[The association between serum bilirubin levels and hearing loss in the patients with bilateral sudden deafness].

Objective:The aim of this study was to investigate the association between serum bilirubin levels and the severity of bilateral sudden sensorineural hearing loss （BSSHL）. Method:A total of 113 patients with bilateral axillary sputum were enrolled, and the relationship between serum bilirubin levels and initial hearing levels was explored using a univariate and multivariate linear regression model. Result:Compared with the group with moderate and below hearing loss （≤70 dB HL）, patients with severe profound HL（>70 dB HL） were more likely to have lower levels of total and indirect bilirubin level, magnesium and relative hearing gain, higher levels of final hearing, white blood counts, neutrophil, platelet and alkaline phosphatase. After adjusting for possible confounders, only serum indirect bilirubin levels were significantly negatively correlated with initial hearing loss in patients with bilateral axillary sputum. 1 µmol/L increase of IBIL was associated with 1.1 dB （95%CI: -2.2, 0.0） reduction in initial hearing loss. Conclusion:Within the normal or mildly elevated range, higher levels of IBIL are independently and significantly associated with less severe hearing loss in BSSHL. It suggested a beneficial effect of bilirubin on auditory system.

Pathologic erythrocyte deformability in patients with sudden sensorineural hearing loss.

OBJECTIVE: To evaluate if viscoelastic properties of blood influence suffering sudden sensorineural hearing loss and the capacity to respond after a specific therapy. PATIENTS AND METHODS: A longitudinal prospective study included 85 ears bearing sudden deafness. In them, the mean hearing loss compared to the healthy ear and the recovery ratio were measured at the onset and 6 months after a treatment with corticoids and piracetam. In addition, tinnitus or vestibular symptoms, whole blood filterability (WBF) and erythrocyte deformability -by means of the erythrocyte rigidity index (ERI)- were determined and noted at the beginning and the end of the study. RESULTS: Mean hearing loss was 30.3±19.7% at the onset, and 25.8±39% at the end. Forty-one ears showed a recovery of more than 75%. In these (48% of the entire study group), an increase in WBF and a decrease in ERI were observed (P<.001). Ears without tinnitus or vestibular crisis recovered more hearing at 6 months and showed a significant improvement in WBF and ERI, not detected among patients with these clinical findings. There were good correlations between mean hearing loss at onset and WBF, and between recovery and ERI at 6 months, but without statistical significance. Patients with arterial hypertension, cardiopathy and hypercholesterolemia were the most frequently detected, while hypertension and hyperuricaemia showed a better hearing recovery ratio. CONCLUSIONS: The blood viscosity parameters WBF and ERI offer useful information about the risk of suffering sudden deafness and the capacity to recover hearing with reactive therapies.

Cytomegalovirus DNA detection in Guthrie cards: role in the diagnostic work-up of childhood hearing loss.

INTRODUCTION: Cytomegalovirus (CMV) infection is the leading cause of congenital nongenetic sensorineural hearing loss (SNHL) and a major cause of prelingual SNHL that is not present at birth. Polymerase chain reaction (PCR) analysis of dried blood samples on the Guthrie card has been proposed as a sensitive and specific method to screen for congenital CMV infection. METHODS: Prospectively, consecutive infants who failed universal neonatal hearing screening and children referred for a noncongenital SNHL (NCHL) were included and underwent a standard audiometric and etiologic work-up. DNA was extracted from dried blood spots on neonatal Guthrie cards and amplified by real-time PCR. Data were available for 96 cases. RESULTS: Mean age of the universal neonatal hearing screening group was 3.8 +/- 2.4 months (n = 41). Auditory brain stem response thresholds were 72.9 +/- 20.2 dB nHL. A CMV-positive PCR was obtained in 4 babies. One test was considered false-positive. This resulted in a 7.3% prevalence of congenital CMV infections.Mean age of the NCHL group was 4.9 +/- 3.2 years (n = 55). Hearing loss was moderate in 37, severe in 5, and profound in 13 children. A CMV-positive PCR was obtained in 4 children (7.3%). Other causes of SNHL were excluded in the PCR positive cases of both study groups. CONCLUSION: We advocate PCR for CMV DNA detection on Guthrie cards in the etiologic work-up of childhood SNHL and recommend serologic confirmation to exclude false-positive PCR results. 7.3% of SNHL in babies with congenital hearing loss and children with NCHL could be attributed with this technique to congenital CMV infection.

Immune thrombocytopenic purpura-related hemotympanum presenting with hearing loss.

A 5-year-old boy was admitted to our center with a major complaint of bilateral hearing loss for 2 days. He was diagnosed with acute immune thrombocytopenic purpura 3 months before the admission and treated with high-dose methylprednisolone 2 months ago. Physical examination revealed wet purpura in the oral mucosa, serous nasal discharge, multiple petechiae and ecchymosis of the lower lip. Otomicroscopic ear examination revealed the presence of bilateral hemotympanum. The patient denied head trauma, ear pain, fever, hypertension and medications, including salicylates. The patient received high-dose intravenous methylprednisolone because of low platelet count and wet purpura for 7 days and oral prophylactic amoxicillin-clavulanate for 14 days. The onset of the response to corticosteroids was rapid, and significant hematologic improvement was observed within a few days. The 2-week follow-up examination revealed intact tympanic membranes with normal color and mobility, and the patient restored normal hearing. In this patient, hemotympanum developed rapidly, and no predisposing cause other than immune thrombocytopenic purpura was found. However, presence of a serous nasal discharge may be a sign of viral upper respiratory tract infection. Therefore, it can be speculated that sneezing or coughing might have caused bilateral hemotympanum by increasing the middle ear pressure abruptly. We would like to emphasize that bleeding may occur in unusual sites and, unlike in healthy people, may cause bizarre symptoms in patients with bleeding diathesis. Hemotympanum can be considered among the indications to start treatment in patients with acute immune thrombocytopenic purpura.

The association of codeine, macrocytosis and bilateral sudden or rapidly progressive profound sensorineural deafness.

CONCLUSIONS: Codeine shows a similar association with profound deafness to other opiates. Good cochlear implant outcomes suggest a sensory disorder. Although macrocytosis could be due to confounding factors, the lack of other consistent findings may signify a vascular pathology. OBJECTIVES: To describe a series of patients identified as codeine users after referral for cochlear implantation. PATIENTS AND METHODS: This was a retrospective case series review. Patients were identified by the senior audiologist. Information regarding mode of onset of deafness, past medical history, drug and alcohol history, investigations and audiological outcomes following cochlear implantation was collected from hospital records and patient questionnaires. RESULTS: Ten patients were included in the study. All patients had taken codeine phosphate and paracetamol in combination for several years, usually at greater than recommended daily dose. All patients presented with sudden or rapidly progressive bilateral deafness. All patients had a significant macrocytosis at the time of deafness (mean cell volume (MCV): mean 115 fL; range 105-132 fL). No other investigation was consistently abnormal. Four patients had a history of alcoholism. Seven patients had abnormal liver function tests. Patients usually performed well with cochlear implants (CUNY sentence scores without lip reading >90% in 9 of 10 patients).

Progressive bilateral sensorineural hearing loss induced by an antithyroid drug.

We report a patient with antithyroid drug-induced progressive bilateral sensorineural hearing loss associated with myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA). While antithyroid drugs have been linked to MPO-ANCA-associated small-vessel vasculitis, sensorineural hearing loss rarely was noted. A 36-year-old man treated for hyperthyroidism with propylthiouracil (PTU) developed progressive bilateral sensorineural hearing loss accompanied by fever and arthritis. MPO-ANCA were demonstrated in serum. Distortion product otoacoustic emissions test results suggested dysfunction of outer hair cells of the organ of Corti. Inner ear blood flow impairment from ANCA-associated small-vessel vasculitis presumably caused cochlear dysfunction. PTU withdrawal and high-dose methylprednisolone administration greatly improved hearing on both sides.

Sudden bilateral hearing loss and sporadic mitochondrial DNA deletion.

Several studies have indicated that a number of different mitochondrial DNA (mtDNA) mutations may be responsible for human pathologies. Sensorineural Hearing Loss (SNHL) may be associated with known syndromes (syndromal SNHL) or represent the only manifestation of mitochondrial damage (non-syndromal hearing loss). Moreover, mtDNA alterations may be responsible for aminoglycoside-induced deafness. We describe a patient harbouring a single sporadic mtDNA deletion, who presented with sudden adult-onset bilateral, although non-simultaneous SNHL, that was partially responsive to corticosteroids. Increased values of rest, and exercise, blood lactic acid were decisive for diagnosis, prompting muscle biopsy that revealed the mtDNA deletion. The case underscores the importance of investigating a mitochondrial disease in cases of SNHL of unknown origin and points out the importance of an increased blood level of lactic acid as a screening test.

Lead exposure and hearing effects in children in Katowice, Poland.

The objective of the study was to investigate the relationship between lead exposure and hearing in children in the Katowice region, an industrial area in Poland. Blood lead was determined using inductively coupled plasma mass spectrometry, with appropriate quality control. The concentrations of lead in blood (B-Pb) in 155 children, aged 4-14, ranged from 19 to 281 microg/L (0.09 to 1.4 micromol/L), with a median of 72 microg/L (0.34 micromol/L). The hearing thresholds increased significantly with increasing blood lead levels at all investigated frequencies (0.5, 1, 2, 4, 6, and 8 kHz). The relationship also remained significant for B-Pb below 100 microg/L (0.48 micomol/L; n=107). The brainstem auditory evoked potential latency of wave I was significantly increased (also after adjustment for age) in the group of children with the highest blood lead levels (B-Pb above 100 microg/L, 0.48 micromol/L; n=51), compared to the group with the lowest ones (B-Pb below 46 microg/L, 0.22 micromol/L; n=51). The audiometric results clearly indicate that auditory function in children is impaired at a blood lead concentration even below 100 microg/L (0.5 micromol/L).

Serum antibodies to heat shock protein 70 in sensorineural hearing loss.

OBJECTIVE: To identify the 68-kd target of antibody in serum samples from patients with idiopathic, progressive, bilateral sensorineural hearing loss. DESIGN: To purify target protein from renal extracts using gel filtration, ion-exchange chromatography, and polyacrylamide gel electrophoresis and to transfer to nitrocellulose membranes. The purified protein was digested with trypsin, and peptide fragments were separated by high-pressure liquid chromatography. RESULTS: One fraction obtained by high-performance liquid chromatography contained a peptide of 2776 molecular weight. The sequence of a stretch of 22 amino acids within this peptide was identical to that of amino acids 424 through 445 of heat shock protein 70 (HSP70). On Western blotting, monoclonal antibody directed against HSP70 (but capable of recognizing both constitutive HSP70 [HSC70] and stress-inducible HSP70) reacted with the purified 68-kd protein. We compared the reactivity of serum samples from six patients with idiopathic, progressive, bilateral sensorineural hearing loss, as well as monoclonal antibody to HSC70, and monoclonal antibody to HSP70 with renal extract. The pattern obtained suggested that patient antibodies are preferentially directed at HSP70. CONCLUSION: The target of antibody in serum samples from patients with idiopathic, progressive, bilateral sensorineural hearing loss is HSP70.