Moderate Conductive Hearing Loss Research Articles

Novel likely pathogenic variant in the EYA1 gene causing Branchio oto renal syndrome and the exploration of pathogenic mechanisms

ObjectiveBranchio-oto-renal syndrome (BOR, OMIM#113,650) is a rare autosomal dominant disorder that presents with a variety of symptoms, including hearing loss (sensorineural, conductive, or mixed), structural abnormalities affecting the outer, middle, and inner ear, branchial fistulas or cysts, as well as renal abnormalities.This study aims to identify the pathogenic variants by performing genetic testing on a family with Branchio-oto-renal /Branchio-otic (BO, OMIM#602,588) syndrome using whole-exome sequencing, and to explore possible pathogenic mechanisms.MethodsThe family spans 4 generations and consists of 9 individuals, including 4 affected by the BOR/BO syndrome. Phenotypic information, including ear malformation and branchial cleft, was collected from family members. Audiological, temporal bone imaging, and renal ultrasound examinations were also performed. Whole-exome sequencing was conducted to identify candidate pathogenic variants and explore the underlying molecular etiology of BOR/BO syndrome by minigene experiments.ResultsIntra-familial variability was observed in the clinical phenotypes of BOR/BO syndrome in this family. The severity and nature of hearing loss varied in family members, with mixed or sensorineural hearing loss. The proband, in particular, had profound sensorineural hearing loss on the left and moderate conductive hearing loss on the right. Additionally, the proband exhibited developmental delay, and her mother experienced renal failure during pregnancy and terminated the pregnancy prematurely. Genetic testing revealed a novel heterozygous variant NM_000503.6: c.639 + 3 A > C in the EYA1 gene in affected family members. In vitro minigene experiments demonstrated its effect on splicing. According to the American College of Medical Genetics (ACMG) guidelines, this variant was classified as likely pathogenic.ConclusionThis study highlights the phenotypic heterogeneity within the same family, reports the occurrence of renal failure and adverse pregnancy outcomes in a female patient at reproductive age with BOR syndrome, and enriches the mutational spectrum of pathogenic variants in the EYA1 gene.

The presence of a submucous cleft palate in patients with isolated cleft lip and middle ear dysfunction

PurposeRecent studies have suggested that children with an isolated cleft lip (CL) are more likely to develop middle ear disease and eustachian tube dysfunction (ETD) compared to the general population. This may be related to abnormal palatal musculature or an undiagnosed submucosal cleft palate (SMCP). We aim to determine the prevalence of SMCP in patients with CL who exhibit ETD. Materials and methodsA retrospective chart review was performed for children with an isolated CL requiring tympanostomy tubes over a 20-year period at an academic tertiary care medical center. Demographic, clinical, and surgical data were collected. ResultsThree hundred twelve patients had an isolated CL, and 29 (9.3 %) children required tympanostomy tubes. Of those, nine (31 %) were found to have a SMCP (7 males, 6 Caucasian). The average age at CL repair was 3.94 ± 1.03 months, and the average age at tympanostomy tube placement was 13.68 ± 13.8 months. All nine patients had chronic otitis media, with four having mild conductive hearing loss and three having moderate conductive hearing loss. The SMCP was diagnosed at the time of CL diagnosis (4), after CL diagnosis with the diagnosis of chronic otitis media/ETD (2) and after a diagnosis of chronic otitis media/ETD. ConclusionMiddle ear disease or eustachian tube dysfunction in a patient with an isolated cleft lip should raise suspicion for an accompanying undiagnosed SMCP.

Severity and Types of Hearing Loss in Patients with Hypertrophic Adenoids

Background: Hearing loss in children can significantly impact language development and social interaction. Hypertrophic adenoids are a common pediatric condition that can contribute to hearing impairments. Understanding the prevalence and severity of hearing loss in this demographic is crucial for effective management and intervention strategies. Objective: The study aimed to determine the prevalence and severity of hearing loss among children with hypertrophic adenoids, assessing the demographic characteristics and the types of hearing loss observed in this population. Methods: This observational cross-sectional survey was conducted over nine months at the Children's Hospital and Institute of Child Health, University of Lahore Teaching Hospital. The study included a sample size of 339 children aged 5-13 years, calculated based on a prevalence rate of 67.3% for mild hearing loss. Children with comorbidities like Down syndrome, Autism, ADHD, and other disabilities were excluded. Hearing assessments were conducted using an otoscope, a tympanometer, and a diagnostic audiometer (MAICO MA41 model). Data were analyzed using SPSS Version 25.0, focusing on the frequency and percentage of various types of hearing loss. Results: The study included 183 males (54%) and 156 females (46%), with a higher representation from rural areas (60%, n=203). Tympanometric analysis revealed 51.9% (n=176) with Type A tympanograms, 43.1% (n=146) with Type B, and 5% (n=17) with Type C. Audiometric results showed that 53.1% (n=180) of participants had normal hearing levels, while 27.1% (n=92) experienced mild hearing loss, 16.2% (n=55) had moderate hearing loss, 3.5% (n=12) had moderately severe hearing loss, 1.5% (n=5) had sensorineural hearing loss, and 2.1% (n=7) had mixed hearing loss. Conclusion: The study demonstrates a significant occurrence of mild to moderate conductive hearing loss in children with hypertrophic adenoids, particularly in rural settings. These findings highlight the need for regular auditory screening and early intervention strategies in this demographic to prevent long-term speech and language complications.

Pyogenic Granuloma of External Auditory Canal: A Case Report

The external auditory canal is an atypical location for pyogenic granuloma (PG) to originate. We report a case of PG in a 15-year-old boy's external auditory canal. He experienced reduced hearing and occasional blood-tinged ear discharge for 8 months. Examination revealed a pinkish-soft mass occluding the canal lumen. Pure tone audiogram showed 42 dB moderate conductive hearing loss in the right ear. High-resolution computed tomography showed a well- defined, homogenously enhancing soft tissue density lesion in the canal. The mass was completely excised, and post-operative histopathology confirmed the diagnosis of PG. No recurrence was observed during the six-month follow-up period. PG should be considered in the differential diagnosis of external auditory canal lesions.

A Comparative Study of Button Hole Technique Versus Underlay Technique in Type 1 Tympanoplasty.

Tympanoplasty is the most common operation performed by an Otolaryngologist worldwide.Type 1 tympanoplasty involves repair of pars tensa of tympanic membrane, when the middle ear is normal. The most widely used method is underlay technique using temporalis facia. In buttonhole tympanoplasty, the temporalis fascia is anchored to the handle of malleus through the buttonhole. To compare and analyze graft uptake and hearing outcome in button hole technique and underlay technique. It is a comparative study done at tertiary care center, where patients suffering from tubotympanic type of chronic otitis media with medium sized perforation with moderate conductive hearing loss, within age group of 18-60 years,were selected. In Button hole tympanoplasty group the mean hearing gain was 9.3dB, and 8.17 dB in Underlay tympanoplasty group which was statistically significant (p < 0.05) but P value between Button hole and Underlay tympanoplasty was not statistically significant.With regard to graft uptake 96.7% showed graft uptake in Buttonhole tympanoplasty group and in underlay tympanoplasty the graft uptake was 93.3%. Buttonhole technique is better in terms of graft uptake since the graft is anchored to the handle of malleus, and postoperatively medialisation of graft and other complications like lateralization of graft, epithelial pearl formation and anterior blunting is prevented. Both techniques are good in terms of hearing improvement for chronic otitis media with medium sized perforation with moderateconductive hearing loss.

Postmeningitic pediatric hearing loss from non-type b Haemophilus influenzae

BackgroundPostmeningitic hearing loss from Haemophilus influenzae (H. influenzae) is increasingly due to encapsulated serotypes other than type b (Hib) and nontypeable strains (collectively, nHiB H. influenzae). Pediatric hearing loss after nHib H. influenzae meningitis remains poorly described. MethodsRetrospecive case series of nHiB H. influenzae meningitis cases identified from a microbiologic database at Children's Hospital Colorado from 2000 to 2020. Literature regarding nHiB H. influenzae and H. influenzae postmeningitic hearing loss was also reviewed. ResultsEleven cases of nHib H. influenzae meningitis (median age 15.9 months) were identified due to serotype f (36 %), serotype a (27 %), and nontypable strains (36 %). Seven (64 %) patients were male, 55 % were white and 18 % were Hispanic or Latino. Hearing loss was initially identified in 4 children (40 %), with two patients with moderate conductive hearing loss (CHL) and one child with unilateral moderate sensorineural (SNHL) hearing loss patients recovering normal hearing. One patient with bilateral profound sensorineural hearing loss and associated labyrinthitis ossificans required cochlear implantation. All children (4) with identified hearing loss were noted to have additional intracranial sequelae, which included empyema (2), sinus thrombosis (2), and seizures (2). Of patients receiving steroids, 25 % had hearing loss on initial testing, compared to 66 % of those who did not receive steroids. ConclusionsnHib H. influenzae can cause both transient and permanent postmeningitic hearing loss. Steroids may offer otoprotection in nHib H. influenzae meningitis similar to Hib meningitis. Given the limited literature, further study is needed to better characterize hearing outcomes after nHib H. influenzae meningitis.

Tympanometric and auditory brainstem response findings among children with down syndrome

Background: The Worldwide prevalence of Down syndrome children with hearing loss is 38–78% with conductive hearing loss accounting for the majority of cases and a documented 20%. Otitis media with effusion (OME) is common in 2-year-olds. Objective: To determine the tympanometric and auditory brainstem response findings among children with Down syndrome. Methodology: Cross sectional observational study was conducted to determine the tympanometric and auditory brainstem response findings among children with Down syndrome on a sample of 98 patients both Male and Female with Non-probability purposive sampling technique. The study was performed at the Children's Hospital and Institute of Child Health in Lahore. The duration of this study was 6 months, from January 2023 to June 2023. Children aged 1 to 6 years were included in this study. A self-structured questionnaire, tympanometry, and auditory brainstem response test were used to accumulate the data. The data was analysed using the SPSS version 24.0 software package. Results: Among the 98 DS patients, there were 52 (70.41%) male patients and 46 (29.59%) female patients. The majority of patients were in the age group 1-3 years, which is 61 (65.31%) in number. From the analysis of the tympanometry test, it was found that the majority of patients had type B tympanograms, which is 55 (56.1%), and type C tympanograms were also shown in 28 (28.6%) patients. Results of BAEP showed that most patients had moderate-degree hearing loss 70 (71.1%) and 15 (15.3%) had normal hearing. Conclusion: This study concluded that the majority of children had moderate degree conductive hearing loss rather than mild to severe degree mixed and sensorineural hearing loss. Key words: Down syndrome, Pediatric Hearing Loss, Tympanometry, Auditory Brainstem Response, Otitis Media with Effusion

Conductive hearing loss in newborns: Hearing profile, risk factors, and occasions of service.

Infants diagnosed with a conductive hearing loss (CHL) are at increased risk of developmental delays. Using a sample of infants diagnosed with CHL through UNHS, this study aimed to investigate the relationship between specific demographic or clinical characteristics and 1) occasions of service to reach a hearing diagnosis and 2) the profile of CHL. Retrospective analysis was conducted for all infants with CHL born between 01/01/2007 and 31/12/2018 who had received UNHS. Chi squared analysis was conducted on data from 1208 records. Infants with ≥1 risk factor for hearing loss were more likely to attend more than three occasions of service. Infants who were bilateral refer/medical exclusion, Torres Strait Islander, had ≥1 risk factors for hearing loss or were born pre-term had greater proportions of bilateral CHL than unilateral CHL. Mild to moderate was the most frequent degree of CHL, although a unilateral or bilateral CHL did not have an association with the severity of CHL. Compared to other risk factors, infants with a syndrome had greater proportions of bilateral than unilateral CHL. Risk factors of craniofacial abnormality, prolonged ventilation, or syndrome had greater proportions of mild to moderate CHL than moderate or greater. On average, infants were diagnosed with a CHL at 37.29 weeks of age. These findings highlight the relationship between clinical/demographic characteristics and occasions of service to diagnose CHL in children, including the CHL profile. An understanding of this relationship may help clinicians to better plan, assess and manage infants diagnosed with a CHL through UNHS.

Factors Leading to Conductive Hearing Loss among Children Living in Delhi Slum Areas

Background: Middle-ear diseases in children pose a significant burden to the healthcare system. Recurrent upper respiratory tract infections, cold, and cough often initiate middle ear disease. It results in mild to moderate hearing impairment that can be detrimental to the child's linguistic development. It can further lead to behavioural and cognitive problems. The study was designed to establish a relationship between common middle pathologies like CSOM and OME with hearing loss in children.&#x0D; Objective: To determine the prevalence of middle ear pathology and its association with hearing loss in slum children of Delhi.&#x0D; Methods: A study was conducted with 269 children aged five to ten years. The children were screened by otoscopic examination, followed by Tympanometry and then by Pure Tone Audiometry.&#x0D; Results: The prevalence of middle ear problems was 24.2%, out of which the prevalence of Chronic Suppurative Otitis Media (CSOM) was 10.8%, Otitis media with effusion (OME) was 12.3% and Acute Otitis Media (AOM) was 1.1%. A significant correlation (p&lt; 0.005) was found between hearing loss and middle ear pathology like CSOM and OME. Out of the 65 children with ear pathology, hearing loss was detected in 55 children, out of which 29 children with CSOM, moderated conductive hearing loss was detected in 28 children and out of 33 OME children, mild to moderate conductive hearing loss was detected in 26 children.&#x0D; Conclusion: The article highlights the high prevalence of middle ear pathology in underprivileged children. The study also concludes the high probability of conductive hearing loss in these children. It is recommended that there is more need for awareness for early detection and treatment of middle ear infection to avoid any language and behavioural problems in the future. Also, the disparity between children living in urban and rural areas needs to be bridged.

Analysis of audiological features in infants with unilateral hearing loss

Objective:To investigate the audiological characteristics and possible causes of unilateral hearing loss in infants and young children. Methods:105 infants from Beijing Maternal and Child Health Care Institution who failed the newborn hearing screening and were referred to the Children's Hearing Diagnosis Center of PLA General Hospital for hearing diagnosis. They were diagnosed with unilateral hearing loss and underwent clinical data collection. A full set of audiological examinations included ABR, 40 Hz auditory event related potential, ASSR, DPOAE, tympanometry. Results:①In initial diagnosis, 45 cases（42.86%） had mild hearing loss, 19 cases（18.10%） had moderate hearing loss, 14 cases（13.33%） had severe hearing loss, and 27 cases（25.71%） had severe hearing loss; Among them, 65 cases（61.90%） were conductive hearing loss or mixed hearing loss, and 40 cases（38.10%） were sensorineural hearing loss. ②83 of 105 cases had follow-up visits: 24 cases were normal, 15 cases with mild hearing loss, 4 cases with moderate hearing loss, 12 cases with severe hearing loss, and 26 cases with extremely severe hearing loss, 2 cases of hearing loss in both ears. ③From the initial diagnosis to the follow-up diagnosis, the change of mild hearing loss was the largest, followed by moderate hearing loss, severe and extremely severe hearing loss basically did not change; the number of mild and severe conductive hearing loss which recovered to normal hearing was most, the number of sensorineural hearing loss changed little. Conclusion:The infants who failed the newborn hearing screening and were diagnosed with unilateral hearing loss were mainly mild to moderate conductive hearing loss and severe to extremely severe sensorineural hearing loss. The hearing of children with hearing loss gradually improved, and severe and extremely severe sensorineural hearing loss remained unchanged.

Cross-Sectional Study of Hearing Status in Primary Students in Mainstream and Special Schools

Background. The only way to prevent the negative outcomes of hearing loss in children (speech, mental, learning, socialization disorders) is early diagnosis of the pathology and rehabilitation onset. It can be provided by hearing screening programs for children of different ages, including school-age children. Hearing screening is not performed among school-age children in Russian Federation. Objective. The aim is to study the prevalence and structure of hearing disorders in students in mainstream and special (for children with visual impairments) schools to determine the strategy for implementing hearing screening of school-age children in Russian Federation. Methods. The study included children of two groups who had not previously been diagnosed with hearing disorders: group 1 — students of 1–4 grades of mainstream school, group 2 — students of 1–4 grades of special school (children with visual impairments) (St. Petersburg). The screening stage included otoscopy, tympanometry, otoacoustic emissions, pure tone audiometry. Children with any problems later underwent a complex hearing examination at Center of Pediatric Audiology (St Petersburg) to confirm the presence of hearing disorder and for disease differential diagnosis. The major indicator in this study is the percent (%) of children with confirmed hearing disorder in each group. The additional indicator is the proportion of various pathological conditions in hearing disorders’ structure (persistent disorders and hearing loss degree included). Study methods: otoscopy, tympanometry, pure tone audiometry, speech audiometry, otoacoustic emissions and auditory brainstem responses. Results. We have revealed abnormalities in 23% of 183 children from mainstream school. Hearing disorders were confirmed in 19.7% of children. Most children were diagnosed with temporary mild and moderate conductive hearing loss, whereas 3.9% were diagnosed with permanent (sensorineural) hearing loss. The total percent of children with confirmed hearing disorder was not significantly different in 148 children from special school. Therefore, they have 2.5 times more cases of permanent hearing loss and children with more severe hearing disorder. Conclusion. The obtained data confirm the hearing screening relevance in school-age children. Further studies are required to develop hearing screening protocol for school-age children considering the features of audiologic service and educational system in Russian Federation.

Hearing Profile of Children Below Three Years Old at Jatinangor Integrative Health Care Center, West Java, Indonesia

Background: Hearing function is one of the most important factors affecting children’s development process. The first three years of life is a golden period of growth and development of children. This study was conducted to investigate the hearing profile of children below three years old at Jatinangor Integrative Health Care Center (Pos Pelayanan Terpadu, Posyandu), West Java, Indonesia. Methods: An observational cross-sectional study was conducted to 86 children below three years old who was selected by using multistage cluster sampling at 12 Posyandu in Jatinangor sub-district by using in-depth interview related to hearing loss risk factors, reaction test, and tympanometry test. Results: The hearing loss prevalence of children below three years old in the Jatinangor sub-district was 7.0%. Of the 86 children, there were 2.3% (n=2) children with moderate conductive hearing loss, 1.2% (n=1) children with moderate sensorineural hearing loss, and 3.5% (n=3) children with very severe sensorineural hearing loss. Fifty percent of children with sensorineural hearing loss were discovered without any risk factor. Conclusions: The prevalence of hearing loss in children under three years old at the Jatinangor Integrative Health Care Center is slightly higher than the national prevalence. Fifty percent of children are found without risk factors, therefore Universal Newborn Hearing Examination (UNHS) needs to be implemented at the national level in accordance with the guidelines of the Joint Committee for Infant Hearing (JCIH) in which are integrated with primary health care units, tertiary health care centers otorhinolaryngology, and also the audiological center.

Correlation of size of tympanic membrane perforation &amp; degree of hearing loss and frequency specific hearing gain following tympanoplasty

Introduction: The tympanic membrane (TM) serves as a key component of the tympano-ossicular system. Chronic otitis media is known to cause mild to moderate conductive hearing loss. Material &amp; Methods: The proposed study was conducted on 50 patients of either sex in age group of 15-50 years having unilateral or bilateral chronic inactive (mucosal) otitis media after obtaining their consent. Pure tone audiogram was done to confirm the degree, type and frequency specific hearing loss from frequency 250Hz to 8000Hz for air conduction and 250Hz to 4000Hz for bone conduction. TM perforations were divided into Group I (small), II (medium), III (Large) according to size of perforation. Results: Average Hearing loss in Group I, II, &amp; III was 33.86, 43.84 &amp; 46.75 dB respectively. Hearing improvement following tympanoplasty at 250, 500, 1000, 2000, 4000, 8000 Hz was 20.50, 18.1, 16.7, 14.3, 12.0, 1.6 dB respectively. Conclusion: Hearing threshold level depends on size of the tympanic membrane perforation. Hearing improvement following tympanoplasty is frequency-dependent also.

Hearing impairment in patients with oral submucous fibrosis

Abstract Introduction: Oral submucous fibrosis (OSMF) is a premalignant condition of the oral cavity. The pathological changes in OSMF often affect mucosa, submucosa, and underlying muscles in the oral cavity and pharynx. The pathological changes of the tubal and para-tubal muscles can affect the function of the Eustachian tube. The Eustachian tube dysfunction in OSMF often may result in hearing impairment. Aim: This study aims to evaluate the effect of OSMF on Eustachian tube function resulting in hearing impairment. Materials and Methods: This prospective study was carried out at a tertiary care teaching hospital of Eastern India from June 2019 to July 2021. There were 68 patients with OSMF enrolled in this study by simple random sampling. In this study, 68 patients (136 ears) suffering from OSMF with hearing impairment or fullness/blockage feeling in the ear who participated in this study. The clinical profile of these patients was worked out by taking a detailed history and clinical examination. All the patients underwent audiological evaluation with pure-tone audiometry and tympanometry. Results and Discussion: Out of 68 patients, 42 (61.76%) were male and 26 (38.23%) were female with a male-to-female ratio of 1.61:1. The age ranges of the patients who participated in this study were between 18 years and 65 years. Pure-tone audiometry showed normal hearing in 86 (63.24%) ears, mild conductive hearing loss in 31 (22.79%) ears, moderate conductive hearing loss in 14 (10.29%) ears, and sensorineural hearing loss in 5 (3.68%) ears. Conclusion: The Eustachian tube may be blocked by the effect of the OSMF and result in hearing loss. Hence, while treating OSMF, hearing impairment in OSMF has to be kept in mind.

Congenital Absence of Stapedius Muscle and Tendon: Case Report with Review of Literature

Absence of stapedius muscle and its tendon is an extremely rare congenital anomaly, with only nine indexed reports in live patients. It presents with non progressive, occasionally bilateral conductive deafness, although this cannot be confirmed from Pure Tone Audiometry (PTA) and High Resolution Computed Tomography (HRCT). This makes it one of the least probable differential diagnosis of conductive deafness with unremarkable tympanic membrane. The definitive diagnosis is made only at time of surgery. The condition often co-exists with other genetic/acquired disorders of the middle/inner ear (otosclerosis, tympanosclerosis, etc.,). In absence of standardised, evidence based management protocol, diagnosis and appropriate treatment become challenging, and are predominantly situational. Its occurrence follows complex embryologic sequences where the internal portion of the interhyale- the mesenchymal condensation separating the cranial mesenchyme of the second pharyngeal arch (stapedial anlage) from Reichert’s cartilage- remains either absent or undergoes regression. Here, authors presents a case of 19-year-old female with congenital absence of stapedius muscle and tendon. She presented with congenital, bilateral, non progressive, moderate conductive hearing loss, and was diagnosed with this condition at surgery. There was co-existent tympanosclerosis in the middle ear that resulted in ossicular chain fixation and absence of round window reflex. The tympanosclerotic plaques were meticulously removed, ossicular chain mobility was restored, and a type II ossiculoplasty was done, along with reconstruction of the posterosuperior bony canal wall with cartilage perichondrium complex. Apart from the clinical presentation and its management, the present report also emphasises the plausible theories on relevant embryology explaining such an unusual event.

Simultaneous Bilateral Bone Bridge Implantation in a Two-Year-Old Child with Atresia of the External Auditory canal: A Case Report

Introduction: Active transcutaneous bone conduction implants are indicated for mild to moderate conductive or mixed hearing loss (intact inner ear and auditory nerve) for subjects 5 years or older. The aim of the here presented study was to share our results with the Bonebridge in a child aged 2 years at time of surgery. Especially the new generation of the implant being half of the size, a re-evaluation of the given indication for children 5 years or older is highly recommended.

A Rare Case of Severe Post Adenoidectomy Secondary Haemorrhage

Tonsillectomy and adenoidectomy are amongst the most common surgeries performed in children. Haemorrhage, after and during these procedures, is a major and known complication. Most commonly, the haemorrhage presents intraoperatively or in the immediate postoperative period and is self-limiting. However, secondary haemorrhage presenting after adenoidectomy where adenoidectomy alone was performed have been reported in very few patients. In this case, a 10-year-old male presented with history of upper airway obstruction including sleep apnoea, adenoid facies, and bilateral moderate conductive hearing loss since four years. He had no co-morbidities, history of bleeding diathesis. The pre-operative evaluation of the adenoids (clinically and radiologically) revealed no abnormal vasculature or anatomical variations. Conventional adenoidectomy and bilateral grommet insertion was performed under General Anaesthesia (GA). The postoperative period was entirely uneventful, and the patient was discharged on postoperative day 3. Despite having no risk factors, he presented with profuse posterior epistaxis after conventional adenoidectomy on postoperative day 5. Immediate management was done by posterior nasal packing and intravenous amoxicillinclavulanate Definitive treatment was done by re-exploration and debridement of the remnant adenoid tissue using a microdebrider under GA the next day. The patients had no complaints postoperatively or at 6 months follow-up. The rarity of complications makes adenoidectomy a fairly safe procedure. However, despite being uncommon, secondary complications of adenoidectomy should be anticipated by surgeons to avoid dire consequences for the patient.

Cortical auditory function in children with unilateral congenital aural atresia

ABSTRACT Objective: This study explored cortical auditory evoked potential (CAEPs) elicited by speech sounds in children with unilateral congenital aural atresia (CAA). Design: Twelve children (8 males) aged 7–16 years (M age 10.0, SD 3.1) with unilateral CAA took part in the study. Results were compared to a control group of 15 children 5–16 years (M age 9.5, SD 3.4 years) with normal hearing bilaterally. CAEPs to speech in noise (+5 dB signal-to-noise ratio) were recorded. Results: P1 amplitudes were larger for frontal locations regardless of the stimulus, ear of presentation, group (CAA vs. normal hearing), or age. Younger participants had larger P1 amplitude across electrodes. For N250 responses, CAEPs evoked by /ti/ had the shortest latency, followed by /di/and/gi/. Older children presented with shorter N250 latencies for /ti/, regardless of electrode location or group or ear of presentation. Conclusions: CAEPs in children with a moderate conductive congenital unilateral hearing loss showed typical developmental and stimulus effects. We did not find group differences when CAEPs of children with atresia were compared to control participants. Better understanding of typical development and changes in CAEPs over time in children with CAA is needed to ascertain whether unilateral hearing loss due to CAA has effects on CAEPs that correlate with perceptual effects.

Allergic Fungal Otomastoiditis: A Case Report and Review of Literature.

Allergic fungal otomastoiditis, a rare known entity has only been published as case reports in literature. There have been 3 cases of adult and 1 case of pediatric allergic fungal otomastoiditis reported as of now. A young male had unilateral otorrhea and hearing difficulty for four years. The audiogram revealed moderate conductive hearing loss and radiograph showed complete opacification of tympanic cavity. He underwent tympano-mastoidectomy, showing polypoidal granulations and thick mucoid discharge. The histopathology was a surprise diagnosis of Allergic fungal otomastoiditis. The clinical and histopathological appearance of this entity matches allergic fungal rhinosinusitis. After extensive review of literature, we propose a working criterion for early identification of allergic fungal otomastoiditis in otolaryngology setting.

Ceruminous Adenoma: A Rare Mass that Impair Hearing.

Ceruminous adenoma is described as a glandular neoplasm of ceruminous glands. It is seen for less than one percent of all external ear tumours. Ceruminous adenoma cases were reported to have recurrence and residual tumour, however there was no malignant transformation known in ceruminous adenoma up to this date. Here, we report a young adult woman with two years history of progressive reduced hearing and tinnitus of the right ear. She was proven to have right moderate conductive hearing loss with pure tone audiometry. There was a cystic mass with serous content arising from the posterior wall of the right ear canal. We proceeded with wide local excision of the mass via trans-canal approach. Ceruminous adenoma was confirmed with histopathology and immunohistochemistry of CK7 staining. The hearing impairment was resolved completely post excision and there was no recurrence of the tumour on one year follow-up. We concluded wide local excision with appropriate margin of the mass is adequate to prevent recurrence in ceruminous adenoma cases.