intervention and the prognosis, better prognosis was obtained in patients whose initial intervention was within 3 days of the disease, good prognosis was achieved within 1 or 2 weeks of the disease, poor prognosis was noted beyond 2 weeks of this disease. Furthermore, comparison with the initial intervention within 3 week, 1 month and 1 month later, the prognosis among them was not statistical different. 97.7% hearing recovery was achieved in the concave subgroup with the initial hearing threshold >40 dB group. Comparison with the other subgroup (except total deafness subgroup), the cure rate and recovery rate was 23.8% and 57.9% respectively in the profound subgroup. Poor prognosis was demonstrated in the total deafness subgroup and inefficacy rate was 67.4% in the total deafness subgroup. Comparison with patients without companying complications, the prognosis of patients with companying complications such as diabetes or high blood pressure has negative impact in hearing recovery. The age was correlated with the prognosis, elder had poor prognosis, patients more than 50 years old present with worse hearing than that less than 50 years old (H = 7.851, P = 0.0051).

CONCLUSIONSThe initial intervention beyond 2 weeks had negative impact on the prognosis. The initial audiogram patterns and hearing threshold were both significant factors on the prognosis of idiopathic sudden sensorineural hearing loss. In addition, old patient had poor prognosis. The companying complications such as high blood pressure and diabetes had negative impact on the prognosis of idiopathic sudden hearing sensorineural loss.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Audiometry ; Auditory Threshold ; Child ; Female ; Hearing Loss, Sensorineural ; complications ; diagnosis ; physiopathology ; Hearing Loss, Sudden ; complications ; diagnosis ; physiopathology ; Humans ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Risk Factors ; Young Adult

OBJECTIVEWe aimed to determine whether blood lipid parameters were related to the severity and the prognosis of idiopathic sudden sensorineural hearing loss (ISSNHL) patients.

METHODSA retrospective cohort study of 258 patients with ISSNHL from December 2013 to February 2015. The distribution characteristics of lipids [total cholesterol (TC), triglyceride (TG), high density lipoprotein cholesterol (HDL-C), low density lipoprotein cholesterol (LDL-C), Non-high-density lipoprotein cholesterol (Non-HDL-C), et al] in different degree of deafness (mild, moderate, severe, and profound), hearing curve types (low frequency, high frequency, full range frequency, and completely deafness type) and prognosis of recovery (complete, partial, slight, and no recovery) were analyzed by IBM SPSS 22.0 ANOVA analysis, chi square test and multiple regression analysis.

RESULTSTG level in mild hearing loss group was significantly lower than that in severe and profound hearing loss group (P = 0.017 and P = 0.007). There were no correlation between curve types and lipid indexes (P > 0.05). Non-HDL-C level was elevated in no recovery and slight recovery groups (P = 0.026 and 0.021). TC levels in partial recovery group and no recovery group were significantly higher than that in the complete recovery group (P = 0.049 and 0.042), TG was higher in slight recovery group (P = 0.014).

CONCLUSIONSTG has significant correlation with the severity of hearing loss. There are negative correlations between hearing recovery and Non-HDL-C, TC and TG levels. Non-HDL-C, TC and TG might be a prognostic factor for treatment outcome in ISSNHL patients.

Cholesterol, HDL ; blood ; Cholesterol, LDL ; blood ; Deafness ; Hearing Loss, Sensorineural ; blood ; diagnosis ; physiopathology ; Hearing Loss, Sudden ; blood ; diagnosis ; physiopathology ; Humans ; Lipids ; blood ; Prognosis ; Retrospective Studies ; Treatment Outcome ; Triglycerides ; blood

Adolescent ; Adult ; Audiometry, Pure-Tone ; methods ; Auditory Cortex ; physiopathology ; Case-Control Studies ; Female ; Hearing Loss, Sensorineural ; diagnosis ; physiopathology ; Humans ; Magnetoencephalography ; Male ; Young Adult

OBJECTIVE: To explore the technique that distinguish pseudo-anacousia. METHODS: 60 cases were divided into three groups (normal group, deafness sensorineura group and pretending hearing loss group). They all had been tested by distortion products otoacoustic emissions technique. RESULTS: (1) The DPOAE incidence of normal group is 94.00%. the DPOAE incidence of deafness sensorineura group is 22.00%. There were significant differences at statistics in DPOAE incidence (P < 0.01). those proved that patients (deafness sensorineura group) were in hearing problem. (2) The DPOAE incidence of pretending hearing loss group is 93.00%, there were no significant differences at statistics in DPOAE incidence between normal group and pretending hearing loss group (P > 0.01), furthermore when behavior threshold is over 50 dBHL, DPOAE incidence is no score. CONCLUSION This study shows the technique can distinguish pretending hearing loss. The application of DPOAE can be used as a new tool in cases assessment of clinic forensic medicine.
Audiometry, Pure-Tone ; Auditory Threshold ; Deafness/physiopathology* ; Diagnosis, Differential ; Evoked Potentials, Auditory, Brain Stem ; Forensic Medicine ; Hearing Loss, Sensorineural/physiopathology* ; Humans ; Otoacoustic Emissions, Spontaneous