Case of oculo-auriculo-vertebral spectrum: rare clinical features

A 2-month-old boy presented to us with bilateral microtia, left lower motor neuron facial palsy, micrognathia, hemivertebra, bifid rib, bifid thumb and absent/hypoplastic right-sided depressor anguli oris. He had bilateral external auditory canal atresia, although response to loud sound was present. Brain stem evoked response audiometry (BERA) was advised at 3 months of age. Karyotype was normal. We diagnosed him as a case of oculo-auriculo-vertebral spectrum. Child was discharged on request by the family with the plan for bone-anchored hearing aid after BERA and plan for pinna and ear canal reconstruction at a later age but child did not come for any follow-up visit. On telephonic enquiry, it was found that he is thriving well but has developmental delay including speech delay. We conclude that children presenting with external ear abnormalities should be screened for multiple congenital anomalies so that a multidisciplinary approach to management can be planned.

BRAIN STEM EVOKED RESPONSE AUDIOMETRIC FINDINGS IN POST NEONATAL MENINGITIS

Background: Hearing loss is an important complication of meningitis. We usually advise BERA study for detection of hearing loss particularly sensorineural type. BERA is superior to pure tone audiometry in malingering patients. Objective: To evaluate the sensorineural hearing following meningitis in children between 1 month to 2 years. Material and methods: This was a cross sectional, observational study of children aged 1month to 2 years who were admitted in Department of Paediatrics, R. G Kar. Medical college, Kolkata during the period of January 2017-June 2018, the study includes 30 babies who were suffering from Meningitis. Result: We found that out of 30 patients 23(76.7%) patients had no hearing loss, 3(10.0%) patients had mild hearing loss, (3.3%) patient had moderate hearing loss and 3(10.0%) patients had severe hearing loss in right ear. Conclusion: BERA is a helpful tool for assessing the severity of Hearing loss in post-meningitis patient. It is a useful test for screening of SNHL, especially in the young children and infants in whom other conventional methods may not be of much use.

A clinico-epidemiological study of hearing loss in adult patients presenting to a tertiary healthcare center in India

Background: The objectives of the study are: 1. To determine the profile of patients presenting with hearing loss. 2. To determine the causes of hearing loss in adult patients of various age groups. The cases for the study comprised of adult patients aged >/=20years, presenting to the ENT Out-patient Department with complaint of hearing loss. Detailed history thorough ENT examination including Examination of the ear, nose and throat with Bull’s eye lamp, Otoscopic/Otomicroscopic/Otoendoscopic examination with photo-documentation of the ear status when required, tuning fork tests, and a battery of audiological tests including pure-tone audiometry, impedance audiometry and Brain-stem Evoked Response audiometry (when required) were conducted and the reports of the same were enclosed with the Pro forma of the patients. Results and Discussion: Hearing loss is more common in old age and more male patients presented with hearing loss. The most common symptom seen with hearing loss was tinnitus which was commonly ringing type and this was commonly seen in older patients associated with sensori-neural hearing loss. The most common type of hearing loss was sensori-neural hearing loss seen more commonly in older patients and associated with tinnitus. The definitive diagnosis for patients with sensori-neural hearing loss was difficult to specify with the most common diagnosis being presbyacusis, followed by noise induced hearing loss. The second common diagnosis was Chronic Otitis Media(COM).

Evaluation of effect of brainstem evoked response audiometry in hyperlipidemic patients

<p class="abstract"><strong>Background:</strong> Brain stem evoked response audiometry (BERA) is a useful objective assessment of hearing. The major advantage of this procedure is its ability to test even infants in whom conventional audiometry may not be useful. This investigation can be used as a screening test for deafness at high risk. This study is to correlate changes in brainstem evoked response audiometry parameters concerning lipid profile.</p><p class="abstract"><strong>Methods:</strong> The study was conducted between January 2019 to June 2020 on 50 patients attending ENT OPD in Vinayaka Mission’s Kirupanandha Variyar Medical College and Hospital, Salem. All patients with auditory and/or vestibular complaints were seen at the otorhinolaryngology OPD and underwent an otorhinolaryngological examination, audiological studies (pure tone audiometry) and an electrophysiological assessment BERA. </p><p class="abstract"><strong>Results:</strong> The results of BERA was considered. A total of 5 waveforms and 3 interpeak latency waves were calculated. It was recorded from both the ears. There was a significant increase in the values of absolute waves II, III, IV, and V and interpeak latency wave values I-II and I-V.</p><p class="abstract"><strong>Conclusions:</strong> Early identification of hyperlipidemic patients is useful in preventing disease progression and associated morbidity and mortality. BERA is a non-invasive method which can help us to detect central auditory pathway dysfunction at early stages in hyperlipidemic patients even before the patient's experience symptomatic hearing loss.</p>

Correlation between serum electrolyte levels and brain stem evoked response audiometry in chronic renal failure: a pilot study

<p class="abstract"><strong>Background:</strong> The effect of electrolyte imbalance on hearing thresholds and its objective manifestation, as delayed latencies or inter-peak intervals in evoked response audiometry is studied.</p><p class="abstract"><strong>Methods:</strong> The present prospective study was undertaken in a period of one and a half years, to analyze the audiological profile in patients of chronic renal failure and renal allograft recipients. 60 patients were randomly selected from the out- patient and indoor services of nephrology, urology and oto-rhino-laryngology, Dayanand Medical College and Hospital, Ludhiana. Brain stem evoked response audiometry was performed and the latencies were tabulated. </p><p class="abstract"><strong>Results:</strong> A significant delay in the absolute latency of wave V was noted in hyponatremic patients of CRF on comparison with patients of CRF having a normal serum Na⁺ levels. The I-V interpeak interval was also seen to be significantly delayed on comparison. A statistically significant delayed I-III inter-peak latency was also observed in hypernatremic patients in comparison to patients having a serum Na⁺ level in the normal range. No significance of serum creatinine levels and wave latencies was noted on comparison between the three categories of patients of CRF as categorized by their serum creatinine levels.</p><p class="abstract"><strong>Conclusions:</strong> There is a definite deterioration of the audiological function in patients of chronic kidney disease, and some reversal of these abnormalities following a successful renal transplantation; indirectly pointing towards uremic milieu being the culprit.</p>

867. Upregulated Matrix Metalloproteinase-2 Relates to Milder Hearing Impairment in Bacterial Meningitis

Background Hearing impairment is a well-recognized sequela caused by bacterial meningitis, but the underlying pathophysiology remains largely unknown. Matrix metalloproteinase-2 (MMP-2) is known to affect neuronal cell damage and survival in different diseases of the brain. We investigated whether levels of MMP-2 in the cerebrospinal fluid (CSF) relate to the extent of hearing impairment in children with bacterial meningitis. Methods Clinical data of 179 children were obtained from a previous clinical trial examining the adjuvant treatment of bacterial meningitis in Latin America in 1996–2003. At discharge or shortly thereafter, the ability to hear was measured with brain stem evoked response audiometry or traditional pure tone audiometry. Levels of CSF MMP-2 on admission (CSF1, n = 161) and 12–24 hours later (CSF2, n = 133) were assessed by zymography. The combined results for the detected pro-form and active MMP-2 were compared with the audiological outcome of the patients. Results MMP-2 was detected in half of both the CSF1 and CSF2 samples. The median densitometric values with interquartile ranges (IQRs) were 0.04 (IQR 0.00–0.29) for CSF1 and 0.00 (IQR 0.00–0.33) for CSF2. Detectable MMP-2 associated with milder hearing impairment in CSF1 (P = 0.05), but not in CSF2 (P = 0.1). Patients who were deaf at discharge had lower levels of MMP-2 in both samples (CSF1, P = 0.05; CSF2, P = 0.04), compared with patients who were not deaf. A MMP-2 level over the 75th percentile in CSF1 predicted lower odds of any audiological sequelae (odds ratio 0.30, 95% confidence interval 0.14–0.68, P = 0.004). Conclusion The upregulation of MMP-2 in the CSF associated with a better audiological outcome in children with bacterial meningitis. The results suggest that MMP-2 might play a protective role in the development of hearing sequelae due to bacterial meningitis. Disclosures All authors: No reported disclosures.

Hearing Evaluation of Neonates with Hyperbilirubinemia by Otoacoustic Emissions and Brain Stem Evoked Response Audiometry

Introduction: Neonatal hyperbilirubinemia is one of the most important factors affecting the auditory system and can cause sensorineural hearing loss. This study evaluated the hearing status in neonates with hyperbilirubinemia and results obtained from Otoacoustic emissions (OAE) and Brain stem evoked response audiometry (BERA) analysis were compared with each other’s.Material and Methods:This study was performed on fifty eligible term neonates with hyperbilirubinemia requiring either phototherapy or exchange transfusion or both. Hearing analysis was done by OAE and BERA.Results: Out of fifty eligible neonates, twenty one (42%) had hearing impairment as per OAE analysis, eight (16%) had hearing impairment; two (4%) neonate had inconclusive hearing analysis as per BERA analysis. Out of twenty one neonates having hearing impairment by OAE only five had hearing impairment as per BERA analysis. While the eight neonates who had hearing impairment by BERA analysis, five had hearing impairment by OAE.Conclusion: The study showed hearing impairment by OAE analysis in 42% neonates while the BERA analysis showed hearing impairment in 16 % neonates. The increased level of Serum bilirubin has not shown the increase in percentage of hearing impairment.J Nepal Paediatr Soc 2016;36(3):310-313