Background: This retrospective study was carried out to know the characteristics of patients suffering from unilateral sensorineural hearing loss (USNHL) (type and degree). Aims and Objectives: (1) To know the incidence of pure USNHL (other ear being normal) in patients complaining of HL. (2) To establish the type and degree of USNHL in patients. Materials and Methods: This study was conducted over a period of more than 1 year to find out the pattern of pure USNHL (other ear being normal) in the patients attending ear, nose, and throat outpatient department (OPD) with a complaint of HL. Pure tone audiometry data of 1800 cases between August 2014 and December 2015 with HL were analyzed for this study. Demographic characteristics were compared with types and degrees of unilateral sensorineural hearing impairment. Results: A total of 1800 OPD patients who had a complaint of HL and had undergone pure tone audigram (PTA) in a period of more than 1 year in the Department of Otorhinolaryngology and Head and Neck Surgery were studied. SNHL type was the highest with 802 cases (44.55%). Out of 802 cases of SNHL, 257 (32.05%) were USNHL - out of which, only 155 patients (60.31%) had pure USNHL with other ear being normal; this constituted the study group, i.e., 155 patients (8.6%) out of 1800. Age of patients ranged from 9 years to 76 years (mean age - 41.5 years). Male:female ratio was 1.31:1. Both ears were almost equally involved. The highest numbers of USNHL patients were seen in the age group of 31-40 years (23.87%). Most cases presented with mild (34.2%), followed by profound (31.6%) USNHL. Conclusion: Our study concluded that SNHL type is the most common type (44.55%) of hearing impairment among patients complaining of HL. The incidence of pure USNHL (i.e. other ear being normal) was 60.31% (155/257) cases of USNHL, 19.32% (155/802) cases of SNHL, and 8.6% (155/1800) cases of PTA performed on patients complaining of HL. Limited work has been published on incidence, characteristics, and causes of pure USNHL. Further studies and researches are required in the field of USNHL to know the incidence, functional and psychological impact, and treatment options.

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Objective: An important factor determining the efficacy of speech therapy for children with severe hearing loss is the type and the method of educating children in the use of speech language items. Given the shortage of golden time for language learning, it is necessary to use efficient speech therapy approaches for improving the language of the child. The aim of this study was to investigate the effect of adding gestures to auditory-verbal training (AVT) in speech therapy for children with severe hearing loss. Methods: In this double-blinded clinical trial, 66 children with severe hearing loss were randomly assigned to two groups of 33 subjects. Receptive and expressive language competence for some simple Persian verbs was evaluated by the Newsha test. The AVT was accompanied by gesture for the intervention group and no accompanying gesture for the control group. Simple verbs include fifty early action words such as sit, run, hold, and go. Results: In both the experimental and control groups, a significant difference was observed for receptive language of simple verbs before and after the intervention (P = 0.001, P = 0.045). However, no significant difference was found before the intervention (P = 0.532), there was a significant difference between mean receptive score of the two groups after the intervention (P = 0.032). Conclusion: Adding gestures to AVT significantly changed the mean expressive score in 2-3-year-old children with severe hearing loss. But, both the synthetic approach and the AVT had the same degree of positive effect for education of receptive language of simple verbs for these children.

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Aim: To correlate the sequence of embryological development of ear with radiological imaging. Materials and Methods: The study enrolled 23 patients of age group 11 months to 27 years with malformed external ear/microtia and hearing loss. The children with postoperative changes, acute hearing loss (such as infection, trauma) were excluded from the study. We used high-resolution computed tomography, with axial and coronal sections to examine the temporal bones of patients. Results: Of the 23 cases, 12 cases had external ear anomaly and 11 cases had cochlear anomaly. In patients with the external ear anomaly, seven cases had associated middle ear malformations, one patient had associated middle and inner ear anomaly, one had anomalous course of facial nerve, and three cases had isolated external ear anomalies. In patients with inner ear anomalies, one patient had complete labyrinthine aplasia, one had cochlear dysplasia with incomplete cochlear turns, four had common cavity malformations, one had cystic featureless cochlea with dilated and cystic vestibule, two patients had small cochlea with the middle and apical turns coalescing to form a cystic apex, one patient had small rudimentary cochlea, and the other one had dilated vestibule and enlarged endolymphatic duct and sac with cochlear dysmorphism. Conclusion: Most of the children with malformed pinna had external auditory canal atresia with associated middle ear anomalies. Though the inner ear development is independent of external and middle ear development, we insist on the fact that insult during the 1 ^st month of embryogenesis can result in associated abnormalities involving external, middle, and inner ear. Developmental arrest at various stages of inner ear development results in various types of cochlear anomalies with associated vestibular, semicircular canal abnormalities, and rarely associated with middle and external ear anomalies. Hence, clear knowledge about embryology will help to guide the management.

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Context: Auditory brainstem response is the most important tool in differential diagnosis and degree of hearing impairment. Many studies have been carried out to ascertain the effects of noise on human beings but very less on the transportation workers; hence, considering the need of time and use of brainstem auditory-evoked potentials (BAEP), this study was conducted to analyze the effect of noise pollution on auto-rickshaw drivers (ARDs). Aim: The aim of this study was to evaluate I, II, III, IV, and V wave latencies in ARDs and comparing it with control subjects in Central India. Settings and Design: This was a case-control study done on ARDs as participants and compared it with normal healthy individual BAEP pattern. Materials and Methods: We recorded BAEP from fifty healthy control subjects and fifty ARDs from the community of same sex and geographical setup. The absolute latencies were measured and compared. Recording was done using RMS EMG EP MARK II machine manufactured by RMS recorders and Medicare system, Chandigarh. Statistical Analysis Used: All the data related with subjects were filled in Excel sheet and analyzed with the help of EPI 6.0 info software with Student's t-test. Results: There were prolongations of all absolute wave latencies of II, III, IV, and V in the ARDs as compared to control subjects. Conclusions: The prolongation of all absolute latencies of II, III, IV, and V suggests abnormality in brainstem auditory pathway mainly affecting the retrocochlear pathways in group of ARDs (noise exposure >10 years) than other group who had exposed for <10 years and is more significant on the right ear than left.

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Glutaric acidemia Type 1 is a condition due to inborn error of metabolism. It is generally characterized by elevated urinary excretion of glutaric acid, 3-hydroglutaric acid, and glutarylcarnitine. This can result in medical issues along with speech and language deficits. The extent of problems often varies from individual to individual. This study attempts to highlight the varying language characteristics and prognosis demonstrated in a child with glutaric acidemia Type 1.

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First branchial cleft fistula is a rare congenital malformation of the head and neck with an incidence of <8% of all branchial cleft defects. The patient presenting with discharging sinus in the neck with discharging ear should be investigated for a possible branchial cleft anomaly. Rarity and diverse presentation often lead to misdiagnosis and inadequate treatment. Recurrent infection and scarring make intraoperative identification of facial nerve made very difficult. We report a case of a 5-year-old boy with a first branchial cleft fistula, in which discharge was observed through the fistulous opening on the right side of the face and along the ear canal.

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Aim: The objective of the present study is the assessment of otoacoustic emissions (OAEs) and auditory brainstem responses (ABRs) for hearing screening of high risk infants. Study Design: Prospective, hospital-based. Materials and Methods: Distortion product OAEs (DPOAEs) and brainstem evoked response audiometry (BERA) recordings were obtained for 30 controls and 100 infants with one or more high risk factors, in a sound treated room and the results were interpreted. ABR peak latencies, amplitudes, and waveform morphology in high risk infants were compared with those in control group. DPOAE as screening test was evaluated in terms of various parameters with BERA/ABR taken as gold standard. Results: Absolute latencies of Wave I and Wave V and interpeak latency of I-V were significantly prolonged in high risk group as compared to control group. The most common causes to contribute significantly for hearing impairment were found to be hyperbilirubinemia, birth asphyxia, meningitis/septicemia. DPOAE when compared with ABR taken as gold standard showed that sensitivity of the test was 87.7% (74.5%-94.9%) and specificity was 74.5% (60.0%-85.2%). Positive predictive value was 76.7% (63.2%-86.6%) and negative predictive value of the test was 86% (71.9%-94.3%). Positive likelihood ratio was 0.29 (0.18-0.46) and negative likelihood ratio was 6.08 (2.82-13.09). Conclusion : ABR/BERA, though highly reliable, is a tedious and time consuming test. DPOAE is a simple and rapid test with relatively higher acceptability but low sensitivity and specificity; therefore, limits its role as independent screening test. DPOAE-ABR test series is an effective way to screen all the high risk infants at the earliest.

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Albinism is a congenital disorder characterized by complete or partial absence of pigments in the skin, eyes, and hair due to the absence or defective melanin production. As a result of that, there will be disruption seen in auditory pathways along with other areas. Therefore, the aim of the present study is to highlight the underlying auditory neural deficits seen in albinism and discuss the role of audiologist in these cases.

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Goldenhar-Gorlin syndrome (GGS) is a rare developmental anomaly, characterized by numerous anomalies affecting the first and second brachial arches which results in hemifacial atrophy in majority of cases (85%) and associated with cervical spine vertebral deformities, cardiovascular malformation, and sternocleidomastoid muscle abnormalities. Hereby, we report such a rare case of GGS in a 20 year old male patient, with all these classical features.

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Tubercular otitis media is a rare disease and its association with retropharyngeal abscess is rarer. We have not come across any such case in the English literature of the dual presentation of tuberculosis to the best of our knowledge so far. Early diagnosis and prompt management are the key to reduce the disease burden and also to avoid life-threatening complications.

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Introduction: Vestibular migraine (VM) is a vestibular syndrome seen in patients with migraine and is characterized by short spells of spontaneous or positional vertigo which lasts between a few seconds to weeks. Migraine and VM are considered to be a result of chemical abnormalities in the serotonin pathway. Neuhauser's diagnostic criteria for vestibular migraine is widely accepted. Research on VM is still limited and there are few studies which have been published on this topic. Materials and Methods: This study has two parts. In the first part, we did a retrospective chart review of eighty consecutive patients who were diagnosed with vestibular migraine and determined the frequency of auditory dysfunction in these patients. The second part was a prospective case control study in which we compared the audiological parameters of thirty patients diagnosed with VM with thirty normal controls to look for any significant differences. Results: The frequency of vestibular migraine in our population is 22%. The frequency of hearing loss in VM is 33%. Conclusion: There is a significant difference between cases and controls with regards to the presence of distortion product otoacoustic emissions in both ears. This finding suggests that the hearing loss in VM is cochlear in origin.

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Context: The occlusion effect (OE) is a well-known phenomenon in audiology clinical practice. Hence, some authors recommend the application of a correction factor to compensate for the OE. However, only two studies have assessed the OE using auditory steady-state responses (ASSRs). Aims: The aim of this study is to confirm the findings from previous ASSRs studies of the OE for a larger sample of normal-hearing adults. Settings and Design: Thirty-two normal-hearing adults (32 ears) with a mean age of 21 ± 2 years participated in this study. For each participant, one ear was selected at random, and the first measures to be obtained (occluded or unoccluded) were randomly determined. Subjects and Methods: The stimulus comprised a combination of four sinusoidal carrier tones, 500, 1000, 2000, and 4000 Hz, modulated in amplitude (95% depth) at the following rates: 104.2, 107.8, 111.4, and 115 Hz, respectively. It was presented through bone conduction for each participant under two different conditions (occluded and unoccluded ear). The overall ambient noise was 52 dB sound pressure level. Statistical Analysis Used: Repeated-measures analysis of variances was performed to compare occluded and unoccluded ASSR thresholds and amplitudes at 30 dB hearing level for 500, 1000, 2000, and 4000 Hz. Results: Occlusion caused a significant decrease of bone-conducted ASSR thresholds at low frequencies and a significant increase at 4000 Hz. Mean ASSR amplitudes were significantly higher after occlusion at low frequencies. However, some participants showed no OE at frequencies at which it is expected to be present. Conclusions: Despite the high ambient noise issue, results of previous studies are confirmed here in a larger sample of cases.

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Introduction: This observational study was carried out to determine the sensitivity and specificity of MB11 BERAphone^® , when used for neonatal hearing screening in a postnatal ward setting in comparison against the gold standard, auditory brainstem response (ABR). Materials and Methods: Thirty-seven consecutive newborns (74 ears) who either unilaterally or bilaterally failed hearing screening with MB11 BERAphone in the postnatal ward were recruited and a second screening with BERAphone was performed after 1 week along with confirmatory testing using ABR. Results: MB11 BERAphone showed sensitivity of 92.9%, specificity of 50%, positive predictive value of 30.23%, and negative predictive value of 96.77% for the diagnosis of hearing loss. The prevalence of confirmed hearing impairment was 18.9%. The rate of unilateral impairment was 10.8%, and the rate of bilateral impairment was 13.5%. The average ambient noise levels in the postnatal ward setting was 62.1 dB. Conclusion: Although the sensitivity of MB11 BERAphone is good, the specificity is significantly lower when the test is performed in the postnatal ward setting with high ambient noise. Neonates who fail the two-step screening should undergo auditory response for confirming the diagnosis of hearing loss.

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Objective: To correlate the size and site of perforation with hearing loss in chronic suppurative otitis media. Materials and Methods: This cross-sectional prospective study was carried out in a Tertiary Care Hospital in Western Uttar Pradesh. One hundred consecutive patients fulfilling the inclusion criteria were included in the study. In all the patients, a detailed history and a thorough ENT examination was followed by hearing assessment and measurement of the size of the perforation. Results: Infection was the most common etiology of tympanic membrane perforation in 92 (92%) cases and trauma in 08 (08%) with otorrhea as the most common presenting complaint. Half of the cases were in the age group of 20-30 years. Seventy-nine percentage cases had mild hearing loss whereas 19% had moderate hearing loss. Anterior quadrant perforations on an average had 31.4 dB hearing loss, whereas there was 43.9 dB average hearing loss in posterior quadrant perforations. Conclusions: It was observed that there is direct relationship between size and site of perforation and loss of hearing. There was less hearing loss in small sized perforations. Posterior quadrant perforations and malleolar perforations (MLs) had a greater hearing loss than anterior, multiple quadrant, or non-MLs.

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