Profound bilateral sensorineural hearing loss during gentamicin therapy

A case of profound bilateral sensorineural deafness occurring during gentamicin therapy is presented. Considerable recovery of the hearing in the right ear was observed but the left ear remained "dead". Electrocochleography confirmed the end-organ nature of the damage. Apart from the profound sudden deafness the unusual feature of this case was the total absence of vestibular effects. The ototoxic effects of gentamicin are discussed including the predisposing factors. While the incidence of ototoxicity with gentamicin is low (2-3%) this figure will only remain acceptable if the use of the drug is restricted to those patients who might die or suffer severe morbidity without it. The factors which make side-effects more likely to occur should always be borne in mind and due attention paid to the monitoring of renal function and serum antibiotic levels.     

Electroconvulsive therapy in therapy refractory depression in the elderly. Report of experiences

The aetiology of idiopathic facial nerve palsy (Bell's palsy) and sudden deafness are not known, although viruses have been suspected as a cause of them. We investigated the relationship between Bell's palsy or sudden deafness, and reactivation of cytomegalovirus, human herpesvirus 6 (HHV-6) and 7 (HHV-7). Paired sera were collected from 22 patients with Bell's palsy and 24 patients with sudden deafness. IgG antibody titres to HHV-7 were increased in one patient with Bell's palsy. IgG antibody titres to HHV-6 were increased in one patient with Bell's palsy and two with sudden deafness. IgG antibody titres to cytomegalovirus were increased in one patient with sudden deafness. Titres of the three viral antibodies were not increased simultaneously in any patients. These viruses may contribute to the development of Bell's palsy or sudden deafness in some cases. It is, however, unlikely that these viruses are the main cause of Bell's palsy and sudden deafness in the majority of patients.     

Human herpesvirus-6 antibodies in idiopathic facial nerve palsy and sudden deafness

Human herpesvirus-6 (HHV-6) is the causative agent for exanthem subitum. This study investigated the relationship between idiopathic facial nerve palsy (Bell's palsy), sudden deafness and HHV-6 infection. Both Bell's palsy and sudden deafness are syndromes which causes are unknown. Both of them are suspected viral infection as causative agents. Paired sera from 22 patients of Bell's palsy and 39 patients of sudden deafness were examined for reactivity to HHV-6 by the indirect immunofluorescence test. On a case of Bell's palsy and two cases of sudden deafness each of the HHV-6 antibody titers was increased.     

The management of sudden deafness (author's transl)

The mean hearing gain (125-8,000 Hz) in 46 cases of sudden deafness treated with low molecular weight dextran was found to be significantly greater (20.9 dB) than that obtained in twelve cases treated differently (9.4 dB). These alternate measures of care included stellate ganglion blockade, vasoactive drugs, sodium bicarbonate infusions, and CO2-insufflation. Early diagnosis and treatment are considered to be crucial for successful therapy: the mean hearing gain in those patients who came for dextran treatment later than one week was less than half of the gain that resulted after treatment beginning within the first week. The results obtained conform with the hypothesis of cochlear microcirculation impairment or "sludging" as the prevalent cause of sudden deafness.     

Mosaic isochromosome 20q and normal outcome: a new case ascertained by fluorescence in situ hybridization and a review of the literature

OBJECTIVE: To explore the mechanisms of sudden deafness treated with integrated traditional Chinese and Western medicine (TCM-WM). METHODS: One hundred and forty-six patients suffering from sudden deafness were divided into two groups, 64 cases of treated group treated with the method of integrated TCM-WM and the other 82 cases treated with Western medicine alone for control. RESULTS: The total effective rate of the treated group was 92.19%, that of the control group was 75.61%. The difference between two groups was significant, P < 0.01. CONCLUSION: The integrated method is very effective in treating sudden deafness. Its mechanism might be: (1) Decrease the blood viscosity and increase the red blood cell's tenacity, so that the blood stream is passing through unimpeded. (2) Decrease the plasma TXA2 level and increase the PGI2 level, keep the blood supply for inner ear. (3) Increase the activity of SOD and clear away the free radicals so as to reduce the damage of inner ear.     

Quantification of aortic stenosis in mechanically ventilated patients using multiplane transesophageal Doppler echocardiography

OBJECTIVE: The development of sudden postoperative hearing loss as a complication of microvascular decompression (MVD) operations in the cerebellopontine angle has already been reported. A sudden hearing loss of vascular origin may also occur hours or days after such operations, but even in such cases an improvement of hearing over the following weeks is possible. Here we report on a gradual deterioration of hearing over a period of two weeks after MVD which has not been described in the literature up to now. CLINICAL PRESENTATION: A MVD operation was performed twice on a 36 year old patient with trigeminal neuralgia. After the second operation the patient developed a slight hearing impairment 3 days postoperatively which increased over a period of two weeks and ended up with total deafness. The course of intra-operative brainstem auditory evoked potentials and postoperative audiograms is documented. CONCLUSION: Because of gradual development of the delayed hearing loss, we conclude that postoperative tissue scarring may be the underlying pathology.     

Immunological abnormalities in patients with etiology unknown sensorineural hearing loss

An immunological study was carried out in 50 patients with etiology unknown sensorineural hearing loss, including the following: 12 low tone deafness, 7 sudden deafness, 8 unilateral deafness, 7 idiopathic bilateral progressive sensorineural hearing loss and 14 other bilateral sensorineural hearing loss. Twenty-five out of 50 of the cases demonstrated immunological abnormalities. A full array immunological tests were performed with the following results. High immnunoglobulin titers showed in 18 out of 50 cases. Six cases were positive for antinuclear antibody. The anti-DNA antibody assay revealed high titers in 2 cases. Also, anti-rheumatoid factor antibody assay showed high titers in 6 cases while abnormal values of complements in serum were detected in 6 cases, though an immune complex was demonstrated in only one case. Also, it was noted that six patients with immunological abnormalities have autoimmune diseases. The above results indicated that some cases of etiology unknown sensorineural hearing loss may have an immunological link.     

Geographical incidence of infection with Borrelia burgdorferi in Europe

Sudden deafness and vertigo are a challenge for the otolaryngologist. With its high fluid content, the membranous labyrinth is best evaluated through Magnetic Resonance Imaging (MRI). Recently gadolinium-enhanced MRI has opened new perspectives in the imaging of the pathological labyrinth, able to directly detect labyrinthine lesions. To date the main role of MRI has been to rule out the presence of retrocochlear involvement in patients with sudden deafness and/or vertigo. However, in the past few years, several authors, reporting on gadolinium-enhanced MRI, have described better labyrinthine imaging, revealing direct labyrinth or nerve bundle involvement in all such patients. On the contrary, other studies have not revealed any enhancement of post-contrast labyrinthine images upon MRI but have shown an increase in signal intensity upon T2-weighted images. In this light, the purpose of the present study has been a) to evaluate the prevalence of labyrinthine abnormalities found upon MRI in consecutive patients with sudden deafness and vertigo and b) to assess the correlation between the severity of clinical and audiological findings and the MRI abnormalities encountered. Twelve consecutive patients with sudden hearing loss and/or vertigo were included in the study (age range 9-59 years; 6 males and 6 females). All subjects underwent complete otoneurological examination. MRI was performed with a 0.5 T superconducting magnet system allowing 2 mm-thick sections through the temporal bone. Within 29 days of onset of the complaints all patients were studied both before and after administration of the contrast medium (gadolinium-DTPA, 0.01 mmol/l, i.v.). Two of the 12 subjects had sudden deafness, 5 sudden deafness and vertigo while 5 had vertigo alone. Gadolinium enhancement and/or a high signal intensity upon T2-weighted images of the pathological labyrinth and nerve bundle was observed in 4 patients. The patients were divided into groups by etiology. MRI abnormalities were only found in the group where the etiology was viral. There was no correlation between the severity of clinical findings and the presence of MRI abnormalities nor between MRI and ABR findings. Analysis of such controversial data is discussed, stressing the importance of gadolinium-enhanced MRI of the labyrinth as a new diagnostic tool in sudden deafness and vertigo.     

Confidentiality and privacy breaches in a university hospital emergency department

Six temporal bones and a brain tissue sample removed at autopsy from four patients with bilateral sudden hearing loss related to gastric adenocarcinoma were histologically studied. The pathological remains suggest that the sudden hearing loss of these patients may have occurred via one of two different mechanisms: (1) metastasis to the internal auditory meatus damaging the auditory nerve or (2) inner ear haemorrhage damaging Corti's organ. These two mechanisms may cause bilateral sudden deafness in patients with gastric adenocarcinoma.     

Changes in otoacoustic emissions in patients with idiopathic sudden deafness

Transiently click-evoked (TEOAE), distortion product (DPOAE) and spontaneous otoacoustic emissions (SOAE) were recorded and changes in these tests were studied during the recovery process in 15 cases of idiopathic sudden deafness (ISD). In all these cases the amplitudes of TEOAEs and DPOAEs increased concurrently with the recovery of the hearing threshold. Ears with ISD were not different in their OAE characteristics from ears with other forms of sensorineural hearing loss (SNHL). In 4 of the 15 cases, SOAEs could be detected when hearing had recovered. These results suggest that the function of outer hair cells (OHCs) had deteriorated when the hearing threshold was elevated and that OHC activity recovered as hearing improved to nearly normal levels in ISD cases with good outcome.     

Linkage of congenital, recessive deafness (DFNB4) to chromosome 7q31 and evidence for genetic heterogeneity in the Middle Eastern Druze population

Clinically significant hearing loss affects 1 in 1000 infants and it is estimated that at least 50% of these cases are due to a genetic cause. Some forms of inherited deafness are syndromic and affected individuals have a specific pattern of additional features while in other families the deafness is non-syndromic and there is no other recognizable phenotype. Analysis of several large families with syndromic and non-syndromic forms of deafness have been used in genetic linkage analysis to identify genes or gene locations that cause deafness. Here, we describe a large Middle-Eastern Druze family with recessive non-syndromic deafness and demonstrate linkage between deafness in this family and human chromosome 7q31 with a lod score exceeding 5.5. This is the first evidence for a gene at this location that causes deafness. In addition, we found that deafness in three other Druze pedigrees, one related to the linked family, is not linked to this chromosomal location. This suggests that there are multiple nonallelic mutations for deafness in this genetic isolate.     

Deafness in the Muckle-Wells syndrome

Typical of the syndrome described by Muckle and Wells is a combination of progressive perceptive deafness appearing at various ages in a family, but usually at the same age in the same family, arthralgia, urticarious eruption and renal amyloidosis. The existence of incomplete forms, either without deafness or without renal amyloidosis should be known. This perceptive deafness is very probably due to lesion of the internal ear but the histological characteristics do not seem to be very clear at the present time. Finally, as certain of our observations show, in a few cases, conduction may be impaired, either in isolation, or associated with impairment of perception. Only an analysis of a large number of cases will enable the audiological characteristics of this curious syndrome to be defined.     

Mitochondrial ribosomal RNA gene mutation in a patient with sporadic aminoglycoside ototoxicity

PURPOSE: Aminoglycoside-induced deafness has been described in a number of Chinese pedigrees. In nearly all of these families, affected individuals were related through the maternal side. Because mitochondrial DNA is transmitted exclusively through mothers, it had been speculated that a mutation in the mitochondrial DNA might predispose these maternally related family members to aminoglycoside ototoxicity. Recently, we analyzed three such families with multiple cases of ototoxic deafness and identified a pathogenic mutation in the mitochondrial 12S ribosomal RNA gene at nucleotide position 1555. The purpose of the current study is to analyze individuals with no family history of deafness, who had severe hearing loss after aminoglycoside exposure, for presence or absence of this particular mitochondrial DNA mutation. MATERIALS AND METHODS: Blood was obtained from 36 Chinese individuals who became deaf after aminoglycoside exposure and had no family history of deafness. The DNA of these individuals was extracted, amplified by the polymerase chain reaction, and analyzed for the mitochondrial ribosomal RNA gene mutation by allele-specific oligonucleotide hybridization and Southern blot analysis. RESULTS: In one of these 36 sporadic cases, we identified the nucleotide 1555 A-->G mutation in the mitochondrial genome. CONCLUSION: This finding implies that a small proportion of individuals at risk for aminoglycoside ototoxicity harbor the specific mitochondrial DNA mutation identified in the familial cases. In these individuals, a genetic susceptibility to the ototoxic effects of aminoglycosides can be diagnosed, and deafness can be prevented in maternal relatives by avoiding the use of these antibiotics.     

Hemorheological disorders in patients with sudden deafness]

Fourteen hemorheological and related parameters of blood samples derived from 59 patients with sudden deafness were observed and were compared with those of blood samples got from 512 healthy persons. The ratio viscosity of whole blood (6.50 +/- 1.07), reduced viscosity of whole blood (12.37 +/- 1.80), and plasma viscosity (1.84 +/- 0.14) in male patients were significantly higher than those (6.05 +/- 0.63, 11.06 +/- 1.04, and 1.70 +/- 0.07, respectively) in male controls. The reduced viscosity of whole blood (16.62 +/- 3.87) and plasma viscosity (1.92 +/- 0.13) in female patients were also significantly higher than those (14.40 +/- 2.27 and 1.72 +/- 0.06, respectively) in female controls. The findings above suggest that there is an intimate relationship between the microcirculationary disorder resulting from hemorheological changes and the onset of sudden deafness. The parameters related to plasma viscosity revealed that there were higher concentrations of plasma fibrinogen and triglyceride in male patients than those in male controls, while the concentrations of plasma cholesterol and triglyceride were higher in female patients than in female controls. These results indicate that the factors which induce the change of plasma viscosity in male patients could not be the same as those in female patients. Thus, in the process of treating sudden deafness, besides the use of common devices such as reducing blood viscosity and improving microcirculation, some other special steps based on the hemorheological parameters derived from different sexes or individuals, should also be considered.     

Immune-mediated inner ear disease: report of clinical cases

Many clinical and experimental studies have obtained evidence of immune-mediated inner ear disease. Discrepancies between theories of the mechanisms of injury to the inner ear and the laboratory tests that identify it mean that the diagnosis is based on clinical symptoms and a positive response to treatment. We report four cases of immune-mediated inner ear disease characterized by endolymphatic hydrops, fluctuating hearing loss, sudden deafness (first symptom of primary Sj?gren's syndrome), and rapidly progressive sensorineural hearing loss.     

Implantation of multiple intracochlear electrodes for rehabilitation of total deafness: preliminary report

Many instances of total deafness are due to destruction of the organ of Corti but with partial or complete preservation of the function of the cochlear nerve. In such cases, it is possible to restore some hearing by electrically stimulating the fibers of the cochlear nerve with the help of implanted electrodes. Preoperative testing with electric shocks applied to the round window have aroused sensations of noise in 45 cases of total bilateral deafness with a great variety of etiologies. The only negative results were in two cases of operated acoustic neuromas. Our operation places up to eight intracochlear electrodes, each with a separate fenestration opening into an electrically isolated compartment of the scala tympani. Stimulation of each electrode yields a different sound sensation of a pitch that depends on its location along the cochlea. Electric filters direct different frequency bands to the appropriate electrodes, with the necessary compression of dynamic range. In three experimental cases of unilateral deafness, pitch matches to the normal ear were made. In seven therapeutic operations on adult cases of acquired total bilateral deafness, speech recognition was usually relearned within a month or two. Improvement of voice quality was also dramatic. The intracochlear electrodes have been well tolerated for months, but the method of connection to the external equipment still presents difficulties.     

Juvenile psychogenic hearing disorders

Psychogenic hearing problems exist even in childhood. They must be differentiated from the stimulated or aggravated hearing losses. A characteristic is the discrepancy between the understanding during conversation and the bad results of the hearing tests. The psychical case history of the patient shows his conflict-situation and personal problems. If the psychogenic hearing problems do not improve on their own or by means of oto-rhinolaryngical placebo-therapy, psycho-therapy will be needed. Taking the example of 2 children, where the suspected primary diagnosis was sudden deafness, the problems of diagnosis was shown.     

Sudden deafness and Barlow disease

From January 1993 to December 1994 twelve patients were evaluated for sudden hearing loss. The median age was 49 years with a range of 18 to 71. All had severe or profound initial hearing loss. The incidence of bilateral disease was 25%. Total deafness occurred in five (33%) ears. Nine (75%) patients had vestibular symptoms and eight (67%) admitted experiencing tinnitus. Two-dimensional echocardiography revealed mitral prolapse in eight (67%) patients; another patient showed moderate to severe ischemic left ventricular dysfunction with apical aneurysm.     

Cardiomyopathic lentiginosis/LEOPARD syndrome presenting as sudden cardiac arrest

A 26-year-old apparently healthy man with numerous pigmented skin lesions collapsed during an evening party and was resuscitated from ventricular fibrillation. Hypertrophic cardiomyopathy and subaortic tunnel were disclosed by angiocardiography. A diagnosis of cardiomyopathic lentiginosis/lentigines (multiple), electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of the genitalia, retardation of growth, and deafness (sensorineural) syndrome was made. The patient then underwent treatment with an implantable pacer-cardioverter-defibrillator device. Further evaluation revealed several well-established features of the disorder. This is the first reported case of survival from ventricular fibrillation associated with this rare and little known multifaceted syndrome. Disseminated lentiginosis must prompt clinicians to evaluate such cases further since underlying disorders may be associated with considerable morbidity and, apparently, sudden death.     

Mitochondrial DNA mutation at nucleotide 1555 in a patient with bilateral sensorineural hearing loss of unknown etiology

A mitochondrial DNA mutation at nucleotide 1555 in the ribosomal RNA gene was recently reported as a cause of maternally inherited non-syndromic sensorineural deafness. We assumed that the 1555 mutation is also associated with sporadic non-syndromic deafness and screened for the mutation in seven randomly selected sporadic cases with bilateral sensorineural hearing loss of unknown etiology. The mutation was found in one patient, who first noticed hearing loss when she was in her early teens with subsequent gradual progression. The results suggest that the 1555 mutation may contribute to the etiology of idiopathic bilateral sensorineural hearing loss in some cases.