Waardenburg Syndrome Type 2 in Paediatrics: A Case Highlighting Diagnostic Complexities and the Efficacy of Cochlear Implantation.

Waardenburg Syndrome Type 2 (WS2) is a rare hereditary condition with a low prevalence, characterized by abnormalities in both auditory function and pigmentation. We present a case of a 2-year-old female child who exhibited reduced vocalizations, delayed speech development, and distinctive heterochromic irides. Initial auditory assessments revealed bilateral severe to profound hearing loss. Subsequent MRI findings confirmed bilateral aplasia of the posterior semicircular canals, consistent with a diagnosis of Waardenburg syndrome type 2. While standard treatments using bilateral Behind-The-Ear (BTE) power hearing aids yielded only modest improvements, cochlear implantation significantly enhanced auditory perception and speech abilities within 18 months. This report underscores the diagnostic intricacies of WS2 and highlights the profound benefits of cochlear implantation in addressing associated auditory challenges.

Hearing Aids and Quality of Life: A Psychological Perspective.

Objective: This research assessed the relationship between hearing aid usage and quality of life in individuals with hearing impairment, exploring associated demographic and clinical factors. Methodology: Data from 500 individuals at a tertiary care facility were collected over a year through medical records and an online questionnaire. Quality of life was gauged using WHOQOL-BREF and HHIE. Multiple linear regression analysed the correlation between hearing aid usage and quality of life, adjusting for age, gender, hearing loss severity, and socioeconomic factors. Results: Descriptive statistics showed varying degrees of hearing impairment, socioeconomic status, hearing aid usage, and quality-of-life scores. Bivariate analyses found significant correlations between hearing impairment level, socioeconomic status, hearing aid usage, and quality of life. Linear regression highlighted a positive relationship between daily hearing aid usage duration and quality-of-life scores, even after adjusting for covariates. Variations in quality-of-life ratings were observed between hearing aid users and non-users, spanning different hearing loss levels and socioeconomic statuses. Subgroup analysis showed a positive correlation between hearing aid use and quality of life across age groups. Conclusion: The study confirms the positive influence of hearing aids on the quality of life in hearing-impaired individuals, emphasizing the benefits of consistent use. Further studies should probe the specific domains and long-term adherence impacts. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-04297-w.

Management of Rhinosporidiosis: by Coblation.

Rhinosporidiosis is a granulomatous disease commonly affecting the mucous membrane. It is caused by Rhinosporidium seeberi, an aquatic parasite & seen affecting the nose, paranasal sinuses most commonly. A retrospective study was conducted at a tertiary care hospital situated in Indian peninsula and five patients who were diagnosed and treated for rhinosporidiosis were analysed. Surgical excision by coblator along with medical management using Dapsone 100 mg once daily for 6 months given promising results in view of reducing recurrence. Combined approach of management including surgical excision using coblator and medical therapy with dapsone is effective in managing the rhinosporidiosis with no recurrence.

Endoscopic Assisted Cold Steel adenoidectomy - A Cost Effective Modification for Better Outcome.

Introduction: Adenoidectomy is most commonly performed in children to alleviate the symptoms pertaining to adenoid hypertrophy. The conventional cold steel method utilizing adenoid curette is the most commonly performed method in the world even after the invention of endoscopes & powered instruments like coblator & microdebrider due to the cost & time factors. The conventional method being a blind procedure, carries higher rate of recurrence due to remnant tissues. The visualization of the adenoid tissue in nasopharynx through the nasal endoscope helps in better engagement of adenoids into the curette & adequate tissue clearance with reduced recurrence of symptoms. Aim: To study the effect of visualization of adenoid tissue for better tissue clearance in conventional adenoidectomy. Objectives: To compare the duration of surgery, blood loss & recurrence rate following conventional cold steel adenoidectomy (CSA) & endoscope assisted cold steel adenoidectomy (EACSA). Method: 50 patients who underwent adenoidectomy with various indications were grouped into two groups with 25 patients each. Group A underwent (CSA)with or without tonsillectomy & Group B underwent (EACSA) with or without tonsillectomy were followed up for the duration of 3 months. The patients were evaluated for duration of surgery & post operatively for the recurrence at 3rd month of follow up. Results: In our study, it was found that the tissue clearance in Group A was significantly low. The recurrence rate of 48% was observed in CSA group compared to 0% in group B with EACSA. The duration of surgery in both the procedures were comparable. Conclusion: EACSA is an effective modification to CSA. It adds the benefits of endoscopic visualization of adenoid for the conventional curettage. The high recurrence rates of CA can be effectively reduced with no significant variation in duration of surgery.

Outpatient Clinical Markers for Laryngopharyngeal Reflux Disease: A Correlational Study.

Laryngopharyngeal reflux disease (LPRD) is the result of retrograde flow of gastric contents to the laryngopharynx which comes in contact with tissues of the upper aerodigestive tract. Due to ill defined criteria for diagnosis & followup, LPRD patients are underdiagnosed & undertreated. Reflux Symptom Index (RSI) and the Reflux Finding Score (RFS) are two clinical methods which can be utilised especially in the outpatient setup. This study was done with the aim to assess various laryngoscopic findings in patients with LPRD diagnosed symptomatically and examine the correlation between the RSI & RFS by comparing these two indices. This prospective analytical study was conducted at a tertiary care centre in Bangalore in the Department of ENT for a period of 24 months between Dec 2020 to Dec 2022. The study included patients aged 18 to 60 years diagnosed with LPRD based on symptoms as per RSI score (> 13). RSI & RFS were assessed on diagnosis and patients were followed up for 1, 3 & 6 months for assessment. Total 96 patients were enrolled, with mean age of be 42.49 ± 11.33 years. Prevalence was found to be more in females (61.5%). The most common symptom according to RSI was frequent throat clearing & globus sensation (sensation of something sticking in throat) and most common finding according to RFS was erythema/hyperemia. The mean score of RSI and RFI was found to reduce with treatment at different intervals in follow-up visits. There was a significant strength of association between the RSI and RFS at baseline, 1st month, 3rd month and 6th month of follow-up (r = 0.568, r = 0.684, r = 0.774, r = 0.736 respectively) (p < 0.001).The RFS and RSI showed statistically significant strong relationships between total scores and sign and symptom characteristics. On follow-up, there was a significant reduction in the RSI which was also correlated with a reduction in RFS.

A Study on Comparison of Conventional Micro Laryngeal Micro Dissection Surgery with Carbon Dioxide Laser in Management of Benign Lesions of Larynx.

Dysphonia is a common symptom in benign and pre malignant lesions of larynx affecting vocal cords. Surgical excision is the main stay of treatment. Only a limited number of studies have compared the efficacy of microdissection versus CO2 laser surgery in the larynx. In the present study we have compared conventional micro-laryngeal surgery and carbon dioxide laser assisted microsurgical technique in the management of these lesions. A randomized prospective study was conducted in 88 cases of benign and pre malignant lesions affecting vocal cords were divided randomly to undergo surgery either by conventional micro dissection or CO2 laser assisted techniques in a tertiary care hospital. The groups were assessed through vocal cord morphological observation by videostroboscopy and subjective voice assessment parameters GRBAS score and VHI10 index preoperatively and 2 weeks and 3 months postoperatively by a panel of blinded viewers and listeners. Surgical and recovery times were compared between the two groups. Intraoperative blood loss was compared by gauze visual analogue. Patients recovered remarkably well following both the techniques as denoted by the voice parameters. Peroperative bleeding was observed to be significantly reduced in the laser excision group; operating time was significantly increased in this group. Duration of hospital stay was similar in both groups. No differences in clinical outcomes are identified when comparing microdissection with laser excision of benign lesions.

Can Nose Scale Be Used as a Predictor of Successful Surgery in Patients Undergoing Septoplasty.

The complaint of nasal obstruction or difficulty in nasal breathing is highly subjective. The benefits of surgery vary depending upon patient perception and satisfaction. Along with objective methods like nasal endoscopy improvement in subjective score of patients symptoms is definitely an important outcome parameter of management. Assessing the disease specific quality of life outcomes of patients undergoing septoplasty by means of a questionnaire using nose scale and correlating it with diagnostic nasal endoscopic findings and clinical examination thirty patients above 17 years of age, with anatomical deviation of the nasal septum as the sole cause of obstruction and symptoms persisting for more than 3 months, underwent septoplasty. Degree of septal deviation was classified as per Mladina classification. Patients Nasal Obstruction & Symptom Evaluation (NOSE) score were documented preoperatively and postoperatively and analysed. In our study it was found that there was significant improvement in nose scores which correlated with the improvement in nasal endoscopic findings. Use of NOSE score will help in letting the patient know his expected outcome following septoplasty and can be used as a predictor of successful surgery/an adjunct to clinical examination and nasal endoscopy in patients undergoing septoplasty. Disease specific health status instruments like Nose scale are needed along with objective outcome measures to compare disease-specific health status and symptom severity in patients before and after treatment. Similarly it could be used to assess differences in outcome when different surgical techniques are used. These subjective scales like nose scale can be used as along with to clinical examination and nasal endoscopy in the management of patients undergoing septoplasty.

An Unusual Case of Bilateral First Branchial Cleft Anomaly: A Case Report with Review of Literature.

We report a case of 32 year old male who presented to us with first branchial cleft anomalies on both sides with discharging cutaneous openings on the right side; and cystic swelling on the left side. High index of suspicion is required in such congenital cysts. Surgical exploration and excision is the definitive treatment of a collaural fistula.

A Study on Role of NOSE Scale in the Management of Allergic Rhinitis.

Allergic rhinitis is a global health problem and patients present with varied symptoms. The severity of symptoms is evaluated by a 5 point Nasal Obstruction Symptom Evaluation Scale (NOSE) and levels of absolute eosinophil count (AEC). A prospective observational study was conducted in 140 patients in a tertiary care hospital over a period of 1 year. A detailed clinical examination was performed, NOSE scale scoring based on symptomatology was done, and AEC values were assessed. Subjects were treated with intra nasal steroid spray and symptom evaluation done by NOSE scale at 3 months of use for follow up. NOSE scale and AEC values were correlated in our study. The mean NOSE 1 scale was 64.07 ± 16.71 in the study population, the mean AEC value was 633.07 ± 152.77. In our study correlation between intra nasal steroid spray and NOSE scale has been done. NOSE scale evaluation is simple, economical, and non-invasive. Therefore, it may be used in the management of allergic rhinitis.

Use of Bilateral Nasolabial Flaps with Coronoidectomy as a Novel Approach to a Case of Oral Cancer with Severe Trismus.

Patients with oral malignancy often have associated trismus. Trismus is a debilitating condition that needs to be treated adequately with the release of fibrosis and interposition of vascularized tissue to provide long-term improvement in the mouth opening. Treatment of oral cancer in patients with severe trismus becomes challenging because of difficult access and further deterioration of mouth opening following treatment of the malignancy. Herein, the planning of access to oral cancer and management of trismus in 9 oral cancer patients with severe trismus using nasolabial flaps and coronoidectomy are described. Bilateral nasolabial flaps and coronoidectomy were used successfully as an approach in these 9 patients with oral carcinoma with severe trismus. The advantages of this procedure are described.

Use of Clavien-Dindo Classification System in Assessing Head and Neck Surgery Complications.

Improving quality of postoperative surgical care in head and neck surgery requires reporting of complications. Clavien-Dindo classification system can be used in grading complications related to head and neck surgery and to assess interobserver variability in grading complex complication scenarios. Data was collected from 242 patients who underwent Head and Neck Surgery from 2015 to 2018 at Dept. of ENT and Head and Neck Surgery at a tertiary care hospital. 177 patients had complications were graded based on Clavien-Dindo classification system, into a 5-scale classification system. Interobserver reliability scores for the complication grading scenarios were found to be statistically significant. Construct validity was confirmed as the length of stay in the hospital was statistically related to complication grade (P = 0.032) Reporting of complications is critical to quality improvement in surgical practice. The Clavien-Dindo complication grading scale system was found to be a useful tool for grading head and neck surgery complications.

A Study of Application of Preoperative Clinical Predictors of Difficult Laryngeal Exposure for Microlaryngoscopy: The Laryngoscore in the Indian Population.

Good exposure for visualisation of the glottis is an essential prerequisite for effective microlaryngoscopy. There is no definitive clinical predictor scoring system to predict difficult microlaryngoscopy. The aim of the study was to study the validity of the Laryngoscore scoring system as a preoperative clinical predictor score in Indian population for difficult laryngeal exposure during microlaryngoscopy. A prospective study was carried out in 32 patients undergoing microlaryngoscopy in a tertiary care hospital who were evaluated by a standardized preoperative assessment protocol (Laryngoscore) which included 11 parameters interincisors gap, thyro-mental distance, upper jaw dental status, trismus, mandibular prognathism, macroglossia, micrognathia, degree of neck flexion-extension, history of previous open-neck and/or radiotherapy, Mallampati's modified score, and body mass index. Each parameter was assessed to obtain a total score (Maximum-17). Patients were divided into five classes according to the anterior commissure visualization: class 0 to class IV. Class 0 being complete and class IV being impossible AC visualization. When the Laryngoscore was < 6, good laryngeal exposure was observed in 94% of patients. Laryngoscore had a sensitivity of 87.5% and specificity of 75% in predicting difficult laryngeal exposure. The positive and negative predictive values of the same are 53.8% and 94.7% respectively. Our study found that the Laryngoscore scoring system as a preoperative indicator for predictor of Difficult Laryngeal Exposure during Micro Laryngeal surgeries is very useful in the Indian population.

Reconstruction of Orbital Suprastructure Maxillectomy Defects by Temporalis Myofascial Flap.

Oncological surgery being radical is often mutilating in form and function especially in the maxillary/orbit region reconstruction of maxillo-orbit defects are challenging due to the complex three dimensional anatomy. Free flaps are de-rigueur but a technical resource with constraints. The temporalis myofascial flap (TMFF) is a locally available, safe and reliable flap which can be used for the reconstruction of various orbital and supramaxillary facial defects. To study the use of the temporalis myofascial flap in the reconstruction of various orbital and supramaxillary facial defects. Temporalis myofascial flap was harvested and successfully used in reconstruction of three patients who had undergone orbital exenteration with or without suprastructural maxillectomy. There was no morbidity related to flap loss. Temporalis flap (TMFF) can be considered as a first line reconstructive option for limited resection of upper maxilla with palatal preservation. Its proximity to the oral cavity, palate and mid third face and the technical ease makes the TMFF valuable for reconstruction. The techniques and outcomes of TMFF are discussed.

A Study of Effect of Consanguinity on Cochlear Morphology in Patients with Congenital Bilateral Profound Sensorineural Hearing Loss.

Consanguinity has been considered as one of the risk factors predisposing to the development of congenital hearing loss. Effect of consanguinity on cochlear morphology has been subject of speculation, though many studies have provided insight into functional aspect of cochlea. This study was conducted to know the effect of consanguinity on cochlear morphology, if any. A prospective, observational study, including prelingually deaf children with bilateral profound sensorineural hearing loss who are registered as candidates for cochlear implantation at a tertiary care centre, was conducted. Seven children born out of consanguineous marriages and having bilateral congenital sensorineural hearing loss were imaged for their inner ears by MRI and various cochlear measurements were compared with that of seven control children born out of non-consanguineous marriages and who had congenital bilateral sensorineural hearing deafness. No statistically significant differences were noticed in the measurements of mean cochlear length, cross sectional diameter of cochlea at apex, middle turn and at basal turns; between children born of consanguineous and non-consanguineous marriages. Study revealed normal cochlear morphology in all the children born out of consanguineous marriages. Consanguinity, as a risk factor for development of sensorineural hearing loss, may not result in gross structural anomaly of cochlea. The genetic testing of these patients need not be extensive and can be limited to selective screening of genes responsible for functioning of cochlea rather than its anatomical development.

Study of efficacy of anaesthesia with propofol and fentanyl for rigid bronchoscopy in foreign body bronchus removal in children.

Anaesthesia for removal of foreign body bronchus by rigid bronchoscopy in children is a challenging procedure because it is difficult to maintain adequate ventilation and oxygenation in patients in whom pulmonary gas exchange is already reduced. The choice between spontaneous and controlled ventilation and type of drug used are still subjects of discussion. We report a study in five children of use of a technique of spontaneous ventilation using propofol with fentanyl, midzolam and sevoflurane without the use of muscle relaxant. In our study no side effects were seen in the study group. Spontaneous ventilation using propofol, fentanyl, midzolam and sevoflurane can be safely used in children for rigid bronchoscopy for foreign body bronchus removal.

Cervical sympathetic chain schwannoma: a case report.

Tumors originating in the parapharyngeal space are rare and are fascinating to the surgeon in view of the anatomical complexity of the area, varied histological type of tumors encountered and the impressive size which some of these tumors attain before becoming clinically evident. We report a rare case of schwannoma arising from the cervical sympathetic chain presenting as a mass in the parapharyngeal space.

Endoscopic sinus surgery in chronic rhinosinusitis and nasal polyposis: a comparative study.

Nasal polyposis are common presentations in patients of chronic rhinosinusitis and are considered to be associated with more severe forms of disease with poor treatment outcome. The presentation and treatment outcome after endoscopic sinus surgery in patients of chronic rhinosinusitis and nasal polyposis have been analysed in this study. A prospective analysis of 90 patients of chronic rhinosinusitis who were classified into two groups depending on presence and absence of nasal polyps was performed in the study. The two groups were evaluated using subjective (patient complaints) and objective (computed tomography scan and endoscopy scores) criteria. Preoperative data were compared with data obtained 12 months post endoscopic sinus surgery. The study included 38 patients of chronic rhinosinusitis and 52 patients of nasal polyps. The patients of nasal polyp group presented with increased severity of symptoms of nasal blockage, nasal discharge and reduced sense of smell as compared to the chronic rhinosinusitis group who had significantly higher presentation of headache and facial pain. The preoperative CT scan revealed significantly higher bilateral disease with increased involvement of multiple sinuses in nasal polyp group. Post endoscopic sinus surgery both the groups showed significant improvement in their symptoms with the nasal polyp group demonstrating reduction in improvement on 1 year follow up. In our study we have found the patients with chronic rhinosinusitis and nasal polyp have varied severity of symptoms with the nasal polyp group having higher nasal symptoms and increased severity as compared to chronic rhinosinusitis group. Though the universal rationale of management by adequate drainage and ventilation of sinus is similar in both groups, there is a reduction in both objective and subjective scores during 1 year follow up in the nasal polyp group.

Antrolith in the maxillary sinus: an unusual complication of endoscopic sinus surgery.

Antrolith of the paranasal sinuses are rare entity which are usually asymptomatic, caused by calcification of a nidus and are detected incidentally on radiological examinations. We report a case which presented to us with features of pansinusitis six months after endoscopic sinus surgery. Radiological examination revealed a discrete bony density in the maxillary sinus blocking the ostiomeatal complex. The bony mass was removed endoscopically from the maxillary sinus with drainage of discharge and debris from the sinuses. Histopathological examination revealed an antrolith with bony nidus and calcium deposited around it. We present the imaging and review the present world literature on this rare complication of endoscopic sinus surgery.