Sentinel Surveillance for Congenital Rubella Syndrome - India, 2016-2017.

Rubella infection during pregnancy can result in miscarriage, fetal death, stillbirth, or a constellation of congenital malformations known as congenital rubella syndrome (CRS). The 11 countries in the World Health Organization (WHO) South-East Asia Region are committed to the elimination of measles and control of rubella and CRS by 2020. Until 2016, when the Government of India's Ministry of Health and Family Welfare and the Indian Council of Medical Research initiated surveillance for CRS in five sentinel sites, India did not conduct systematic surveillance for CRS. During the first 8 months of surveillance, 207 patients with suspected CRS were identified. Based on clinical details and serologic investigations, 72 (34.8%) cases were classified as laboratory-confirmed CRS, four (1.9%) as congenital rubella infection, 11 (5.3%) as clinically compatible cases, and 120 (58.0%) were excluded as noncases. The experience gained during the first phase of surveillance will be useful in expanding the surveillance network, and data from the surveillance network will be used to help monitor progress toward control of rubella and CRS in India.

Flunarizine in the prophylaxis of migrainous vertigo: a randomized controlled trial.

Migrainous vertigo is a common cause of dizziness presenting to an otorhinolaryngology/otoneurology clinic. Although it causes a substantial burden to the individual and society there are no randomized controlled trails on prophylactic medication for this condition. Flunarizine, a calcium channel blocker has been used effectively in both migraine and vestibular conditions. This randomized control trial was undertaken in a tertiary academic referral center to evaluate the efficacy of flunarizine in patients with migrainous vertigo when compared to non-specific vestibular treatment of betahistine and vestibular exercises. The effect of flunarizine on two particularly disabling symptoms of vertigo and headache was studied. A total of 48 patients who were diagnosed with definitive migrainous vertigo completed the study of 12 weeks duration. Patients in arm A received 10-mg flunarizine daily along with betahistine 16 mg and paracetamol 1 gm during episodes, and arm B received only betahistine and paracetamol during episodes. Symptom scores were noted at the start of the study and at the end of 12 weeks. Analysis of the frequency of vertiginous episodes showed a significant difference between arm A and arm B (p = 0.010) and improvement in severity of vertigo between the two groups (p = 0.046). Headache frequency and severity did not improve to a significant degree in arm A as compared to arm B. The main side effects were weight gain and somnolence and this was not significantly different between the two groups. Flunarizine (10 mg) is effective in patients with migrainous vertigo who suffer from considerable vestibular symptoms.

A Randomised Controlled Trial Comparing Intratympanic Gentamicin with Methylprednisolone in Meniere's Disease with Good Hearing.

A randomized prospective parallel group trial was done to compare the efficacy of intratympanic low dose gentamicin with methylprednisolone in treating intractable unilateral Meniere's disease with serviceable hearing. STUDY DESIGN: Randomised prospective parallel group trial. SETTING: Tertiary care centre in South India. SUBJECTS AND METHODS: Forty patients with unilateral Meniere's disease and serviceable hearing with vertigo following 6 months of conservative therapy were enrolled between November 2018 and March 2020. Twenty patients were administered with one dose of intratympanic Gentamicin (40 mg/ml) and the other half were given intratympanic Methylprednisolone (40 mg/ml, 4 injections given on alternate days). Pure tone audiogram, speech discrimination score, number of vertigo episodes, dizziness handicap inventory, tinnitus handicap inventory and functional scores were compared before treatment, 3 months later and up to 24 months. There was no significant difference between the two treatments with regard to short term as well as long term DHI scores, THI scores, Functional level score and average pure tone audiogram of patients. In patients with unilateral Meniere's disease who have good hearing, one dose of Gentamicin had equivalent effect to that of four doses of Methylprednisolone in vertigo and tinnitus control, hearing preservation and quality of life.

Recurrent Meningitis in a Non-user Cochlear Implantee.

Meningitis is a known complication in patients with inner ear malformations. Here we present a case of recurrent meningitis following cochlear implantation, in a patient with cochleovestibular anomaly. Good knowledge in radiology to identify the inner ear malformations, presence of cochlea and cochlear nerve is essential before planning cochlear implantation and meningitis can occur several decades after cochlear implantation.

Cochlear Implant in Klippel-Feil Syndrome: Challenges and Concerns.

Cochlear implantation (CI) can be safely performed in patients with syndromic hearing loss. Here we present a case of CI in a child with Klippel-Feil syndrome with various skeletal, extraskeletal, cochleovestibular and Arnold-Chiari malformations. Multidisciplinary approach and good preoperative imaging play a key role in planning for surgery.

Standard Programming of Retrograde Electrode Insertion via Middle-Turn Cochleostomy in Labyrinthitis Ossificans.

Successful cochlear implantation in the setting of labyrinthitis ossificans is challenging. Various surgical techniques are described to circumvent the region of ossification and retrograde insertion of the electrode array is one such option. While reverse programming is often recommended in the case of retrograde electrode insertion, we present our experience of retrograde electrode insertion for labyrinthitis ossificans, where standard programming was adopted due to patient preference and provided satisfactory outcomes.

Comparing Intratympanic Gentamicin with Methylprednisolone in Meniere's Disease with Non-Serviceable Hearing.

Abstract: To compare the effectiveness of high dose fixed alternate day intratympanic gentamicin with methylprednisolone in the treatment of patients with unilateral, intractable Meniere's disease with poor hearing. Randomized single blind prospective parallel group trial in a tertiary referral centre. Twenty-two patients with definite unilateral Meniere's disease with average pure tone thresholds worse than 50 dB in the affected ear were enrolled. Eleven patients were treated with intratympanic buffered gentamicin and the other eleven were administered intratympanic methylprednisolone (both 4 injections, 40 mg/ml, on alternate days). Patients were assessed pre-intervention, 3 months post intervention and subsequently followed up for 2-4 years. Both groups of patients had significant control of vertigo, DHI scores and THI scores after treatment while the functional scores in the methylprednisolone group was not better than the pre- treatment scores in the long-term follow-up. 9 of 11(82%) patients in gentamicin group and 3 of 11(27%) patients in the methylprednisolone group achieved Class A vertigo control. The gentamicin group had better post intervention DHI scores (p = 0.016, 3 months and p = 0.046, long term) and Functional score (p = 0.014, 3 months and p = 0.05, long term). The hearing in both groups and THI scores, post intervention was similar between both groups. In patients with unilateral intractable MD with non-serviceable hearing, high fixed doses of both intratympanic gentamicin and methylprednisolone are effective in alleviating disease symptoms in long term follow-up. However, intratympanic gentamicin resulted in better control of vertigo, total DHI score and functional level scores than intratympanic methylprednisolone with no significant difference in hearing levels. Trail Registration Number: Clinical Trials Registry of India (CTRI- REF/2016/10/012363).

Dimensions of the Posterior Tympanotomy and Round Window Visibility Through the Facial Recess: Cadaveric Temporal Bone Study Using a Novel Digital Microscope.

To measure the width of the posterior tympanotomy in cadaveric temporal bones using the digital microscope and classify the round window visibility through it. In 17 cadaveric wet adult temporal bones, cortical mastoidectomy followed by posterior tympanotomy was performed, delineating the facial and chorda tympani nerves. Antero-posterior width of the facial recess was measured at the levels of oval window and round window with the help of a digital microscope and its software. Visibility of the round window through the facial recess was assessed and classified according to the St. Thomas Hospital classification. The mean antero-posterior width of the facial recess measured was 4.7 ± 0.6 mm at the level of oval window and 4.3 ± 0.7 mm at the level of round window. Round window visibility grading in bones studied were as follows-Type 1 (53%), Type 2a (24%), Type 2b (18%) and Type 3 (5%). Interobserver variability of the posterior tympanotomy measurements using the digital microscope was found to be 91.1% with a 95% confidence interval of 79 to 97% at the level of oval window and 94.1% with a 95% confidence interval (CI) of 87 to 98% at the level of round window. The visibility of the round window is not entirely dependent on the facial recess width at the round window level, suggesting that other factors like cochlear rotation may also contribute to its actual location. Measurements of micro distances with the help of digital microscope seems to be convenient, cost effective and accurate with good inter observer reliability.

An Effective Home-Based Particle Repositioning Procedure for Posterior Canal Benign Paroxysmal Positional Vertigo (BPPV).

Patients with benign paroxysmal positional vertigo (BPPV) find it difficult to visit the hospital many times for a standard Epley's maneuver performed only by a specialist. The aim of this study was to compare the efficacy of a home-based particle repositioning procedure (HBPRP) with the standard Epley's maneuver in treating patients with posterior canal BPPV. A prospective non-blinded randomized controlled study was conducted. Patients were randomized into two groups, where one group received the standard treatment and other received a new HBPRP. The vertigo scale, duration of nystagmus during Dix-Hallpike test and frequency of vertigo, were documented on first, second and third visits, with complications noted during the second and third visits. These parameters were compared between both the groups following the treatment, during all visits. The patients were randomized into 2 arms with 15 each. Those belonging to group 1 received Epley's maneuver and group 2 received HBPRP. There was no significant difference in the baseline characteristics of patients in both groups. Both groups of patients had significant improvement of symptoms at the end of the study. A comparison of both groups at 2nd and 3rd visits showed no differences in frequency of vertigo, reduction in vertigo scale and duration of nystagmus following Dix-Hallpike test between both groups. HBPRP is a safe and effective procedure and can be taught as a home-based treatment for patients diagnosed with posterior canal BPPV.

Etiopathology and Prevalence of Pulsatile Tinnitus in a Tertiary Care Referral Hospital.

The diverse etiopathogenesis of pulsatile tinnitus (PT) makes it a difficult condition to diagnose and treat. To describe the clinical features, investigations and diagnosis of patients presenting with pulsatile tinnitus (PT). Retrospective chart review in an otology unit of a tertiary care referral centre. All medical records of patients who had a complaint of pulsatile tinnitus during the period 1st January 2014-1st May 2020 were included in the study. Data regarding history, characteristics of tinnitus, examination findings, investigations and diagnosis were collected and analyzed. Sixty-four patients with complaints of PT presented to our clinic during this time period and were included in the study giving a prevalence of 0.09%. Definite diagnosis was made in 62 (96.8%) cases with a detailed history, clinical examination and tailored investigations. Pathologies diagnosed were paraganglioma (25%), superior semicircular canal dehiscence (20.3%), anterior inferior cerebellar artery loop (7.8%), sigmoid sinus wall dehiscence (10.9%), sigmoid sinus diverticulum (6.25%), jugular bulb anomalies (7.8%) and hyperpneumatised petrous apex (3.1%) among others. Rare causes encountered were IgG4 disease, far advanced otosclerosis, vestibular aqueduct dehiscence and idiopathic intracranial hypertension. Pulsatile tinnitus is a rare complaint in the Otology clinic. Almost all cases of PT can be diagnosed correctly and appropriate treatment initiated with a logical approach to investigations.

A Comparative Study of Outcomes and Quality of Life in Canal Wall up Mastoidectomies and Canal Wall down Mastoidectomies.

Abstract: Chronic Otitis Media (COM) of the squamosal type was primarily managed by performing a canal wall down mastoidectomy; however, the latter era otolaryngologist envisioned benefits in preserving the posterior canal wall. Our primary objective was to assess the disease specific quality of life following canal wall up (CWU) mastoidectomy and canal wall down (CWD) mastoidectomy surgery after a 6 month post-operative period. A prospective observational study was done from September 2017 to August 2018 where the charts of 380 patients from Christian Medical College, Vellore were reviewed. Details of patients above 18 years who had undergone the above surgeries for COM active squamosal disease from the period of January 2014 to December 2016; and had their post-operative follow up (average of 16 months) during the period of study were looked at. The CWU group had a significantly better disease-specific quality of life in the symptoms subscale than the CWD group (p value < 0.01). The CWU group showed a significant air-bone gap closure to 23.3 dB as compared to 27.7 dB in CWD (p value = 0.005). The recurrence rates were 4.5% (9 cases) in the CWU group and 3.9% (7 cases) in the CWD group, which was not statistically significant. Both CWU and CWD methods of mastoidectomy for COM squamosal type give comparable outcomes in terms of recurrence with the CWU group having a better disease-specific quality of life after surgery. Level of Evidence: 2a.

Prevalence, clinical profile, and diagnosis of pediatric dizziness in a tertiary care hospital.

INTRODUCTION: Pediatric dizziness is an uncommon complaint presenting to the Otolaryngology clinic. While the term dizziness may be used to describe any altered sensation of orientation to the environment which includes presyncope, light-headedness and ataxia, vertigo refers to a false sensation of motion of self or surroundings. Although a variety of etiologies are known to cause dizziness and vertigo, evaluation of this symptom becomes challenging in children who are unable to clearly explain what they experience, the provoking factors, associated symptoms and the duration of attacks. Vestibular tests are also difficult to conduct in the pediatric age group leading to apathy from the clinician. OBJECTIVES: To ascertain the prevalence of pediatric vertigo in children under 18 years of age, presenting to the Otolaryngology Clinic of a tertiary care hospital, and to describe the clinical profile, investigations and diagnosis in these children. METHODS: A prospective cross-sectional, descriptive clinical study was undertaken from January 1, 2018 to April 30, 2019. All children below the age of 18 years presenting to our department with primary complaints of dizziness were included in the study. After a thorough history and physical examination, screening methods and diagnostic tests were conducted to make a diagnosis. Referrals were sought from other specialties when necessary. RESULTS: The number of children visiting the Department for various ENT ailments during the study period was 10,950. Among these 89 children presented with a primary complaint of dizziness. Their ages ranged from 3 to 18 years; mean age was 11.42 years (SD 3.45). A diagnosis was made in all except two children. The most common cause of dizziness in the age group less than 6 years was benign paroxysmal vertigo of childhood (BPVC) and in the older children was migraine associated vertigo, which was also the commonest overall diagnosis made (28.1%). This was followed by circulation related dizziness like orthostatic hypotension and vasovagal syncope (15.7%). CONCLUSIONS: The prevalence of pediatric dizziness in children presenting to the Otolaryngology clinic was 0.8%. The diagnosis of pediatric vertigo may be challenging, but careful history and examination along with guided investigations and referrals results in correct diagnosis in almost all patients.

Skull base osteomyelitis with secondary cavernous sinus thrombosis: a rare presentation of an animate foreign body in the ear.

A 42-year-old woman presented with fever, left ear pain, restricted mouth opening, difficulty in swallowing and inability to open her left eyelid for a period of 10 days. She was treated with antibiotics for the same at a local medical facility; however, a sudden decrease in her left eye vision prompted her to visit our tertiary centre. Her history was insignificant except for having multiple left ear syringing for an insect removal 10 days before onset of her current symptoms. On examination, she had ptosis of the left eye with chemosis, dilated pupil with only perception of light and restricted ocular mobility. Oral examination revealed trismus and bulge in the left peritonsillar region. Left ear examination revealed a large central perforation with mucopurulent discharge. CT of the neck with contrast demonstrated a collection in the left peritonsillar space with left internal carotid artery thrombosis. MRI of the brain with gadolinium revealed left cavernous sinus thrombosis with acute infarcts in the left frontal lobe. An emergency incision and drainage of the left peritonsillar abscess was performed. Culture grew broad aseptate fungal hyphae. Despite starting on antifungal therapy, she succumbed to her illness.

Normative Data for Cortical Evoked Response Audiometry of a Heterogeneous Indian Population and Its Comparison with Behavioral Audiometry.

OBJECTIVE: The objective of the study is to establish normative values of cortical evoked response audiometry (CERA) in a heterogeneous Indian population and correlate CERA threshold with pure tone audiometric (PTA) threshold values. MATERIALS AND METHODS: A prospective study was carried out on 31 volunteers (n = 62) who had no otological or neurological complaints. Two study groups were formed; Group 1 with individuals from 20 to 40 years (mean age of 29.1 years) and Group 2 with individuals from 41 to 60 years (mean age of 46.2 years). The latencies and amplitudes of the waves of P1, N1, and P2 at threshold and 70 dBnHL were measured. RESULTS: Twenty-nine participants (94% of the ears) had CERA threshold within 20 dB of true behavioral threshold with only 6% having a difference of >20 dB with their PTA thresholds. There was a significant difference (P < 0.05) at 70 dB in amplitudes for waves P1, N1, and P2 at 2 kHz and additionally at 1 kHz for N1 between the two groups. CONCLUSION: Normative values for CERA in a heterogeneous Indian population at 70 dB nHL using tone burst stimulus was found to have an average latency of 46.5, 90.1, and 155.5 ms for P1, N1, and P2, respectively. The average amplitude of P1 at 70 dB nHL was 4.3 µV, N1, was 6.5 µV and P2 was 3.2 µV. Hearing threshold obtained with CERA gave a good indication of the actual behavioral hearing threshold of the normal controls, and the age of an individual had a significant effect on the values obtained during CERA testing with N1 being significantly larger at 1 kHz and 2 kHz in older adults when compared to young adults.

Labyrinthine Fistulae in Squamosal Type of Chronic Otitis Media: Therapeutic Outcome.

INTRODUCTION: Labyrinthine fistulae (LF) are the common complications of chronic otitis media (COM) of squamosal variety. The final therapeutic outcome of this condition is to preserve the cochlear and vestibular functions. Herein, we present the data of the cases managed at our institute with respect to their presenting complaints, adopted therapeutic approaches and outcomes. MATERIALS AND METHODS: A retrospective chart review was conducted on all cases with COM squamosal type in adult patients. A total of 275 patients were reviewed, out of whom 30 cases had LF. The results were mainly studied with respect to the postoperative improvement of hearing and vertigo. RESULTS: The incidence rate of LF in the present study was obtained at 10.9%. Only 50% of the cases had the symptoms of vertigo. Furthermore, positive fistula test was elicited in 3.3% of the cases. All cases undergoing preoperative imaging were diagnosed successfully. In addition, 42.85% of the cases had profound hearing loss preoperatively, which sustained after the operation. However, 47.61% of the cases showed an improvement of at least ≥ 10 dB in the air-bone gap. Out of the 15 LF cases with vertigo as the main complaint, only 11 cases referred for follow-up. In this regard, 63.63% of the cases had no postoperative vertigo symptoms. CONCLUSION: Patients with LF may not have complaints of vertigo and a positive fistula sign upon admission. Pre-operative imaging facilitates the diagnosis of this condition. The removal of the matrix under constant irrigation, followed by repair with bone wax and/or autologous tissue, is sufficient to preserve the cochlear and vestibular symptoms postoperatively.

A Smartphone-based gaming system for vestibular rehabilitation: A usability study.

BACKGROUNDVestibular dysfunctions result in a wide range of impairments and can have debilitating consequences on a person's day-to-day activities. Conventional vestibular rehabilitation is effective but suffers from poor therapy compliance due to boredom. Virtual reality technology can make training more engaging and allow precise quantification of the training process. However, most existing technologies for vestibular rehabilitation are expensive and not suitable for use in patients' homes and most clinics. In this pilot study, we developed and evaluated the usability of a smartphone-based head-mounted display (HMD) for vestibular rehabilitation and quantified the simulator sickness induced by the system.METHODSTwo adaptive training games were developed to train discrete and rhythmic head movements in the pitch and yaw planes. The usability and simulator sickness associated with the system were evaluated in a single testing session on healthy subjects and patients with unilateral vestibular dysfunction. Additionally, the head movement kinematics measured during training was also analyzed using different movement quality measures.RESULTSA total of 15 healthy subjects and 15 patients underwent testing with the system. Both groups found the system to be highly usable (>80 score on the system usability scale). Following 20-30 min training with the system, healthy subjects reported minimal simulator sickness symptoms. On the other hand, patients reported a higher incidence rate for symptoms, which could have been the result of their vestibular condition.CONCLUSIONThe current study demonstrated the usability and safety of a smartphone-based system for vestibular rehabilitation. The system is compact, and affordable thus has the potential to become an excellent tool for home-based vestibular rehabilitation.

Posterior semicircular canal dehiscence: a diagnostic and surgical conundrum.

Third window defects have increasingly been identified as a cause of vertigo. These defects are bony dehiscences that occur in the bony labyrinth, resulting in abnormal pressure gradient in the inner ear fluids leading to sound (Tullio's phenomenon) or pressure (Hennebert's sign) induced vertigo. The superior semicircular canal dehiscence syndrome is a well-described entity in this regard, however defects of the posterior semicircular canal are rare and may have overlapping symptomatology. We describe the history, clinical profile and management of a patient who had importunate symptoms despite being on conservative management for a year and had resolution of vestibular symptoms following surgical management.

Congenital Cholesteatoma in Adults-Interesting Presentations and Management.

To report a series of adult patients diagnosed with congenital cholesteatoma (CC) with respect to symptoms, different varieties of presentation, surgical findings and approach used, complications and the postoperative results. A retrospective chart review of adult cases of CC who were treated in the period from January 2014-2017 was carried out in a tertiary care center. Levenson's criteria were used for diagnosis. Diagnosis was confirmed by imaging and intraoperatively. Postoperative results and complications were also analyzed. Six adult cases of CC were studied with a mean follow up of 10 months. Interesting presentations included otitis media with effusion, non-resolving facial nerve palsy, post aural discharge and meningitis. It included 3 cases of petrous apex cholesteatoma, 2 patients with cholesteatoma involving both the middle ear and mastoid and 1 patient with mastoid cholesteatoma. The operative procedures included canal wall up mastoidectomy (1 patient), atticotomy (1 patient), canal wall down mastoidectomy (1 patient), translabyrinthine and transotic excision of mass with blind sac closure (2 patients) and partial labyrinthectomy (1 patient). Complications encountered during surgery were cerebrospinal fluid leak and worsening of hearing in 2 patients and 1 patient respectively. CC can have variety of interesting presentations in adult population and they may or may not have the classical white mass behind the tympanic membrane. Appropriate individualized surgical planning and intervention gives good results.

Radiological Evaluation of Cochlear Orientation and Its Implications in Cochlear Implantation.

To test whether there are variations in cochlear orientation with respect to age and sex, and its relevance in cochlear implant surgery. Implant otologists rely upon the anatomic landmarks including the facial recess and round window niche and round window membrane for accessibility and placement of electrode array into scala tympani of basal turn of cochlea. Anecdotally, surgeons note variations in cochlear orientation with respect to age. Cochlear orientation studied radiologically by pre-operative CT scan of temporal bone can guide a Surgeon's approach to cochlear implantation. To investigate the changes in cochlear orientation with respect to age and sex; and its relevance in cochlear implantation. A retrospective analytical study was performed on CT scans of temporal bones in patients (of our hospital from July 2013 to January 2015 i.e. for a period of 18 months) with no congenital or radiological abnormalities of cochlea. The basal turn angulations of cochlea varied with age and majority of change occurred during early age. The basal turn angulations of cochlea in difficult situations during cochlear implantation were correlated with the data. There is a significant variation in cochlear orientation as measured radiologically by basal turn angulations relative to midsagittal plane. The more obtuse and acute basal turn angulations have implications like difficulty in cochleostomy and electrode placement during cochlear implantation.

Audiological Profile in Primary Sjögren's Syndrome in a Tertiary Care Setting and its Clinical Implications.

This study aims to assess the frequency and the profile of hearing loss among patients with primary Sjögren's syndrome in a tertiary care hospital in India and to look for an association between hearing loss and immunological parameters (anti-SSA antibody, anti-SSB antibody, anticardiolipin antibodies, complements C3 and C4). This prospective observational study was done from January 2011 to October 2011 on consecutive patients diagnosed with primary Sjögren's syndrome in our tertiary care hospital. All patients underwent a puretone audiogram, tympanogram and acoustic reflex testing. The results of the tests were correlated with clinical and immunological findings. The frequency of audiometrically confirmed hearing loss in primary Sjögren's syndrome was estimated to be 78.38 %, though only 17.24 % complained of hearing loss; minimal to mild sensorineural hearing loss were the most common varieties. The commonest finding on tympanometry was 'A' type curve and acoustic reflex was absent in 18.92 % of cases. There was no association between hearing loss and age, sicca symptoms, systemic symptoms or immunological test results in primary Sjögren's syndrome. There was a high prevalence of hearing loss among patients with primary Sjögren's syndrome, but most patients were unaware of this. Hearing assessment and regular monitoring of hearing thresholds is advisable for all patients with primary Sjögren's syndrome.