Hearing Loss, Central ; physiopathology ; Humans ; Mitochondria ; pathology

Hearing Loss, Noise-Induced ; physiopathology ; Humans

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

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

Hearing loss caused by cochlear damage is the main symptom of sudden deafness (SD). Some patients also suffer from vestibular symptoms. In recent years, more attention has been paid to the vestibular dysfunction in patients with SD. The lesions could involve the whole inner ear in SD patients with and without vertigo. Comprehensive evaluation of vestibular function may help us understand the extent of lesions in sudden deafness and analyze the pathogenesis of disease. A less involvement of inner ear lesion may indicate a better hearing recovery.
Cochlea ; injuries ; Hearing Loss, Sudden ; diagnosis ; pathology ; Hearing Tests ; Humans ; Vertigo ; Vestibule, Labyrinth ; physiopathology

OBJECTIVETo 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.

METHODSWe 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.

RESULTAmong 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.

CONCLUSIONSSSHL 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.

Benign Paroxysmal Positional Vertigo ; complications ; Cochlea ; physiopathology ; Deafness ; Dizziness ; Hearing Loss, Sensorineural ; Hearing Loss, Sudden ; complications ; Hearing Tests ; Humans ; Prognosis ; Retrospective Studies ; Vestibular Function Tests ; Vestibule, Labyrinth ; physiopathology

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

BACKGROUNDHearing impairment has been reported to be common in patients with mitochondrial disorders, a group of diseases characterized by pleiomorphic clinical manifestations due to defects in oxidative phosphorylation of mitochondria. This study aimed to investigate the audiological characteristics in a large cohort of patients with mitochondrial disease.

METHODSComprehensive audiological evaluations, including pure tone audiometry, tympanometry, speech audiometry, otoacoustic emissions, electrocochleography and auditory brainstem evoked potentials, were performed in 73 Chinese patients with mitochondrial encephalomyopathy and with confirmed mitochondrial DNA (mtDNA) defects.

RESULTSAmong the patients, 71% had hearing impairment. However, the incidence rate and severity of hearing impairment were much less in the chronic progressive external ophthalmoplegia (CPEO) subtype than in the mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), myoclonic epilepsy with ragged red fibers (MERRF) and Kearns-Sayre syndrome (KSS) subtypes. While most of our patients had a predominantly cochlea origin for the hearing deficit, five patients had an auditory neuropathy spectrum disorder and three patients had impairment of both cochlea and auditory cortex.

CONCLUSIONSVarious portions of the auditory system could be involved in patients with mitochondrial diseases, including cochlea, auditory nerve, auditory pathway and cortex. Hearing loss was more associated with multisystem involvement. Genotype, mutant load of mtDNA and other unknown factors could contribute to heterogeneity of hearing impairment in mitochondrial disease.

Adolescent ; Adult ; Aged ; Child ; Female ; Hearing Loss ; physiopathology ; Hearing Loss, Central ; physiopathology ; Humans ; Male ; Middle Aged ; Mitochondrial Encephalomyopathies ; physiopathology ; Young Adult

OBJECTIVE: Amplitude modulation of auditory cortical responses was evaluated with functional MRI (fMRI) in subjects of unilateral sensorineural hearing loss (USNHL) and those of normal hearing (NH). METHOD: Twenty-one subjects with USNHL and 11 with normal hearing were examined with fMRI in response to amplitude modulation tones of 500 Hz with the modulation frequency at 8 Hz. An event related design was combined with a sparse clustered volume acquisitioning paradigm in data collection in order to reduce the influence of acoustic scanner noise. SPM2 software was used for offline data analyzing. RESULT: Significant activation, including volume and intensity, were found in the temporal lobe of control subjects, and significant differences in the volume and intensity were noted between the contralateral and ipsilateral activated auditory cortexes in them, exhibiting clearly contralateral predominance. When the normal ear with unilateral sensorineural hearing loss received signals, while significant activations in bilateral auditory cortexes, greater activation in the contralateral auditory cortexes was found in the normal ear. CONCLUSION The difference in the lateralization between the two groups suggests the plasticity of auditory cortex with unilateral sensorineural hearing loss.
Adult ; Aged ; Auditory Cortex ; physiology ; physiopathology ; Case-Control Studies ; Female ; Hearing Loss, Sensorineural ; physiopathology ; Hearing Loss, Unilateral ; physiopathology ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Young Adult