OBJECTIVE: To analysis the characteristics of sudden sensorineural hearing loss (SSHL) patients with tinnitus, and explore the relationship of characteristics of tinnitus and audiology. METHOD: Patients diagnosed as SSHL with tinnitus were studied in the research. All patients' clinical features were analyzed, such as tinnitus frequency, pure tone audiometry, tinnitus, hearing loss degree, results of residual inhibition test. RESULT: Thirty cases were identified as mild degree hearing loss, 13 cases as moderate degree, 28 cases as severe degree and 34 cases as profound degree. And hearing impaired frequency of 13 cases was ascertained at low-frequency, 39 cases at middle-high-frequency, and 53 cases at full-range-frequency. The incidence of patients with low-frequency was about 41. 9% (44/105), and it was about 21. 9% (23/105) in those with middle-frequency. And it was 36. 2% (38/105) in cases of high-frequency tinnitus. The chi-square test show statistically significant differences between patients with the low-frequency, middle-frequency and high-frequency of the hearing loss (P<0. 05). In tinnitus residual inhibition test, positive rate of convergence type masking curve was about 72.0%, tinnitus separated type masking curve 20.0%, overlapping type was 57.9%, and the spacing type was 43.5%. There was a statistically significant difference among cases with different type masking curve (P<0. 05)with the spacing residual inhibition test positive rate. CONCLUSION There are individual differences of clinical characteristics among SSHL patients with tinnitus. Tinnitus frequency is consistent with the frequency of hearing loss. Patients had the more serious the degree of hearing loss, who had more serious tinnitus grading. Cases with the converged type curve will be fit for tinnitus masking. Therefore, combining the tinnitus detection with the audiological tests, we could obtain the clinical characteristics of SSHL patients with tinnitus.
Audiometry, Pure-Tone ; Deafness ; Hearing Loss ; Hearing Loss, Sensorineural ; complications ; pathology ; Humans ; Tinnitus ; complications ; pathology

Cadherins ; genetics ; Hearing Loss, Sensorineural ; genetics ; pathology ; Humans

BACKGROUND AND OBJECTIVES: Intratympanic steroid serves as a good therapeutic alternative for various inner ear diseases presenting with vertigo, hearing loss, and tinnitus. This study reports the effect of intratympanically injected dexamethasone (ITDEXA) on tinnitus in various cochlear pathologies. SUBJECTS AND METHOD: 19 patients with cochlear tinnitus (7 sudden sensorineural hearing loss (SSNHL), 5 noise induced hearing loss, 4 Meniere's disease and 3 acoustic trauma) were subjected to the IT-DEXA. The injection was performed with a spinal needle through the posterior superior quadrant of the tympanic membrane for 2 to 5 consecutive days. RESULTS: The overall improvement rate was 31.6%. Tinnitus was improved in 4 patients with SSNHL and 2 patients with Meniere's disease. Tinnitus was not improved in noise induced hearing loss or acoustic trauma. CONCLUSION: IT-DEXA is a therapeutic modality for tinnitus in SSNHL and Meniere's disease.
Acoustics ; Dexamethasone* ; Ear, Inner* ; Ear, Middle ; Hearing Loss ; Hearing Loss, Noise-Induced ; Hearing Loss, Sensorineural ; Humans ; Labyrinth Diseases* ; Meniere Disease ; Needles ; Noise ; Pathology ; Tinnitus* ; Tympanic Membrane ; Vertigo

OBJECTIVE: To compare the auditory steady-state response (ASSR) test data of severe sensorineural hearing loss children with cochlear lesion and those with retrocochlear lesion and find some specific phenomena of retrocochlear lesion in ASSR, then to improve diagnostic accuracy in clinical practice. METHOD: Between 2008 to 2012. 96 children (179 ears) were diagnosed with "retrocochlear auditory nerve lesion" and recieved ASSR test (" retrocochlear lesion" group). Eighty-one (143 ears) the same age children (143 ears) were diagnosed as "cochlear auditory nerve lesion" and selected them as the "cochlear lesion" group. Twenty-six (50 ears) normal hearing children at the same age who had ASSR test records selected from the pediatric hearing center database of our hospital were selected as "normal control" group. Compare the difference of ASSR threshold, ASSR elicit rate and ASSR audiogram among the three groups. RESULT: (1) ASSR threshold: Compared each frequency threshold in ASSR test with cochlear.lesion group, the retrocochlear lesion group with wave V and wave I had no significant difference in 500 Hz and 1000 Hz but had significant difference in 2000 Hz and 4000 Hz. As for the retrocochlear lesion group without wave V and wave I, there was a decline in 500 Hz, 1000 Hz, 2000 Hz and 4000 Hz compared with cochlear lesion group. (1) ASSR elicit rate: When compared to retrocochlear lesion group, the cochlear lesion group had a significance lower elicit rate in the four frequency. (3) Number of elicit reactions in ASSR test . In the retrocochlear lesion group, the elicit reactions in all the four frequency in ASSR test was significantly higher than ochlear lesion group. (4) ASSR audiogram type. The proportion of rising curve audiogram in retrocochlear lesion group was 26.83% (with wave I and wave V), 40% (with wave I and without wave V ) and 33.80% (without wave I and wave V). CONCLUTION (1) Children with a severe hearing loss in ABR test and a rise type audiogram in ASSR test should be most possibly considered as retrocochlear lesion. (2) ASSR threshold cannot be used in determinate the severity of hearing loss in children with retrocochlear lesion.
Auditory Threshold ; Child ; Cochlea ; pathology ; Hearing Loss, Sensorineural ; diagnosis ; Hearing Tests ; Humans ; Labyrinth Diseases ; Vestibulocochlear Nerve Diseases ; diagnosis

Hearing loss is one of the most common sensory disorders and has numerous environmental and genetic factors that influence its onset and development. Hearing loss can be classified by either the affected anatomic or functional lesion of hearing loss, or as conductive or sensorineural hearing loss (SNHL). Genetic factors account for about 50% of congenital SNHL, and are therefore the most common cause. Molecular genetics research has identified more than 100 genes related to hearing and hearing loss, and shown that the risk of hearing loss caused by non-genetic factor is modified by genetic susceptibility. About 30% of genetic hearing loss is syndromic related and has affected phenotypic markers in other organs that make it easier to correctly diagnose the etiology of the hearing loss. In some cases, hearing loss can precede the pathologies of other organs and in these cases, hearing loss acts as a predictor of the syndrome associated pathologies of other organs. Inheritance of nonsyndromic hearing loss follows common inheritance patterns such as autosomal dominant, autosomal recessive, sex chromosome related, and mitochondrial inheritances. The paucity of predominant phenotypes and ethnic specificity of the prevalence and types of mutations may hinder the genetic diagnosis in nonsyndromic hearing loss. However, progress in elucidating the causal mutations is going forward using stratified genetic diagnostic strategies of candidate genes identified by hearing phenotypes and patterns of inheritance.
Diagnosis ; Fibrinogen ; Genetic Predisposition to Disease ; Genetics ; Hearing ; Hearing Loss* ; Hearing Loss, Sensorineural ; Inheritance Patterns ; Molecular Biology ; Pathology ; Phenotype ; Prevalence ; Risk Factors ; Sensation Disorders ; Sensitivity and Specificity ; Sex Chromosomes ; Wills

OBJECTIVETo observe the function of the otolithic end organs and their input pathways in sudden sensorineural hearing loss (SSHL) patients.

METHODSForty cases of unilateral SSHL were enrolled as the observing group from May, 2011 to May, 2012. Thirty age- and gender-matched normal subjects were recruited as the control group. Both patients and normal subjects underwent conventional air-conducted ocular vestibular evoked myogenic potential (oVEMP) and cervical vestibular evoked myogenic potential (cVEMP) in bilateral ears. The results were compared between the affected ears, the contralateral ears and the normal controls.

RESULTSOverall, oVEMP was elicited in 16 affected ears (40.0%), 23 contralateral ears (57.5%) and 43 normal ears (71.7%). cVEMP could be elicited in 25 affected ears (62.5%), 30 contralateral ears (75.0%) and 49 normal ears (81.7%) respectively. Significant statistical significance could be found in the oVEMP response rate between the affected ears and the normal ears (χ(2) = 9.949, P = 0.002) and in the cVEMP response rate between the affected ears and the normal ears (χ(2) = 4.582, P = 0.032). Significant statistical difference could not be found in all oVEMP and cVEMP parameters (threshold, N1 latency, P1 latency, latency interval and amplitude) among groups (P > 0.05).

CONCLUSIONSThe otolithic vestibular end organs and their input pathways could be damaged in SSHL patients. Such damages could be monitored objectively by cVEMP and oVEMP examinations.

OBJECTIVES: The aim of this study was to detect the genetic cause of deafness in a large Iranian family. Due to the importance of SLC26A4 in causing hearing loss, information about the gene mutations can be beneficial in molecular detection and management of deaf patients. METHODS: We investigated the genetic etiology in a large consanguineous family with 9 deaf patients from Fars province of Iran with no GJB2 mutations. Initially, linkage analysis was performed by four DFNB4 short tandem repeat markers. The result showed linkage to DFNB4 locus. Following that, DNA sequencing of all 21 exons, their adjacent intronic sequences and the promoter of SLC26A4 was carried out for mutation detection. RESULTS: Two novel mutations (c.863-864insT and c.881-882delAC) were identified in exon 7 of the gene, in both homozygous and compound heterozygous state in patients. CONCLUSION: Our results supported the importance of the SLC26A4 mutations in the etiology of hearing loss among the Iranian patients and therefore its mutation screening should be considered after GJB2 in the molecular diagnostics of hearing loss, especially when enlarged vestibular aqueduct or goiter is detected.
Deafness ; Exons ; Goiter ; Goiter, Nodular ; Hearing Loss ; Hearing Loss, Sensorineural ; Humans ; Introns ; Iran ; Mass Screening ; Microsatellite Repeats ; Pathology, Molecular ; Pedigree* ; Sequence Analysis, DNA ; Vestibular Aqueduct

BACKGROUND AND OBJECTIVES: Injection of endotoxin into the middle ear of experimental animals results in otitis media with effusion and transient reversible hearing loss, which occurs in the higher concentration of endotoxin than the concentration found in human middle ear effusion. The purpose of this study was to investigate the histopathologic changes of the cochlea in guinea pigs with experimental otitis media with effusion induced by E.coli endotoxin. MATERIALS AND METHODS: Concentrations of lipopolysaccharides (LPS) from E.coli, such as 0.1 mg/ml, 1 mg/ml, and 10 mg/ml, were instillated into the middle ear cavity of 30 normal guinea pigs. Histopathologic changes of the cochlea were observed at 1 week after endotoxin instillation, using both light and electron microscope. Six normal guinea pigs were used as a control group whose middle ears were instillated with the physiologically sterile saline. RESULTS: There were deformation in the organ of Corti, partial loss of stereocilia in the hair cells, and vacuolation in stria vascularis at a concentration of 10 mg/ml lipopolysaccharides from E. coli. Mild morphologic abnormality of the cochlea was observed at the concentrations of 1 mg/ml and 0.1 mg/ml. CONCLUSION: A higher concentration of LPS in the middle ear cavity may produce severe inner ear damage, possibly resulting in the sensorineural hearing loss.
Animals ; Cochlea ; Ear, Inner ; Ear, Middle ; Guinea Pigs ; Hair ; Hearing Loss ; Hearing Loss, Sensorineural ; Humans ; Lipopolysaccharides ; Organ of Corti ; Otitis Media with Effusion* ; Otitis Media* ; Otitis* ; Pathology* ; Stereocilia ; Stria Vascularis

Pendred syndrome is an autosomal recessive genetic disorder, which is characterized by sensorineural hearing loss, goiter and a positive perchlorate discharge test. It is caused by mutations of the PDS gene, and its clinical characteristics vary widely. The thyroid function in most cases is normal, or shows only mild hypothyroidism. In Pendred syndrome, there is an organification defect that leads to defective thyroid hormone synthesis, followed by chronic TSH stimulation. Herein is reported a case of a follicular thyroid carcinoma associated with Pendred syndrome. To our knowledge, this is the first case reported in Korea. The patient presented with a huge anterior neck mass, sensorineural hearing loss and a positive perchlorate discharge test. Fine needle aspiration cytology suggested malignancy of the thyroid, and a total thyroidectomy, with central compartment node dissection, was performed. The pathology from the thyroid mass showed a poorly differentiated follicular thyroid carcinoma
Adenocarcinoma, Follicular* ; Biopsy, Fine-Needle ; Goiter ; Hearing Loss, Sensorineural ; Humans ; Hypothyroidism ; Korea ; Neck ; Pathology ; Thyroid Gland ; Thyroidectomy

Pendred syndrome is an autosomal recessive genetic disorder, which is characterized by sensorineural hearing loss, goiter and a positive perchlorate discharge test. It is caused by mutations of the PDS gene, and its clinical characteristics vary widely. The thyroid function in most cases is normal, or shows only mild hypothyroidism. In Pendred syndrome, there is an organification defect that leads to defective thyroid hormone synthesis, followed by chronic TSH stimulation. Herein is reported a case of a follicular thyroid carcinoma associated with Pendred syndrome. To our knowledge, this is the first case reported in Korea. The patient presented with a huge anterior neck mass, sensorineural hearing loss and a positive perchlorate discharge test. Fine needle aspiration cytology suggested malignancy of the thyroid, and a total thyroidectomy, with central compartment node dissection, was performed. The pathology from the thyroid mass showed a poorly differentiated follicular thyroid carcinoma
Adenocarcinoma, Follicular* ; Biopsy, Fine-Needle ; Goiter ; Hearing Loss, Sensorineural ; Humans ; Hypothyroidism ; Korea ; Neck ; Pathology ; Thyroid Gland ; Thyroidectomy