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   Table of Contents - Current issue
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January-March 2022
Volume 28 | Issue 1
Page Nos. 1-102

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EDITORIAL  

Minimum access mastoidectomy Highly accessed article p. 1
Mahendra Kumar Taneja
DOI:10.4103/indianjotol.indianjotol_171_21  
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REVIEW ARTICLE Top

An update on autosomal recessive hearing loss and loci involved in it p. 6
Mahbobeh Koohiyan, Masih Hoseini, Fatemeh Azadegan-Dehkordi
DOI:10.4103/indianjotol.indianjotol_115_21  
Hearing plays an important role in human development and childhood speech learning for the proper functioning and development of people in society. Hearing loss (HL) is one of the most abnormal disabilities that affect the human senses. This disability may be due to genetic or environmental factors or both. Congenital HL is a disorder that occurs in at least 1 in 1000 births. At least 42 genetic loci are associated with syndromes, while more than 163 are associated with nonsyndromic HL (NSHL), and no specific gene therapy treatment has yet been proposed. Investigate the types of genes involved in regulating hair cell adhesion “and evaluate functions such as intracellular transport, the release of neurotransmitters, ion homeostasis, and hair cell cytoskeleton, and whether defects in them can impair cochlear and inner ear function.” Can help diagnose and treat the disease through various methods, including gene therapy. Given the complex internal and external structures of the ear, nervous system, and auditory mechanisms, it is not surprising that abnormalities in hundreds of different genes may lead to HL. In recent years, with the increasing number of studies on genes involved in congenital HL, counseling and treatment options with the help of gene therapy have increased. In this study, we aimed to describe genes and proteins and their functions in NSHL in the inner ear for screening and diagnostic programs of live birth and classify the genes involved in this model of deafness to open the door to gene therapy. It is on these genes. We hope to develop new molecular and gene therapies for autosomal recessive NSHL.
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ORIGINAL ARTICLES Top

Comparison between autologous and artificial graft ossiculoplasty in canal wall down tympanomastoidectomy: A 10 year's personal experience p. 18
Mohamed Tawalbeh, Lubna Khreesha, Elnagi Ali, Ahmad Al Nsour, Jareer Shatnawi, Abdulrahman Al-Shudifat
DOI:10.4103/indianjotol.indianjotol_219_20  
Background: Ossiculoplasty is the reconstruction of ossicular chain: Malleus, incus, and stapes. This procedure attempts to reestablish the continuity between the tympanic membrane and the oval window. Different materials were used in ossiculoplasty; whether to use autologous grafts or synthetic prosthesis. Aims: This study aimed to evaluate hearing changes in patients' undergone ossiculoplasty as a part of Tympanomastoidectomy. Also, to appraise the autologous reconstruction materials used in ossiculoplasty at Jordan University Hospital (JUH) in comparison to other materials. Materials and Methods: A retrospective study done for a total of 100 patients underwent ossiculoplasty using autologous and artificial graft during canal wall down tympanomastoidectomy at JUH from 2009 to 2019. Results: Overall average mean of air-bone gap (ABG) closure (Hearing Gain) for Group A was 11.07 dB, while for Group B was 7.5 dB; this decrease was found to be highly significant (P = 0.002). In Group A, ABG mean was (34.44 dB) preoperative and reduced to 23.37 dB postoperative, while in Group B was (32.95 dB) preoperative and reduced to 25.45 dB postoperative. Conclusions: Autologous grafts can be used safely in reconstruction of middle ear with favorable outcome. Our study is one of the few reports that compare outcome between artificial and autologous graft in ossiculoplasty.
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Microbial flora of mastoid cavity after canal wall down (CWD) mastoidectomy p. 23
Behrouz Barati, Matin Ghazizadeh
DOI:10.4103/indianjotol.indianjotol_247_20  
Introduction: Many scientists have worked on microbial flora of the ear canal or middle ear in nonoperated patients, but it is not clear that what changes would be occurred in the flora of the mastoid cavity postoperatively. It can help the surgeons to choose the appropriate drugs in the perioperative period. The study was designed to investigate the microbial flora of the middle ear and mastoid cavities after canal wall down (CWD) mastoidectomy. Materials and Methods: The survey was designed as a prospective cross-sectional study. All patients with chronic otitis media who had undergone CWD mastoidectomy in a public university hospital from 2014 to 2017 were enrolled in the study. They were periodically followed up postoperatively at least for 6 months. Swab sampling from ear cavity was performed, and the samples were sent for culture and antibiogram. Each specimen was sent for bacterial and fungal analysis. If bacterial culture result was positive, the sensitivity of the cultured organisms to common antibiotics was also assessed. Results: Seventy-four patients entered into the survey. Twenty-six (35%) of samples were positive for bacteria, and 11 (14.5%) were positive for fungi. Furthermore, 3 (4%) of the studied ears were positive simultaneously for both bacterial and fungal culture. Among cultured bacteria, Staphylococcus epidermidis was the most common species (29%). Candida species was the most common fungal species (8%). Conclusions: Microbial infection is a common finding after CWD mastoidectomy. S. epidermidis and Candida species should be considered as the primary targets of treatment.
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A study of vestibular evoked myogenic potential and clinical features in benign paroxysmal positional vertigo: An institutional experience p. 26
Poonam Kumar Saidha, Dali Chandran, Natashya Sima, Ophelia D'souza
DOI:10.4103/indianjotol.indianjotol_17_21  
Background: Benign paroxysmal positional vertigo (BPPV) is the most common vestibular disorder in adults caused by degeneration of the otolithic organs in most cases but may also be a consequence of head injury, labyrinthitis, and ischemia in the distribution of the anterior vestibular artery or prolonged bed rest. The aim of the study is to find the correlation of alterations in vestibular evoked myogenic potential (VEMP) recordings in BPPV patients, with other associated symptoms. Materials and Methods: The study was conducted in 29 BPPV patients over a 3-year period. All patients were investigated with pure-tone audiometry, bithermal caloric test with Electronystagmography (ENG) recording, and VEMP recording. Clinical tests included Romberg, Sharpened Romberg, and Dix–Hallpike tests. Associated parameters were recorded, and any association with abnormal VEMP was evaluated. Results: VEMP was abnormal in three patients. There was no association of abnormal p or n latency with BPPV. No significant relationship with any clinical parameter was observed. Conclusions: BPPV is associated with the occurrence of abnormal VEMP recordings, possibly due to degeneration of the saccular macula, but a reproducible association is not demonstrable in a significant way.
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Persistent postural-perceptual dizziness: A multispecialty survey of clinician awareness and practices in Malaysia p. 32
Carren S Teh, Salim Iffah, Narayanan Prepageran
DOI:10.4103/indianjotol.indianjotol_149_21  
Objectives: Persistent postural-perceptual dizziness (PPPD) is a chronic functional vestibular disorder which is also the most common cause of chronic dizziness. Effective treatment is dependent on the awareness of the provider's knowledge of PPPD and correct provision of treatment. This study was aimed to assess the awareness of PPPD and practice patterns among a diverse group of providers who care for patients with chronic dizziness in the outpatient clinics. Materials and Methods: A 12-question web-based survey was distributed to a cross-sectional convenience sample of medical providers from primary care including general practitioners, otorhinolaryngology (ORL) and internal medicine all over Malaysia. We analyzed the responses using multivariate logistic regression. Results: Of the 186 included responses, only 46.7% (85 respondents) replied they have heard of PPPD and 158 (84.9%) have actually encountered patients with symptoms describing PPPD. Those in primary care had less odds of having heard of PPPD (adjusted odds ratio [aOR] 0.37 confidence interval [CI] 0.18–0.76) nor treated patients with PPPD symptoms (aOR 0.18 CI 0.07–0.49) when compared to the General Medical and ORL providers. In terms of treatment of PPPD, gender, level of training, and practice setting predicted the provision of therapy. Conclusion: PPPD awareness is still lacking among our providers and the current practices reflect that the management of chronic dizziness is focused on ruling out systemic causes. Now is the time to invest in health communication strategies to improve the awareness and knowledge of managing chronic dizziness especially PPPD.
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Comparison of tragal cartilage and conchal cartilage in tympanoplasty p. 41
Sohil I Vadiya, Parth Makwana, Sridhar Khetani, Nisarg Mehta
DOI:10.4103/indianjotol.indianjotol_142_21  
Aim and Objectives: The aim and objective of the study are to compare results in terms of graft uptake and hearing improvement in cases undergoing tympanoplasty with the use of conchal or tragal cartilage. Materials and Methods: A total of 74 cases of more than 18 and <50 years of age included in this study, where full-thickness cartilage tympanoplasty was performed. Thirty-seven cases included in Group A where tragal full-thickness cartilage was used and 37 cases included in Group B where full-thickness conchal cartilage was used. Results: Thirty-six (97.3%) cases in Group A and 35 (94.6%) cases in Group B had successful and complete graft uptake in this study. Hearing improvement was found to be better with the cases in Group B than Group A. Conclusion: Full-thickness conchal cartilage and tragal cartilage give good graft uptake results and hearing improvement is significantly better with the use of conchal cartilage.
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Speech recognition, quality of hearing, and data logging statistics over time in adult cochlear implant users p. 45
Giulia Elvira Malzanni, Caterina Lerda, Rosa Alessia Battista, Chiara Canova, Omar Gatti, Mario Bussi, Lucia Oriella Piccioni
DOI:10.4103/indianjotol.indianjotol_51_22  
Objective: The objective of this study was to examine improvement in speech recognition and “the Speech, Spatial and Qualities of Hearing Scale” (SSQ) scores in monolateral cochlear implant (CI) users and its correlations to data logging statistics. Materials and Methods: A prospective observational, nonrandomized, study was conducted analyzing speech tracking with shielded mouth in noise (SMn), speech audiometry, and SSQ questionnaire (Italian version) at 1, 3, and 6 months postactivation of CI of 31 patients. The overall data of average daily use and acoustic scene analyses were extracted from data logging system. Data of 6-month cumulative speech in quiet-, speech-in-noise-, and noise-listening time of 19 patients were correlated to speech perception scores and SSQ scores. Results: An improvement was observed in all outcomes (speech tracking with SMn, speech audiometry, and SSQ scores). Listeners used their device on for a mean time of 13.45 h per day. Quiet scene was the most represented listening environment. A significant positive correlation was found between noise exposition (in hours) and hearing quality score of SSQ and between exposition to 60–69 dB noise and scores resulted from each part of SSQ. Discussion: The analysis failed to demonstrate a correlation between auditory performances and both exposition to speech scene and total exposition. However, time spent in noise using the device correlated with SSQ scores. Conclusion: Cochlear implantation ensures good results in speech recognition and quality of life, with progressive scores' improvement after activation. A predominant use in quiet was observed together with a correlation between hearing performance and exposition to noise.
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Changes of serum levels of Caspase-3 after trauma and ototoxic damage of the cochlea in rabbits: An in vivo study p. 52
Pavlos Pavlidis, Haralampos Gouveris, Vasileios Nikolaidis, Gregor Alexander Schittek
DOI:10.4103/indianjotol.indianjotol_29_22  
Background: Caspase-3 is one of the most important enzymes for the regulation of apoptosis. Aims and Objectives: Aim of our study was to examine the changes in serum levels of this factor during apoptotic phenomena in the cochlea, caused by traumatic or ototoxic causes. Materials and Methods: A cohort of 24 rabbits was studied for this purpose for 31 days. Eight animals were implanted with a cochlear implant electrode (group A), 8 were treated with intramuscular amikacin for 14 days (group B) and another 8 were the control group (group C). In all groups DPOAEs and serum levels of caspase-3 were tested every second day. Results: Serum levels of caspase-3 rise immediately after implantation, while a 3-day latency in levels was seen in group B. Caspase-3-levels in both groups remained elevated until the 31st day of the experiment. Levels of caspase-3 showed a moderate negative correlation with DPAOE amplitudes. Conclusion: Caspase-3 rises after traumatic and ototoxic causes and moderately correlates with cochlear outer hair cell function in rabbits. Therefore, serum caspase-3 levels should be tested as a surrogate marker of structural integrity of the cochlea after trauma or administration of ototoxic drugs in humans as well.
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CASE REPORTS Top

Facial palsy following onyx embolization of a glomus tympanicum tumor: A case report and literature review p. 57
Abdulaziz AlEnazi, Yazeed Alshawi, Hawra Alnasser, Mohammed AlAftan, Munahi AlQahtani
DOI:10.4103/indianjotol.indianjotol_220_20  
The embolization of a glomus tumor (GT) is a known preoperative procedure aimed at decreasing the blood supply to the tumor and reducing operative time and blood loss. Liquid embolic material is the preferable embolic agent. However, the incidence of cranial neuropathy due to denaturation with Onyx is not well established. This report seeks to illustrate the outcomes of a combined preoperative Onyx embolization of a glomus tympanicum tumor and highlight the related complications of facial palsy. A 49-year-old woman developed left-sided facial palsy following the preoperative embolization of a left-sided GT with selective transarterial embolization using ethylene vinyl alcohol (EVOH; Onyx 18), which was complicated with left-sided facial weakness 1 h post embolization. An additional uneventful surgical resection of the GT was performed successfully 72 h later. Embolization material poses a risk of cranial neuropathy. Further studies are recommended to support the knowledge of well-established embolization agents that will provide maximal occlusion while minimizing the risk of complications.
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Auditory neuropathy spectrum disorder in two siblings with congenital cytomegalovirus infections: Two rare cases p. 62
Ronny Suwento, Kartika Hajarani, Semiramis Zizlavsky, Tri Juda Airlangga
DOI:10.4103/indianjotol.indianjotol_25_21  
Auditory neuropathy spectrum disorder (ANSD) is a rare hearing disorder when signal transmission is disrupted on the auditory pathway. Infection is one of the multifactorial causes of ANSD. Only a few researchers have reported the cases of ANSD caused by congenital cytomegalovirus (CMV) infection, and fewer have presented cases involving siblings. In our study, the two patients were siblings, aged 3 years and 7 months and 8 months. They were born prematurely, had microcephaly; anti-CMV IgG levels were reactive. Distortion product otoacoustic emissions test was normal. A click auditory brainstem response showed that no wave was detected with an 80 dB stimulus, and a cochlear microphonic response was obtained. No abnormality in the middle ear was observed, and negative acoustic reflexes were noted. Hearing habilitation with a low-gain hearing aid, speech therapy, and motoric habilitation was planned, and the patients were referred to the pediatric neurology for CMV management.
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Management of earlobe pseudocysts using the bolster technique p. 65
Al Hafiz Djosan, Dolly Irfandy, Ferdy Azman
DOI:10.4103/indianjotol.indianjotol_129_21  
Pseudocysts of the ear are asymptomatic noninflammatory cystic enlargements without pain, are idiopathic, and relatively rare, usually located in the lateral or posterior region of the earlobe. The definitive etiology is still unclear, but it is thought that constant compressed trauma to the earlobe can be a trigger for the pseudocyst's development. Management is mainly carried out to achieve complete resolution, preventing the possibility of recurrence and maintaining the structure of the auricle. Corticosteroid injection, needle aspiration, and suppression techniques are common management modalities. Bolster compression technique is an emphasis on both sides using gauze. There were two cases of patients reported who performed procedures with a bolster. The first case was a 35-year-old male patient with right ear pseudocyst. The second case was a 49-year-old male patient with recurrent right ear pseudocysts. Management of ear pseudocysts with suppression using a bolster is safe and effective, inexpensive, and has minimal risk for recurrence.
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Bilateral traumatic delayed facial nerve palsies: Challenges in management p. 69
Siti Nazira Abdullah, Azliana Aziz, Nik Adilah Nik Othman
DOI:10.4103/indianjotol.indianjotol_241_20  
Trauma causing bilateral temporal bone fracture has a unique mechanism. The type of fracture can predict the probability of facial nerve palsy. Commonly facial nerve palsy is managed by surgery or conservative management, depending on the onset, grading, and value of the objective electrodiagnostic test. The dilemma arises in our case when the young patient presented with delayed onset, bilateral incomplete facial nerve palsy, whereby the symmetrical monotonous looking can be misleading at initial diagnosis. We discussed several options of test available to give the prognostic value in the case if there is no normal side to be compared to.
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Atypical manifestation in vestibular schwannoma p. 74
Addina Mohd Baki, Wan Nur Anis Wan Draman, Zulkiflee Salahuddin, Nik Adilah Nik Othman
DOI:10.4103/indianjotol.indianjotol_99_21  
The cerebellopontine angle (CPA) is a cerebrospinal fluid-filled space, found at the junction between the cerebellum and pons. Vestibular schwannoma (VS) is the most common CPA tumor. Typical initial symptoms are unilateral or asymmetrical hearing impairment, tinnitus in the affected ear, or imbalance or vertigo. We report an unusual case of the patient who presented with right sensorineural hearing loss but was found to have left VS in the normal hearing ear.
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Bilateral otomastoid tuberculosis with pulmonary tuberculosis in immunocompetent young patient p. 77
Ahmad Mahfuz Zamaili, Nik Adilah Nik Othman, Safinaz Zainor
DOI:10.4103/indianjotol.indianjotol_147_21  
Tuberculous (TB) otitis media is rare and usually seen secondary to pulmonary tuberculosis or associated with it. TB is one of the major infectious diseases with predominant involvement of lung and lymph nodes which is common in Malaysia. Regarding the tubercular otitis media or tubercular mastoiditis, Mycobacterium tuberculosis is the principal causative agent, however, other atypical agents such as Mycobacterium bovis, Mycobacterium avium and Mycobacterium fortiutum are also responsible. TB otitis media may present with other middle ear problems whereby clinicians also unfamiliar with its typical presentation. Diagnosis is difficult because it needs specific culture and pathologic studies. Early diagnosis and effective treatment may prevent ear damage as well as central nervous system complication. The objective of this study was to report a rare case of bilateral TB otitis media to alert physicians to the rare presentation of the disease as well as to discuss the diagnosis and management plan based on the literature review.
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High-dose-rate brachytherapy boost following external radiotherapy in a case of carcinoma external ear p. 80
Jayesh Singh, Aastha Shah, Ankita Parikh, Isha Shah, Dinesh Anand, Himanshi Jain, U Suryanarayan
DOI:10.4103/indianjotol.indianjotol_104_21  
Squamous cell carcinoma of the external ear is rare and the combination of external beam radiotherapy and brachytherapy has been beneficial for tumors in this location due to the advantage of organ preservation and thereby cosmesis. The incorporation of brachytherapy allows the delivery of doses large enough for the eradication of tumor and the advantage of rapid dose fall off due to brachytherapy minimizes the radiation damage to the nearby organs at risk. Herein we report a case of elderly male with early stage squamous cell carcinoma of external ear treated with a combination of external beam radiation with brachytherapy. An eighty nine year old male reported to our hospital with the complaints of left sided ear ache the presence of some mass in the left external ear. A diagnostic Contrast enhanced Computed Tomography (CECT) scan of head and neck showed a 1.6*1.5*1.4 cm mass lesion (maximum diameter) involving anterior cartilaginous part of left external auditory canal and preauricular soft tissue,thereby staged as T2N0,stage II. Patient was planned for External Beam Radiotherapy (EBRT) using 3D CRT(Conformal Radiotherapy) technique by 6 MV photon beam to a dose of 50 Gy in 25 fractions (2 Gy/#) and following this,a gap of seven days was given and after that four fractions of HDR brachytherapy using Iridium 192 of 3 Gy/# keeping a gap of six hours between two fractions on a single day,Two flexible plastic tubes were used for radiation delivery. The size of the catheter used for radiation delivery was 6 French. Planning CT scan was taken and target volume defined as PTV and a dose of 3 Gy per fraction to a total of 4 fractions was prescribed to the target volume. The spike of intravenous site was used for fixing the plastic tube in the mould. The tumor responded ver well to radiotherapy. Brachytherapy is an effective modality to boost high risk areas without significant damage to the surrounding area. External Beam Radiotherapy in case of early stage carcinoma external auditory canal combined with brachytherapy boost can be considered as a better modality with less severe side effects and better cosmesis.
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Unilateral double-barreled internal auditory meatus with severe sensorineural hearing loss: A diagnostic challenge p. 84
G Selvarajan, R Vaishnavi, Mithun Anand Prateep, Balaji Jeevanandham
DOI:10.4103/indianjotol.indianjotol_154_21  
Internal auditory meatus duplication is an exceedingly rare temporal bone anomaly. All of them being bilateral duplication or narrowing of internal auditory meatus with profound sensorineural hearing loss. We report a very rare case of unilateral double-barreled internal auditory meatus with severe sensorineural hearing loss without any inner ear anomalies in an adolescent, being benefited with an air conduction hearing aid.
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Temporal bone rhabdomyosarcoma mimicking chronic suppurative otitis media p. 88
Isha Shah, Siddharth Malukar, Jayesh Singh, Ankita Parikh, U Suryanarayana Kunikullaya
DOI:10.4103/indianjotol.indianjotol_151_21  
Rhabdomyosarcoma (RMS) is one of the most commonly encountered soft-tissue sarcomas in childhood. The most common location is in the head-and-neck region, followed by the urogenital system, extremities, and torso. Head and neck is a common site though the temporal bone is an extremely rare site with only a few reported cases in the literature till now. Herein, we report a case of a sclerosing type of temporal bone RMS in an 8-year-old young girl. We discuss clinical, pathological, radiological findings and management of temporal bone RMS. Misdiagnosis is common because of this aggressive temporal bone lesion's similarity to more common benign diseases. Clinicians should maintain suspicion for RMS in patients with chronic otitis media not responsive to medical therapy and should refer a specialist multidisciplinary team in a timely manner as an early diagnosis will optimize the chance of survival. Clinical features, radiologic findings, and histopathological examination using immunohistochemistry are all important for the correct diagnosis of temporal bone RMS. It is important to be aware of and to know how to deal with such rare cases.
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Cortical emissary vein: An important vessel to look for before bone anchored hearing aid implantation p. 91
Ahmad Faiz Dahlan, Izny Hafiz Zainon, Zhi Xiang Yeoh, Mohd Khairi Md Daud
DOI:10.4103/indianjotol.indianjotol_146_21  
Bone anchored hearing aid (BAHA) is a device that combines a sound processor with a small titanium fixture implanted at the mastoid area. It is indicated for those having conductive hearing loss, especially in those not suitable for hearing aids. Emissary veins are residual connections between intracerebral veins and their extracranial drainage. The vein may be injured at the time of drilling procedure such as during insertion of implant at the mastoid area. We report a case of difficult BAHA surgery after encounter with cortical emissary vein. Mastoid emissary vein is a rare but definite entity which if not diagnosed preoperatively could be a cause of severe hemorrhage intraoperatively.
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Cochlear implantation in a case of jervell and lange-nielsen syndrome presenting with absence seizure and anemia p. 94
Murundi Basavarajaiah Bharathi, Shankar Medikeri, Sandhya Dharmarajan, Kumar Shankar De
DOI:10.4103/indianjotol.indianjotol_167_21  
Here, we describe the case of a pediatric patient who presented with an episode of absence seizures and was later diagnosed to have Jervell and Lange-Nielsen syndrome (JLNS) and underwent cochlear implantation (CI). A 4-year-old male child born out of a third degree consanguineous marriage presented with congenital profound sensorineural hearing loss and an episode of absence seizures. Electrocardiogram showed a prolonged QT interval of 509 ms. The patient subsequently underwent CI. Here, we outline the challenges anticipated and the precautions to be taken when performing implantation on a patient with JLNS. JLNS is a rare autosomal recessive form of congenital long QT syndrome associated with congenital sensorineural deafness and a high incidence of sudden cardiac death in childhood. Patients with JLNS face added intra- and postoperative challenges during CI which require multidisciplinary and intensive care unit care.
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Congenital atresia of external auditory canal with choristoma: A rare case report p. 98
T Dinesh Kumar, Priya Kanagamuthu, Gunakeerthana Ramesh, Rajasekaran Srinivasan
DOI:10.4103/indianjotol.indianjotol_180_21  
Congenital aural atresia (CAA) is the abnormality of the auricle and/or external auditory canal where there is closure of the external auditory canal which may be associated with microtia and anomalies in the middle ear, inner ear, or temporal bone. It can be solid or membranous type of atresia. To identify the type of defect, high-resolution computed tomography (HRCT) temporal bone is useful. After identifying the defect, the patient can be planned for canaloplasty or atresiaplasty with split skin graft. A 6-year-old boy was brought to the otorhinolaryngology outpatient department with complaints of deformity of the left ear since birth. HRCT temporal bone showed isolated left external auditory canal atresia. Echocardiogram showed dextrocardia with situs solitus and USG abdomen revealed situs inversus of the abdominal organs with no significant abnormality. After proper evaluation of the child, after obtaining preanesthetic fitness, the child had undergone atresiaplasty with split skin graft under general anesthesia. Soft tissue that was excised from the external auditory canal was sent for histopathological examination, which revealed the presence of salivary gland tissue with lymph nodes showing reactive changes. These features were suggestive of possibility of choristoma of the left external auditory canal. CAA occurs in the defect during embryological development which may result in deformity of the auricle and external auditory canal. The surgical management of congenital external auditory canal atresia is atresiaplasty, and hearing rehabilitation (bone anchored hearing aid) should be given in case of no improvement in hearing. The surgery will be challenging because of altered anatomy of surgical landmarks. Choristoma is a tumor-like growth of normal tissue in an abnormal place. It is a benign condition, which may be found commonly in the head-and-neck region such as presence of salivary gland tissue in the middle ear, presence of gastric mucosa in the tongue and presence of osseous or cartilaginous masses in the intraoral soft tissues. Total excision can be done for small and pedunculated tumors, and care should be taken not to injure facial nerve. CAA may not necessarily present with deformities in the external ear (pinna), but Congenital aural atresia cases may not always present with deformities of the pinna,but it is crucial not to miss such cases. These patients may be evaluated by doing High Resolution Computed Tomography temporal bone and audiometric assessment such as. Pediatric evaluation is equally important to rule out for any syndromic association. Atresiaplasty or canaloplasty has shown great result for correcting the cosmetic defect. Hearing can be facilitated further using bone anchored hearing aids or cochlear implantation.
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