Low-frequency Sensorineural Hearing Loss Research Articles

Impact of Endolymphatic Hydrops and Semicircular Canal Dysfunction on the Incomplete Recovery of Acute Low-Frequency Sensorineural Hearing Loss.

The reasons for short-term, incomplete recovery of some acute low-frequency sensorineural hearing loss (ALFHL) are unclear. We investigated the impact of endolymphatic hydrops and semicircular canal dysfunction on the incomplete recovery of ALFHL. The impact of electrocochleography (EcochG), caloric test, video head impulse test (vHIT), cervical vestibular-evoked myogenic potential (cVEMP), and ocular vestibular-evoked myogenic potential (oVEMP) results on the incomplete recovery of 86 patients with ALFHL was analyzed. The correlation between a high abnormal rate of vestibular-evoked myogenic potential (VEMP) and aural fullness, dizziness, and level of hearing loss was also examined. Abnormal oVEMP with an odds ratio (OR) of 6.071 (P = .002) and EcochG with an OR of 5.919 (P = .005) were independent risk factors for incomplete recovery in ALFHL, but abnormal cVEMP, caloric test, and vHIT were not independent risk factors for ALFHL. There was no correlation between a high abnormal rate of VEMP and aural fullness, dizziness, or level of hearing loss in ALFHL. Abnormal oVEMP and EcochG values rather than caloric test and vHIT were risk factors for incomplete recovery of ALFHL, which may be related to the difficulty of successfully treating utricular and cochlear hydrops. High abnormal rate of VEMP was not correlated with hearing loss level, aural fullness, or dizziness in ALFHL. Abnormal oVEMP and EcochG values can indicate poor short-term prognosis of ALFHL.

Olfactory function in Susac syndrome

ObjectivesSusac syndrome is a rare autoimmune endotheliopathy involving the brain, retina, and inner ear. Olfactory dysfunction is a common early manifestation of several central nervous system diseases, including neurodegenerative diseases and autoimmune-mediated diseases such as Multiple Sclerosis. While the literature is abundant about the Susac syndrome classic triad of encephalopathy, branch retinal artery occlusion, and low-frequency sensorineural hearing loss, little is known about the extent of olfactory sense involvement. MethodsUsing the Sniffin' Sticks test, this study evaluated olfactory function (identification and threshold) in ten recovering Susac syndrome patients under our clinic surveillance with a median of 3.1 (SD=1.53) years post-disease onset. Resultsolfactory assessment by threshold and odor identification were within the normal range. No differences between recovering Susac syndrome patients to standard norms of odor identification and threshold were found. ConclusionsOur findings do not support olfactory dysfunction in Susac syndrome and thereby, do not support olfactory assessment as a reliable biomarker for this condition.

Does labyrinthectomy have an impact on hearing in the contralateral ear during long-term follow-up?

ObjectiveTo quantify contralateral hearing outcomes after labyrinthectomy for unilateral Ménière's disease (MD). Study designRetrospective case series. SettingTertiary neurotology referral center. PatientsLabyrinth removal for the management of MD or translabyrinthine (TLAB) acoustic neuroma resection between 2008 and 2012. Main outcome measureLong-term hearing changes via pure tone averages (PTA). ResultsUpon comparison of low-frequency PTA (250, 500, 1000 Hz), MD patients experienced a greater degree of hearing loss during the follow-up period when compared to the TLAB lab group (7.54 ± 2.11 dB vs 2.39 ± 1.10 dB, p = 0.035). This difference as attributable to 12 (28.6 %) MD patients experiencing a ≥30 dB increase in low-frequency PTA, whereas none (0.0 %) of the TLAB surpassed this threshold. ConclusionsAt 10 years post-labyrinthectomy there is a heightened risk for MD patients to develop low-frequency sensorineural hearing loss. Clinicians should monitor for audiometric changes through regular testing in the decade following labyrinth removal.

Menière's Syndrome Associated With Fibrous Dysplasia of the Temporal Bone.

Menière's Syndrome Associated With Fibrous Dysplasia of the Temporal Bone.

Tinnitus, Aural Fullness, and Hearing Loss in a Patient with Acoustic Neuroma and Pituitary Macroadenoma

We report the case of a 51-year-old woman with multiple otologic and vestibular symptoms. She presented with two different types of tinnitus in her right ear, vertigo, and fluctuating aural symptoms in the left ear. She also complained of disequilibrium; chronic headache; hyperhidrosis; amenorrhea; insomnia; broadened hands and feet; and widened, thickened, and stubby fingers. The patient underwent careful collection of medical history, otomiscroscopy, pure tone audiometry, tympanometry, reflex threshold measurements, vestibular assessments, blood tests, magnetic resonance imaging (MRI), and cone beam computed tomography (CBTC) of the head. The audiogram showed: (1) a mild low-to-mid frequency conductive hearing loss, and a sharply sloping sensorineural hearing loss above 4000 Hz in the right ear; (2) a mild low-frequency sensorineural hearing loss in the left ear. MRI with 3D FLAIR sequences detected an acoustic neuroma (7.4 mm × 5.2 mm) in the middle-third of the right internal auditory canal, a pituitary macroadenoma (13 mm × 10 mm × 10 mm) and left saccular hydrops. The CBCT scan documented an outbreak of otosclerosis (3 mm) around the fissula ante fenestram in the right ear. Therefore, acoustic neuroma (right ear), growth hormone-secreting macroadenoma of the pituitary gland, Menière’s disease (left ear), and otosclerosis (right ear) were diagnosed/strongly suspected. A watch-and-wait strategy was adopted for acoustic neuroma and otosclerosis, while transsphenoidal surgery was successfully performed to remove the pituitary macroadenoma. This case report confirms that multiple otologic disorders can occur simultaneously in the same patient, requiring prompt audiological and imaging evaluations.

Advances in the study of fluctuating sensorineural hearing loss

Fluctuating sensorineural hearing loss（FSNHL） is a special type of sensorineural hearing loss, which can be manifested in many clinical diseases. In this paper, some clinical diseases associated with FSNHL are summarized, such as Meniere's disease, large vestibular aqueduct syndrome, acute low frequency sensorineural hearing loss, delayed endolymphatic hydrops, autoimmune inner ear disease and syndromes leading to FSNHL. The pathogenesis, diagnosis and treatment of FSNHL were summarized in order to improve clinicians' understanding of FSNHL, reduce the probability of misdiagnosis of related diseases and improve the prognosis of patients.

Genotype and Phenotype Analyses of a Novel WFS1 Variant (c.2512C>T p.(Pro838Ser)) Associated with DFNA6/14/38.

The aim of this study is to contribute to a better description of the genotypic and phenotypic spectrum of DFNA6/14/38 and aid in counseling future patients identified with this variant. Therefore, we describe the genotype and phenotype in a large Dutch-German family (W21-1472) with autosomal dominant non-syndromic, low-frequency sensorineural hearing loss (LFSNHL). Exome sequencing and targeted analysis of a hearing impairment gene panel were used to genetically screen the proband. Co-segregation of the identified variant with hearing loss was assessed by Sanger sequencing. The phenotypic evaluation consisted of anamnesis, clinical questionnaires, physical examination and examination of audiovestibular function. A novel likely pathogenic WFS1 variant (NM_006005.3:c.2512C>T p.(Pro838Ser)) was identified in the proband and found to co-segregate with LFSNHL, characteristic of DFNA6/14/38, in this family. The self-reported age of onset of hearing loss (HL) ranged from congenital to 50 years of age. In the young subjects, HL was demonstrated in early childhood. At all ages, an LFSNHL (0.25-2 kHz) of about 50-60 decibel hearing level (dB HL) was observed. HL in the higher frequencies showed inter-individual variability. The dizziness handicap inventory (DHI) was completed by eight affected subjects and indicated a moderate handicap in two of them (aged 77 and 70). Vestibular examinations (n = 4) showed abnormalities, particularly in otolith function. In conclusion, we identified a novel WFS1 variant that co-segregates with DFNA6/14/38 in this family. We found indications of mild vestibular dysfunction, although it is uncertain whether this is related to the identified WFS1 variant or is an incidental finding. We would like to emphasize that conventional neonatal hearing screening programs are not sensitive to HL in DFNA6/14/38 patients, because high-frequency hearing thresholds are initially preserved. Therefore, we suggest screening newborns in DFNA6/14/38 families with more frequency-specific methods.

Полиэтиология низкочастотной сенсоневральной тугоухости

Despite the successes of modern medical science, a powerful arsenal of diagnostic techniques (including neurophysiological methods and neuroimaging), the diagnosis for cochleovestibular disorders usually depends on the personal experience of the doctor and is based on the results of a clinical examination and symptomatic criteria. Given the complexity of identifying idiopathic Meniere’s disease, this paper discusses a number of comorbid and conjugate conditions accompanied by low-frequency sensorineural hearing loss. Due to the variety of causes of the development of this type of hearing loss, emphasis is placed on the expediency of expanding the examination of patients in this category.

A Novel Missense WFS1 Variant: Expanding the Mutational Spectrum Associated with Nonsyndromic Low-Frequency Sensorineural Hearing Loss.

Background Nonsyndromic low-frequency sensorineural hearing loss (LFSNHL) is an uncommon form of hearing loss (HL) that typically affects frequencies at 2000 Hz and below. Heterozygous variants in the WFS1 gene at the DFNA6/14/38 locus are considered a common cause of LFSNHL. To date, 34 different pathogenic genetic variants have been reported to cause LFSNHL with seven of these variants identified in the Chinese population. However, limited reports are available on the association between WFS1 gene and LFSNHL. Here, we report a five-generation Chinese family with an autosomal dominant inheritance pattern of postlingual and progressive LFSNHL. Methods Routine clinical and audiological examinations were performed on 16 affected and 7 healthy members in this family. The targeted next-generation sequencing of 127 known deafness genes was performed to identify variants in affected individuals. Sanger sequencing were further employed to confirm the pathogenic variant identified. Results A novel heterozygous pathogenic genetic variant c.2530G > T (p.Ala844Ser) was identified in the WFS1 gene in all patients of this family. The mutated Ala residue is evolutionarily conserved and cosegregated with HL. The variant was predicted to be deleterious by MutationTaster, PolyPhen-2, LRT, and Fathmm software. Conservation analysis and 3D protein structure model indicated that the variant caused a structural change in the protein. Conclusions Our present study identifies a novel heterozygous WFS1 variant associated with LFSNHL in a Chinese family.

A Comparison of Local Endolymphatic Sac Decompression, Endolymphatic Mastoid Shunt, and Wide Endolymphatic Sac Decompression in the Treatment of Intractable Meniere's Disease: A Short-Term Follow-Up Investigation.

BackgroundMeniere's disease (MD) is an inner ear disorder, characterized by recurrent attacks of vertigo, low-frequency sensorineural hearing loss, tinnitus, and aural fullness. Endolymphatic sac surgery is an effective treatment to control vertigo attacks but without causing a hearing loss for intractable MD. However, the methods and effects of endolymphatic sac surgery have been controversial for many years, and the relationship between the vertigo control rates of different endolymphatic sac surgery methods is not well-documented.ObjectivesThis study compared the vertigo control rate, hearing outcome, and quality of life (QOL) among different endolymphatic sac surgery, such as local endolymphatic sac decompression (LESD), endolymphatic sac mastoid shunt (ESMS), and wide endolymphatic sac decompression (WESD).Materials and MethodsWe retrospectively analyzed the patients who underwent endolymphatic sac surgery from January 2008 to June 2019. The control rate of vertigo and QOL scores were compared after 2 years of follow-up. The QOL was scored with validation of the MD patient-oriented symptom-severity index (MDPOSI). The pure tone thresholds of all patients at pre- and postoperation were also compared.ResultsIn total, 83 MD patients with complete follow-up data were included in the study, i.e., 20 patients with LESD, 28 patients with ESMS, and 35 patients with WESD. Results showed a better vertigo control with WESD than the other groups (70% with LESD, 71.4% with ESMS, and 88.6% with WESD). The QOL was improved after surgery in all groups in which the difference was statistically significant (QOL, preoperative vs. postoperative, 38.2 vs. 10.1 with LESD, 37.8 vs. 9.6 with ESMS, and 37.6 vs. 8.3 with WESD), respectively. After endolymphatic sac surgery, the hearing was well-preserved in the three groups [pure tone averages (PTAs), dB, preoperative vs. postoperative, 41.0 ± 19.3 vs. 40.8 ± 17.9 with LESD, 39.7 ± 16.4 vs. 40.8 ± 18.2 with ESMS, and 38.5 ± 18.7 vs. 36.6 ± 19.5 with WESD].ConclusionWide endolymphatic sac decompression has a higher vertigo control rate, better improvement of QOL, and relatively higher hearing stability or improvement rate after surgery in patients with MD compared with LESD and ESMS.

Chronic Obstructive Pulmonary Disease is a Risk Factor for Sensorineural Hearing Loss: A US Population Study.

The goal of this study is to determine if chronic obstructive pulmonary disease (COPD) is associated with sensorineural hearing loss (SNHL) in a national database. Cross-sectional study. National sample of the United States population. Adults with audiometric and spirometry data from the National Health and Nutrition Examination Study (NHANES) database. None. A total of 2,464 adults with spirometry and audiometry data from the NHANES database (2009-2012) were studied. Outcome measures included hearing, measured by high-frequency pure tone average (HFPTA; 3, 4, 6, 8 kHz) and low-frequency pure tone average (LFPTA; .5, 1, 2 kHz) frequencies. SNHL was defined as a HFPTA or LFPTA threshold more than 25 decibels (dB) in the better ear. Multivariable regression analyses explored the association between hearing loss and COPD. The prevalence of COPD was 19.8% in individuals with SNHL in the better ear and 4.7% in individuals with normal hearing (p < 0.001). Presence of COPD was associated with elevated hearing thresholds (worse hearing) at each individual frequency. The presence of COPD was independently associated with a 3.29 dB (95% CI: 1.48, 5.09) increase in HFPTA (p < 0.001), and 2.32 dB (1.13, 3.50) increase in LFPTA (p < 0.001) after controlling for medical, social, and environmental covariates. The presence of COPD was independently associated with a 1.85-fold (1.12, 3.06) increased odds of isolated low-frequency SNHL (p = 0.017). COPD was independently associated with sensorineural hearing loss after controlling for multiple confounding factors. These results contribute to the evidence that COPD and pulmonary dysfunction can be comorbid with hearing decline.

Rare coding variants involving MYO7A and other genes encoding stereocilia link proteins in familial meniere disease

The MYO7A gene encodes a motor protein with a key role in the organization of stereocilia in auditory and vestibular hair cells. Rare variants in the MYO7A (myosin VIIA) gene may cause autosomal dominant (AD) or autosomal recessive (AR) sensorineural hearing loss (SNHL) accompanied by vestibular dysfunction or retinitis pigmentosa (Usher syndrome type 1B). Familial Meniere's disease (MD) is a rare inner ear syndrome mainly characterized by low-frequency sensorineural hearing loss and episodic vertigo associated with tinnitus. Familial aggregation has been found in 6–8% of sporadic cases, and most of the reported genes were involved in single families. Thus, this study aimed to search for relevant genes not previously linked to familial MD. Through exome sequencing and segregation analysis in 62 MD families, we have found a total of 1 novel and 8 rare heterozygous variants in the MYO7A gene in 9 non-related families. Carriers of rare variants in MYO7A showed autosomal dominant or autosomal recessive SNHL in familial MD. Additionally, some novel and rare variants in other genes involved in the organization of the stereocilia links such as CDH23, PCDH15 or ADGRV1 co-segregated in the same patients. Our findings reveal a co-segregation of rare variants in the MYO7A gene and other structural myosin VIIA binding proteins involved in the tip and ankle links of the hair cell stereocilia. We suggest that recessive digenic inheritance involving these genes could affect the ultrastructure of the stereocilia links in familial MD.

MR Imaging of Cochlear Modiolus and Endolymphatic Hydrops in Patients With Menière's Disease.

Background: Menière's disease (MD) is an inner ear disorder characterized by recurrent episodes of spontaneous vertigo, unilateral low-frequency sensorineural hearing loss, tinnitus, and aural fullness. Current diagnosis still often has to rely on subjective and audiometric criteria only, although endolymphatic hydrops is recognized as the pathophysiological substrate of the disease, having been demonstrated in anatomical pathological studies and by magnetic resonance (MRI). The modiolus has a close functional and anatomical relationship with the cochlear nerve and membranous labyrinth and can be evaluated with MRI but no data exist on the modiolar size in MD.Purpose: Our purpose is to examine the following hypothesis. Is cochlear modiolus smaller in symptomatic ears in MD?Methods: We used a retrospective 3 Tesla MR study (heavily T2-weighted 3D fast asymmetric spin-echo images and 0.5 mm slice thickness) comparing the mean modiolar area (MMA) in the index and best ears of eight patients with definite MD based on audiometric data. The obtained MMA values were compared against the audiometric data and the presence of vestibular endolymphatic hydrops.Results: No differences were seen in MMA between best and worst ears. Ears with a pure tone average (PTA) ≥25 dB and more pronounced endolymphatic hydrops showed lower MMA (not statistically significant). Two patients with extreme endolymphatic hydrops showed a noteworthy ipsilateral decrease in the cochlear modiolus area.Conclusion: No differences were seen in MMA between best and worst ears in definite MD. Worse hearing function (PTA ≥ 25dB) and more pronounced endolymphatic hydrops seem to be associated with lower MMA. This might be related to bone remodeling as a consequence of endolymphatic hydrops. Further research is needed to corroborate and explore these findings.

Deficiency of Klc2 Induces Low-Frequency Sensorineural Hearing Loss in C57BL/6J Mice and Human.

The transport system in cochlear hair cells (HCs) is important for their function, and the kinesin family of proteins transports numerous cellular cargos via the microtubule network in the cytoplasm. Here, we found that Klc2 (kinesin light chain 2), the light chain of kinesin-1 that mediates cargo binding and regulates kinesin-1 motility, is essential for cochlear function. We generated mice lacking Klc2, and they suffered from low-frequency hearing loss as early as 1month of age. We demonstrated that deficiency of Klc2 resulted in abnormal transport of mitochondria and the down-regulation of the GABAA receptor family. In addition, whole-genome sequencing (WGS) of patient showed that KLC2 was related to low-frequency hearing in human. Hence, to explore therapeutic approaches, we developed adeno-associated virus containing the Klc2 wide-type cDNA sequence, and Klc2-null mice delivered virus showed apparent recovery, including decreased ABR threshold and reduced out hair cell (OHC) loss. In summary, we show that the kinesin transport system plays an indispensable and special role in cochlear HC function in mice and human and that mitochondrial localization is essential for HC survival.

Missense Variant of Endoplasmic Reticulum Region of WFS1 Gene Causes Autosomal Dominant Hearing Loss without Syndromic Phenotype.

Objective Genetic variants in the WFS1 gene can cause Wolfram syndrome (WS) or autosomal dominant nonsyndromic low-frequency hearing loss (HL). This study is aimed at investigating the molecular basis of HL in an affected Chinese family and the genotype-phenotype correlation of WFS1 variants. Methods The clinical phenotype of the five-generation Chinese family was characterized using audiological examinations and pedigree analysis. Target exome sequencing of 129 known deafness genes and bioinformatics analysis were performed among six patients and four normal subjects to screen suspected pathogenic variants. We built a complete WFS1 protein model to assess the potential effects of the variant on protein structure. Results A novel heterozygous pathogenic variant NM_006005.3 c.2020G>T (p.Gly674Trp) was identified in the WFS1 gene, located in the C-terminal domain of the wolframin protein. We further showed that HL-related WFS1 missense variants were mainly concentrated in the endoplasmic reticulum (ER) domain. In contrast, WS-related missense variants are randomly distributed throughout the protein. Conclusions In this family, we identified a novel variant p.Gly674Trp of WFS1 as the primary pathogenic variant causing the low-frequency sensorineural HL, enriching the mutational spectrum of the WFS1 gene.

Association Between Sigmoid Sinusoidal Tinnitus and Low-Frequency Sensorineural Hearing Loss: A Retrospective Study at a Single Center.

BackgroundWe aimed to explore the correlation between patients’ sigmoid sinusoidal tinnitus (SST) and low-frequency sensorineural hearing loss (LFSHL) and illustrate the underlying mechanism.Material/MethodsSeven healthy volunteers with normal hearing were subjected to 125-, 250-, and 500-Hz pure sound and different white noise-masking intensities. A retrospective analysis was made on the clinical data and postoperative follow-up data of 59 patients with SST in the First Affiliated Hospital of Chongqing Medical University. The patients’ sex, age, chief complaints, affected site, concomitant symptoms, course of disease, pure-tone audiometry (PTA) results, tinnitus discomfort loudness scale results, imaging examination, and complications were collected.ResultsThe results of the simulation experiment showed that the threshold of each frequency segment was higher after noise masking than before masking; the intensity of noise masking was positively correlated with hearing loss, and the changes of the hearing threshold of the 3 frequencies before and after masking were statistically significant (P<0.05). Fifty-nine patients with SST were documented between January 2015 and January 2020. After the operation, their low-frequency hearing was recovered to normal; 11 cases had significantly alleviated tinnitus and 9 cases were cured.ConclusionsSST often causes corresponding pseudo-low-frequency hearing loss due to the noise-masking effect. The center frequency of tinnitus appears not to be 250-Hz or 500-Hz octave frequency of PTA, barring the detection of the pseudo-hearing loss in the audiometry chart of most patients. Surgery positively affects patients with SST, and the pseudo-LFSHL can be completely recovered after the operation as a result of tinnitus elimination.

Analysis of prognostic factors of low-frequency type of sudden sensorineural hearing loss

Objective: To investigate the prognostic factors of patients with low-frequency type of sudden sensorineural hearing loss. Methods: From February 2017 to February 2019, adult patients with unilateral low-frequency type of sudden sensorineural hearing loss in Department of Otological Medicine, Shandong Provincial ENT Hospital, Shandong University were selected. All patients were examined by audiology, vestibular function evaluation, imaging examination and serum thyroid function test; the same treatment program was given, the curative effect was recorded and followed up for more than 3 months. SPSS 20.0 software was used to analyze concomitant symptoms (tinnitus, ear tightness, echo, rotatory vertigo), degree of deafness, inducement of deafness, basic disease, vestibular function (caloric test), electrocochleogram, inner ear gadolinium enhanced MRI radiography and thyroid function on prognosis of patients. Results: Among the 155 patients, 76 cases were cured (49.0%), 1 case was markedly effective (0.6%), 19 cases were effective (12.3%), and 59 cases were ineffective (38.1%). The total effective rate was 61.9%. Among them, 24 cases (15.5%) had hearing fluctuations during follow-up, and 1 case (0.6%) developed Meniere's disease. Univariate analysis showed that vestibular function, electrocochleogram and inner ear MRI were correlated with prognosis. Multivariate logistic analysis showed that ear tightness, vestibular function, electrocochleogram and inner ear MRI were correlated with the prognosis of the patients. The two analyses showed that tinnitus, echo, rotational vertigo, degree of deafness, predisposing factors and underlying diseases were not significantly correlated with the prognosis of the patients (all P>0.05). Rotational vertigo was closely related to gender, and women had a high incidence. There was a significant correlation between the degree of deafness and prognosis in patients with course of less than 1 week (P<0.05). The abnormal rate of vestibular function in patients with course of disease ≤ 1 week was significantly different from that>1-≤2 and>2-≤4 weeks (P<0.05). The rate of abnormal thyroid function was significantly higher than that of normal people (P<0.05), but there was no significant correlation between thyroid dysfunction and hearing prognosis (χ(2)=0.009, P=0.923) . Conclusions: The prognosis of low-frequency sudden sensorineural hearing loss is not related to clinical symptoms, inducements, underlying diseases and serological abnormalities of thyroid function, but the degree of deafness is positively related to the prognosis within 1 week from onset. Abnormal thyroid function is one of the risk factors of happening with low-frequency descending sudden deafness. Abnormal vestibular caloric test, electrocochleogram and endolymph hydrops are the factors of poor prognosis.

Is Endolymphatic Sac Surgery Beneficial For Meniere's Disease?

Is Endolymphatic Sac Surgery Beneficial For Meniere's Disease?

Diagnosis and outcome analysis of diseases characterized by recurrent low-frequency sensorineural deafness

Objective:The aim of this study was to explore the clinical features, hearing prognosis and differential diagnosis of recurrent low frequency sensorineural hearing loss （RLFD） . Method:The clinical characteristics, clinical manifestations, audiological examination and auxiliary examination of RLFD patients were retrospectively analyzed. We summarized clinical features, draw the pure tone audiometry curves, and analyze the diagnosis of RLFD. Result:Forty-seven patients （53 ears） with RLFD had a hearing review time of 1-124 months. The course of disease ranged from 3 to 320 months with an average course of 29 months. ①The incidence of tinnitus in the accompanying symptoms was 93.6%（44 cases）, and the ear suffocation was 83.0%（39 cases）, 38.3% （18 cases） of the patients developed vestibular symptoms during the course of the disease. ②During the observation period, 27 cases（57.4%） were diagnosed with related diseases: 7 cases（14.9%） Meniere's disease, 6 cases（12.8%） vestibular migraine, 2 cases（4.3%） with Meniere's disease and migraine, and 1 case（2.1%） with idiopathic intracranial hypotension 11 cases（23.4%） were possible cochlear migraine; ③Migraine-related RLFD had a younger onset age, more common in women; ④83.0%（44 ears）of the affected ears had stable or improved low-frequency hearing during the observation period, 17.0%（9 ears）of the affected ears experienced low-frequency hearing; ⑤18.9%（10 ears）of the affected ears had high-frequency hearing loss; ⑥RLFD had 6 types of audiological outcomes: low-frequency improvement combined with high-frequency stability; low-frequency stability combined with high-frequency stability; low frequency progress combined with high frequency stability type; low frequency improvement combined with high frequency progress type; low frequency stability combined with high frequency progress type; low frequency progress combined with high frequency progress type; ⑦Rising type hearing curve low frequency hearing prognosis is good, mountain type and descending low frequency hearing prognosis is poor. Conclusion:Tinnitus and ear stuffiness are the early symptoms and the most disturbing symptoms in patients with RLFD. The mechanism of Migraine may play an important role in the pathogenesis of RLFD. The rare causes such as intracranial hypotension syndrome should not be ignored. Most of the patients with RLFD had stable or improved hearing after long-term fluctuation, but some patients with low or high frequency hearing might decline. The type of initial hearing curve was a prognostic factor. Long-term hearing follow-up is helpful for prognosis evaluation.

Comparison of Treatment Outcomes between Intratympanic Steroid Injection and Oral Diuretics in Patients with Acute Low Frequency Sensorineural Hearing Loss with Vertigo

Comparison of Treatment Outcomes between Intratympanic Steroid Injection and Oral Diuretics in Patients with Acute Low Frequency Sensorineural Hearing Loss with Vertigo