N-acetylcysteine as a single therapy for sudden deafness

Conclusion: Like NAC ameliorates hearing loss from acoustic trauma in the inner ear, NAC may also rescue hearing loss from sudden deafness confined to the inner ear.

Objective: This study assesses the effect of N-acetyl-L-cysteine (NAC) as a single therapy for sudden deafness.

Methods: Thirty-five sudden deafness patients with neither systemic disorders nor central signs in electronystagmography were treated with NAC alone and assigned to Group A. For comparison, another 35 sudden deafness patients treated by corticosteroids and plasma expander were assigned to Group B. There were no significant differences between the two groups in terms of age, sex, laterality, and pre-treatment mean hearing level. All patients underwent an inner ear test battery comprising audiometry, and ocular vestibular-evoked myogenic potential (oVEMP), cervical VEMP (cVEMP), and caloric tests.

Results: Groups A and B did not significantly differ in the pre-treatment mean hearing level, and percentages of abnormal oVEMP, cVEMP, and caloric tests, indicating that the involvement severity of sudden deafness between the two groups was similar. However, Group A (43?±?27?dB) showed significantly greater mean hearing gain than Group B (21?±?28?dB), and Group A (91%) revealed better improved rate of hearing than Group B (57%).     

Acute sensorineural hearing loss in hemodialysis patients

Abstract

Background: Approximately, 30–40% of patients experienced hearing loss under regular hemodialysis.

Objective: This study reviewed our experience on treating acute hearing loss in patients under regular hemodialysis over the past two decades.

Methods: Twenty-six patients having acute hearing loss under hemodialysis were divided into two groups based on their etiologies. Sixteen patients (16 ears) with sudden sensorineural hearing loss (SSHL) were assigned to Group A and 10 patients (13 ears) with endolymphatic hydrops (EH) were assigned to Group B.

Results: No significant difference was noted between Groups A and B, regardless of hemodialysis duration, clinical manifestation, underlying systemic diseases, blood examination, and vestibular test battery. In contrast, serum osmolality was significantly lower in Group B (292?±?11 mOsm/kg) than in Group A (310?±?11 mOsm/kg). Furthermore, Group B (40?±?14?dB) had better mean hearing level than Group A (87?±?21?dB) in the initial audiogram, and a higher hearing improvement rate (69%) than Group A (19%).

Conclusions and significance: Both SSHL and EH are major causes for precipitating acute hearing loss in hemodialysis patients. Compared to SSHL, the less deteriorated MHL and lower serum osmolality in EH provide two clues for differentiating acute hearing loss in hemodialysis patients.     

Sensory organization test principally reflects utricular function

Conclusion: Our study showed that sensory organization test (SOT) could principally reflect utricular function, and our cut-score of VEST ratio (the indicator of inputs from the vestibular system in balance performance) in SOT could help to screen patients with unilateral peripheral vestibular dysfunction in non-fallers.

Objectives: We aimed to know SOT reflects the function of which vestibular end organs and to assess the possibility of screening patients with peripheral vestibular dysfunction in non-fall population by means of SOT.

Methods: A total of 75 patients with unilateral peripheral vestibular dysfunction underwent SOT, ocular vestibular-evoked myogenic potential (oVEMP), cervical vestibular-evoked myogenic potential (cVEMP) and caloric tests.

Results: Regression model indicated that oVEMP, as an independent variable, exerted the greatest influence on VEST ratio, followed by age and cVEMP. There were no significant differences in VEST ratio among groups with different extents of peripheral vestibular involvement (p?>?.05). VEST ratio in the vestibular impairment groups was significantly lower than in the control group and significantly higher than in fall group (p?p?=?.013). The cut-score between populations with functional and dysfunctional vestibule was 0.649.     

Acute hearing loss in patients with hematological disorders

Objective: This study adopted an inner ear test battery to investigate the causes of acute sensorineural hearing loss in patients with hematological disorders. Methods: During the past 20 years, the authors have experienced 14 patients with hematological disorders, i.e. leukemia or aplastic anemia, having acute sensorineural hearing loss. An inner ear test battery comprising audiometry and cervical vestibular-evoked myogenic potential (cVEMP), ocular VEMP (oVEMP), and caloric tests was performed. Results: Diagnoses comprised of sudden sensorineural hearing loss in 12 patients and endolymphatic hydrops in four patients (two patients had one ear with sudden sensorineural hearing loss while the other ear had endolymphatic hydrops). Percentages of recruitment phenomenon showed a significant difference between endolymphatic hydrops and sudden sensorineural hearing loss. Abnormal percentages for mean hearing level (86%), cVEMP test (71%), oVEMP test (25%), and caloric test (14%) exhibited a significant sequential decline in these patients. Conclusion: Acute sensorineural hearing loss in a patient with leukemia or aplastic anemia may be related to either sudden sensorineural hearing loss or endolymphatic hydrops. A significant sequential decline in the function of the cochlea, saccule, utricle, and semicircular canals indicates that the pars inferior is more vulnerable to blood insult than the pars superior.     

Analysis of audio-vestibular assessment in acute low-tone hearing loss

Conclusion This study demonstrated excellent hearing recovery following the combined treatment of diuretic and oral steroid, and electrocochleography (ECoG) was significantly higher than normal side. This study reports characteristics of acute low-tone hearing loss (ALHL) that show the greater low-tone hearing loss, the higher ECoG, and excellent recovery, even-though low-tone hearing loss is worse, which can be different compared with sudden deafness. Objective To analyze ALHL without vertigo, this study compared the ALHL group with all patients exhibiting low-tone hearing loss and ear fullness. Hearing changes and vestibular functions were analyzed. Materials and methods ALHL was defined as a mean hearing loss of?≥?30?dB at 125, 250, and 500?Hz, and?≤?20?dB at 2, 4, and 8?kHz. From 156 cases of low-tone hearing loss of more than 10?dB without vertigo, 31 met the ALHL criteria and were subjected to audio-vestibular assessments including PTA, ECoG, vestibular evoked myogenic potential (VEMP) testing, and caloric testing. Results In ALHL, low-tone hearing loss was 42.7?±?9.5?dB, and 83.9% of ALHL significantly recovered by more than 10?dB. The ECoG in ALHL was 0.334?±?0.11 (higher than 0.25?±?0.08 on the normal side) and ECoG abnormality was 35.5% (the greater low-tone hearing loss, the higher ECoG value).     

Progressive familial hearing loss in Muckle-Wells syndrome

Conclusion. The tumor size of acoustic neuroma correlates with cochleovestibular deficits. Those tumors with global frequency hearing loss, bilateral gaze nystagmus, or absent caloric and VEMP responses may indicate a tumor size >2.5 cm. Objective. This study aimed to investigate the correlation between cochleovestibular deficits and the size of acoustic neuroma. Patients and methods. A total of 44 patients with acoustic neuroma were enrolled in this study. Pure tone audiometry, electronystagmography, caloric test, vestibular evoked myogenic potential (VEMP) test, and MRI were conducted. Results. There is a trend of correlation between tumor size and audiographic configuration, with small-sized tumor in normal and rising types, medium-sized tumor in mid- and high-frequency hearing loss, and large-sized tumor in flat and deafness types. Five patients with bilateral gaze nystagmus had significantly larger tumor size than those without nystagmus. When 1 and 0 are used to represent abnormal and normal responses, respectively, the relationship between tumor size and vestibular function can be expressed as: tumor size (cm)=1.43 (caloric response)+1.35 (VEMP response), indicating that the estimated tumor size for those with abnormal caloric or VEMP responses increased by 1.43 or 1.35 cm, respectively.     

Different prognoses in patients with profound sudden sensorineural hearing loss

Background: Inner ear hemorrhage is increasingly recognized as a cochlear lesion that can cause profound sudden sensorineural hearing loss (SSNHL).

Objectives: To investigate changes of cochlear and vestibular function and to compare therapeutic recovery from profound SSNHL induced by different etiologies.

Material and methods: Eighty patients with profound SSNHL (≥90?dB) were divided into an inner ear hemorrhage group and a non-inner ear hemorrhage group by MRI. Statistical analysis was performed to compare the therapeutic effects from vertigo and hearing loss and the outcomes of follow-up in the two groups.

Results: There were significant differences between the two groups in terms of the overall 14-day therapeutic response rate (20 vs. 48%), the incidence of imbalance (26.7 vs. 6%), the incidence of semicircular canal dysfunction on the affected side (60 vs. 20%), the incidence of abnormal C-VEMP and O-VEMP on the affected side (63.3 vs. 38%; and 60 vs. 30%, respectively), the average hearing threshold (74.2?±?10.7 vs. 53.6?±?11.4?dB), and the word recognition score (65.5?±?21.7 vs. 83.5?±?24.5%) at a 12-month follow-up.

Conclusions and significance: A higher percentage of patients with profound SSNHL induced by inner ear hemorrhage were associated with vertigo and had a poor prognosis.     

Lesion site in idiopathic bilateral vestibulopathy: A galvanic vestibular-evoked myogenic potential study

Conclusion The result suggests that patients with idiopathic bilateral vestibulopathy may have nerve lesions when the inferior nerve system is affected, while the inferior vestibular nerve system may be spared.

Objective To clarify the lesion site in idiopathic bilateral vestibulopathy, an acquired bilateral vestibulopathy of unknown cause.

Material and methods Two 75-year-old males diagnosed with idiopathic bilateral vestibulopathy were enrolled. Both showed absent or highly decreased responses on the caloric test on both sides. They underwent vestibular-evoked myogenic potential (VEMP) testing by means of acoustical and electrical stimulation. As acoustic stimulation, 95 dB nHL clicks and short tone bursts (500 Hz) were presented, while 3 mA (1 ms) short-duration galvanic stimuli were presented as electrical stimulation. Responses were recorded on the sternocleidomastoid muscles.

Results Both patients showed unilateral absence of VEMPs with both acoustic and short-duration galvanic stimuli.     

Comparisons among vestibular examinations and symptoms of vertigo in sudden sensorineural hearing loss patients

ObjectivesVertigo in sudden sensorineural hearing loss (SSNHL) is hypothesized as an extension of the disease caused by the anatomical proximity of the cochlea and vestibule. The present study aimed to demonstrate the association of vestibular function test (VFT) results with SSNHL disease severity and prognosis.Materials and methodsThis study assessed clinical records of 263 SSNHL patients admitted to our hospital, between January 2010 and October 2017. Steroid treatment comprised high-dose intravenous dexamethasone (16 mg/d) or oral methylprednisolone (64 mg/d) for 4 days and tapered oral methylprednisolone for 8 days after discharge. Caloric tests were performed in all patients, and cervical vestibular-evoked myogenic potential (c-VEMP) and ocular VEMP (o-VEMP) tests were performed in 209 and 144 patients, respectively.ResultsNinety six patients had vertigo, and caloric abnormalities were observed in 119 patients. Initial PTA in patients with vertigo were worse than in those without vertigo (63.0 dB vs 72.7 dB, P = .002). Initial PTA in patients with abnormal o-VEMP was worse than in those with normal o-VEMP (61.4 dB vs 73.0 dB, P = .004). PTA improvement after steroid treatment in patients with vertigo was lower than in those without vertigo (25.0 dB vs 20.9 dB, P = .028). PTA improvement after treatment in patients with abnormal caloric results was lower than in those with normal caloric results (26.0 dB vs 18.4 dB, P = .013).ConclusionThe functions of vestibular organs, particularly the utricle and lateral semicircular canal, are associated with disease severity and hearing outcome in SSNHL patients.     

Function of semicircular canals,utricles and saccules in deaf children

Objective To study vestibular function in deaf children.

Material and Methods In 36 deaf children the function of the semicircular canals, saccules and utricles was measured by means of caloric testing, recordings of vestibular-evoked myogenic potentials (VEMPs) and measurements of the subjective visual horizontal (SVH) at different body tilts, respectively.

Results In total, 30% of subjects had caloric hypo- or areflexia and 24% had a caloric asymmetry. VEMPs were weak or absent bilaterally in 22% of cases, and asymmetric in 19%. Regarding the utricle, 17% of subjects had a pathologically reduced perception of roll tilt to both sides and 25% had an asymmetry. In total, 30% of subjects were pathologic in all 3 tests and 30% were completely normal. Semicircular canal function correlated best with the function of the saccule. If hearing was better than 90 dB (pure-tone average of 0.5, 1.0 and 2.0 kHz) vestibular function was often normal. For hearing levels of 100–120 dB, otolith function declined significantly.

Conclusions Vestibular function tends to be preserved up to a point where hearing is nearly extinct. Hearing level correlates more closely with otolith function, especially that of the utricle, than with semicircular canal function.     

Influence of cochlear implantation on vestibular function

Conclusion Vestibular function in patients can be damaged following cochlear implantation. Therefore, assessing the pre-operative vestibular status, carefully choosing the side of implantation, and preserving function by using minimally invasive surgical techniques are important. Objectives The aim of this study was to assess the influence of cochlear implantation on vestibular function in patients with severe and profound sensorineural hearing loss, and to analyze a possible correlation between the changes in vestibular testing and post-operative vestibular symptoms. Methods Thirty-four patients were evaluated for vestibular function using the cervical and ocular vestibular-evoked myogenic potentials (cVEMP and oVEMP, respectively), and 29 patients underwent caloric tests pre-operatively and 4 weeks post-operatively. Results Before surgery, the cVEMPs were recorded bilaterally in 22 patients, unilaterally in eight patients, and absent bilaterally in four patients. The oVEMPs were recorded bilaterally in 19 patients, unilaterally in six patients, and absent bilaterally in nine patients. After implantation, the cVEMPs were absent in 10 patients and the oVEMPs were absent in seven patients on the implanted side. Caloric tests demonstrated canal paresis in 17 patients, and normal responses were recorded in 12 of the 29 patients pre-operatively. There was a significant decrease post-implantation in the ear implanted, with the exception of two patients. Two patients presented with vertigo and another two patients reported slight unsteadiness post-operatively, but all symptoms resolved within 7 days. The impaired vestibular function did not correlate with vestibular symptoms, age, or gender. Function on the contralateral side remained unaffected.     

Bilateral Meniere’s disease assessed by an inner ear test battery

Conclusion: An inner ear test battery may suggest bilateral involvement and aid in ‘grading’ of potential disease in a patient with actively symptomatic Meniere’s disease (MD). Objectives: This study applied an inner ear test battery in patients with bilateral MD to map the inner ear deficits in each ear. Methods: From 2009 to 2012, 100 (20%) of 498 MD patients were diagnosed with bilateral involvement, which was defined as established MD in one ear, and the opposite ear had inner ear symptoms combined with documented hearing loss. Each patient underwent audiometry, and ocular vestibular-evoked myogenic potential (oVEMP), cervical VEMP (cVEMP), and caloric tests. Results: Grading of inner ear deficits was based on the number of abnormal results in the inner ear test battery. Of 100 patients with bilateral MD, 54% had the same grade and 46% had different grades in their 2 ears. On the other hand, based on four-tone average, 79% had the same Meniere stage and 21% had different stages in their two ears. The difference between ears with asymmetric grades (46%) and ears with asymmetric stages (21%) accounts for 25% of bilateral MD patients, which may be caused by the saccular hydrops in the opposite ear.     

Sacculo-collic pathway dysfunction accompanying auditory neuropathy

Conclusions. In a patient with bilateral auditory neuropathy (AN), the vestibular-evoked myogenic potential (VEMP) was probably absent because of a neuropathy involving the inferior vestibular nerve and/or its end organ, the saccule. Our result can therefore be interpreted as a concomitant unilateral sacculo-collic neuropathy. We suggest the use of more precise terms to characterize AN patients with involvement of different parts of the inner ear and its innervations. We encourage detailed vestibular assessment in patients with AN in order to assess the co-existence of any symptomatic or asymptomatic vestibular disorder. Information such as that provided in this report will be valuable for clinicians caring for this group of patients. Objective. AN is a disorder characterized by the absence or severe impairment of auditory brainstem responses in the presence of normal cochlear outer hair cell function as revealed by otoacoustic emissions (OAEs) and/or electrocochleography (ECoG). A variety of processes and etiologies are thought to be involved in its pathophysiology. In most literature reports the auditory profile of patients with AN is discussed. However, the extent of vestibular involvement, especially that involving the saccule, is not known. We performed vestibular tests to assess the status of the saccule in a patient with AN. Material and methods. One patient with AN was studied. The patient was a right-handed 21-year-old female with chief complaints of hearing loss and speech perception difficulty. Results. The auditory test results were consistent with the diagnosis of AN, i.e. absent auditory brainstem responses, moderate hearing loss, an inappropriately profound speech discrimination score and the presence of OAEs and measurable cochlear microphonics on ECoG. On neurological examination, gait and balance tests were normal. Ice-water caloric testing induced a sensation of dizziness in both ears. Short tone-burst VEMPs showed no response on left-ear stimulation and a biphasic response with normal latency and amplitude on right-ear stimulation.     

Evolution of Meniere’s Disease from MD 1.0, via MD 1.5, to MD 2.0

Abstract

Background: Elder Meniere’s disease (MD) patients ultimately lose their vestibular function.

Objective: This study utilized an inner ear test battery to investigate evolution of MD.

Methods: Total 278 elder MD patients aged >65 years were divided into three groups. Ninety-four patients with bilateral MD (188 ears) were assigned to Group A. The remaining 184 patients with unilateral MD were further divided into two groups. Group B consisted of 20 affected ears with normal vestibular function on the opposite ears, while Group C indicated 184 unaffected ears. All patients underwent an inner ear test battery.

Results: Inner ear deficits in Group B declined from the cochlea via the saccule, utricle to semicircular canals. In contrast, Groups A and C did not significantly differ in the abnormality rates of cervical vestibular-evoked myogenic potential (cVEMP), ocular VEMP and caloric tests, indicating that Group C (unaffected ears) may partly share the same mechanism like Group A (affected ears), namely aging and hydropic effects.

Conclusion and significance: Evolution of MD may progress from unilateral MD (MD 1.0), via unilateral MD coupled with asymptomatic hydrops on opposite ear (MD 1.5), toward bilateral MD (MD 2.0), where the number 1.0-2.0 means the number of clinically affected ears.     

Sealing of the round and oval window niches with triamcinolone-soaked fascia as salvage surgical therapy in sudden sensorineural hearing loss

Conclusions: Triamcinolone-soaked fascia seems to show better hearing improvement when added to tympanotomy for sudden idiopathic sensorineural hearing loss (SSHL), compared to fascia round window occlusion without triamcinolone.

Objectives: To analyse if adding triamcinolone to sealing the round and oval window niches with fascia results in improved audiological outcome for acute SNHL.

Methods: Fifty-three patients (27m:43?±?12 years, 26f:45?±?14 years) with acute SSHL ≥50dB over 3 frequencies, who failed primary therapy, underwent transcanal tympanotomy. Twenty-five patients (Group A;cortisone:14m, 11f:46?±?9 years) received sealing of the round and oval window with fascia soaked in triamcinolone (1ml; 40mg/ml) and 28 controls (Group B;no-cortisone:13m, 15f, 42?±?12 years) without triamcinolone. Frequency specific and pure tone average (PTA =500–1000–2000–3000Hz) results were compared between Group A and B pre- and postoperatively.

Results: In Group A the PTA improved by ≥10dB in 21/25(83%) cases; in Group B 18/28(63%). Group A showed a statistically significantly better improvement across all frequencies, while linear regression revealed a significant decrease of posttherapeutic PTA to 94.96% of the initial PTA (p?=?.037). The overall PTA improved by 24dB. Group A improved from 73dB to 41dB(-32dB) PTA, Group B improved from 76dB to 56dB PTA (-20dB) (p?p?相似文献   

全聋型突发性耳聋患者的预后与前庭症状及前庭功能关系的回顾性分析

目的 探讨全聋型突发性耳聋患者的预后与前庭症状及前庭功能的关系。 方法 回顾性分析52例单侧全聋型突发性耳聋患者的前庭症状,以及眼肌前庭诱发肌源性电位(oVEMP)、颈肌前庭诱发肌源性电位(cVEMP)、视频头脉冲试验(vHIT)和冷热试验结果,观察这些患者的听力结果与前庭症状及前庭功能的关系。 结果 在52例单侧全聋型突发性耳聋患者中,有前庭症状、前庭功能异常的全聋型患者与无前庭症状者、功能正常者相比疗效差异有统计学意义。oVEMP、cVEMP、vHIT和冷热试验四项结果异常的全聋型患者显示出更低的治疗总有效率,oVEMP、cVEMP、vHIT和冷热试验正常的突发性耳聋患者有更高的听力恢复数值。 结论 有前庭症状、前庭功能异常的全聋型突发性耳聋患者疗效较差,oVEMP、cVEMP、vHIT和冷热试验正常的全聋型突发性耳聋患者有更好的听力恢复。前庭功能异常提示突发性聋患者的内耳病变范围较大、程度较重。     

Vestibular evoked myogenic potentials in patients with bilateral profound hearing loss

We report vestibular evoked myogenic potentials (VEMPs) in 3 patients with bilateral profound hearing loss in order to confirm that they are not of cochlear origin. All of the 3 patients (31-year-old man, 67-year-old man and 47-year-old woman) had bilateral profound hearing loss. They were diagnosed as having congenital hearing loss, bilateral Ménière's disease and inner ear syphilis. Their pure-tone hearing ranged from 81 dB HL to nearly total hearing loss. Stimulation by click (95 dB nHL) evoked biphasic myogenic responses (p13-n23) on the sternocleidomastoid muscle ipsilateral to the stimulated ear. The ear in which the stimulation did not evoke biphasic myogenic responses did not have a caloric response either. These results suggested that VEMPs are not likely of cochlear origin but of vestibular origin.     

Lermoyez syndrome revisited: 100-year mystery

Abstract

Objective: This study revisited seven patients with Lermoyez syndrome over the past 25 years using an inner ear test battery to elucidate its mechanism.

Methods: From 1992 to 2017, we have experienced 4096 patients with Meniere’s disease (MD) and seven patients (5 males and 2 females, 8 ears) with Lermoyez syndrome. Two of the Lermoyez patients were elderly, aged 71 and 85 years. An inner ear test battery comprising audiometry, ocular and cervical vestibular-evoked myogenic potential (oVEMP and cVEMP) tests, and caloric test were performed.

Results: Significant improvement of mean hearing levels (MHLs) was identified at low and middle frequencies after vertiginous attack, but not at high frequencies. Inner ear deficits in Lermoyez patients ran from abnormal hearing (100%) to abnormal cVEMP (43%), caloric (38%) and oVEMP (0) tests, exhibiting a significantly declining sequence. This declining sequence differed from that in MD.

Conclusions: Lermoyez syndrome is extremely rare, with prevalence relative to MD of 0.2%. The mechanism is considered as blockage in the ductus reuniens caused by dislodged saccular otoconia. Aging and trauma are two precipitating factors for the dislodged saccular otoconia, which may explain why Lermoyez syndrome occurs most frequently in males and some elderly.     

Vestibular evoked myogenic potential induced by bone-conducted stimuli in patients with conductive hearing loss

Conclusions. Bone-conducted vestibular evoked myogenic potentials (B-VEMPs) showed high specificity for the presence of vertigo in patients with unilateral chronic otitis media (COM). These results suggest that vestibular function can be evaluated with B-VEMPs, even in patients with conductive hearing loss. Objective. The purpose of this study was to investigate the VEMPs induced by bone-conducted stimuli (B-VEMPs) in patients with conductive hearing loss due to perforated COM. Subjects and methods. The subjects were 48 patients with unilateral perforated COM and conductive hearing loss. The disequilibrium group consisted of 25 patients, and the non-disequilibrium group consisted of 23 patients. The control group comprised 35 healthy volunteers. B-VEMPs were stimulated with tone burst sound of 60 dB nHL and 250 Hz delivered from a bone vibrator and were recorded for each subject. The results of B-VEMP were compared between disequilibrium and non-disequilibrium groups. Results. The mean interaural ratio was 16.5±12.1% in the control group, thus the normal range was <40.7%. Abnormal results were not found in any subject in the non-disequilibrium group but were found in 13 patients (54.0%) in the disequilibrium group (p<0.001). The ear with COM showed lower responses than the intact ear in all subjects with abnormal results.     

An acoustically evoked short latency negative response in profound hearing loss infants

OBJECTIVE: A large negative deflection with a 3-4 ms latency within the auditory brainstem response has been reported in some profound hearing loss ears under intense stimuli in adult subjects. The wave has been termed the N3 potential or acoustically evoked short latency negative response and it is assumed to be a vestibular-evoked potential. The purpose of the current study was to investigate the relationship between the vestibular-evoked myogenic potentials and the acoustically evoked short latency negative response in infants with a functionless cochlea and normal or impaired semicircular canal. METHODS: Seventeen 3 months old infants with profound bilateral sensorineural hearing loss had acoustically evoked short latency negative responses and vestibular-evoked myogenic potentials recorded and caloric tests performed. RESULTS: No spontaneous symptoms of vestibular dysfunction were found in the examined infants. ASNR with medium latency 3.3 ms and threshold value 80-90 dB normal hearing level was elicited from 10 ears. VEMPs were present in 12 ears. ASNRs and VEMPs were absent in two ears with normal response to caloric stimulation. No response to caloric stimulation was elicited from other two ears with normal saccular function. For the ears with absence of ASNR, four had normal VEMP and the rest were considered to have saccular afunction. Significant correlation was found between the presence of ASNRs and VEMPs. CONCLUSION: Acoustically evoked short latency negative responses could be a valuable tool for assessing vestibular function in infants with profound sensorineural hearing loss.