Intravenous gentamicin therapy in bilateral Menière disease

BACKGROUND: The treatment of severe forms of bilateral Menière's disease remains an especially challenging task. Similar problems also occur in debilitating Menière's disease in the only hearing ear. The intramuscular titration therapy with streptomycin has been the means of choice since 1984 to minimize the risk of total hearing loss in cases of severe bilateral disease. METHOD: Since 1989 we have treated six out of 21 cases of bilateral Menière's disease by intravenous application of 2 x 120 mg gentamicin in Ringer's solution for several days. Additionally we reported on two cases in 1988. Only minor amounts of gentamicin were applied to sedate the function of both vestibular organs while avoiding damage to the cochlea. RESULTS: In two cases hearing approved approximately about 10 dB, in two cases hearing remained stable, and in two cases hearing worsened about 10 dB. Five of six patients showed minor excitability in caloric tests on both sides, they did not complain of vertigo attacks one to five years after therapy. CONCLUSION: Given that only very small amounts of gentamicin are applied to sedate the function of the vestibular organ while causing almost no damage to the cochlea, this method seems to be an excellent means for treatment of bilateral Menière's disease. Patients do not experience severe problems with equilibrium afterwards, and the treatment can be repeated as often as necessary.     

Mutations in the COCH gene are a frequent cause of autosomal dominant progressive cochleo-vestibular dysfunction, but not of Meniere's disease

The COCH gene is the only gene identified in man that causes autosomal dominantly inherited hearing loss associated with vestibular dysfunction. The condition is rare and only five mutations have been reported worldwide. All affected families showed a similar progressive hearing loss and vestibular dysfunction. Since Meniere's disease-like symptoms have also been described in some families, it was suggested that COCH mutations might be present in some patients diagnosed with Meniere's disease. In this study, using a Japanese population, we performed a COCH mutation analysis in 23 patients from independent families with autosomal dominant hearing impairment, four of whom reported vestibular symptoms, and also in 20 Meniere's patients. While a new point mutation, A119 T, was found in a patient with autosomal dominant hearing loss and vestibular symptoms, no mutations were found in the Meniere's patients. Like all other previously identified COCH mutations, the mutation identified here is a missense mutation located in the FCH domain of the protein. The current mutation is located in close spatial proximity to W117, in which a mutation (W117R) had previously been associated with autosomal dominant hearing loss. Model building suggests that, like the W117R mutation, the A119 T mutation does not affect the structural integrity of the FCH domain, but may interfere with the interaction with a yet unknown binding partner. We conclude that mutations in the COCH gene are responsible for a significant fraction of patients with autosomal dominantly inherited hearing loss accompanied by vestibular symptoms, but not for dominant hearing loss without vestibular dysfunction, or sporadic Meniere's disease.     

Large-scale purification and preliminary x-ray diffraction studies of human aspartylglucosaminidase

Selective vestibular neurectomy is an effective treatment for intractable vertigo of peripheral vestibular origin when preservation of hearing is a goal. The retrolabyrinthine and retrosigmoid-intracanalicular approaches have been used predominantly at our institutions over the last 10 years. The results and complications of these two techniques were compared. No significant differences were found between hearing results in these two patient groups. The retrosigmoid-internal auditory can approach yielded better control of recurrent episodic vertigo, as well as superior ablation of postoperative ice-water caloric responses (p < 0.05). Surgical complications, including the incidence of cerebrospinal fluid leakage (greater in retrolabyrinthine approach) and postoperative headache (more prevalent in retrosigmoid approach), were also analyzed. To further evaluate the results of this study, data were reanalyzed and compared with previously published reports of selective vestibular nerve section.     

Neonatal hearing impairment complicated by neonatal pneumonia. Application of auditory evoked potentials in neonatology

A group of 433 neonates with pneumonia were studied through the monitoring of audiology on the basis of epidemiology in perineonatal hearing impairment and applied auditory evoked potentials. The results showed that 29 cases had a threshold of more than 30 dBnHL in primary auditory brainstem response test (6.7%), and 19 cases with threshold, more than 40 dBnHL in primary 40Hz-auditory event related potential (40Hz-AERP) test (4.4%). The follow-up between the 3rd and 12th month after the initial test demonstrated that 12 cases had hearing impairment to various degrees.     

Acoustic neuroma: correlations between morphology and otoneurological manifestations

Forty-two patients with acoustic neuroma (AN) were studied to determine whether different types of neuroma could be correlated with specific signs and symptoms of the disease. Based on gadolinium-enhanced TI-weighted MRI sequences, the 42 cases of AN could be divided into three groups, either by size (small: 11.9%, medium: 50%, and large: 38.1%) or by site of origin of the tumour (lateral: 16.7%, intermediate: 69%, and medial: 14.3%). Relations were found between the size and the site of origin of the neuromas and certain clinical, audiological and vestibular findings. The clinical presentation seemed to vary with the site of origin and the size of the tumour: patients with lateral neuromas generally had small tumours, sometimes only located in the internal auditory canal (IAC), and presented early subjective hearing loss while patients with medial neuromas had larger tumours which grew without causing significant audiological symptoms. Normal hearing function was seen only in the patients with medial ANs; however, a significant relation between the size or the site of origin of the AN and the average hearing threshold was not demonstrated. The sensitivity of the stapedial reflex test (SR) was higher for lateral ANs. Anomalies in the brainstem auditory evoked potentials (BAEPs) did not seem to be related to either the size or the site of origin of the AN. The vestibular tests demonstrated a higher frequency of central vestibular involvement in the large tumours, while normal function was more frequent in the lateral tumours. In the group studied the combination of BAEPs and vestibular tests allowed us to identify all the ANs with an optimal level of sensitivity.     

Mixed effect modeling of sumatriptan pharmacokinetics during drug development. I: Interspecies allometric scaling

A sample of 22 subjects was studied from a population of adults who had suffered from bacterial meningitis in childhood. Audiovestibular, oculomotor and neuropsychological investigations were performed and quality of life was assessed. An age-matched control group of 20 subjects was recruited. In the meningitis group, nine subjects had abnormal pure tone audiograms. One was previously undiagnosed and a progression was found in four. There was an overrepresentation of subclinical vestibular pathology (6 out of 9 (67%)) in this group. Audiovestibular test results showed a peripheral pattern and oculomotor tests were normal. The quality of life scores of those with hearing loss were significantly higher than those in the control group. Neuropsychological tests of brain dysfunction were abnormal in six out of 22 (27%) who had recovered from meningitis. The prevalence of such dysfunctions was not related to audiovestibular disorder. The quality of life scores of those with brain dysfunctions were similar to those of the control group. The findings of reduced auditory memory and tone level perception in four out of 22 (18%), suggest that lesions of central auditory pathways may follow from bacterial meningitis. The results support the idea that inner ear damage is the major cause of hearing loss after bacterial meningitis. Despite the absence of brainstem involvement, central nervous system lesions with disturbed auditory processing and language functions can be of significance. The high frequency of discrete brain dysfunctions indicate that a thorough neuropsychological investigation is required after bacterial meningitis.     

Vestibular habituation in paralyzed cats.

Studied the role of the eye movement response in the production of vestibular habituation in 12 cats. One group of Ss was subjected to an habituation series of 15 unilateral caloric irrigations while paralyzed with gallamine triethiodide. A 2nd group underwent an identical habituation procedure but was not paralyzed. Tests conducted following recovery from paralysis showed that the responses of the previously is Ss were habituated and did not differ from the responses of Ss habituated in a normal manner. Data support a central origin for the phenomenon of vestibular habituation. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Region-specific changes in GABAA receptor delta subunit mRNA level by tolerance to and withdrawal from pentobarbital

This paper is, aiming at understanding disturbances of and correllation between colchear and vestibular functions in Meniere's disease. Auditory and vestibular functions in 121 Meniere cases (70 males and 51 females, age ranging from 19 to 63 years), diagnosed according to the AAO-HNS criteria, were analysed. The results were as follows: 1)There was positive correlation between semicircular canal paresis and hearing impairment. 2) There was statistically significant positive correlation between hearing impairment and the -SP/AP ratio. 3) There was statistically significant correlation between hearing impairment and glycerin test result, the rate of positive result being higher in those with advanced hearing loss than in those with mild loss. 4) There was correlation between the rate of positive glycerin test result and audiogram configuration. The rate with rising audiogram pattern was higher than with that falling pattern (69.0% vs 33.3%). It was also found that ECochG was far more effective than glycerin test in diagnosing Meniere's disease. The physiologic basis and significance of ECochG and glycerin test in Meniere's disease were discussed.     

Functional mapping of the U3 small nucleolar RNA from the yeast Saccharomyces cerevisiae

The 2 f1-f2 distortion product otoacoustic emission (DP) was measured in 20 normal hearing subjects and 15 patients with moderate cochlear hearing loss and compared to the pure-tone hearing threshold, measured with the same probe system at the f2 frequencies. DPs were elicited over a wide primary tone level range between L2 = 20 and 65 dB SPL. With decreasing L2, the L1-L2 primary tone level difference was continuously increased according to L1 = 0.4L2 + 39 dB, to account for differences of the primary tone responses at the f2 place. Above 1.5 kHz, DPs were measurable with that paradigm on average within 10 dB of the average hearing threshold in both subject groups. The growth of the DP was compressive in normal hearing subjects, with strong saturation at moderate primary tone levels. In cases of cochlear impairment, reductions of the DP level were greatest at lowest, but smallest at highest stimulus levels, such that the growth of the DP became linearized. The correlation of the DP level to the hearing threshold was found to depend on the stimulus level. Maximal correlations were found in impaired ears at moderate primary tone levels around L2 = 45 dB SPL, but at lowest stimulus levels in normal hearing (L2 = 25 dB SPL). At these levels, 17/20 impaired ears and 14/15 normally hearing ears showed statistically significant correlations. It is concluded that for a clinical application and prediction of the hearing threshold, DPs should be measured not only at high, but also at lower primary tone levels.     

Disturbed eye movements after whiplash due to injuries to the posture control system

Self-reports after whiplash often indicate associations with vertigo and reading problems. Neuropsychological and otoneurological tests were applied to a group of whiplash patients (n = 26) and to a carefully matched control group. The whiplash group deviated from the control group on measures of eye movements during reading, on smooth pursuit eye movements with the head in normal position, and with the body turned to the left or to the right. Clinical, caloric, and neurophysiological tests showed no injury to the vestibular system or to the CNS. Test results suggest that injuries to the neck due to whiplash can cause distortion of the posture control system as a result of disorganized neck proprioceptive activity.     

Delayed endolymphatic hydrops: an analysis of 12 cases

Delayed Endolymphatic Hydrops (DEH) is a clinical entity which is characterized by an early phase with a profound sensorineural hearing loss in one ear. After a prolonged period of time (from 1 to 68 years) a late phase of the disease appears with different otologic symptoms. The ipsilateral type of endolymphatic hydrops appears in the deaf ear with consequent episodic vertigo. The contralateral type develops symptoms of endolymphatic hydrop in the previously normal ear, with the onset of fluctuating hearing loss and/or episodic vertigo. This paper is a review of 12 cases of DEH (7 ipsilateral and 5 contralateral, age ranging from 19 to 79 years). Diagnosis was established on the basis of clinical history and the results of audio-vestibular investigations, specifically designed to detect signs of endolymphatic hydrops (including the glycerol-test and neuroradiological imaging of the cerebello-pontine angle). In 8 patients the levels and frequency of anti-collagen I-II-IV-V, anti-laminin autoantibodies, complement, Ig and immuno-complexes were measured. Viral infections (measles, mumps, influenza) were thought to have caused the early phase in 6 cases. In the entire group of 12 patients the delay between the onset of the ear loss and vertigo averaged 12 years. In the group of the ipsilateral DEH, caloric tests showed in 5 cases a reduced or absent response of the deaf ear. About contralateral DEH, caloric tests showed bilateral reduced response in 2 cases, and, in 3 cases, a decreased response only in the previously normal ear. Regarding immunological study, an abnormal level of Ig and Complement was detected in 3 patients.(ABSTRACT TRUNCATED AT 250 WORDS)     

Hearing, speech, language, and vestibular disorders in the fetal alcohol syndrome: a literature review

Fetal alcohol syndrome (FAS) is characterized in part by mental impairment, as well as craniofacial and ocular anomalies. These conditions are traditionally associated with childhood hearing disorders, because they all have a common embryonic origin in malformations of the first and second branchial arches, and have similar critical periods of vulnerability to toxic insult. A review of human and animal research indicates that there are four types of hearing disorders associated with FAS. These are: (1) a developmental delay in auditory maturation, (2) sensorineural hearing loss, (3) intermittent conductive hearing loss due to recurrent serous otitis media, and (4) central hearing loss. The auditory and vestibular systems share the same peripheral apparatuses (the inner ear and eighth cranial nerve) and are embryologically and structurally similar. Consequently, vestibular disorders in FAS children might be expected. The evidence for vestibular dysfunction in FAS is ambiguous, however. Like other syndromes associated with craniofacial anomalies, hearing disorders, and mental impairment, FAS is also characterized by a high prevalence of speech and language pathology. Hearing disorders are a form of sensory deprivation. If present during early childhood, they can result in permanent hearing, language, and mental impairment. Early identification and intervention to treat hearing, language, and speech disorders could therefore result in improved outcome for the FAS child. Specific recommendations are made for intervention and future research.     

Hearing impairment in two boys with Cornelia de Lange syndrome

We analyzed brainstem auditory evoked potentials in 2 boys with Cornelia de Lange syndrome. One patient showed no responses with 100 dB hearing level (HL) stimuli. The other showed a normal latency of wave I, a normal I-V interpeak latency with 80 dB HL stimuli, and an elevated threshold of wave V (40 dB HL). It is likely that these abnormal findings reflect the presence of sensorineural hearing impairment.     

Comparison of methods for identifying transcription units and transcription map of the Werner syndrome gene region

OBJECTIVE: The audiologic presentation of vestibular schwannoma (VS) associated with neurofibromatosis type 2 (NF2) has not been well characterized. The goal of this study was to investigate the audiologic features of NF2-associated VS and to determine their relationship to the size of the tumor. STUDY DESIGN: A retrospective case review. SETTING: Quaternary governmental medical research institute evaluating patients fitting specific criteria for ongoing clinical studies. PATIENTS: Audiologic and magnetic resonance imaging data were available for 40 patients (25 males, 15 females), with an average age of 32 years, who had been recruited for ongoing clinical and genetic studies of NF2. MAIN OUTCOME MEASURES: The audiologic profile and magnetic resonance imaging characteristic of tumor were retrospectively reviewed. RESULTS: The average size of the tumor at presentation was 7.26 +/- 16.58 cm3 and measured 1.2, 1.6, and 1.1 cm in the anterior/posterior, lateral/medial, and superior/inferior dimensions, respectively. An increase in lateral/medial size of the tumor most significantly correlated with deterioration in mid- (1,000-2,000 Hz) and high- (4,000-8,000 Hz) frequency hearing levels, elevated speech reception threshold, and prolonged auditory brain stem response waves III and V latency. CONCLUSIONS: Patients with NF2 demonstrate a more predictable audiologic profile for a given size tumor than has been previously described with spontaneous or sporadic VS.     

Diabetic complications and hypoacusia

The relationship between diabetes mellitus and hypacusia has been discussed since the work of Jordao, in 1857. Numerous authors are openly pro this viewpoint, whereas other deny it. The hypoacusis is typically bilateral, progressive and of neurosensory nature, affecting the higher frequencies. In the etiologic aspect some authors bet for an angiopathic origin, but others suppose a neuropathic cause. We have achieved a comparative study with the aim to get some conclusions that could enlighten this field. Forty patients with diabetes mellitus type II were audiometrically examinees (at threshold and with supraliminar tests) and the results compared with those of a control group. We also assessed several complications of the disease and their possible report with the auditive impairment (retinopathy and neuropathy). We found out a sensorineural, bilateral and symmetrical hearing impairment. Employing the Man Whitney's U test we compare distinct groups and explain the gained outcomes. Bibliographic review of authors defending the vascular or the nervous starting point.     

Audiometric and ophthalmological findings in rubella deafness

In an attempt to study the relevance of ophthalmological changes to the diagnosis of cochlear hearing impairment due to fetal rubella infection, a survey of the literature combined with a clinical investigation was carried out. Based on the investigation of 57 patients, 31% had congenital hearing impairment due to fetal rubella infection. Of these patients, 61% had typical rubella retinal changes. In the remaining 69%, ophthalmoscopy revealed no abnormality, except in 1 patient. As the cause of the hearing impairment in this patient was unknown, it was concluded that the mother had suffered from subclinical rubella infection in the first trimester of her pregnancy. It is concluded that rubella retinitis is found with such a high incidence in rubella children that it can be used as a tool in the diagnosis of cochlear hearing impairment due to fetal rubella infection. Patients with congenital hearing impairment ought to undergo a routine ophthalmoscopy which will detect eventual pigmentary changes.     

Total unilateral deafness in children. Etiologies and long-term consequences

A retrospective study of 72 children with a profound (average hearing loss > 90 dB) unilateral sensorineural hearing loss (PUSNHL) was carried out in the ENT department at the Rouen hospital in 1988 and 1994. This study intended to investigate the etiologies, how it was recognized, the age of the onset and the results of the medical evaluation of these PUSNHL. We found, in 2 cases, that vestibular lesions of the healthy ear were present before the onset of a hearing loss on this side, turning the PUSNHL into a bilateral deafness. We tried to correlate the etiologies, age of onset of the PUSNHL, status of the opposite ear and possible scholar problems in the group of the 42 children followed-up for more than 2 years (mean follow-up time = 7 years). Among children suffering from PUSNHL, we specified a group with higher risk of school failures, allowing ENT physicians to propose more accurate management to these children.     

Macrolides in the management of streptococcal pharyngitis/tonsillitis

OBJECTIVE: This study aimed to compare the hearing changes in the long term after vestibular neurectomy, endolymphatic mastoid shunt, and medical treatment in classic Meniere's disease. STUDY DESIGN: A retrospective case review was conducted based on audiologic follow-up between 5 and 21 years. SETTING: The study was performed at two centers in Bari University Hospital, one performing vestibular neurectomy as the first surgical procedure for Meinere's disease and the other, endolymphatic mastoid shunt. PATIENTS AND INTERVENTIONS: Of 68 patients with intractable idiopathic Meniere's disease, 29 underwent middle fossa vestibular neurectomy, and 17 had endolymphatic mastoid shunt; 22 were offered surgery but declined. MAIN OUTCOME MEASURES: Outcome measures were puretone average (PTA), speech reception threshold, and speech discrimination score before and after treatment. RESULTS: PTA declined by an average of 9.3 dB in neurectomy patients, 13.3 dB in patients undergoing endolymphatic mastoid shunt, and 18.1 dB in patients who were offered surgery but declined. Patients were subdivided into two cohorts based on their preoperative or initial PTA. In the patients who had PTA scores worse than 50 dB initially, the PTA declined an average of 4.3 dB in the vestibular neurectomy group, 11.5 dB in the endolymphatic sac group, and 4 dB in the nonsurgical group. In the patients with PTA > or = 50 dB initially, the PTA declined an average of of 25.3 dB in the vestibular neurectomy group, 16.1 in the endolymphatic sac group, and 26.2 dB in the nonsurgical group. Although shunt patients with good hearing initially deteriorated less than neurectomy patients and less than patients who declined surgery, the difference was not significant. CONCLUSIONS: These results indicate that patients with poor hearing stabilized, while patients with good hearing continued to deteriorate. The same conditions were observed in the patients who had surgery and those who were offered surgery but declined.     

Some hematimetric findings in human Giardia lamblia infection

During an one-hour lecture 277 pupils between 16 and 25 years of age, were questioned about their hearing behaviour and instructed in the consequences of hearing impairment resulting from listening to loud music. Furthermore audiometrics were offered and there was possibility to measure the loudness of the walkman used. A majority visited "discos" and/or used walkman. Over 80% claimed their hearing is good, over 75% knew that loud music can cause damage to hearing. The walkman users suffered more frequently from tinnitus and failed to hear a door bell more often. After the instruction 75% of the pupils were willing to protect their hearing in future. Eight of 89 pupils had a hearing reduction of up to 40 dB(A). The average sound pressure level of the walkman was 104 dB(A).     

Intensity and frequency discrimination monitored by evoked response audiometry: clinical application

Delta 1 and delta f repetitive stimuli superimposed on continuous 0.5-, 1- and 4-kHz carrier tones were administered to 20 normally hearing persons, 8 adults with sensorineural hearing loss, 10 adults with conductive hearing loss and 22 children subjected to evoked response audiometry (ERA) for assessment of hearing acuity. The intensity modulation depth ranged from + 1 to 10 dB. In the subjects with normal hearing and in the adult patients, three carrier tone levels were used whenever possible: 20, 40, and 60 dB SL. The frequency modulation depth ranged from -1 to -10% of the carrier tone frequency. The same levels for the carrier tone were used: 20, 40, and 60 dB SL. With a carrier tone level of 20 dB SL, a clear-cut vertex response was noted in 80% of the tests carried out in the normal adults with 5-dB delta i stimuli and with delta f stimuli of -3 to -5% in magnitude; the same figures are valid for the patients with sensorineural and conductive hearing loss. The patients with sensorineural hearing loss showed a tendency to give vertex responses with lower delta i stimuli than subjects with normal hearing. However, this difference was not statistically significant. According to our results, the children tested can be roughly divided into two groups, the first including subjects with responses to delta i stimuli of +5 and +10 dB and delta f stimuli of -5% and -10%; the second group including subjects with no clear-cut responses to these stimuli.