Occurrence Of Sensorineural Hearing Loss Research Articles

Mitochondrial Syndromic Sensorineural Hearing Loss

Mitochondrial diseases (MD) are a clinically heterogeneous group of disorders that arise as a result of dysfunction of the mitochondrial respiratory chain. Sensorineural hearing loss (SNHL) is often associated to mitochondrial dysfunctions both in syndromic, nonsyndromic forms. SNHL has been described in association to different mitochondrial multisystemic syndromes, often characterized by an important neuromuscular involvement. Because of the clinical relevance of the associated neurological symptoms, the occurrence of SNHL is often underestimated and undiagnosed. In this study we evaluated the incidence of SNHL in a group of 17 patients with MD. We detected some degree of hearing impairment in 8/17 patients (47%), thus confirming the frequency of hearing impairment in MD. Furthermore, we want to highlight the role of the audiologist and otolaryngologist in the diagnosis and characterization of a MD, which should be suspected in all the cases in which the hearing loss is associated to signs and symptoms characteristic of mitochondrial dysfunction, especially if the family history is positive for hearing loss or MD in the maternal line.

The occurrence of sensorineural hearing loss in a patient with myeloperoxidase-anti-neutrophil cytoplasmic antibody-related microscopic polyangiitis

Microscopic polyangiitis, deWned as a necrotizing vasculitis aVecting small vessels (i.e., capillaries, venules, or arterioles), commonly manifests as rapidly progressive glomerulonephritis with necrotizing glomerular tuft, or alveolar hemorrhage or interstitial pneumonia with pulmonary capillaritis. Anti-neutrophil cytoplasmic antibodies (ANCA) are positive in 50–80% of patients with microscopic polyangiitis [1]. Here we describe a patient with myeloperoxidase (MPO)ANCA-related microscopic polyangiitis whose dominant manifestation was sensorineural hearing loss. A 77-year-old Japanese woman was admitted because of progression of renal dysfunction. At age 74 she had developed dyspnea on eVort, and a chest computed tomography (CT) scan revealed bilateral pulmonary Wbrosis in a local hospital. As no marked clinical and laboratory abnormalities (i.e., constitutional symptoms, renal dysfunction, urinary abnormalities, inXammatory signs, or auto-antibodies) had been identiWed, she was diagnosed as having idiopathic interstitial pneumonia and was closely observed without any medication. One month prior to the admission she had started to report constitutional manifestations (i.e., fever, general myalgia, and weight loss). Moreover, a marked increment of serum creatinine levels with active urinary abnormalities, from 0.66 to 1.03 mg/dl during the last 1 month, had been found. As a test for MPO-ANCA was positive (101 EU; normal value, <10 EU), she was referred to our hospital. On admission, she was in mild distress and complained of generalized mild myalgia and emerging left hearing loss. Her height was 135 cm, weight was 28.0 kg, temperature was 38.0 °C, blood pressure was 150/ 90 mmHg, pulse rate was 72 beats per minute with regular rhythm, and respiratory rate was 20 times per minute. No skin lesions or joint abnormalities were identiWed. Fine crackles were heard on bilateral lung Welds. No neurological abnormalities except left hearing loss were found. Laboratory data revealed mild anemia (hemoglobin, 8.5 g/dl) and renal dysfunction (blood urea nitrogen 34 mg/dl; serum creatinine, 1.36 mg/dl). Urinalysis disclosed nephritic urinary sediments [erythrocytes, 30–50/ high-power Weld (HPF); leukocytes, 3–10/HPF; and granular casts, 2–3/HPF] with proteinuria (0.75 g/day). The serological test showed elevation of C-reactive protein (CRP, 9.35 mg/dl), hypergammaglobulinemia, and normal complement concentrations. MPO-ANCA was positive (49.0 EU), but anti-nuclear antibody, anti-hepatitis B/C antibodies, syphilis serology, anti-glomerular basement membrane antibody, and proteinase-3 ANCA were negative. A chest CT scan revealed bilateral pulmonary Wbrosis, but head CT scanning, brain magnetic resonance imaging, and ophthalmologic examination failed to reveal any remarkable abnormalities. Otolaryngological examination revealed marked high-frequency sensorineural hearing loss in the left ear (Fig. 1a). On the basis of these Wndings, she was diagnosed as having MPO-ANCA-related microscopic polyangiitis associated with interstitial pneumonia and rapidly T. Sugimoto (&) · M. Sakaguchi · N. Deji · T. Uzu · Y. Nishio · A. Kashiwagi The Department of Internal Medicine, Shiga University of Medical Science, Seta, Otsu, 520-2192 Shiga, Japan e-mail: toshiro@belle.shiga-med.ac.jp

Characteristics of hearing-impairment among patients in Ghana

The causes, and characteristics of hearing-impairment were determined prospectively among six thousand, four hundred and twenty-eight (6,428) patients who reported at the Komfo Anokye Teaching Hospital (KATH) with hearing problems. The purpose of the study was to determine the characteristics and some causes of hearing loss of patients who report for management at Komfo Anokye Teaching Hospital. The procedure adopted included a detailed case history, Otoscopy and Pure-tone Audiometry. Of the 6,428 patients, 5,734 (89.9 %) were diagnosed as having significant hearing loss. There were more hearing impaired women than men at all ages. Majority of the patients had mild hearing loss. The overall prevalence of Sensorineural Hearing Loss was more in worse ear than better ear. Again, the occurrence of Sensorineural Hearing Loss was more than other types of hearing loss. Noise, Fever, Presbycusis, Sickness, Meningitis and Meniere's diseases were the major causes of Sensorineural Hearing Loss. Conductive Hearing Loss was attributed in the main to Wax, Foreign Bodies, Otitis Media, and Traumas. These findings have important implications on the need of resources for rehabilitation.

Sensorineural hearing loss in patients with chronic otitis media

Objectives: Chronic otitis media is currently associated with an increase in air conduction thresholds. However, a few groups reported a decrease in sensorineural function in these patients. This study evaluates the occurrence of sensorineural hearing loss in patients with chronic otitis media. Methods: The records of patients with unilateral chronic otitis media were reviewed. Eighty-four patients met the inclusion criteria that were normal otoscopy and normal hearing in the contra lateral ear. Bone conduction threshold averages were calculated over 500 Hz, 1000 Hz, 2000 Hz, 3000 Hz and 4000 Hz frequencies. We compared the bone conductions threshold averages between the normal ear and the ear with chronic otitis media. Thresholds were examined separately for each frequency. Results: The bone conduction threshold averages in the normal side were smaller than those of the chronic otitis media ear. The threshold shift was statistically significant in each frequency ( P < 0.001 t Student test) but in 500 Hz ( P = 0.16). There were no differences between the groups when we analyzed the role of age or in the role of the cholesteatoma presence in the sensorineural hearing loss. Conclusions: This study shows that chronic otitis media is associated with decrease in cochlear function. Neither the presence of cholesteatoma in the middle ear nor the patients’ age showed any influence in the sensorineural hearing loss.

Auditory function in a group of adults infected with HIV/AIDS in Gauteng, South Africa

Numerous international studies have demonstrated a relationship between HIV/AIDS and auditory function. The aim of this study was to explore this relationship in a group of adults infected with HIV/AIDS attending an outpatient clinic in a hospital located in Gauteng, South Africa. The prevalence of hearing loss; the type, degree and configuration of the hearing loss; the relationship between the hearing symptoms and the progressive stages of the disease, and the type of onset of hearing problems were examined. The results of the study indicated a prevalence rate of hearing loss which was as high as 23% in the sample surveyed. The types of hearing loss included conductive and sensorineural, while the degree of severity ranged from slight to profound in nature. The configuration of the hearing loss was not frequency-range-specific, and the degree of severity did not seem to worsen with the progression of the HIV/AIDS disease. However, there did seem to be an increase in the occurrence of sensorineural hearing loss with the deterioration of patients' immunological status. Analysis of patients' audiological results along with their case history data suggested that their hearing loss may have been caused by opportunistic infections and/or their treatments. These results are discussed in terms of their implications for the clinical management of patients with HIV/AIDS; education of team members; and policy formulation.

Cisplatin-induced ototoxicity: Morphological evidence of spontaneous outer hair cell recovery in albino guinea pigs?

Cisplatin is frequently used in the treatment of various forms of malignancies. Its therapeutic efficacy, however, is limited by the occurrence of sensorineural hearing loss. Little is known about the course of hearing loss over longer time intervals after cessation of cisplatin administration. Infrequently, recovery of hearing has been described in animals and humans. Stengs et al. (1997) treated guinea pigs with cisplatin at a daily dose of 1.5 mg/kg for 8 consecutive days and subsequently studied cochlear function after survival times varying from 1 day to 16 weeks. Spontaneous improvement of the hair cell-related potentials (cochlear microphonics and summating potentials) was observed starting 2 weeks after cessation of treatment. In the present study we examined light microscopically the cochleas used in the study of Stengs et al. (1997). One day after cessation of cisplatin administration outer hair cell (OHC) loss in the basal cochlear turn averaged 66%. In the 1-week survival group, OHC counts were similar to those of the 1-day survival group. In the 4-week survival group, however, a relatively small loss of OHCs was found in the basal cochlear turn; OHC loss averaged only 15%. A similar loss was found after 8 weeks. In the 16-week survival group, OHC loss in the basal turn increased to 48%, but this was not statistically significant. Our histological observations are in line with the electrophysiological data from the same animals. Our findings suggest that OHCs recover from cisplatin-induced damage 1–4 weeks after treatment. However, the results do not allow a conclusion as to whether the observed recovery is due to the formation of new OHCs or to (self-)repair of damaged OHCs.

Sensorineural Hearing Loss in Children After Liver Transplantation

DEUTSCH, ELLEN S.; BARTLING, VICTORIA; LAWENDA, BRIAN; SCHWEGLER, JOHN; FALKENSTEIN, KATHLEEN; DUNN, STEPHEN Author Information

Sensorineural hearing loss in children after liver transplantation.

To investigate risk factors for sensorineural hearing loss (SNHL) in children after liver transplantation. Retrospective medical record review. Pediatric tertiary care hospital. One hundred twenty-five consecutive children who received liver transplants between March 1, 1987, and June 30, 1996. The presence of SNHL (bone conduction threshold of >35 dB of hearing loss in at least 1 frequency) and the cause of the liver abnormality in all 125 patients. In addition, among the subset of children who had biliary atresia and underwent transplantation before 2 years of age, the total dose (milligrams per kilogram of body weight) of aminoglycoside antibiotic medications (tobramycin sulfate, gentamicin sulfate, and amikacin sulfate) and of intravenous loop diuretic agents (furosemide) was compared between children with and without SNHL. Audiologic evaluations were available for 66 of 125 patients, 15 (12%) of whom have SNHL. Of 5 survivors with the short-bowel syndrome, 4 have severe to profound SNHL. Of 46 children who have biliary atresia and who underwent transplantation before 2 years of age, 8 (17%) have SNHL. Among the 26 evaluable children with biliary atresia undergoing liver transplantation before 2 years of age, logistic regression analysis revealed that the most important risk factor for SNHL was the cumulative dose of amikacin (P = .05). Children receiving liver transplants are at an increased risk for SNHL. Those with the short-bowel syndrome have the greatest prevalence of SNHL. Among the subset of children with biliary atresia receiving liver transplants before 2 years of age, statistical analysis demonstrates a dose-response relationship between the receipt of amikacin and the occurrence of SNHL.

Hearing Disorders in Children With Fetal Alcohol Syndrome: Findings From Case Reports

Fourteen children with the fetal alcohol syndrome were evaluated by standard audiologic procedures. Thirteen of the 14 children had childhood histories of hearing disorders. All 13 of these children (93%) had clinically significant histories of bilateral recurrent serous otitis media (ie, they were otitis prone), and at least four children (29%) had bilateral sensorineural hearing losses in addition to being otitis prone. Many of the children with recurrent serous otitis media required repeated myringotomies with placement of ventilation tubes, and those with sensorineural hearing losses required sound amplification during childhood. Recurrent respiratory infections (secondary to immune deficiencies) and eustachian tube dysfunction (secondary to embryonic malformations of the first and second branchial arches) are discussed as possible etiologic factors in the presence of the recurrent serous otitis media. An alcohol-induced neuroectoderm syndrome and alcohol ototoxicity are discussed as possible etiologic factors in the occurrence of sensorineural hearing loss. The findings suggest that hearing disorders are a heretofore unrecognized characteristic of the fetal alcohol syndrome. Such hearing disorders may contribute to the speech and language and learning difficulties seen in children with fetal alcohol syndrome. The present study also provided confirmatory evidence of visual, health, and speech and language disorders in children with this syndrome.

Sensorineural hearing loss in the unoperated‐on otosclerotic ear

Severe sensorineural hearing loss occurs in less than 1% of stapedectomized ears. This low percentage remains as an irreducible minimum even among the most experienced and competent surgeons. The etiology has been hypothesized; however, the actual cause remains unknown. The rare occurrence of sensorineural hearing loss of undetermined etiology in the unoperated-on side was reported first in 1967 by Armstrong who presented a series of three patients. No other references have been found since this initial report. Recently three unilateral stapedectomized patients who developed sudden severe sensorineural hearing loss in the unoperated-on ear were studied during the years 1971 through 1979. The hearing loss occurred within 1 week, 6 weeks, and 12 years postoperatively. Although the number is small, a study of this group, in addition to Armstrong's, leads to several interesting considerations: 1. Is the incidence of sudden sensorineural type hearing loss greater, the same as, or less than that which develops in the non-otosclerotic general population? 2. Is there a possibility that the sudden sensorineural hearing loss of undetermined origin would occur at the time of surgery? Would this then be considered as a predisposing if not the actual etiology? The present series of six cases is so small that a conclusion is not possible and inference is only conjecture. It is hoped, however, that this may stimulate past, present, and future search for this unusual occurrence. This may help determine whether or not there is a causal or merely a coincidental relationship.

Inhibition of Na+,K+-stimulated ATPase in the cochlea of the guinea pig. A potential cause of disturbed inner ear function in terminal renal failure.

The frequent occurrence of sensorineural hearing loss in patients with chronic renal insufficiency prompted us to study the influence of chronic renal failure upon Na+,K+-ATPase in the inner ear of guinea pigs. Na+,K+-activated ATPase was defined as the ouabain-sensitive part of total ATPase, the activity of which was obtained in the presence of sodium, potassium and magnesium. A significant reduction of Na+,K+-activated ATPase was found in the inner ear of uremic animals. Such inhibition could be demonstrated as early as 12 hours after subtotal nephrectomy. An inverse correlation was found between serum creatinine levels and Na+,K+-activated ATPase. A similar inhibition of Na+,K+-activated ATPase in uremia is also found in other tissues (erythrocytes, renal tubules, intestinal mucosal cells, sarcolemma). Na+,K+-ATPase in the cochlea plays a key role in the maintenance of cochlear cationic gradients. It is suggested that inhibition of this enzyme system may contribute to the inner ear dysfunction in uremia.

Sensorineural hearing loss in chronic ear surgery.

The author's series of 3000 consecutive operations of tympanoplasty from 1960 to 1975 were reviewed in regard to the occurrence of sensorineural hearing loss as a consequence of the surgical procedure. Worsening of bone conduction thresholds by 10 dB through the frequencies 500 to 4000 cps, or a 10% reduction in speech discrimination scores were considered significant. Whereas in transcanal tympanoplasty the incidence of cochlear damage was greater in ears when initially the ossiculare chain was incomplete, by contrast in combined approach tympanoplasty the risk was greater when the chain was intact initially. It was concluded that cochlear trauma was usually due to 1) the hydraulic effect of excessive stapes manipulation during the removal of disease, and 2) the development of a perilymph fistula. The unpredictable predisposing threat of cochlear fragility due to genetic and inflammatory factors was emphasized and the poor results of tympanoplasty in tympanosclerosis were underlined. The current methods of treating sensorineural hearing loss after tympanoplasty were enumerated and discussed. It was concluded that although those aimed at improving labyrinthine circulation had theoretical backing, there is as yet little experimental or clinical evidence to support the claims of their protagonists.