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

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Pathophysiological and Clinical Aspects of Hearing Loss Among 85-Year-Olds.

PURPOSE: This study aimed to identify the prevalence of conductive/mixed and sensorineural hearing loss, with an attempt to differentiate between sensory and neural components in 85-year-olds. METHOD: A comprehensive auditory test protocol, including pure-tone audiometry, speech audiometry, auditory brainstem response (ABR), and distortion product otoacoustic emission (DPOAE), was used to identify different types of hearing loss in 85-year-olds. This study comprised a subsample (n = 125) selected from an unscreened cohort of 85-year-olds born in 1930, within the Gothenburg H70 Birth Cohort Studies in Sweden. RESULTS: Test results were reported descriptively. Sensorineural hearing loss was present in one or both ears in almost all participants (98%), and the majority had absent DPOAEs. Only approximately 6% had additional conductive hearing loss, that is, mixed hearing loss. Approximately 20% of the participants with a pure-tone average at 0.5-4 kHz < 60 dB HL had worse word recognition scores compared with predicted scores by the Speech Intelligibility Index (SII), whereas only two participants were classified with neural dysfunction with the use of ABR. CONCLUSIONS: Sensorineural hearing loss, likely related to outer hair cell loss, was present in the vast majority of 85-year-olds. Conductive/mixed hearing loss appears to be relatively rare in advanced age. Poor word recognition scores in relation to SII-predicted scores were relatively common (20%) in 85-year-olds, whereas auditory neuropathy was only rarely identified (1.6%) by the use of ABR latencies. To explain abnormal word recognition and to identify the neural component of hearing loss among the older-old population, future research should consider factors such as listening effort and cognition among the older-old population.

Clinical characteristics of infants identified with a conductive hearing loss through universal newborn hearing screening: A population-based sample.

BACKGROUND: Universal Newborn Hearing Screening (UNHS) aims to identify infants born with a permanent hearing loss. However, many are also diagnosed with a conductive hearing loss (CHL) and are at subsequent risk for developmental delays. The aim of this study was to investigate the prevalence of CHL and determine which clinical characteristics collected at birth, predict CHL within UNHS. MATERIALS AND METHODS: Retrospective analysis was conducted on all infants born between January 01, 2007 and December 31, 2018. During this period, 731,234 infants were screened, 9802 were direct referrals, and 1208 identified with a CHL. Chi squared analysis and logistic regression was conducted to determine CHL prevalence and identify which clinical characteristics predict CHL. RESULTS: The prevalence of CHL was 12.32%. Following adjustments for collinearity, clinical characteristics that could predict CHL were: bilateral referrals/medical exclusions to screen (Odds ratio, OR 1.89; 95% CI: 1.65-2.1), ≥1 risk factor for hearing loss (OR 2.03; 95% CI: 1.76-2.34), pre-term birth (OR 1.82; 95% CI: 1.57-2.10), male (OR 1.21; 95% CI: 1.07-1.37), and Indigenous status: 'Aboriginal (not Torres Strait Islander)' (OR 1.27; 95% CI:1.03-1.57 and 'not stated' (OR 2.95; 95% CI: 2.02-4.30). CONCLUSION: CHL within UNHS was highly prevalent, with six clinical characteristics that can predict that likelihood of an infant being diagnosed with a CHL. This data could be used to create alternative care pathways for infants with CHL, enabling early and targeted assessments, thereby reducing the risk of developmental delays for these infants.

Hearing impairment and ear anomalies in craniofacial microsomia: a systematic review.

The aim of this systematic review was to review the literature on hearing impairment and ear anomalies in patients with craniofacial microsomia and to determine their prevalence. Sixty-two records including 5122 patients were included. Ear anomalies were present in 52-100% of patients. The most reported external ear malformations were microtia, pre-auricular tags, and atresia of the external auditory canal. Ossicular anomalies were the most reported middle ear malformations, whereas the most reported inner ear malformations included oval window anomalies, cochlear anomalies, and anomalies of the semicircular canals. Hearing loss in general was reported in 29-100% of patients, which comprised conductive hearing loss, mixed hearing loss, and sensorineural hearing loss. Between 21% and 51% of patients used hearing aids, and 58% underwent a surgical intervention to improve hearing. The relationship between different phenotypes of craniofacial microsomia and the type and severity of hearing loss is mostly unclear. In conclusion, the high prevalence of ear and hearing anomalies in patients with craniofacial microsomia underlines the importance of audiological screening in order to facilitate individual treatment.

Exploring the relationship between conductive hearing loss and cleft speech characteristics in children born with cleft palate.

BACKGROUND: Children with cleft palate are at high risk of both conductive hearing loss and cleft speech characteristics (CSCs) yet there is limited research to understand whether hearing loss impacts speech development in this population by contributing to the development of CSCs. AIMS: This study used data from a large national cohort study in the UK (The Cleft Collective) to explore the relationship between those with a history of diagnosed hearing loss and presence of CSCs in children with cleft palate (+/- cleft lip) aged between 18 and 24 months. METHOD: Speech and Language Therapists (SLTs) provided uniformed information from assessment for 123 participants who had been recruited to the Cleft Collective study. History of diagnosed hearing loss, intervention for hearing loss, and presence of CSCs were reported. A consonant inventory for each participant was completed. Statistical analysis of relationships between hearing loss and CSCs and analysis of consonant inventories was undertaken to provide information related to speech acquisition and its relationship with hearing. RESULTS: There was a statistically significant relationship between history of diagnosed hearing loss and presence of CSCs (p < 0.05). Analysis of the consonant inventories highlighted that children with diagnosed hearing loss used fewer oral consonants compared to those with normal hearing (p < 0.05) CONCLUSION: This study provides some evidence that a conductive hearing loss can affect speech in children with cleft palate resulting in CSCs developing. Therefore, children with cleft palate should be closely monitored by audiology from birth to ensure that hearing is optimised for speech and language development. SLT services should also closely monitor the speech development of those children where a hearing loss has been identified, so that early intervention can be provided if appropriate. Continued data collection, with a large sample of children, will provide additional evidence regarding how this hearing loss is best managed. It will also allow increased knowledge of the long term impact of conductive hearing loss on speech development in children with cleft palate.

Efficacy of middle-ear packing in success of type 1 tympanoplasty: a prospective randomised study.

OBJECTIVE: A prospective randomised study was undertaken to compare the results of type 1 tympanoplasty with and without middle-ear packing with gelfoam. METHOD: Eighty patients undergoing type 1 tympanoplasty were randomised into two groups according to packing in the middle ear: with gelfoam and without gelfoam. The data in terms of graft uptake rate, hearing gain and subjective improvement were analysed at one and three months. RESULTS: The graft uptake rate between both groups did not show a statistically significant difference. There was conductive hearing loss in the gelfoam group in the early post-operative period. Subjectively, patients were more comfortable with respect to heaviness and hearing gain than in the non-gelfoam group. CONCLUSION: Gelfoam use in middle-ear packing is not an essential step and causes more discomfort in patients during the early post-operative period. It should be a surgeon's choice to use it when and where it is necessary.

Multicenter Study of Congenital Middle Ear Anomalies. Report on 246 Ears.

OBJECTIVES/HYPOTHESIS: Congenital middle ear anomalies represent a relatively rare condition. This study aimed to describe the characteristics and the surgical outcomes for patients with middle ear anomalies. METHODS: A multicenter study was conducted of consecutive patients with congenital middle ear anomalies who underwent primary surgical treatment between January 2008 and December 2017. Demographics, surgical procedures, and audiometric data were registered into the institutional database. Hearing changes and postoperative air-bone gap (ABG) were evaluated 1 year after surgery. RESULTS: A total of 246 patients (246 ears) (median age: 14 years, range: 4-75 years old) were included in this study. Anomalies were subdivided using the Teunissen and Cremers classification: 53 ears (22%) were categorized as class I, comprising only stapes ankylosis; 35 ears (14%) as class II, having ossicular chain anomalies with stapes ankylosis; 139 ears (57%) as class III, having ossicular chain anomalies with a mobile stapes-footplate; and 19 ears (8%) as class IV, with aplasia of the oval window. Evaluation of hearing outcomes for 198 ears with more than 1 year of follow-up revealed that good postoperative ABG (≤20 dB) was achieved in 82% of class I, 68% of class II, 74% of class III, and 23% of class IV anomalies. The postoperative ABG in class IV was significantly worse than in class I (P < .001) or class III (P < .01). CONCLUSIONS: This study demonstrated that class III anomalies comprised the majority of middle ear anomalies and surgical outcomes for class IV anomalies are unfavorable. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2323-E2328, 2021.

Prevalence of Otological Disease in Turner Syndrome: A Systematic Review.

INTRODUCTION: Girls and women with Turner syndrome (TS) present with multiple ear and hearing problems, ranging from external morphologic abnormalities to sensorineural or conductive hearing loss. The exact pathophysiology behind these otological diseases is not yet completely understood. The aim of this study is to provide a systematic review on the prevalence of otological disease in TS. METHODS: We conducted a systematic review according to the PRISMA guidelines. A database search was performed in PubMed, Embase, Web of Science, and Cochrane library. RESULTS: The prevalence of otological disease as external ear deformities (20-62%), recurrent otitis media (24-48%), and hearing loss (36-84%) is high in TS. The auditory phenotype in TS is complex and seems to be dynamic with CHL due to middle ear disease at young age and sensorineural hearing loss later in life. CONCLUSION: This systematic review of the literature confirms that otological disease is definitely part of the widely variable phenotype in Turner patients. Strong evidence is lacking on the exact prevalence numbers, emphasizing the need for more prospective data gathering. Growing insights in its pathophysiology will help in the understanding and management of hearing problems in TS across lifespan.

Reconstruction of the external auditory canal using full-thickness rolled-up skin graft after lateral temporal bone resection for T1 and T2 external auditory canal cancer.

OBJECTIVE: To present our results of the external auditory canal (EAC) reconstruction procedure using rolled-up full-thickness skin graft with tympanoplasty after lateral temporal bone resection (LTBR) for early-stage EAC carcinoma. PATIENTS AND METHODS: A retrospective review of 15 patients who had undergone LTBR with reconstruction of the EAC for T1 and T2 EAC cancer between 2016 and 2020. RESULTS: Postoperative mean air-bone gap was 30.7 decibel hearing level. Although a few patients experienced chronic granulation, persistent otorrhea, and/or laterization of the tympanic membrane, most patients showed no serious complications related to the EAC reconstruction. CONCLUSION: EAC reconstruction using a full-thickness skin graft in combination with tympanoplasty is useful for minimizing the hearing loss, maintaining the cosmetic appearance, and facilitating the observation into the ear cavity.

Prevalence of Hearing Loss, and Middle Ear Disease in 1351 School Age Children in Mekelle, Ethiopia.

OBJECTIVES: The objective of this study was to begin to measure the prevalence of elevated hearing thresholds in Ethiopia, with audiometric equipment, including bone oscillators and tympanometers. To that end Ethiopian nationals were trained in audiometric techniques to obtain sensorineural hearing loss (SNHL) and conductive hearing loss (CHL) rates in school age children. METHODS: Five mainstreamed public grade schools in northern Ethiopia were the sites for audiometry campaigns examining 1351 children and testing them with behavioral audiometry. RESULTS: Seven percent of students had SNHL > 20 dB in at least 1 frequency in at least 1 ear, and 0.6% of ears had SNHL > 50 dB. Eighteen percent8% of students had CHL > 20 dB in 1 or both ears. The frequency with the highest incidence of elevated pure tone thresholds was at 8000 Hz. Seven percent of students had an air bone gap at 2000 and/or 4000 Hz of at least 15 dB. Air bone gap is not obtained at 8000 Hz. There were 22 perforated tympanic membranes in 17 children (0.8% of tympanic membranes, 1% of students). CONCLUSION: This study gives a baseline for the prevalence of hearing loss in school age children in mainstreamed northern Ethiopian schools. It is the first of its kind and perhaps can help in estimated hearing health needs there. LEVEL OF EVIDENCE: 2.

Renal Syndromic Hearing Loss Is Common in Childhood-onset Chronic Kidney Disease.

BACKGROUND: Hearing loss (HL) in children may adversely affect their development. HL is more prevalent in patients with chronic kidney disease (CKD) than in the general population. This study evaluated the prevalence of HL and its underlying diseases in patients with childhood-onset in CKD. METHODS: In this retrospective study of a tertiary referral center, childhood-onset CKD patients (stage 2-5, age at onset of renal symptom < 18 years) were recruited. We referred to the "renal" syndromic HL as cases with genetic or syndromic diseases, or extra-renal anomalies in addition to HL and CKD. RESULTS: A total of 421 patients (male:female = 279:142) were reviewed according to the causes of CKD: congenital anomalies of the kidney and urinary tract (CAKUT; n = 184, 43.7%), glomerulopathies (GP; n = 105, 24.9%), cystic kidney diseases (CYST; n = 39, 9.3%), perinatal problems (PP; n = 29, 6.9%), and others (n = 64, 15.2%). HL was detected in 82 (19.5%) patients, including 51 (12.1%) patients with sensorineural hearing loss (SNHL), 30 (7.1%) with conductive hearing loss (CHL), and 1 patient with mixed HL. The prevalence of HL in each group was as follows: 16.8% in the CAKUT group, 28.6% in the GP group, 12.8% in the CYST group, 24.1% in the PP group, and 14.1% in the others group. HL was more common in higher CKD stages, especially CHL in end-stage renal disease. SNHL was more prevalent in CKD from GP. Of the 82 patients with HL, 50% had renal syndromic HL: 58.8% of SNHL and one-third of CHL were renal syndromic HL. CONCLUSION: One-fifth of the childhood-onset CKD had HL. Collectively, renal syndromic HL comprised half of the HL in this study. To improve the quality of life in patients with childhood-onset CKD, we suggest that HL should be considered, requiring surveillance, and if necessary, early intervention.

Association of rheumatoid arthritis with hearing loss: a systematic review and meta-analysis.

Rheumatoid arthritis (RA) is a systemic autoimmune disease affecting up to 1% of the worldwide population. RA is associated with multiple extra-articular manifestations (EAMs). Middle ear, cochlea and the auditory nerve are suspected sites of RA activity and hearing loss is a possible novel EAM of RA. Objective was to investigate the association of RA with the different subtypes of hearing loss. This systematic review was performed according to the PRISMA guidelines. A random effects model meta-analysis was conducted and the I2 was used to assess heterogeneity. Twelve studies comprising 20,022 RA patients and 79,244 controls were included in this systematic review. All studies were observational and were rated to a moderate rate of bias. RA patients had nearly fourfold increased odds of sensorineural hearing loss (SNHL) compared with controls (OR 3.42; 95% CI 2.50-4.69; I2 = 13). RA patients also had a significantly increased risk of SNHL (RR 2.28; 95% CI 1.88-2.76; I2 = 0). RA patients did not have increased odds of conductive hearing loss (CHL) and mixed hearing loss (MHL) (OR 1.36; 95% CI 0.52-3.55; I2 = 22); (OR 2.73; 95% CI 0.78-9.58; I2 = 0%). RA is significantly associated with SNHL. RA is not associated with CHL and MHL. Early screening of RA patients with pure tone audiometry should be considered.

Multiple synostoses syndrome: Clinical report and retrospective analysis.

Multiple synostoses syndrome (SYNS1; OMIM# 186500) is a rare autosomal dominant disorder reported in a few cases worldwide. We report a Chinese pedigree characterized by proximal symphalangism, conductive hearing loss, and distinctive facies. We examined the genetic cause and reviewed the literature to discuss the pathogeny, treatment, and prevention of SYNS1. Audiological, ophthalmological, and radiological examinations were evaluated. Whole-exome sequencing (WES) was performed to identify mutations in the proband and her parents. Sanger sequencing was used to verify the results for the proband, parents, and grandmother. The literature on the genotype-phenotype correlation was reviewed. The patient was diagnosed with multiple synostoses syndrome clinically. WES and bioinformatic analysis revealed a novel missense mutation in the NOG gene, c.554C>G (p.Ser185Cys), cosegregated in this family. The literature review showed that the phenotype varies widely, but the typical facies, conductive hearing loss, and proximal symphalangism occurred frequently. All reported mutations are highly conserved in mammals based on conservation analysis, and there are regional hot spots for these mutations. However, no distinct genotype-phenotype correlations have been identified for mutations in NOG in different races. Regular systematic examinations and hearing aids are beneficial for this syndrome. However, the outcomes of otomicrosurgery are not encouraging owing to the regrowth of bone. This study expanded the mutation spectrum of NOG and is the first report of SYNS1 in a Chinese family. Genetic testing is recommended as part of the diagnosis of syndromic deafness. A clinical genetic evaluation is essential to guide prevention, such as preimplantation genetic diagnosis.

Hearing outcomes in children of diabetic pregnancies.

OBJECTIVE: Children of diabetic pregnancies (CDPs) face numerous risk factors for hearing loss (HL). The objective of this study was to investigate the hearing outcomes of CDPs on a population scale. METHODS: Using the Audiological and Genetic Database, the prevalence, severity, and progression of HL in CDPs was compared against children of non-diabetic pregnancies (CNDPs) who served as controls. RESULTS: Among 311 CDPs, 71.1% demonstrated evidence of HL compared to 45.5% in CNDPs (p < 0.001). The mean age at which CDPs received audiograms was 3.6 years compared to 5.4 years for CNDPs (p < 0.001). Compared to CNDPs, CDPs were similarly affected by common otologic conditions such as acute otitis media (25.7%), chronic otitis media (38.3%), and Eustachian tube dysfunction (41.8%) (all p > 0.05). CDPs were more likely to have bilateral HL (81%) and sensorineural hearing loss (SNHL) (8%) relative to CNDPs (p < 0.001 and p = 0.004, respectively). Rates of conductive HL and mixed HL were not significantly different between groups (p = 0.952 and p = 0.058, respectively). CDPs were at significant risk for the development of HL (aOR 1.66 [1.28-2.17], SNHL (aOR 1.63 [1.01-2.52], and high-frequency HL (aOR 1.32 [1.03-1.68]). Of the comorbidities evaluated, CDPs with hyperbilirubinemia (aOR 1.85 [1.18-2.84]), perinatal asphyxia (aOR 1.90 [1.06-3.16]), or congenital heart disease (aOR 1.21 [1.07-1.37]) demonstrated higher risk of SNHL. CONCLUSION: Children of diabetic pregnancies face increased risks of developing HL, particularly bilateral and sensorineural hearing loss. Given these findings, we recommend close audiologic follow-up for these children, especially those with complicated birth histories or additional medical problems.

Hearing loss in ankylosing spondylitis.

AIM: Several studies have described sensorineural deafness in ankylosing spondylitis (AS) patients while conductive hearing loss has been reported to be rare. In our study we have studied the prevalence of hearing loss (HL) among AS patients and its association with non-steroidal anti-inflammatory drug (NSAID) intake. METHODS: We studied 100 AS patients and 40 controls. HL was defined as pure-tone thresholds greater than 20 dB (decibel) in at least two frequencies of the audiogram. Severity of HL was defined as given by World Health Organization. Clinical details and cumulative NSAID doses were noted. RESULTS: Ninety-six of the 100 patients were male with mean age of 32 ± 12 years and mean duration of illness of 8.2 ± 6 years. Median Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI), Bath Ankylosing Spondylitis Metrology Index (BASMI) and cumulative NSAID dose were 3.5 ± 2.2, 2.75 ± 2, 3.7 ± 2.6 and 1202 ± 1290, respectively. Of the 48 with HL, 28 patients had bilateral HL. Twenty-nine patients had pure conductive HL while 16 had mixed HL (components of both sensory and conductive) and 3 had pure sensorineural HL. HL was mild in 38 patients, and moderate to severe in 10 patients. The presence of HL was associated with higher age (P ≤ 0.05). Conductive HL was at low frequency (0.25, 0.5, 1 kHz) in 70% of cases. Sensorineural HL was at high frequency (4, 8 kHz) in 75% of cases. There was no association of HL with disease parameters such as BASMI, BASDI, BASFI or cumulative NSAID dose. CONCLUSION: HL is common in AS. Conductive HL is more common than sensorineural HL. It is usually mild and occurs at low frequencies.

The emergence and prevalence of hearing loss in children with homozygous sickle cell disease.

OBJECTIVES: Many children with sickle cell disease (SCD) experience the effect of cochlear insult due to hemolytic anemia and vaso-occlusion that accompanies the disease. As a result, hearing sensitivity eventually decreases. The purpose of this study was to investigate the emergence of hearing loss and the prevalence of such in children with homozygous SCD. METHODS: A large pediatric database was utilized to gather audiometric outcomes in children with homozygous SCD. Children were identified by primary diagnosis ICD codes. Demographic and audiometric data was drawn to determine presence, type, degree, and laterality of hearing loss. Hearing loss was defined as an elevated audiometric threshold >15 dB HL for pure tone air conduction audiometry or >20 dB HL minimal response level for sound-field testing. RESULTS: One hundred and twenty-eight children were identified. The prevalence of hearing loss ranged from 28.8% to 50.8% depending on the calculation method (i.e., individual vs. ear specific prevalence and any elevated threshold vs. a three-frequency pure tone average). Conductive hearing loss and bilateral loss were most prevalent. The degree of hearing loss ranged from slight to profound. The mean age of identification of sensorineural hearing loss was 9.6 years. CONCLUSION: The prevalence of hearing loss in children with homozygous SCD is higher than in normal children. Regular hearing assessments of children with SCD is warranted and should be advocated in early infancy and as part of their ongoing care.

Dosimetric parameters associated with conductive or sensorineural hearing loss 5 years after intensity-modulated radiation therapy in nasopharyngeal carcinoma.

BACKGROUND: Most previous studies are separate dosimetric analyses of conductive or sensorineural hearing loss, and they are not conducive to a comprehensive assessment of auditory radiation damage. AIMS/OBJECTIVES: Our study aimed to evaluate the long-term incidence of sensorineural hearing loss (SNHL) or conductive hearing loss (CHL) in patients with nasopharyngeal carcinoma (NPC) after intensity-modulated radiation therapy (IMRT), and to investigate the relationship between SNHL or CHL and patient factors, treatment-related factors, and radiation dose parameters. MATERIAL AND METHODS: Seventy patients (117 ears) with NPC, who were also treated with IMRT in our hospital from 2006 to 2014, were retrospectively analyzed. Radiation doses to the Eustachian tube (ET), middle ear (ME), cochlear (Co), and internal auditory canal (IAC) were assessed. Pure tone audiometry and impedance audiometry were performed before and during the follow-up period. The relationships between low-frequencies (0.5-2 kHz) or high-frequency (4 kHz) SNHL/CHL and radiotherapy dose parameters were analyzed. RESULTS: Of the 117 ears studied, 7.69% had low-frequency SNHL, 35.9% had high-frequency SNHL, 23.93% had low-frequency CHL, and 18.80% had high-frequency CHL. The incidence of high-frequency CHL was higher in the T4 group than in the T (1-3) group (p < .05). When IAC Dmax > 42.13 Gy or IAC Dmean > 32.71 Gy, the risk of high-frequency SNHL increased in NPC patients. When ME Dmax > 44.27 Gy, ME Dmean > 29.28 Gy, or ET Dmax > 57.23 Gy, the risk of high-frequency CHL in NPC patients increased. CONCLUSIONS AND SIGNIFICANCE: SNHL and CHL remain common ear complications after IMRT for NPC. IAC Dmax, IAC Dmean, ME Dmax, ME Dmean, and ET Dmax all need to be carefully considered during the IMRT treatment protocol.

Malformations of the lateral semicircular canal correlated with data from the audiogram.

OBJECTIVES: Lateral semicircular canal (LSCC) malformations  are one of the most common inner ear malformations. The purpose of this study is to analyze the prevalence and type of hearing losses associated with LSCC malformations, compared to a control group. MATERIALS AND METHODS: We retrospectively included 109 patients (166 ears) presenting with a CT-confirmed LSCC malformation, compared to a control group (24 patients). The bony island surface and the width of the inner portion of the LSCC were measured to confirm the malformation. There results were correlated to audiogram data: sensorineural (SHNL), mixed (MHL) or conductive hearing loss (CHL) by an otologist. RESULTS: In the LSCC group, 60.9% of patients presented with an audiogram-confirmed hearing loss, especially SNHL (39.2%, n = 65) and MHL (12.7%, n = 21). Hearing was normal in 39.2% (n = 65) of the cases. Bilateral LSCC malformations (n = 57) were frequently associated with hearing loss (80.7%), SNHL in most of the cases (33.3%). Unilateral LSCC malformations were associated with hearing alterations (51.9%, n = 27), but we also observed a high rate (81%, n = 42) of contralateral abnormalities of the audiogram. CONCLUSION: LSCC malformations are commonly associated with hearing loss (61%), especially SHNL (39%). The high rate (81%) of contralateral hearing disturbances in unilateral LSCC malformations should be taken into account in the patient's daily life to avoid triggering or exacerbating any hearing loss. Otologists and radiologists must cooperate to ensure that all malformations are correctly described on CT, especially to improve the patient's education regarding hearing preservation.

Hearing status and behavioural patterns among school aged children with cleft lip and/or palate.

OBJECTIVE: There is a dearth of studies on long term hearing status and behavioural patterns among cleft lip and/or palate children after their primary lip and palate closure in Malaysia. This study describes the audiology status and behavioural patterns in a group of school aged children with cleft lip and/or palate. METHOD: A cross sectional study was carried out where caretakers of cleft lip and/or palate were asked to complete the translated Malay language version of Strength Difficulties Questionnaire. The hearing status of the children was analyzed based on recent pure tone audiometric and tympanogram results. The patients' age, gender, type of cleft pathology, age of palatal surgery and behavioural patterns were examined for their potential relationship with hearing status. RESULTS: A total of 74 children (148 ears) aged between 7 and 17 years with cleft lip and/or palate were recruited. The result showed 37 ears (25.0%) had hearing loss with majority suffered from mild conductive hearing loss. There were 16 ears (10.8%) that had persistent middle ear effusion. Hearing improvement occurred when palatal repair was performed at the age of less than 1 year old. (p = 0.015) There was no significant relationship between patients' gender, age, type of cleft and history of myringotomy with their hearing status. In terms of behavioural patterns, 16.3% were abnormal for total behavioural score, 39.2% for peer problem and 17.6% for conduct problem. For prosocial behaviour, 16.3% were rated low and very low. There was fair correlation between age and hyperactivity problems (r = 0.44). Patients' gender, type of cleft pathology, had been teased apart and hearing status was found not related to behavioural problems. CONCLUSION: Cleft lip and/or palate patients have a good longterm hearing outcome. Majority had normal hearing and if there is hearing impairment, it is only a mild loss. Early palatal repair surgery before the age of 1 year can significantly reduce the risk of hearing loss. Cleft lip and/or palate patients experienced peer problems. There was no significant correlation between behavioural difficulty and hearing status among school-aged children with cleft lip and palate.

Renal ultrasound abnormalities in children with syndromic and non-syndromic microtia.

OBJECTIVE: Renal abnormalities are commonly considered in the work up of pediatric patients with external ear malformations. However, there is little consensus regarding an appropriate renal screening protocol for patients with microtia. We sought to characterize renal abnormalities detected on ultrasonography in pediatric patients with microtia. METHODS: We conducted a retrospective cohort study of pediatric patients diagnosed with microtia who underwent renal ultrasound from 1991 to 2014 at a single tertiary academic institution. Renal ultrasound reports and medical records were reviewed to assess for renal abnormalities and to determine whether patients required specialist follow-up or interventions. Audiograms and otolaryngology notes were used to determine patterns of hearing loss. The following additional information was recorded from the electronic medical records: patient sex, microtia grade (I-IV), microtia laterality, and known associated syndromes. Characteristics were compared between those who did and did not have renal ultrasound findings using Fisher's exact test. Univariate logistic regression analysis was performed to determine factors associated with renal ultrasound findings. RESULTS: The majority of patients in this cohort were syndromic (n = 51, 64%) with grade III microtia (n = 46, 58%) and conductive hearing loss (n = 58, 72%). Syndromic children with microtia demonstrated a higher crude rate of renal ultrasound abnormalities (22%) than children with isolated microtia (7%). Of these patients, 69% required specialist follow-up. Univariate logistic regression analysis did not identify predictors that were significantly associated with renal ultrasound findings. CONCLUSION: Fairly high rates of abnormalities in syndromic and non-syndromic patients may warrant screening renal ultrasound in all patients with microtia, especially given the high percentage of findings requiring renal follow-up. A prospective study to formally evaluate screening efficacy is needed.