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 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

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 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

OBJECTIVETo analyze clinical pure tone test data in patients with Meniere disease and study the changes in the audiometic curve configuration.

METHODSA retrospective analysis was conducted of the pure tone test data in 75 cases (82 ears) from 1984 to 2008. The audiometic curve configuration was divided into 6 categories to analyze the relationship between the main curve types and the clinical stages. RESULTS The most common audiogram was the peak type (46.34%), followed by the falling type (19.51%) and the mountain type (12.19%), and the dip type was relatively rare (2.43%). No significant differences were found in the rate of peak audiograms between the clinical stages. The common audiogram peak frequency occurred at 2 kHz (47.39%). The top curve of 1-2 kHz had the largest proportion in mountain type audiograms (70%, 7/10 ears).

CONCLUSIONThe peak type and mountain type configuration are one of the audiological characteristics of Meniere disease, especially for 2 kHz peak, which may serve as the diagnostic features of Meniere disease in the vertigo patients on their first visit.

Adolescent ; Adult ; Aged ; Audiometry, Pure-Tone ; methods ; Female ; Hearing Loss, Sensorineural ; diagnosis ; etiology ; Humans ; Male ; Meniere Disease ; complications ; physiopathology ; Middle Aged ; Retrospective Studies ; Young Adult

OBJECTIVE: To analyse the clinical audiological significance in the diagnosis of large vestibular aqueduct syndrome (LVAS) by the auditory brain stem response (ABR) testing. METHOD: Patients with sensorineural hearing loss were examined by temporal bone CT scanning from January, 2008 to September, 2009. The result of CT scanning of 70 cases inner ear malformation were analysed. Patients were divided into two groups, LVAS group including 38 cases (76 ears) and other inner ear malformation group including 32 cases (62 ears). All patient accepted clinical audiology analysis and auditory brainstem response (ABR) test. RESULT: Twenty-four cases (41 ears) of LVAS group were detected with ASNR in 2 3 cm by the ABR testing, the positive rate was 54%, while ASNR was not detected in patients of other inner ear malformations group. There was significant differences (P=0.01) of the ASNR between two groups. CONCLUSION There is high incidence of LVAS on the patients with non-syndromic deafness. ASNR by ABR testing could help diagnosing the LVAS.
Child ; Child, Preschool ; Ear, Inner ; abnormalities ; Evoked Potentials, Auditory, Brain Stem ; Female ; Hearing Loss, Sensorineural ; diagnosis ; physiopathology ; Humans ; Infant ; Infant, Newborn ; Male ; Syndrome ; Vestibular Aqueduct ; abnormalities

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

Adolescent ; Adult ; Brain Hemorrhage, Traumatic ; complications ; etiology ; Craniocerebral Trauma ; complications ; diagnostic imaging ; Hearing Loss, Sensorineural ; diagnosis ; etiology ; physiopathology ; therapy ; Humans ; Male ; Radiography

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

OBJECTIVETo explore the etiology, clinical aspects, diagnosis and therapeutic strategies of acute low-tone sensorineural hearing loss (ALHL).

METHODSThirty patients (31 ears) with ALHL were selected for this study. Detailed history collection, otological examination and systematic audiological evaluations were conducted. The hearing tests included pure tone audiometry, acoustic immittance, auditory brainstem response (ABR) and otoacoustic emissions (OAE). All cases received therapeutic trial of corticosteroid for 15 days with 6 to 14 months' following-up.

RESULTSALHL mainly affected young people. Low-tone tinnitus, a sensation of ear fullness and hearing impairment were the frequent complains. Otological examinations showed normal results. Mild to moderate sensorineural hearing loss at low frequencies and type "A" tympanograms were found in all patients. Acoustic stapedial reflexes were elicited in 26 of 31 affected ears, and 14 of them had positive results on the Metz test. ABR responses were normal in all 20 tested ears. In 14 out of 20 ears, TEOAEs were absent and DPOAE grams at low frequencies (0.5, 0.75 kHz) were abnormal on the first visit. After steroid therapy, 24 ears demonstrated complete recovery, but 4 ears showed partial recovery and 3 ears unchanged. The total improvement rate was 90.3%.

CONCLUSIONSALHL patients are clinically characterized by low-tone tinnitus, aural fullness and hearing loss, which mainly involved unilateral ear. Audiological findings indicate a cochlear impairment, which only invades low frequency region. The basic pathological feature may be endolymphatic hydrops involves immune response. Conflicting data exist on whether ALHL is an independent disorder or a subtype of Meniere's disease. Ideal therapeutic strategy has not been established by now and corticosteroid is probably an effective agent.

Acute Disease ; Adrenal Cortex Hormones ; therapeutic use ; Adult ; Audiometry, Evoked Response ; Endolymphatic Hydrops ; etiology ; Female ; Hearing Loss, Sensorineural ; diagnosis ; drug therapy ; physiopathology ; Humans ; Male ; Meniere Disease ; diagnosis ; drug therapy ; physiopathology ; Middle Aged ; Otoacoustic Emissions, Spontaneous