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Fulltext Anterior external bony ear canal bulge

Rajagopalan R

Indian J Otolaryngol Head Neck Surg, 2010 Jan;62(1):90-1.
PMID: 23120691 DOI: 10.1007/s12070-010-0001-0

To study the prevalence of anterior external bony ear canal bulge.
Bilateral nontuberculous mycobacterial middle ear infection: a rare case

Tang IP, Singh S, Rajagopalan R

Ear Nose Throat J, 2014 Sep;93(9):390-4.
PMID: 25255345

Nontuberculous Mycobacterium (NTM) middle ear infection is a rare cause of chronic bilateral intermittent otorrhea. We report a rare case of bilateral NTM middle ear infection in which a 55-year-old woman presented with intermittent otorrhea of 40 years' duration. The patient was treated medically with success. We conclude that NTM is a rare but probably under-recognized cause of chronic otitis media. A high index of suspicion is needed for the diagnosis to avoid prolonged morbidity. Treatment includes surgical clearance of infected tissue with appropriate antimycobacterial drugs, which are selected based on culture and sensitivity.
Laryngeal metastasis from a rectal carcinoma

Ramanathan Y, Rajagopalan R, Rahman NA

Ear Nose Throat J, 2007 Nov;86(11):685-6.
PMID: 18225631

Metastases to the larynx from distant primaries are rare. We report a case of a laryngeal metastasis from a rectal carcinoma.
Base of tongue tuberculosis: a case report

Hussaini J, Mutusamy S, Omar R, Rajagopalan R, Narayanan P

Acta Med Iran, 2012;50(2):151-2.
PMID: 22359087

We report a rare case of base of tongue tuberculosis following pulmonary tuberculosis. Patient presented to us with chief complaints of sore throat and pain on swallowing for period of 3 months. On examination with 70 degree telescope, we observed an ulcer on right side of base of tongue. The edges of the ulcer appeared to be undermined with whitish slough at the centre of the ulcer. Examination of neck showed a multiple small palpable middle deep cervical lymph nodes on right side of neck. Biopsy of the ulcer was taken, which showed granulomatous inflammation, suggestive of tuberculosis. Laboratory investigations revealed a raise in erythrocyte sedimentation rate, sputum for acid fast bacilli was strongly positive. Chest X ray was performed for patient showed multiple areas of consolidation. Patient was referred to chest clinic for further management of tuberculosis and was started on anti-tuberculous drugs. In conclusion tuberculosis of oral cavity is rare, but should be considered among one of the differential diagnosis of the oral lesions and biopsy is necessary to confirm the diagnosis.
Low lying larynx

Govindaraju R, Rajagopalan R, Omar R, Mukari SA

Congenit Anom (Kyoto), 2010 Sep;50(3):193-6.
PMID: 20507348 DOI: 10.1111/j.1741-4520.2010.00280.x

We report a rare case of laryngotracheal anomaly and its possible etiology and mode of presentation. A teenager presented with voice change and a neck lump. Investigations revealed a laryngeal anomaly in which the larynx was hyperdescended. It was accompanied by low lying thyroid gland and hyoid bone together with an absence of a cervical segment of the esophagus and trachea. The anomaly only became noticeable secondary to pubertal changes in the thyroid cartilage of the teenager. An embryological defect during the formation of the laryngotracheal tube and esophagus is a possible explanation of this anomaly. The present case probably represents the third reported of its kind.
Hearing loss after noise exposure

Govindaraju R, Omar R, Rajagopalan R, Norlisah R, Kwan-Hoong N

Auris Nasus Larynx, 2011 Aug;38(4):519-22.
PMID: 21236610 DOI: 10.1016/j.anl.2010.12.006

The higher field strength magnetic resonance imaging (MRI) such as 3 Tesla (T) and above generates noise that has potential detrimental effects on the hearing. Temporary threshold shifts following MRI examination have been reported for MRI with lower field strength. Such effect, however, have not been reported so far for a 3T MRI. We report a case that exemplifies the possible detrimental effects of a 3 T MRI generated noise on the auditory system. Our patient underwent investigation of his chronic backache in a 3 T MRI unit and developed hearing loss and tinnitus post-MRI examination. Hearing assessment was done using pure tone audiogram, distortion product otoacoustic emission (DPOAE) and brainstem electrical response audiometry (BERA) which revealed a unilateral sensorineural hearing loss which recovered within 3 days. However the tinnitus persisted. This is possibly a case of temporary threshold shift following noise exposure. However a sudden sensorineural hearing loss remains the other possibility.
Comparison of Grafting Success Rate and Hearing Outcomes between Primary and Revision Tympanoplasties

Faramarzi M, Shishegar M, Tofighi SR, Sharouny H, Rajagopalan R

Iran J Otorhinolaryngol, 2019 Jan;31(102):11-17.
PMID: 30783594

Introduction: There are a few studies that compare the outcomes between primary and revision tympanoplasties. The purpose of the present study was to compare the results of type I tympanoplasty (i.e., synonymous to myringoplasty) and revision myringoplasty based on the closure of tympanic membrane perforation and hearing improvement.
Materials and Methods: This prospective single-blind study was carried out on a total of 240 patients with tympanic membrane perforation at a tertiary referral center. The subjects underwent primary or revision myringoplasty. Grafting success rate and hearing results were measured and the comparison between the primary and revision groups was drawn.
Results: Grafting success rate was reported as 96.6% (112 out of 116 cases) for myringoplasty, while in revision myringoplasty the success rate of 78.2% (97 out of 124 patients) was achieved (P=0.001). Speech reception threshold was 23.1±9.2 dB and 24.9±13.1 dB in the primary and revision groups, respectively (P>0.05). However, the percentage of air-bone gap on audiometry≤20 dB were 83.8% and 76% in the primary and revision groups, respectively (P=0.26).
Conclusion: The findings of the present study have shown that although grafting success was reported significantly better in myringoplasty (tympanoplasty type 1), compared to that in revision myringoplasty, it did not reveal any superiority over revision tympanoplasty regarding the hearing outcomes. No consensus was achieved due to a great number of controversies in the literature.
Endoscopic skull base training using 3D printed models with pre-existing pathology

Narayanan V, Narayanan P, Rajagopalan R, Karuppiah R, Rahman ZA, Wormald PJ, et al.

Eur Arch Otorhinolaryngol, 2015 Mar;272(3):753-7.
PMID: 25294050 DOI: 10.1007/s00405-014-3300-3

Endoscopic base of skull surgery has been growing in acceptance in the recent past due to improvements in visualisation and micro instrumentation as well as the surgical maturing of early endoscopic skull base practitioners. Unfortunately, these demanding procedures have a steep learning curve. A physical simulation that is able to reproduce the complex anatomy of the anterior skull base provides very useful means of learning the necessary skills in a safe and effective environment. This paper aims to assess the ease of learning endoscopic skull base exposure and drilling techniques using an anatomically accurate physical model with a pre-existing pathology (i.e., basilar invagination) created from actual patient data. Five models of a patient with platy-basia and basilar invagination were created from the original MRI and CT imaging data of a patient. The models were used as part of a training workshop for ENT surgeons with varying degrees of experience in endoscopic base of skull surgery, from trainees to experienced consultants. The surgeons were given a list of key steps to achieve in exposing and drilling the skull base using the simulation model. They were then asked to list the level of difficulty of learning these steps using the model. The participants found the models suitable for learning registration, navigation and skull base drilling techniques. All participants also found the deep structures to be accurately represented spatially as confirmed by the navigation system. These models allow structured simulation to be conducted in a workshop environment where surgeons and trainees can practice to perform complex procedures in a controlled fashion under the supervision of experts.
3 Tesla magnetic resonance imaging noise in standard head and neck sequence does not cause temporary threshold shift in high frequency

Lim EY, Tang IP, Peyman M, Ramli N, Narayanan P, Rajagopalan R

Eur Arch Otorhinolaryngol, 2015 Nov;272(11):3109-13.
PMID: 25205300 DOI: 10.1007/s00405-014-3232-y

High acoustic noise level is one of the unavoidable side effects of 3 T magnetic resonance imaging (MRI). A case of hearing loss after 3 T MRI has been reported in this institution and hence this study. The objective of this study was to determine whether temporary threshold shift (TTS) in high frequency hearing occurs in patients undergoing 3 T MRI scans of the head and neck. A total of 35 patients undergoing head and neck 3 T MRI for various clinical indications were tested with pure tone audiometry in different frequencies including high frequencies, before and after the MRI scan. Any threshold change from the recorded baseline of 10 dB was considered significant. All patients were fitted with foamed 3 M earplugs before the procedure following the safety guidelines for 3 T MRI. The mean time for MRI procedure was 1,672 s (range 1,040-2,810). The noise dose received by each patient amounted to an average of 3,906.29% (1,415-9,170%). The noise dose was derived from a normograph used by Occupational Noise Surveys. This was calculated using the nomograph of L eq, L EX, noise dose and time. There was no statistically significant difference between the hearing threshold before and after the MRI procedures for all the frequencies (paired t test, P > 0.05). For patients using 3 M foamed earplugs, noise level generated by 3 T MRI during routine clinical sequence did not cause any TTS in high frequency hearing.
Fulltext Radiological Study of the Ethmoidal Arteries in the Nasal Cavity and Its Pertinence to the Endoscopic Surgeon

Kho JPY, Tang IP, Tan KS, Koa AJ, Prepageran N, Rajagopalan R

Indian J Otolaryngol Head Neck Surg, 2019 Nov;71(Suppl 3):1994-1999.
PMID: 31763282 DOI: 10.1007/s12070-018-1415-3

We studied the ethmoidal arteries using preexisting computer tomography of the paranasal sinuses (CT PNS) and statistically scrutinized data obtained between genders. A descriptive study from 77 CT PNS dated January 2016-December 2016 were collected and reviewed by two radiologists. A total of 54 (108 sides) CT PNS were studied of patients aged 18-77 years. 37 are male, 17 are female; with Bumiputera Sarawak predominance of 25 patients, 12 Malays, 16 Chinese and one Indian. Rate of identification are as follows: anterior ethmoidal artery (AEA)-100%, middle ethmoidal artery (MEA)-30%, posterior ethmoidal artery (PEA)-86%. The average distance from AEA-MEA is 8.1 ± 1.52 mm, MEA-PEA is 5.5 ± 1.29 mm and AEA-PEA is 12.9 ± 1.27 mm. The mean distance from PEA-the anterior wall of sphenoid is 7.7 ± 3.96 mm, and PEA-optic canal is 8.5 ± 3.1 mm with no statistical difference when compared between gender. AEA frequently presented with a long mesentery 57.4%, while 87.1% of PEA was hidden in a bony canal. The vertical distance of the AEA-skull base ranges from 0 to 12.5 mm whilst PEA-skull base is 0-4.7 mm. There is no statistical difference in distances of AEA, MEA nor PEA to skull base when analyzed between genders; t(82) = 1.663, p > 0.05, t(32) = 0.403, p > 0.05 and t(75) = 1.333, p > 0.05 respectively. We newly discovered, that 50% of MEA is hidden in a bony canal, and its distance to skull base ranged 0-5.3 mm. MEA and PEA less commonly have a short or long mesentery. Knowledge on the ethmoidal arteries especially in our unstudied population of diverse ethnicity, gains to assist surgeons worldwide, when embarking in endoscopic transnasal surgeries.
Fulltext Six-Year Outcomes in Subjects with Polypoidal Choroidal Vasculopathy in the EVEREST II Study

Teo KYC, Park KH, Ngah NF, Chen SJ, Ruamviboonsuk P, Mori R, et al.

Ophthalmol Ther, 2024 Apr;13(4):935-954.
PMID: 38308746 DOI: 10.1007/s40123-024-00888-0

INTRODUCTION: The EVEREST II study previously reported that intravitreally administered ranibizumab (IVR) combined with photodynamic therapy (PDT) achieved superior visual gain and polypoidal lesion closure compared to IVR alone in patients with polypoidal choroidal vasculopathy (PCV). This follow-up study reports the long-term outcomes 6 years after initiation of the EVEREST II study.
METHODS: This is a non-interventional cohort study of 90 patients with PCV from 16 international trial sites who originally completed the EVEREST II study. The long-term outcomes were assessed during a recall visit at about 6 years from commencement of EVEREST II.
RESULTS: The monotherapy and combination groups contained 41 and 49 participants, respectively. The change in best-corrected visual acuity (BCVA) from baseline to year 6 was not different between the monotherapy and combination groups; - 7.4 ± 23.0 versus - 6.1 ± 22.4 letters, respectively. The combination group had greater central subfield thickness (CST) reduction compared to the monotherapy group at year 6 (- 179.9 vs - 74.2 µm, p = 0.011). Fewer eyes had subretinal fluid (SRF)/intraretinal fluid (IRF) in the combination versus monotherapy group at year 6 (35.4% vs 57.5%, p = 0.032). Factors associated with BCVA at year 6 include BCVA (year 2), CST (year 2), presence of SRF/IRF at year 2, and number of anti-VEGF treatments (years 2-6). Factors associated with presence of SRF/IRF at year 6 include combination arm (OR 0.45, p = 0.033), BCVA (year 2) (OR 1.53, p = 0.046), and presence of SRF/IRF (year 2) (OR 2.59, p = 0.042).
CONCLUSION: At 6 years following the EVEREST II study, one-third of participants still maintained good vision. As most participants continued to require treatment after exiting the initial trial, ongoing monitoring and re-treatment regardless of polypoidal lesion status are necessary in PCV.
TRIAL REGISTRATION: ClinicalTrials.gov identifier, NCT01846273.
Genomic analyses in Cornelia de Lange Syndrome and related diagnoses: Novel candidate genes, genotype-phenotype correlations and common mechanisms

Kaur M, Blair J, Devkota B, Fortunato S, Clark D, Lawrence A, et al.

Am J Med Genet A, 2023 Aug;191(8):2113-2131.
PMID: 37377026 DOI: 10.1002/ajmg.a.63247

Cornelia de Lange Syndrome (CdLS) is a rare, dominantly inherited multisystem developmental disorder characterized by highly variable manifestations of growth and developmental delays, upper limb involvement, hypertrichosis, cardiac, gastrointestinal, craniofacial, and other systemic features. Pathogenic variants in genes encoding cohesin complex structural subunits and regulatory proteins (NIPBL, SMC1A, SMC3, HDAC8, and RAD21) are the major pathogenic contributors to CdLS. Heterozygous or hemizygous variants in the genes encoding these five proteins have been found to be contributory to CdLS, with variants in NIPBL accounting for the majority (>60%) of cases, and the only gene identified to date that results in the severe or classic form of CdLS when mutated. Pathogenic variants in cohesin genes other than NIPBL tend to result in a less severe phenotype. Causative variants in additional genes, such as ANKRD11, EP300, AFF4, TAF1, and BRD4, can cause a CdLS-like phenotype. The common role that these genes, and others, play as critical regulators of developmental transcriptional control has led to the conditions they cause being referred to as disorders of transcriptional regulation (or "DTRs"). Here, we report the results of a comprehensive molecular analysis in a cohort of 716 probands with typical and atypical CdLS in order to delineate the genetic contribution of causative variants in cohesin complex genes as well as novel candidate genes, genotype-phenotype correlations, and the utility of genome sequencing in understanding the mutational landscape in this population.