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Abstracts

Scientific Abstracts:

Page: 12

Hearing Loss

ABSTRACTS

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Evaluation of tinnitus patients by peroral multi-drug treatment.

Ohsaki K, Ueno M, Zheng HX, Wang QC, Nishizaki K, Nobuto Y, Fujimura T.
Division of Clinical Otology, University Hospital, University of Tokushima, School of Medicine, Japan.

Auris Nasus Larynx 1998 May;25(2):149-54

To evaluate patients complaining of subjective tinnitus, this study examined their response to peroral betahistine mesilate, vitamin B complex and diazepam in combination. Because three drugs were used together, it remains to be seen whether a single drug or a combination of drugs was effective. We issued questionnaires to 67 patients with tinnitus associated with sensorineural hearing loss of unknown etiology or tinnitus, despite normal hearing in pure tone audiometry and lack of distinct systemic disorders. Our original questionnaire contained seven items and allotted points for each item to facilitate evaluation. After prescribing the above drugs and observing patients' progress for 5 weeks, 50 of the 67 subjects were evaluated again by the same questionnaire. The present study evaluates tinnitus of patients as an example of clinical applications; this was not a controlled double blind study. It was found that, after patients took the prescribed medication, the total number of points were significantly reduced (paired t-test, P < 0.001). After medication, cases of bilateral tinnitus were significantly reduced from 27 to 14, and cases of two types of tinnitus sound, were significantly decreased from 22 to 11 (chi 2-test, P < 0.05). After 5 weeks of administration, 54% of patients felt treatment had been effective. Preliminary results suggest this peroral multi-drug treatment may provide relief for some patients with subjective tinnitus. However, long-term efficacy of the treatment was not investigated.

Pediatric and Geriatric Tinnitus.

Podoshin L, Ben-David J, Teszler CB.
Otolaryngology, Head &amp; Neck Surgery Department, Bnai-Zion Medical Center, and Technion-B. Rappaport Faculty of Medicine, Haifa, Israel.

Int Tinnitus J 1997;3(2):101-103

The subject of tinnitus in the population extremes-children and the elderly-is ignored by the literature, probably because children do not complain of tinnitus spontaneously, whereas it is only one challenge among other major health problems in the elderly. A short review of the literature on this subject is presented. Presbytinnitus, defined as tinnitus that accompanies the progressive hearing loss of presbycusis is classified as: type 1 (normal aging affecting the cochlea), and type II (preexistent sensorineural hearing loss accompanied by multiple systemic complaints, especially of sensory ones). The incidence of tinnitus in presbycusis is 11%. Like in other age groups, there is no significant gender predilection in the prevalence of tinnitus, but a correlation was demonstrated between the severity of tinnitus and exposure to noise. Hypertension was associated with a lower incidence of tinnitus, as compared to normotension and hypotension. Several treatment modalities of geriatric tinnitus are reviewed: the superiority of the band-noise masker in patients with presbycusis, as compared to electrical promontory stimulation; amino-oxyacetic in presbycusis and Meniere's disease; zinc supplementation in marginally zinc-deficient elderly patients in improving sensorineural hearing loss and tinnitus; aeration of the middle ear in presbycusis caused by secretory otitis media. Pediatric tinnitus has an incidence of 13% in children who passed an audiometric screening test, and 23-60% in those with hearing loss, 44% in secretory otitis media, but only 3% complain spontaneously because that the child considers tinnitus to be a normal event. There is no significant difference between children with tinnitus and those without in terms of hearing level, age, gender, or etiology of the deafness. Despite the fact that often children do not mention it, tinnitus may incite behavioral problems.

Auditory startle response is diminished in rats after recovery from perinatal copper deficiency.

Prohaska JR, Hoffman RG.
Department of Biochemistry, University of Minnesota, Duluth, USA.

J Nutr 1996 Mar;126(3):618-27

Recovery from perinatal copper deficiency was studied in female and male Sprague Dawley rats for 6 mo. Month-old offspring reared by dams on copper-deficient treatment starting d 7 of pregnancy had up to 80% reductions in regional brain copper concentrations compared with offspring from copper-supplemented dams. Liver copper concentrations and plasma ceruloplasmin diamine oxidase activities of copper-deficient rats were restored to control levels within 1 mo of nutritional repletion with dietary copper. However, brain copper concentrations, with the exception of the hypothalamus and medulla, remain lower than in controls even after 5 mo of treatment. Rats were screened for startle responses and foot splay after 1, 3 and 5 mo of repletion. Diminished auditory startle was evident in rats of both sexes at all repletion times tested, whereas tactile startle and preimpulse inhibition of tactile startle were not influenced by prior copper deficiency, suggesting auditory sensory perception abnormalities. In a separate study, postweaning male rats deprived of dietary copper for 5 wk exhibited clear signs of copper deficiency but normal acoustic startle responses and foot splay. Long-term neurochemical and behavioral abnormalities persist in rats after perinatal copper deficiency.

L- and D- methionine provide equivalent long term protection against CDDP-induced ototoxicity in vivo, with partial in vitro and in vivo retention of antineoplastic activity.

Reser D, Rho M, Dewan D, Herbst L, Li G, Stupak H, Zur K, Romaine J, Frenz D,
Goldbloom L, Kopke R, Arezzo J, Van De Water T.
Department of Neuroscience, Albert Einstein College of Medicine, Bronx, NY 10461, USA.

Neurotoxicology 1999 Oct;20(5):731-48

Treatment of metastatic tumors with ionic platinum compounds is hampered by the potent nephrotoxic, ototoxic and neurotoxic properties of these drugs. Recent studies have shown that sulfur-containing antioxidants relieve the dose limiting side effects of cis-diamminedichloroplatinum (CDDP), the most commonly used ionic platinum therapy. Here we report that both isomers of the sulfur-containing antioxidant methionine (MET) completely block the in vivo ototoxic and nephrotoxic effects of CDDP, and the duration of MET otoprotection is longer than has been previously reported. Rats treated with either L- or D-MET in addition to CDDP exhibited no signs of auditory system damage after 7 days, as evaluated by the auditory brainstem response and scanning electron microscopic examination of the organ of Corti, while CDDP-treated rats exhibited pronounced evidence of ototoxic damage after only 3 days. Microscopic examination of kidney tissue revealed moderate to severe nephrotoxic damage to CDDP-treated rats after 5 days, while rats co-treated with either MET isomer showed no evidence of kidney damage. Mortality among CDDP-treated subjects increased steadily over the period of the study, while all of the MET-protected rats survived. Finally, the efficacy of CDDP in the presence of L- or D-MET was evaluated in vitro using cultures of MTLN-3 breast tumor cell lines, and in vivo using implanted MTLN-3 tumors. Both L- and D-MET reduced the ability of CDDP to kill tumor cells in vitro and in vivo, however, our data suggest that a higher proportion of the antineoplastic activity of CDDP is retained in the presence of L- MET.

An epidemic in Cuba of optic neuropathy, sensorineural deafness, peripheral sensory neuropathy and dorsolateral myeloneuropathy.

Roman GC.
Neuroepidemiology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.

J Neurol Sci 1994 Dec 1;127(1):11-28

An epidemic outbreak of peripheral neuropathy affected Cuba in 1992-93 resulting in 50,862 cases (national cumulative incidence rate (CIR) 461.4 per 100,000). Clinical forms included retrobulbar optic neuropathy, sensory and dysautonomic peripheral neuropathy, dorsolateral myeloneuropathy, sensorineural deafness, dysphonia and dysphagia, spastic paraparesis, and mixed forms. For epidemiological purposes, cases were classified as optic forms (CIR 242.39) or peripheral forms (CIR 219.25). Increased risk was found among smokers (odds ratio (OR) 4.9), those with history of missing meals (OR 4.7) resulting in lower intake of animal protein, fat, and foods that contain B-vitamins, combined drinking and smoking (OR 3.5), weight loss (OR 2.8), excessive sugar consumption (OR 2.7) and heavy drinking (OR 2.3). Optic neuropathy was characterized by decreased vision, bilateral and symmetric central or cecocentral scotomata, and loss of color vision due to selective lesion of the maculopapillary bundles. Peripheral neuropathy was a distal axonopathy lesion affecting predominantly large myelinated axons. Deafness produced selective high frequency (4-8 kHz) hearing loss. Myelopathy lesions combined dorsal column deficits and pyramidal involvement of lower limbs with spastic bladder. Clinical features were those of Strachan syndrome and beriberi. Intensive search for neurotoxic agents, in particular organophosphorus esters, chronic cyanide, and trichloroethylene intoxication, yielded negative results. Treatment of patients with B-group vitamins and folate produced rewarding results. Most patients improved significantly and less than 0.1% of them remained with sequelae; there were no fatal cases. Supplementation of multivitamins to the entire Cuban population resulted in curbing of the epidemic. Overt malnutrition was not present, but a deficit of micronutrients, in particular thiamine, cobalamine, folate and sulfur amino acids appears to have been a primary determinant of this epidemic.

The therapeutic effect of vitamins A and E in neurosensory hearing loss.

Romeo G.

Acta Vitaminol Enzymol 1985;7 Suppl:85-92

After an anatomical and physiological outline of the organ of Corti, the pathology of the cochlea related to the sensorineural deafness is described. The role of Vitamin A on cochlear function and the effects of Vitamin E in man are then emphasized, on the base of some experimental results. Clinical and therapeutic efficacy of the combination of Vitamin A + E is shown by a number of papers, pointing out a 5-15 decibel improvement of the pure-tone threshold in patients with sensorineural hearing-loss particularly when the auditory troubles are due to presbyaccusis.

Chemical anatomy of excitatory endings in the dorsal cochlear nucleus of the rat: differential synaptic distribution of aspartate aminotransferase, glutamate, and vesicular zinc.

Rubio ME, Juiz JM.
Instituto de Neurociencias, Universidad Miguel Hernandez, Alicante, Spain.
lrubio@pop.nidcd.nih.gov

J Comp Neurol 1998 Sep 28;399(3):341-58

In order to identify cytochemical traits relevant to understanding excitatory neurotransmission in brainstem auditory nuclei, we have analyzed in the dorsal cochlear nucleus the synaptic distribution of aspartate aminotransferase, glutamate, and vesicular zinc, three molecules probably involved in different steps of excitatory glutamatergic signaling. High levels of glutamate immunolabeling were found in three classes of synaptic endings in the dorsal cochlear nucleus, as determined by quantitation of immunogold labeling. The first type included auditory nerve endings, the second were granule cell endings in the molecular layer, and the third very large endings, better described as "mossy." This finding points to a neurotransmitter role for glutamate in at least three synaptic populations in the dorsal cochlear nucleus. The same three types of endings enriched in glutamate immunoreactivity also contained histochemically detectable levels of aspartate aminotransferase activity, suggesting that this enzyme may be involved in the synaptic handling of glutamate in excitatory endings in the dorsal cochlear nucleus. There was also extrasynaptic localization of the enzyme. Zinc ions were localized exclusively in granule cell endings, as determined by a Danscher-selenite method, suggesting that this ion is involved in the operation of granule cell synapses in the dorsal cochlear nucleus.

Dose dependent protection by lipoic acid against cisplatin-induced ototoxicity in rats: antioxidant defense system.

Rybak LP, Husain K, Whitworth C, Somani SM.
Department of Pharmacology, Southern Illinois University, School of Medicine,
Springfield 62794, USA.

Toxicol Sci. 1999 Feb;47(2):195-202.

This study investigated the alterations that occur in auditory brainstem-evoked responses (ABRs) concurrent with changes in cochlear concentrations of glutathione (GSH), lipid peroxidation, and antioxidant enzyme activity in cisplatin-induced ototoxicity and in dose-dependent otoprotection by an antioxidant lipoate. Male Wistar rats were divided into different groups and were treated as follows, with: (1) vehicle (saline) control; (2) cisplatin (16 mg/kg, i.p.); (3) lipoate (100 mg/kg, i.p.) plus saline; (4) cisplatin plus lipoate (25 mg/kg); (5) cisplatin plus lipoate (50 mg/kg), and (6) cisplatin plus lipoate (100 mg/kg). Post-treatment ABRs were evaluated after three days, the rats were sacrificed, and cochleae were harvested and analyzed. The cisplatin-injected rats showed ABR threshold elevations above the pre-treatment thresholds. Rats treated with lipoate plus cisplatin did not show significant elevation of hearing thresholds. Cisplatin administration resulted in a depletion of cochlear GSH concentration (69% of control), whereas, cisplatin-plus-lipoate treatment increased GSH concentration close to control value. Cisplatin-treated rats showed a decrease in cochlear superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GSH-Px), and glutathione reductase (GR) activities (57, 78, 59, and 58% of control, respectively), and an increase in malondialdehyde (MDA) concentration (196% of control). Cochlear SOD, CAT, GSH-Px, and GR activities and MDA concentrations were restored in the rats injected with cisplatin plus graded doses of lipoate than those with cisplatin alone. It is concluded that cisplatin-induced ototoxicity is related to impairment of the cochlear antioxidant defense system, and the dose-dependent otoprotection conferred by an antioxidant lipoate against cisplatin ototoxicity is associated with sparing of the cochlear antioxidant defense system.

Application of antioxidants and other agents to prevent cisplatin ototoxicity.

Rybak LP, Whitworth C, Somani S.
Department of Surgery, Southern Illinois University, School of Medicine,
Springfield 62794-9638, USA.

Laryngoscope. 1999 Nov;109(11):1740-4.

OBJECTIVE/HYPOTHESIS: To review the recent data from experiments performed in this laboratory to test the hypothesis that cisplatin ototoxicity is related to depletion of glutathione and antioxidant enzymes in the cochlea and that the use of antioxidants or protective agents would protect the cochlea against cisplatin damage and prevent hearing loss.

STUDY DESIGN/METHODS: Data were reviewed from experiments performed in this laboratory. Control rats were treated intraperitoneally with cisplatin 16 mg/kg. Experimental rats were given cisplatin in combination with one of the following protective agents: diethyldithiocarbamate, 4-methylthiobenzoic acid, ebselen, or lipoic acid.
Animals in each group underwent auditory brainstem response (ABR) threshold
testing before and 3 days after treatment. Cochleae were removed after final ABR testing and analyzed for glutathione and activities of the enzymes superoxide dismutase, catalase, glutathione peroxidase, glutathione reductase, and malondialdehyde.

RESULTS: Rats in the control group receiving cisplatin were found to have significant ABR threshold shifts. This was accompanied by a reduction of glutathione and the activity of antioxidant enzymes (superoxide dismutase, glutathione peroxidase, catalase, and glutathione reductase) and an elevation of malondialdehyde. Experimental animals had preservation of ABR thresholds and levels of glutathione, antioxidant enzyme activity, and
malondialdehyde that were similar to untreated animals.

CONCLUSION: Cisplatin ototoxicity appears to be initiated by fee-radical production, which causes depletion of glutathione and antioxidant enzymes in the cochlea, and lipid peroxidation, manifested by an increase in malondialdehyde. These effects were blocked by each of a series of antioxidant compounds given in combination with cisplatin. A mechanism for cisplatin ototoxicity is elaborated with a proposed plan of chemoprevention using agents with different mechanisms of action. These substances could be used alone or in combination to reduce the severity of cisplatin ototoxicity in patients.

Ototoxicity. Amelioration by protective agents.

Rybak LP, Somani S.
Department of Surgery, Southern Illinois University, School of Medicine,
Springfield 62794-9638, USA.

Ann N Y Acad Sci. 1999 Nov 28;884:143-51.

The findings of studies from this laboratory are summarized to compare the efficacy of four chemoprotective agents against the effects of cisplatin-induced hearing loss and biochemical damage in the rat cochlea. A number of studies have shown that cisplatin is ototoxic, resulting in hearing loss, morphologic damage, and biochemical changes in the cochlea. These studies used Wistar rats, which underwent pre- and posttreatment ABR testing using clicks and tonebursts stimuli at 8, 16, and 32 kHz. Controls received i.p. saline injection. Cisplatin-treated rats were given 16 mg/kg cisplatin i.p. Animals received protective agents in the following dosage: DDTC protected rats received 600 mg/kg subcutaneously an hour after cisplatin. MTBA-protected animals were given 250 mg/kg i.p. 30 minutes before cisplatin. Animals protected with ebselen received 16 mg/kg i.p. an hour before cisplatin. One hundred mg/kg of alpha-lipoic acid was injected i.p. 30 minutes before cisplatin. Rats were sacrificed three days after treatment and the cochleae were harvested and frozen in liquid nitrogen and stored at -80 degrees C until analysis of glutathione (GSH), the activity of antioxidant enzymes (superoxide dismutase, catalase, glutathione peroxidase, and glutathione reductase) and malondialdehyde was performed. Cisplatin-treated rats were found to have ABR threshold shifts of 27-40 dB, and rats treated with chemoprotective agents plus cisplatin all had ABR thresholds shifts of less than 10 dB. Significant depletion of glutathione and decrease of the activities of the antioxidant enzymes were observed in cisplatin-treated rats. These changes were accompanied by a marked elevation of malondialdehyde. These changes were almost completely prevented by the use of the chemoprotective agents. These findings suggest that cisplatin ototoxicity is related to lipid peroxidation and that the use of protective agents prevents hearing loss and lipid peroxidation by sparing the antioxidant defense system in the cochlea.

[Hearing recovery in sudden deafness with profound hearing loss.] [Article in Japanese]

Sano H, Okamoto M, Hirayama M, Ono Y, Nitta M.
Department of Otolaryngology, School of Medicine, Kitasato University, Sagamihara.

Nippon Jibiinkoka Gakkai Kaiho 1998 Jun;101(6):836-40

We investigated the hearing recovery in 51 patients with sudden deafness with the initial averaged five-frequency hearing levels worse than or equal to 100 dB. Twenty-two patients were treated in an outpatient setting with a peri-oral steroid, vasodilator, metabolic activator, and vitamin B. The remaining 29 patients were treated in the hospital with additional hyperbaric oxygenation therapy and/or Satellite ganglion block. The results were as follows. 1) The ultimate averaged five-frequency hearing levels were mainly distributed from 55 to 80 dB. Only seven patients showed ultimate hearing levels worse than 100 dB. Two patients achieved complete recovery better than 20 dB. 2) There was no significant difference in the hearing recovery between the patients treated in an outpatient setting and in the hospital. 3) The time interval until the hearing recovery began was distributed broadly between 2 and 28 days from the onset. While most of the patients who began to recover within 14 days from the onset showed ultimate hearing levels better than 80 dB, the patients who recovered after 14 days had worse hearing levels.

Pharmacokinetics of coenzyme Q10 in recovery of acute sensorineural hearing loss due to hypoxia.

Sato K.
Department of Otolaryngology, Kanazawa Medical University, Japan.

Acta Otolaryngol Suppl 1988;458:95-102

Coenzyme Q10 (CoQ10) has already been favorably evaluated in the clinical treatment of heart disease. In the otolaryngological field, it has been reported that CoQ10 is effective in promoting recovery from acute sudden deafness. However, the pharmakinetics of CoQ10 in the inner ear is not yet clarified. The present study focuses upon the pharmacokinetics of CoQ10 using guinea pigs with acute sensorineural hearing loss artificially induced by hypoxia conditions. The respiration of the animals was controlled in an artificial respirator while the ABR, ECG and blood pressure were monitored. Repeated hypoxia caused a gradual disappearance of the ABR. After the experiments, the animals were sacrificed and brain and inner ear were examined by histological and histochemical methods as well as by SEM and TEM. The results indicated that CoQ10 is effective in promoting recovery from damage in auditory hairs as well as preventing respiratory metabolic impairment of hair cell due to hypoxia.

Preventive effect of magnesium supplement on noise-induced hearing loss in the guinea pig.

Scheibe F, Haupt H, Ising H.
Department of Otorhinolaryngology, Charite Hospital, Humboldt University, Schumannstrasse 20-21, 10117 Berlin, Germany. fred.scheibe@charite.de

Eur Arch Otorhinolaryngol 2000;257(1):10-6

The effect of magnesium (Mg) on noise-induced hearing loss was investigated in two groups of adult pigmented guinea pigs maintained either on optimal or suboptimal (physiologically high or low) Mg produced by different diets. The total Mg concentrations of the perilymph (PL), cerebrospinal fluid, blood plasma and red blood cells were measured by atomic absorption spectrometry and were found to differ significantly between the two groups (P < 0.01). One ear of each animal was exposed to either a single shooting impulse at a peak pressure level of 187 dB or two impulse noise series at a rate of 1/s and peak pressure levels of 150 dB (1,000 impulses) and 167 dB (2,280 impulses), respectively. Temporary (TTS) and permanent (PTS) hearing threshold shifts in anesthetized animals were measured 2 h and 1 week after the noise exposure, using auditory brain stem response (ABR) audiometry at a frequency range from 3.75 to 30 kHz. Exposure to the single noise impulse resulted in a mean TTS that was significantly lower in the high Mg group than that in the low Mg group (P < 0.05), although no substantial PTS was observed in either group. In the animals exposed to 150 dB noise, the TTS showed a tendency towards an Mg-related reduction at the higher frequencies. A small difference in PTS was found between the low Mg and high Mg groups, but was not significant. Exposure to the 167-dB noise series caused a considerable TTS, which was significantly lower in the high Mg group at 7.5 and 15 kHz than in the low Mg group (P < 0.05). The mean PTS showed a significant difference between the two Mg groups over the whole frequency range (P < 0.05) and was found to correlate negatively with the total Mg concentrations of both PL and plasma (P < 0.05). Moreover, the high Mg group showed a faster recovery from the hearing threshold shift than the low Mg group. The present findings show that preventive oral Mg supplements can significantly reduce the rate of acoustic trauma caused by high-level impulse noise exposure in the guinea pig.

Preventive magnesium supplement reduces ischemia-induced hearing loss and blood viscosity in the guinea pig.

Scheibe F, Haupt H, Vlastos GA.
Department of Otorhinolaryngology, Charite Hospital, Humboldt University, Berlin, Germany. fred.scheibe@charite.de

Eur Arch Otorhinolaryngol 2000;257(7):355-61

The effect of magnesium (Mg) on ischemia-induced hearing loss was investigated in two groups of adult pigmented guinea pigs of either an optimal or suboptimal (physiologically high or low) Mg status maintained by different diets. Total Mg concentrations of the perilymph, cerebrospinal fluid, blood plasma and red blood cells were found to differ significantly between the two groups, as tested in a previous study. Local vascular impairment was produced by unilateral ferromagnetic thrombosis of cochlear blood vessels. Cochlear blood flow (CBF) and hearing function were measured using laser Doppler flowmetry and auditory brain-stem response audiometry, respectively. Ferromagnetic thrombosis resulted in significant reductions of the mean apical CBF in both experimental groups and of the mean basal CBF in the low Mg group compared to the contralateral ears. In the high Mg group, the basal CBF was not decreased. However, the laser Doppler signals revealed considerable interindividual variations and the differences found between the two experimental groups were not significant. In contrast, the hearing loss in the low Mg group was significantly higher than that in the high Mg group. A correlation was found to exist between the vascular impairment and the hearing threshold shift. In a separate series, we also tested the effect of Mg on hemorheology and found both the blood viscosity and blood viscoelasticity to be significantly lower in the high Mg group than in the low Mg group, depending on the shear rates tested. The present findings show that a preventive oral Mg supplement can significantly reduce the rate of ischemia-induced hearing loss and improve blood viscosity in the guinea pig.

Biologic activity of mitochondrial metabolites on aging and age-related hearing loss.

Seidman MD, Khan MJ, Bai U, Shirwany N, Quirk WS.
Department of Otolaryngology Head & Neck Surgery, Henry Ford Health System,
Detroit, Michigan 48323, USA.

Am J Otol. 2000 Mar;21(2):161-7.

HYPOTHESIS: Compounds that upregulate mitochondrial function in an aging model will improve hearing and reduce some of the effects of aging.

BACKGROUND: Reactive oxygen metabolites (ROM) are known products of oxidative metabolism and are continuously generated in vivo. More than 100 human clinical conditions have been associated with ROM, including atherosclerosis, arthritis, autoimmune diseases, cancers, heart disease, cerebrovascular accidents, and aging. The ROM are extremely reactive and cause extensive DNA, cellular, and tissue damage. Specific deletions within the mitochondrial DNA (mtDNA) occur with increasing frequency in age and presbyacusis. These deletions are the result of chronic exposure to ROM. When enough mtDNA damage accrues, the cell becomes bioenergetically deficient. This mechanism is the basis of the mitochondrial clock theory of aging, also known as the membrane hypothesis of aging. Nutritional compounds have been identified that enhance mitochondrial function and reverse several age-related processes. It is the purpose of this article to describe the effects of two mitochondrial metabolites, alpha-lipoic acid and acetyl L-carnitine, on the preservation of age-related hearing loss.

METHODS: Twenty-one Fischer rats, aged 24 months, were divided into three groups: acetyl-1-carnitine, alpha-lipoic acid, and control. The subjects were orally supplemented with either a placebo or one of the two nutritional compounds for 6 weeks. Auditory brainstem response testing was used to obtain baseline and posttreatment hearing thresholds. Cochlear, brain, and skeletal muscle tissues were obtained to assess for mtDNA mutations.

RESULTS: The control group demonstrated an expected age-associated threshold deterioration of 3 to 7 dB in the 6-week study. The treated subjects experienced a delay in progression of hearing loss. Acetyl-1-carnitine improved auditory thresholds during the same time period (p<0.05). The mtDNA deletions associated with aging and presbyacusis were reduced in the treated groups in comparison with controls.

CONCLUSIONS: These results indicate that in the proposed decline in mitochondrial function with age, senescence may be delayed by treatment with mitochondrial metabolites. Acetyl-1-carnitine and alpha-lipoic acid reduce age-associated deterioration in auditory sensitivity and improve cochlear function. This effect appears to be related to the mitochondrial metabolite ability to protect and repair age-induced cochlear mtDNA damage, thereby upregulating mitochondrial function and improving energy-producing capabilities.

Salicylate attenuates gentamicin-induced ototoxicity.

Sha SH, Schacht J.
Kresge Hearing Research Institute, Department of Otolaryngology, University of Michigan, Ann Arbor 48109-0506, USA.

Lab Invest 1999 Jul;79(7):807-13

Aminoglycosides, primarily gentamicin, are the most commonly used antibiotics worldwide despite their toxicity to the kidney and the inner ear. A preventive therapy against these side effects should combine safety and efficacy with low cost because aminoglycoside-induced deafness is most prevalent in developing countries. We have previously shown that aminoglycosides catalyze the formation of free radicals in an iron-dependent reaction and have delineated the structure of an iron-gentamicin complex. Here we demonstrate that 2-hydroxybenzoate (salicylate), which can act as an iron chelator and antioxidant, effectively protects against gentamicin-induced hearing loss in guinea pigs. Co-therapy with salicylate reduced a profound gentamicin-induced auditory threshold shift of more than 60 dB to less than 20 dB. Morphological assessment of the inner ear confirmed protection of auditory sensory cells. Salicylate altered neither serum levels of gentamicin nor its antibacterial efficacy. Because the required salicylate levels correspond to anti-inflammatory levels in humans, this treatment holds promise for clinical application.

Antioxidants attenuate gentamicin-induced free radical formation in vitro and ototoxicity in vivo: D-methionine is a potential protectant.

Sha SH, Schacht J.
Kresge Hearing Research Institute, Department of Otolaryngology, University of Michigan, 1301 East Ann Street, Ann Arbor, MI, USA.

Hear Res 2000 Apr;142(1-2):34-40

We have recently suggested antioxidant therapy against aminoglycoside-induced hearing loss based on the hypothesis of a redox-active aminoglycoside-iron complex causing ototoxicity. The present study compares seven antioxidants and iron chelators for their ability to attenuate gentamicin-induced free radical generation in vitro and ototoxicity in guinea pig in vivo.Free radical formation by gentamicin was measured by chemiluminescence detection both in a non-enzymatic system in vitro and in cell culture. Deferoxamine, 2,3-dihydroxybenzoate, or salicylic acid suppressed gentamicin-induced luminescence in both tests. This indicated the usefulness of the assay as a screen for potential protectants since these agents had previously been shown to attenuate gentamicin-induced ototoxicity in vivo. Histidine and D-methionine, amino acids with chelating and antioxidant properties, also suppressed gentamicin-mediated luminosity both in vitro and in cell culture. In contrast, the metal chelators succimer (2, 3-dimercaptosuccinic acid (DMSA)) and trientine (N, N'-bis[2-aminoethyl]-1,2 ethanediamine) promoted free radical formation and were excluded from further studies. Histidine and D-methionine were then administered to guinea pigs receiving concurrent treatment with gentamicin (120 mg/kgx19 days). Threshold shifts induced by gentamicin were significantly attenuated by twice-daily injections of D-methionine. Once-daily injections of histidine or D-methionine were less effective, pointing to the importance of pharmacokinetics in antioxidant protection in vivo.The study presents a simple screening system for agents with the potential to attenuate gentamicin-induced hearing loss. It also supports the hypothesis of free radical formation as an underlying cause of gentamicin ototoxicity.

Zinc: the neglected nutrient.

Shambaugh GE Jr.
Shambaugh Hearing and Allergy, Hinsdale, IL 60521.

Am J Otol 1989 Mar;10(2):156-60

Zinc was first recognized as essential for animals at the University of Illinois School of Agriculture in 1916, when it was found that zinc-deficient baby pigs were runty, developed dermatitis on their legs, and were sterile. Zinc deficiency was first recognized in man by Dr. Ananda Prasad of Detroit 26 years ago when he measured serum and hair zinc levels in young male Egyptian dwarfs who had failed to mature and were small in stature. By simply adding zinc to their regular diet, they grew in height and became sexually mature. It is now recognized that dwarfism in males is frequent around the Mediterranean, where wheat is the staple of life and has been grown for 4,000 years on the same soil, thereby resulting in the depletion of zinc. Professor Robert Henkin first suggested that zinc deficiency might cause hearing-nerve impairment. Assay of the soft tissues of the cochlea and vestibule revealed a zinc level higher than that of any other part of the body. Previously, the eye was considered to have the highest level of zinc of any organ. To diagnose zinc deficiency clinically, we use serum zinc assays made at the Mayo Clinic Trace Element Laboratory. With zinc supplementation in patients who are marginally zinc deficient, there has been improvement in tinnitus and sensorineural hearing loss in about one-third of elderly adults. We believe zinc deficiency is one causation of presbycusis; by recognizing and correcting it, a progressive hearing loss can be arrested.

Vitamin B12 deficiency in patients with chronic-tinnitus and noise-induced hearing loss.

Shemesh Z, Attias J, Ornan M, Shapira N, Shahar A.
Institute of Noise Hazards Research and Evoked Potentials Laboratory, IDF, Chaim-Sheba Medical Center, Ramat-Gan, Israel.

Am J Otolaryngol 1993 Mar-Apr;14(2):94-9

INTRODUCTION: This study examines the incidence of vitamin B12 deficiency in three groups of noise-exposed subjects: patients with chronic tinnitus and noise-induced hearing loss (NIHL), patients with NIHL only, and subjects demonstrating normal hearing. MATERIALS AND METHODS: A group of 113 army personnel exposed to military noise was studied. The mean age was 39 years. Chronic tinnitus and NIHL existed in 57 subjects. NIHL alone was observed in 29 subjects, and 27 subjects had normal audiograms. All subjects were queried about noise exposure and dietary habits. Vitamin B12 serum levels were measured. RESULTS: Patients with tinnitus and NIHL exhibited vitamin B12 deficiency in 47% of cases (blood levels < or = 250 pg/mL). This was significantly more (P < .023) compared with NIHL and normal subjects who exhibited vitamin B12 deficiency in 27% and 19%, respectively. CONCLUSION: These observations suggest a relationship between vitamin B12 deficiency and dysfunction of the auditory pathway. Some improvement in tinnitus and associated complaints were observed in 12 patients following vitamin B12 replacement therapy. The authors recommend that routine vitamin B12 serum levels be determined when evaluating patients for chronic tinnitus.

Delayed auditory brainstem response in thiamin-deficient rats.

Shigematsu Y, Nakai A, Kuriyama M, Kikawa Y, Konishi I, Sudo M, Itokawa Y.
Department of Pediatrics, Fukui Medical School, Japan.

J Nutr Sci Vitaminol (Tokyo) 1990 Jun;36(3):209-15

We recorded the auditory brainstem responses of rats fed a thiamin-deficient diet. The interpeak latencies between waves I and III, as well as those between waves I and IV, were significantly prolonged from day 24, while the latency of wave I was prolonged on day 26 of the thiamin-deficient diet. These delayed responses were corrected in 2 to 4 days after the initiation of daily intraperitoneal thiamin injections from day 32. The rats that were fed the thiamin-deficient diet, and then sacrificed on day 32, showed a decrease of total thiamin levels in the brain (26% of the level in control rat brains). Based on these results, we emphasize the value of the auditory brainstem response to detect thiamin deficiency.

Clinical improvement of memory and other cognitive functions by Ginkgo biloba: review of relevant literature.

Soholm B.
Sano-Pharm A/S, Vedbaek, Denmark.

Adv Ther 1998 Jan-Feb;15(1):54-65

Ginkgo biloba is a plant extract used to alleviate symptoms associated with cognitive deficits, e.g., decreased memory performance, lack of concentration, decreased alertness, tinnitus, and dizziness. Pharmacologic studies have shown that the therapeutic effect of ginkgo is based on several active constituents with vasoactive and free radical-scavenging properties. The use of ginkgo extract in either dementias of the Alzheimer or multi-infarct type or in the case of cerebral insufficiency, a symptom complex related to age-dependent
impairment of cerebral circulation, is based mainly on positive results from good-quality placebo-controlled studies that enrolled approximately 1,200 patients with criteria established by International Classification of Diseases (9th and 10th revisions, ICD-9 and ICD-10) or the 3rd revision of the Diagnostic and Statistical Manual (DSM-III-R) (uncomplicated dementia). Effect on cognitive symptoms was within the range of a 25% reduction. Memory, concentration, and alertness were the first symptoms to be relieved, with tinnitus and dizziness improving somewhat later. A minimum of 4 to 6 weeks were needed before a pronounced effect could be expected. The pharmacologic advantage of ginkgo seems to be a very tolerable side-effect profile, with a side-effect frequency at the
placebo level.

Protection from gentamicin ototoxicity by iron chelators in guinea pig in vivo.

Song BB, Anderson DJ, Schacht J.
Kresge Hearing Research Institute, University of Michigan, Ann Arbor 48109-0506, USA.

J Pharmacol Exp Ther 1997 Jul;282(1):369-77

This study details the prevention of gentamicin-induced hearing loss in guinea pig in vivo. The approach is based on our recent demonstrations of a redox-active gentamicin-iron complex in vitro and partial attenuation of gentamicin-induced hearing loss by the iron chelators deferoxamine and 2,3-dihydroxybenzoate. In our study, guinea pigs receiving injections of gentamicin (120 mg/kg body weight daily x 19 days) developed a progressive threshold shift reaching 50 to 70 dB at 18 kHz. Concurrent treatment with different doses of 2,3-dihydroxybenzoate (30-300 mg/kg/day) reduced the threshold shift to 25 to 15 dB. Coinjection of gentamicin with dihydroxybenzoate (100 mg/kg/day) plus mannitol (15 mg/kg/day) yielded complete functional and morphological protection from gentamicin ototoxicity although partial protection was observed with combinations of dihydroxybenzoate and deferoxamine. Dihydroxybenzoate also attenuated gentamicin-induced vestibular toxicity. The iron chelators and radical scavengers affected neither serum levels nor the antimicrobial efficacy of gentamicin against Escherichia coli. These results confirm that iron and free radicals play a crucial role in the toxic side effects of gentamicin. Furthermore, they suggest that iron chelators, which are well-established drugs in clinical therapy, may be promising therapeutic agents to reduce aminoglycoside ototoxicity.

Variable efficacy of radical scavengers and iron chelators to attenuate gentamicin ototoxicity in guinea pig in vivo.

Song BB, Schacht J.
Kresge Hearing Research Institute, University of Michigan, Ann Arbor 48109-0506, USA.

Hear Res 1996 May;94(1-2):87-93

Recent studies from our laboratory have suggested that the ototoxic side effects of gentamicin are caused by a metabolized or 'activated' from the drug. Furthermore, we have postulated that the activation proceeds via the formation of an iron-gentamicin complex and that this complex produces free radicals. The present study assessed the protection effects of free radical scavengers and iron chelators on gentamicin-induced ototoxicity in guinea pigs in vivo. Gentamicin (120 mg/kg per day for 19 days) caused progressive threshold shifts reaching 50-65 dB at 18 kHz. Co-therapy with different radical scavengers yielded results ranging from no protection (with allopurinol, dimethyl sulfoxide, benzoate, lazaroid U74389G) to a moderate attenuation of hearing loss (with mannitol, 4-methylthiobenzoate, WR-2721). This finding agrees well with previous reports of inconsistent effects of scavengers on aminoglycoside-induced ototoxicity although it should be cautioned that only a single dose and route of application was tested. Two iron chelators, deferoxamine and 2,3-dihydroxybenzoate, significantly reduced the gentamicin-induced threshold shifts to about 10 dB or less. Iron chelators markedly decreased total serum iron levels while gentamicin treatment alone had no influence. There were no differences in serum gentamicin levels among all treated groups. This study confirms that iron plays a critical role in gentamicin ototoxicity and suggests that iron chelators, which are well-established drugs in clinical therapy, may be promising therapeutic agents to reduce aminoglycoside ototoxicity.

Iron chelators protect from aminoglycoside-induced cochleo- and vestibulo-toxicity.

Song BB, Sha SH, Schacht J.
Kresge Hearing Research Institute, University of Michigan, Ann Arbor 48109-0506, USA.

Free Radic Biol Med 1998 Jul 15;25(2):189-95

The attenuation of gentamicin-induced hearing loss by iron chelators and radical scavengers has recently been demonstrated in guinea pig in vivo. The present study investigated whether this protective treatment is effective against hearing loss and vestibular damage caused by other aminoglycosides. In a direct comparison, dihydroxybenzoate was chosen over deferoxamine because of its more effective action against gentamicin-induced hearing loss. Guinea pigs received daily injections of kanamycin (250 mg/kg/d) or streptomycin (300 mg/kg/d) for 23 d to induce severe cochlear or vestibular toxicity, respectively. Kanamycin injections resulted in a progressive threshold shift of 60 to 80 dB at 18 kHz, while streptomycin injections induced only a small threshold shift. In contrast, streptomycin abolished almost all vestibular responses. Coinjection of
aminoglycosides with a mixture of dihydroxybenzoate (100 mg/kg/d) and mannitol (30 mg/kg/d) significantly attenuated kanamycin-induced hearing loss and protected against streptomycin-induced vestibulotoxicity. DHB/mannitol did not affect serum levels or the antibacterial efficacy of either aminoglycoside. This study supports the idea that iron and free radicals play a critical role in the toxic side effects of aminoglycoside antibiotics. Furthermore, the previously proposed therapeutic protection is not limited to gentamicin but applicable to other aminoglycosides as well.

[The effect of polikatan on the ototoxic action of kanamycin.] [Article in Russian]

Spasov AA, Lobzov MS, Sanzharovskaia NK, Kozhevnikova EV, Kuzubova EA.
Volgograd State Medical Academy, Russia.

Eksp Klin Farmakol 1999 Jul-Aug;62(4):65-6

Experiments on guinea pigs demonstrated that preliminary injection of polycatan (standardized magnesium solution containing the mineral bischofite) into the parotid region by means of electrophoresis reduces the ototoxic effect of the aminoglycoside antibiotic kanamycin. Polycatan prevents kanamycin-induced degenerative changes of the hair cells found in the labyrinth of the internal ear and improves the local blood flow.

[Noise from a car airbag as a cause of acute acoustic trauma] [Article in Polish]

Stankiewicz C, Przewozny T, Kozlowski J.
Katedra i Klinika Chorob Uszu, Nosa, Gardla i Krtani AM w Gdansku.

Otolaryngol Pol. 2000;54(6):775-81.

A case of acute acoustic trauma in 32 year male, caused by a noise from car airbag, was described. The symptoms occurring directly after the airbag shot were tinnitus and hearing loss in right ear. Patient was admitted to ENT Clinic in Gdansk just after 5 months after an accident. Treatment--vasodilatators and oxygen hyperbaric therapy--was ineffective. Review of literature, concerning the side effects of car airbag shot, was made. A mechanism of airbag action was presented as a source of short time noise.

Iron deficiency and hearing loss. Experimental study in growing rats.

Sun AH, Xiao SZ, Li BS, Li ZJ, Wang TY, Zhang YS.

ORL J Otorhinolaryngol Relat Spec 1987;49(3):118-22

Cochlear changes were studied in 141 growing rats raised on a basic iron-deficient diet for 7-100 days; 130 rats served as normal or chronic anemia controls. Electrophysiological findings showed that the incidence of an auditory threshold elevation of more than 15 dB was 31.85% in the iron-deficient rats, but it was unchanged in all the control animals. The main cochlear histopathological changes induced by iron deficiency were strial atrophy and reduction of spiral ganglion cells. It is concluded that the observed anomalies may be attributed solely to iron deficiency of the cochlear tissue.

Changes in the cochlear iron enzymes and adenosine triphosphatase in experimental iron deficiency.

Sun AH, Li JY, Xiao SZ, Li ZJ, Wang TY.
Otolaryngological Laboratories, Changhai Hospital, Shanghai, People's Republic of China.

Ann Otol Rhinol Laryngol 1990 Dec;99(12):988-92

The influences of iron deficiency on the cochlear iron enzymes and adenosine triphosphatase were studied in 68 iron-deficient rats and 68 control rats (normal and with chronic anemia). A disorderly or topographic distribution and reduction or disappearance of the cochlear succinic dehydrogenase and peroxidase reaction products were found in 37.8% of the rats fed on a basic iron-deficient diet for 14 to 100 days. The activity of cochlear sodium-potassium-dependent adenosine triphosphatase in iron-deficient rats was slightly increased, compared to that in normal controls. These results suggest that iron deficiency would produce significant abnormalities of succinic dehydrogenase and peroxidase activity, which in turn would disturb cell respiration and initiate peroxidative damage to the inner ear cells, result in sensorineural hearing loss, or provide a pathologic basis for cochlear deafness.

Noise-induced hearing loss in iron-deficient rats.

Sun AH, Wang ZM, Xiao SZ, Li ZJ, Lin DY, Liang ZF, Hu ZY, Wang GY, Ye XT.
Otolaryngological Laboratories, Changhai Hospital, Shanghai, China.

Acta Otolaryngol 1991;111(4):684-90

The role of iron deficiency in noise-induced hearing loss (NIHL) was evaluated in 64 rats of four different experimental groups. Iron-deficient rats (ID-rats) and normal rats (N-rats) were simultaneously exposed to a steady state white noise (20-10,000 Hz) at 110 dB SPL for 30 min. Unexposed ID- and N-rats served as controls. In N-rats the temporary threshold shifts (TTS) would have completely disappeared if the animals were allowed to survive for 72 h. No permanent threshold shift (PTS) was seen in any of the N-rats. The ultrastructural correlates in N-rats are stereocilia disarray and mitochondria swelling in outer hair cells (OHCs). The TTS in ID-rats were larger than those in the N-rats, and most ID-rats with larger threshold shifts showed varying degrees of PTSs at 11 days post-exposure. The ultrastructural correlates of NIHL in ID-rats are obvious pathology of the stereocilia, such as segmental coalescence of stereocilia of many continuous OHCs and fusion of the tips of stereocilia of OHCs, and a significant reduction of mitochondria as well as slight degeneration of nucleus in the OHCs. It is concluded that iron deficiency can provide a pathological basis for NIHL.

The effects of coenzyme Q10 treatment on maternally inherited diabetes mellitus and deafness, and mitochondrial DNA 3243 (A to G) mutation.

Suzuki S, Hinokio Y, Ohtomo M, Hirai M, Hirai A, Chiba M, Kasuga S, Satoh Y, Akai H, Toyota T.
Third Department of Internal Medicine, Tohoku University School of Medicine, Sendai, Japan.

Diabetologia 1998 May;41(5):584-8

The characteristic clinical features of diabetes mellitus with mitochondrial DNA (mtDNA) 3243(A-G) mutation are progressive insulin secretory defect, neurosensory deafness and maternal inheritance, referred to as maternally inherited diabetes mellitus and deafness (MIDD). A treatment for MIDD to improve insulin secretory defects and reduce deafness has not been established. The effects of coenzyme Q10 (CoQ10) treatment on insulin secretory response, hearing capacity and clinical symptoms of MIDD were investigated. 28 MIDD patients (CoQ10-DM), 7 mutant subjects with impaired glucose tolerance (IGT), and 15 mutant subjects with normal glucose tolerance (NGT) were treated daily with oral administration of 150 mg of CoQ10 for 3 years. Insulin secretory response, blood lactate after exercise, hearing capacity and other laboratory examinations were investigated every year. In the same way we evaluated 16 MIDD patients (control-DM), 5 mutant IGT and 5 mutant NGT subjects in yearly examinations. The insulin secretory response assessed by glucagon-induced C-peptide secretion and 24 h urinary C-peptide excretion after 3 years in the CoQ10-DM group was significantly higher than that in the control-DM group. CoQ10 therapy prevented progressive hearing loss and improved blood lactate after exercise in the MIDD patients. CoQ10 treatment did not affect the diabetic complications or other clinical symptoms of MIDD patients. CoQ10 treatment did not affect the insulin secretory capacity of the mutant IGT and NGT subjects. There were no side effects during therapy. This is the first report demonstrating the therapeutic usefulness of CoQ10 on MIDD.

Long-term auditory and visual complications of biotinidase deficiency.

Taitz LS, Leonard JV, Bartlett K.

Early Hum Dev 1985 Sep;11(3-4):325-31

The biochemical, dermatological and neurological motor disorders of biotinidase deficiency (multiple carboxylase deficiency) show a dramatic response to pharmacological doses of biotin. This condition is characterised by the accumulation of biocytin and depletion of biotin. Neuromuscular function returns to normal with the reversal of the characteristic organic acidaemia. It would appear that the optic and auditory nerves or their related neurological structures may suffer damage from the excess biocytin and deficient biotin. Despite reversal of the dermatological and psychomotor abnormalities children are likely to be left with auditory and/or visual handicaps if diagnosis and treatment is delayed beyond the first year of life. Treatment with biotin was commenced 6, 18, and 13 months after onset of symptoms. Two children subsequently were found to have visual impairment (acquired retinal dysplasia) and two had sensori-neural deafness. In one patient both defects were present.

Randomized trial of taurine supplementation for infants less than or equal to 1,300-gram birth weight: effect on auditory brainstem-evoked responses.

Tyson JE, Lasky R, Flood D, Mize C, Picone T, Paule CL.
Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas 75235.

Pediatrics 1989 Mar;83(3):406-15

Taurine may be important to the developing eye and brain of the small preterm infant. A blinded randomized trial was conducted to determine whether taurine supplementation of healthy infants of less than or equal to 1,300 g birth weight until their discharge from the hospital increases their growth rate, neurobehavioral development, electroretinographic development, or maturation of auditory brainstem-evoked responses. Infants were fed with Similac Special Care as desired, which was prepared to contain less than 5 mg/L of taurine or 45 mg/L of taurine, a concentration similar to that of human milk. Infants who did not receive taurine supplementation (n = 19) and those who did (n = 18) were similar with respect to condition at study entry, caloric intake, and growth rates throughout the study, and electroretinographic findings and scores on the Brazelton Behavioral Assessment Scale at 37 weeks' postmenstrual age. Infants who received taurine supplementation had greater overall plasma taurine concentrations. The group receiving taurine supplementation also had more mature auditory-evoked responses at 37 weeks' postmenstrual age with a modest (0.2 to 0.5 ms) but consistent reduction (P less than .05) in the interval between stimulus and response at two different stimulation rates. Although further study is needed, taurine intake appears to influence auditory system maturation of preterm infants.

Iodine intakes assessed by urinary iodine concentrations in healthy children aged ten months, two years, and four years.

Valeix P, Preziosi P, Rossignol C, Farnier MA, Hercberg S.
Institut Scientifique et Technique de la Nutrition et de l'Alimentation, CNAM, Paris.

Biol Trace Elem Res 1992 Jan-Mar;32:259-66

Urinary iodine excretion was assessed in 642 healthy children aged 10 mo (n = 243), 2 yr (n = 183), and 4 yr (n = 216) living in the Paris area and originating from continental France (60.3%), North Africa (13.8%), the West Indies (9.1%), West Africa (8.3%), Southeast Asia (4.8%), and southern Europe (3.8%). Mild impairment of neurological (reflexes, tone, audiometry) and intellectual development (Brunet-Lezine scale) was assessed in relation to iodine status. Iodine excretions (median values) were 18.4, 11.9, and 10.9 micrograms/100 mL at 10 mo, 2 yr, and 4 yr, respectively, and risk of mild iodine deficiency (5-10 micrograms/100 mL) was 18.1%, 34.8%, and 38.3% for the same age groups. No relationship was found between anthropometry, global development quotient, and iodine status. High hearing thresholds were more commonly associated with lower iodine excretion, suggesting mild hearing defects. In spite of iodine prophylaxis, the risk of mild to moderate iodine deficiency still exists in France and in a number of European countries. Evaluation of neurological sequels of borderline iodine status is a major public health problem in European communities.

Relationship between urinary iodine concentration and hearing capacity in children.

Valeix P, Preziosi P, Rossignol C, Farnier MA, Hercberg S.
Institut Scientifique et Technique de la Nutrition et de l'Alimentation, CNAM, France.

Eur J Clin Nutr 1994 Jan;48(1):54-9

Urinary iodine excretion was assessed in 1222 healthy children aged 10 months (n = 456), 2 years (n = 368) and 4 years (n = 398) living in the Paris area and originating from continental France (55.2%), North Africa (15.7%), the West Indies (9.7%), West Africa (8.2%), Southeast Asia (5.5%), and southern Europe (5.7%). Iodine excretions (median values) were, respectively, 18.1, 13.4 and 11.6 micrograms/100 ml at 10 months, 2 years and 4 years, and risk of mild to moderate iodine deficiency (< 10 micrograms/100 ml) was 18.0%, 32.3% and 37.2% for the same age groups. Urinary iodine excretion was highest among Southeast Asian children, and lowest among West Africans. Hearing acuity was measured either by conventional mono-aural pure-tone audiometry or by binaural free field testing depending on the child's age. Hearing loss at 4000 Hz and average hearing impairment at speech frequencies (500, 1000 and 2000 Hz) were more severe among children at risk of mild to moderate iodine deficiency (less than 10 micrograms/100 ml) compared with those with urinary excretion above 10 micrograms/100 ml.

Comparison of the developmental changes of the brainstem auditory evoked response (BAER) in taurine-supplemented and taurine-deficient kittens.

Vallecalle-Sandoval MH, Heaney G, Sersen E, Sturman JA.
Department of Developmental Biochemistry, Institute for Basic Research in Developmental Disabilities, Staten Island, NY 10314.

Int J Dev Neurosci 1991;9(6):571-9

A similar development of the brainstem auditory evoked response is present in taurine-supplemented and taurine-deficient kittens between the second postnatal week and the third month of life. Between birth and the second postnatal week kittens from mothers fed the 1% taurine diet showed earlier maturation of the brainstem auditory evoked response as indicated by lower threshold, shorter P1 latency and shorter central conduction time when compared to the kittens from mothers fed the 0.05% taurine diet. These results suggest an important role of taurine in the anatomical and functional development of the auditory system.

Biotinidase deficiency: presymptomatic treatment.

Wallace SJ.

Arch Dis Child. 1985 Jun;60(6):574-5.

Biotinidase deficiency presents with clinical signs of biotin deficiency at the age of 3 months, or soon after. In an infant in whom the diagnosis was made on cord blood, vision and hearing were normal before presymptomatic treatment with biotin. Physical and mental development are good at 14 months.

Improvement in hearing among otherwise normal schoolchildren in iodine-deficient areas of Guizhou, China, following use of iodized salt.

Wang YY, Yang SH.

Lancet 1985 Sep 7;2(8454):518-20

According to a survey in Guizhou province of China, the average hearing level of otherwise normal schoolchildren in an area of endemic iodine-deficient goitre and cretinism was significantly poorer than in a non-endemic control area. After iodine prophylaxis for three years, audiometric tests showed that the average hearing level had improved and approached that of the non-endemic controls. In addition, mean values for thyroid function tests were restored to within the normal range. It is suggested that the most likely cause of the poorer hearing level among the ostensibly normal schoolchildren in endemic areas is subclinical hypothyroidism due to prolonged severe iodine deficiency.

Biotinidase deficiency: a survey of 10 cases.

Wastell HJ, Bartlett K, Dale G, Shein A.
Department of Clinical Biochemistry, Newcastle General Hospital, Newcastle upon Tyne.

Arch Dis Child 1988 Oct;63(10):1244-9

Ten patients with biotinidase deficiency were studied. Clinical findings at presentation varied with dermatological signs (dermatitis and alopecia), neurological abnormalities (fits, hypotonia, and ataxia), and recurrent infections being the most common features, although none of these occurred in every case. Biochemically the disease is characterised by metabolic acidosis and organic aciduria. Treatment with biotin results in pronounced, rapid, clinical and biochemical improvement, but some patients have residual neurological damage comprising neurosensory hearing loss, visual pathway defects, ataxia, and mental retardation. The cause of this permanent damage remains obscure and it is not clear if the early introduction of treatment will prevent it.

[Results of multistep oxygen therapy in the treatment of sudden hearing loss.] [Article in German]

Wolf O, Hanson J.
Abteilung fur Hor- und Gleichgewichtsstorungen, Stadt. Klinikums, Dessau.

Laryngorhinootologie 1991 Sep;70(9):475-8

Oxygen multistep therapy (von Ardenne) was applied in 28 patients suffering from an idiopathic sudden hearing loss. The oxygen therapy consisted of a multistep short procedure, each of which lasted for 15 minutes. The results of our study were evaluated by means of standardised statistics confirming the effectiveness and even the superiority of the oxygen multistep therapy in comparison with any other treatment, and also in view of the spontaneous remission rate in cases of sudden hearing loss.

Role of glutathione in protection against noise-induced hearing loss.

Yamasoba T, Nuttall AL, Harris C, Raphael Y, Miller JM.
Kresge Hearing Research Institute, The University of Michigan, 1301 East Ann Street, Ann Arbor, MI 48109-0506, USA.

Brain Res 1998 Feb 16;784(1-2):82-90

A potential mechanism of hearing loss due to acoustic overstimulation is the generation of reactive oxygen species (ROS). ROS not removed by antioxidant defenses could be expected to cause significant damage to the sensory cells of the cochlea. We studied the influence of the antioxidant glutathione (GSH) on noise-induced hearing loss by using l-buthionine-[S,R]-sulfoximine (BSO), an inhibitor of GSH synthesis, and 2-oxothiazolidine-4-carboxylate (OTC), a cysteine prodrug, which promotes rapid restoration of GSH when GSH is acutely depleted. Pigmented female guinea pigs were exposed to broadband noise (102 dB SPL, 3 h/day, 5 days) while receiving daily injections of BSO, OTC, or saline. By weeks 2 and 3 after noise exposure, BSO-treated animals showed significantly greater threshold shifts above 12 kHz than saline-treated subjects, whereas OTC-treated animals showed significantly smaller threshold shifts at 12 kHz than controls. Histologically assessed noise-induced damage to the organ of Corti, predominantly basal turn row 1 outer hair cells, was most pronounced in BSO-treated animals. High performance liquid chromatographic analysis showed that OTC significantly increased cysteine levels, but not GSH levels, in the cochlea. These findings show that GSH inhibition increases the susceptibility of the cochlea to noise-induced damage and that replenishing GSH, presumably by enhancing availability of cysteine, attenuates noise-induced cochlear damage. Copyright 1997 Elsevier Science B.V.

Influence of intense sound exposure on glutathione synthesis in the cochlea.

Yamasoba T, Harris C, Shoji F, Lee RJ, Nuttall AL, Miller JM.
Kresge Hearing Research Institute, The University of Michigan, 1301 East Ann Street, Ann Arbor, MI 48109-0506, USA.

Brain Res 1998 Aug 31;804(1):72-8

Previous studies have shown that depletion of endogenous glutathione (GSH) potentiates noise-induced hearing loss (NIHL), whereas replenishment of GSH attenuates NIHL (Yamasoba et al., Brain Res. 784 (1998) 82-90). Since these findings indicate an important role of GSH in protection from NIHL, we assessed the influence of intense sound exposure (broadband noise, 105 dB SPL, 5 h) on GSH and cysteine levels in the guinea pig cochlea using high performance liquid chromatography. GSH levels were significantly increased in the lateral wall 2 and 4 h post-exposure and returned to normal 6 h post-exposure. GSH levels in the sensory epithelium and modiolus did not show significant changes following noise. Cysteine levels were unchanged in any of the cochlear segments. For the cochlea as a whole, intense sound exposure did not significantly change GSH or cysteine levels throughout the 6-h measurement period post-exposure. These results indicate that GSH synthesis is markedly upregulated selectively in the lateral wall by noise exposure, presumably in response to the robust consumption of GSH, as it is utilized in scavenging reactive oxygen species. Copyright 1998 Elsevier Science B.V.

L-carnitine treatment improves brain stem auditory evoked potentials in diabetic rats.

Yildiz O, Ozata M, Ozkardes A, Corakci A, Gundogan N, Gundogan MA.
Department of Pharmacology, Gulhane School of Medicine, Ankara, Turkey.

Neuroreport 1996 Nov 25;7(18):2957-9

The effect of L-carnitine (LC) on brain stem auditory evoked potentials (BAEP), was examined in alloxan-diabetic rats. LC (200 mg kg-1, i.p., once daily) was given to diabetic rats starting from the third week after the induction of diabetes, lasting for 4 weeks. Age-matched non-diabetic rats served as controls. The latency of wave I and interpeak latency I-IV were measured once weekly. Diabetes-induced deficits in BAEP latencies (p < 0.05, diabetics vs non-diabetic controls) were improved after LC treatment (p < 0.05, LC-treated diabetic rats vs non-diabetic controls). Weight and glucose levels were not influenced by LC treatment. Our results suggest that LC had beneficial effects on diabetic central neuropathy but these effects are not associated with the regulation of glycaemia in alloxan-diabetic rats.

 

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