Imaging in children with hearing loss. / Bildgebende Diagnostik bei Schwerhörigkeit im Kindesalter.

BACKGROUND: Since the introduction of hearing screening in Germany in 2009, pediatric hearing disorders are detected at an early stage. Early therapy is essential for language development. Imaging plays a central role in diagnosis and therapy planning. METHOD: Imaging findings of the most relevant causes of pediatric hearing disorders are presented. Specific attention is given to the method used in each case - CT or MRI. RESULTS AND CONCLUSIONS: While CT is the method of choice for conductive hearing loss, a combination of CT and MRI with high-resolution T2-3D sequences has been established as the best diagnostic method for sensorineural hearing loss. The most common causes of conductive hearing loss in childhood are chronic inflammation and cholesteatoma. Congenital malformations of the outer or middle ear are less frequent. In the case of sensorineural hearing loss, the cause is located in the inner ear and/or the cochlear nerve or the cerebrum. In these cases, congenital malformations are the most common cause. KEY POINTS: · CT and MRI are necessary to identify morphological causes of hearing disorders and to clarify the possibility of hearing-improving ear surgery or cochlear implantation.. · Contraindications for surgical procedures must be excluded.. · Anatomical variants that may be risk factors for surgery must be described.. CITATION FORMAT: · Sorge I, Hirsch F, Fuchs M et al. Imaging diagnostics for childhood hearing loss. Fortschr Röntgenstr 2023; 195: 896 - 904.

The Correlation of Clinical Features and Endolymphatic Hydrops Visualized by 3D-Real IR MRI in Children With Sudden Sensorineural Hearing Loss.

OBJECTIVES: To investigate the correlation between clinical features and endolymphatic hydrops (EH) in children with sudden sensorineural hearing loss (SSNHL). METHODS: We collected 30 SSNHL children aged ≤17 years old, all of whom underwent intravenous gadolinium injection. After 4 hours, inner ear 3-dimensional inversion recovery sequence with real reconstruction (3D-real IR) magnetic resonance imaging was performed. Combined with their medical history such as gender, age, disease course, hearing loss, and so on, the results were analyzed. RESULTS: Different degrees of EH were shown in the vestibule or different turns of cochlea in the affected ears of SSNHL children, and 12 (40%) of 30 children showed positive EH. Age, low and middle frequency hearing loss, and other clinical symptoms such as dizziness and ear fullness have been shown to be related to a certain degree of EH in vestibule or cochlea, whereas no relationship was found between EH and other clinical features such as high-frequency hearing loss, gender, affected side, and tinnitus. CONCLUSIONS: Endolymphatic hydrops may not reflect the trend of disease progression over time in children with SSNHL, but the age of onset may be an important factor in the presence or absence of EH. Endolymphatic hydrops may be one of the causes of dizziness and ear fullness but has no obvious connection with the occurrence of tinnitus. Older SSNHL children with dizziness and ear fullness should maintain long-term follow-up to dynamically monitor the changes in EH.

A retrospective analysis of speech and hearing in patients receiving surgery for submucous cleft palate.

Submucous cleft palate (SMCP) is an uncommon subtype of cleft palate that is associated with symptoms of velopharyngeal insufficiency (VPI), the most common being hypernasal speech. A high proportion of patients also suffer from conductive hearing loss, which is thought to be due to eustachian tube dysfunction. A number of surgical techniques have been proposed to correct the anatomical defect that is responsible for VPI. This exploratory study aims to describe surgical techniques and clinical outcomes in a series of patients who underwent surgical repair of SMCP at a single regional specialist cleft centre between 1999 and 2018. Through a retrospective case note review, records of 57 patients who underwent SMCP repair between the ages of 6 months and 16 years were examined. Patients underwent one of the three surgical techniques: Intravelar Veloplasty, Furlow or a novel technique we have termed as "Hemi-Furlow". Hypernasality, measured on the Great Ormond Street Speech Assessment, showed evidence of improvement post-operatively in all three surgical groups (P<0.005), with no evidence to favour any specific approach. Post-operative complications, including wound dehiscence and fistulae, occurred in nine patients (15.8%). Nine patients (15.8%) required revisional surgery, either due to post-operative complications or persistent speech problems. Otological disease was present in 54.4% of patients, comprising conductive hearing loss due to otitis media with effusion (52.6%) and sensorineural hearing loss (1.8%). There is now a need for large, multi-centre studies with robust outcomes measures to further examine relationships between surgical techniques and clinical outcomes in people born with SMCP.

[Management of microtia in children]. / Prise en charge de la microtie chez l'enfant.

Microtia is a malformation of the external ear with a prevalence of 1-17/10 000 births. It is mostly unilateral and is responsible for conductive hearing loss. It is present in some congenital syndromes but is most frequently isolated. It can be associated with atresia of the external auditory canal (EAC) as well as middle and inner ear malformations. While cosmetic issues seem to be of primary concern to parents at the time of diagnosis, functional audiological rehabilitation is essential for the development of stereophonic hearing in children. We present here a review of the current ENT management of microtia from birth to adulthood.

La microtie est une malformation concernant l'oreille externe et a une prévalence de 1 à 17/10000 naissances. Présente de manière majoritairement unilatérale, elle est responsable d'un déficit auditif de transmission. Elle est présente dans certains syndromes congénitaux, mais est le plus fréquemment isolée. Elle peut être associée à une atrésie du conduit auditif externe (CAE) ainsi que des atteintes de l'oreille moyenne et interne. Si l'atteinte esthétique semble être au premier plan dans la préoccupation des parents lors du diagnostic, la réadaptation fonctionnelle audiologique est importante pour le bon développement d'une audition stéréophonique chez les enfants. Nous présentons ici un rappel de la prise en charge actuelle ORL de la microtie, de la naissance jusqu'à l'âge adulte.

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.

Etiologies of hearing loss in Fanconi Anemia.

OBJECTIVES: We aim to describe types of hearing loss associated with Fanconi anemia patients who underwent a bone marrow transplant (BMT) to identify possible etiologies of hearing loss. Additionally, we hope to investigate hearing loss early in life as a potential predictor of needing a BMT surgery. Fanconi anemia is a rare autosomal recessive disease that is the most common inherited bone marrow failure syndrome, characterized by bone marrow failure and multiple congenital anomalies, including hearing loss. This is the largest study to date reviewing types of hearing loss in patients with Fanconi anemia, specifically in those who have undergone BMTs. METHODS: A retrospective chart review of patients diagnosed with Fanconi anemia at a single institution, tertiary, referral-based children's hospital with a bone marrow transplant team specializing in Fanconi anemia was conducted from 4/19/1976 to 10/19/2015. History, physical examination, audiometry, and imaging findings were reviewed in patients with and without history of bone marrow transplant. Patient hearing levels, as measured by pure tone audiometry at 500 Hz, 1, 2, and 4 kHz, were evaluated. Patients were grouped by transplant status and results and were assessed to determine type and degree of hearing loss. Statistical analysis was performed to compare the likelihood of bone marrow transplant procedures in Fanconi anemia patients with normal and abnormal hearing. RESULTS: There were 252 patients with Fanconi anemia identified via diagnosis search in institutional electronic medical records using CPT codes and cross referencing with the Fanconi Anemia database, 58 of whom had available audiometric data. Of the 58 Fanconi anemia patients with available audiograms, 21 (36%) had abnormal audiograms; 37 patients had normal audiograms. Twenty out of 21 (95%) patients who had abnormal audiograms had undergone bone marrow transplants. Thirty-one of 37 (84%) patients with normal audiograms had received bone marrow transplants. Statistical analysis showed that patients with hearing loss were more likely to require a BMT in the future (OR = 3.87, p = 0.05). Of the patients with abnormal audiograms and a bone marrow transplant (n = 20), 14 (70%) had conductive hearing loss, 5 (25%) had mixed hearing loss, and 1 patient (5%) had sensorineural hearing loss. 13 of 20 patients (65%) had bilateral hearing loss and eight of 20 (40%) had unilateral hearing loss. Of those patients with conductive hearing loss (n = 15), the most common etiologies were Eustachian tube dysfunction (47%), external auditory canal stenosis (33%), and abnormal middle ear anatomy (13%). CONCLUSIONS: Hearing loss is a common finding in Fanconi anemia patients who have undergone BMTs with conductive hearing loss being the most common audiologic manifestation in our cohort of patients. This demonstrates the necessity of frequent hearing screenings in this population and close collaboration with audiology throughout patient care. Our study indicates that hearing status early in life may be a predictor of needing a bone marrow transplant in the future. Further studies should explore the long-term impact of BMT surgery on hearing status.

Fetal Bovine Collagen Grafts for Repair of Tegmen Defects and Encephaloceles Via Middle Cranial Fossa Approach.

To evaluate the use of commercially available allogenic dural graft materials made of fetal bovine collagen, we present an analysis of our case series with use of autologous and allogenic graft materials. Patients who underwent surgical repair of a tegmen tympani defect associated with ipsilateral conductive hearing loss and cerebrospinal fluid (CSF) otorrhea using a middle cranial fossa (MCF) approach from 2004 to 2018 at Loyola University Medical Center were included. Resolution of CSF otorrhea, audiologic outcomes, facial nerve preservation, and surgical complications was analyzed. Thirty-three patients with an average age of 55.3 years (range: 21-78, standard deviation [SD]: 12.9) and body mass index of 34.4 (range: 22-51, SD: 7.4) underwent an MCF repair of a tegmen and dural defect. All patients presented with CSF otorrhea and conductive hearing loss ipsilateral to the defect. Repairs were made with combinations of allograft and autograft in 17 cases, allograft only in 15 cases, and autograft only in 5 cases. Improvement in hearing was noted in 33 cases, and resolution of CSF otorrhea was noted in 36 cases; one patient required repeat surgery which resolved CSF otorrhea. Three patients had minor complications; all these were in the autograft group. The MCF approach coupled with the use of fetal bovine collagen grafts is a safe and viable method to repair tegmen tympani and associated dural defects with salutary outcomes and low morbidity.

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.

The Role of Body Mass Index on Hearing Outcomes After Stapes Surgery.

BACKGROUND: Increased body mass index (BMI) has been associated with postoperative complications in multiple surgical specialties. In otologic surgery involving the stapes, where appropriate patient positioning and surgical dexterity are necessary for proper prosthesis placement, patients with higher BMI could make surgery more challenging. The purpose of this study is to evaluate the impact of BMI on outcomes after stapes surgery. METHODS: Medical records were examined from January 2015 to December 2017 in patients undergoing stapedectomy or stapedotomy at two tertiary care facilities. A retrospective cohort analysis comparing hearing outcomes on postoperative audiogram following procedural intervention with BMI was performed. The primary outcome was postoperative air-bone gap (dB) on audiogram. RESULTS: Two hundred sixty three stapedotomy and stapedectomy patients were included in the study. Six percent had BMI less than 18.5 (underweight), 30% had BMI between 18.6 and 24.9 (normal weight), 38% had BMI between 25 and 29.9 (overweight), 24% had BMI above 30 (obese). The mean preoperative and postoperative air-bone gap (ABG) was 27.0 dB (standard deviation [SD] 15) and 10.4 dB (SD 10.6), respectively, with a postoperative ABG less than or equal to 20 dB in 87% of cases. There were no significant differences in postoperative ABG results when comparing the BMI categories (p = 0.11). CONCLUSIONS: Stapedotomy and stapedectomy are effective surgeries that can be performed by experienced surgeons with successful hearing improvement. While high BMI patients may be a challenge to position appropriately for their surgery, this does not translate to altered otologic outcomes for these patients.

Changes in Hearing Threshold and Tinnitus Severity after Stapes Surgery: Which Is More Important to the Patient's Quality of Life?

BACKGROUND: Stapes surgery is a common method of treatment of otosclerosis, and its effectiveness is reported based on audiometric hearing thresholds. Audiometric tests do not gauge the impact of tinnitus severity and auditory function on quality of life (QOL) after stapes surgery. OBJECTIVE: To measure self-assessed QOL in otosclerosis patients after stapedotomy in terms of three major factors: change in audiometric hearing threshold, subjective hearing benefit, and tinnitus severity. METHOD: This prospective clinical study included 191 patients who underwent stapedotomy between April and October 2017 due to otosclerosis. All patients were tested by pure tone audiometry and filled in a questionnaire before surgery and 6 months afterwards. Subjective hearing was assessed with the Abbreviated Profile for Hearing Aid Benefit (APHAB); tinnitus severity was established using the Tinnitus Functional Index (TFI), and the QOL was measured by the Glasgow Benefit Inventory (GBI). RESULTS: Statistical analysis showed that the average GBI total score (mean = 33.7; SD = 23.7) was statistically significantly higher than zero (t = 19.7; p < 0.001). Based on a regression model, all the three variables studied - audiometric hearing thresholds change, APHAB change, and TFI change - had a significant effect on QOL after stapedotomy. Interestingly, the highest beta value (b = 0.040; p < 0.001) was for TFI change, implying that TFI change had the greatest effect on QOL. CONCLUSION: Although the improvement of QOL after stapes surgery undoubtedly depended on improvement in both audiometric and self-reported hearing, the reduction of tinnitus severity had the greatest impact on increase in QOL.

Impact of unilateral congenital aural atresia on academic Performance: A systematic review.

BACKGROUND: Little is known about the academic performance of children with unilateral congenital aural atresia (CAA). OBJECTIVE: of review: Our objective was to summarize what is known about the academic performance of children with hearing loss by unilateral congenital aural atresia, in order to provide pragmatic recommendations to clinicians who see children with this entity. TYPE OF REVIEW: Systematic review. SEARCH STRATEGY: We conducted a systematic search in PubMed Medline, EMBASE, and Cochrane Library combining the terms "atresia" and synonyms with "unilateral hearing loss" and synonyms. Date of the most recent search was 16 May 2018. EVALUATION METHOD: Two independent authors identified studies, extracted data, and assessed risk of bias. This review was reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Observational studies on the academic achievements of patients of any age with unilateral conductive hearing loss of any level due to congenital aural atresia were included. We considered grade retention, special education, individualized education plans, and parental report of school performance as outcome measures for academic achievement. RESULTS: Two studies reporting on academic performance of patients with unilateral CAA, which both had a significant risk of bias. One study (n = 140) showed a grade retention rate of 3.6% (n = 5) in total. 15.7% (n = 22) needed special education, and 36.4% (n = 51) used an individualized education program. The second study, reporting on 67 patients with unilateral CAA, showed that 29.9% (n = 20) of the patients received school intervention, and 25.4% (n = 17) had learning problems. CONCLUSION: Current evidence regarding the effect of unilateral congenital aural atresia on academic performance is sparse, inconclusive and has a significant risk of bias. High quality observational studies assessing the effects of aural atresia on academic performance in these patients should be initiated.

The Viability of Percutaneous Bone-Anchored Hearing Systems in the HIV Population.

OBJECTIVE: To determine the viability of percutaneous bone-anchored hearing systems (BAHS), in terms of local soft tissue complications, in the HIV population. STUDY DESIGN: Retrospective folder review. SETTING: Two tertiary-level academic state hospitals in Cape Town, South Africa. PATIENTS: Twenty patients, of which six (30%) were HIV-positive. All HIV-positive patients had CD4 counts greater than 200, and were on highly-active antiretroviral therapy (HAART). INTERVENTION: Percutaneous BAHS implantation surgery was performed on all patients. MAIN OUTCOME MEASURE: Comparisons were made between HIV-positive and HIV-negative patients in terms of incidence of local soft tissue complications post-implantation. RESULTS: Soft tissue complications occurred in 50% of patients, and most were easily managed with topical treatment. No significant differences were found when comparing incidence of local soft tissue complications between HIV-positive and HIV-negative patients (p = 0.314). Similarly, surgical technique did not influence soft tissue complication incidence (p = 0.143). CONCLUSIONS: No significant differences in incidence of local soft tissue complications after percutaneous BAHS implantation were found between HIV-positive and HIV-negative patients. In a resource-constrained country like South Africa, where cost and time management are vital, these results indicate that the use of percutaneous BAHS implantation is a viable option, even in HIV-positive patients.

Natural History of Tympanic Membrane Retraction in Children with Cleft Palate.

OBJECTIVES: The natural history of tympanic membrane retraction is unpredictable. To obtain prognostic information for guiding surveillance and treatment, a cohort of children with retraction from cleft palate were prospectively followed for over 5 years. MATERIALS AND METHODS: This was a prospective observational study at a tertiary academic institution. Children with pars tensa retraction were selected from a cohort of 143 children with cleft palate. Thirty-seven ears were assessed with otoendoscopic image capture and audiometry at a median age of 9 years and reassessed at a median follow-up interval of 6.4 years. The severity of tympanic membrane retraction in the serial images of each ear was compared by four pediatric otolaryngologists blinded to the dates of the images. RESULTS: Initially, 19/37 retractions (51%) demonstrated contact with the incus and/or promontory. Follow-up images were rated as stable (n=16) or better (n=12) for 28/37 retractions (76%). Of the nine retractions that became more extensive, two developed cholesteatoma (5% of the total). No ossicular erosion developed in ears without cholesteatoma. Conductive hearing loss (4-tone average air-bone gap >25 decibels hearing level) was initially present in five ears, worsened in one, and normalized without intervention in others. No ears with initial normal hearing developed hearing loss. CONCLUSION: Most tympanic membrane retractions remained stable or improved over time in this cohort of children who were at a risk of persistent eustachian tube dysfunction. Clinically significant progression occurred infrequently, justifying the conservative approach taken to manage these retractions. Such data are necessary to weigh the potential benefit of preventive intervention over observation.

Cartilage rim augmented fascia tympanoplasty: a more effective composite graft model than temporalis fascia tympanoplasty.

OBJECTIVE: To validate a newly introduced cartilage rim augmented temporalis fascia tympanoplasty technique by statistically comparing it with the morphological and audiological outcomes of traditional temporalis fascia tympanoplasty. METHODS: A retrospective comparative study was conducted on 115 patients who underwent tympanoplasty during 2013 and 2015. Fifty-eight patients underwent temporalis fascia tympanoplasty and 57 underwent cartilage rim augmented fascia tympanoplasty. RESULTS: In the cartilage fascia group, graft healing was achieved in 94.7 per cent of cases; in the temporalis fascia group, the graft take-up rate was 70 per cent. In those with a normal ossicular chain, the post-operative air-bone gap was within 20 dB in 92.6 per cent of cartilage fascia group cases and in 69.7 per cent of the temporalis fascia group cases, which was a statistically significant difference. Among the defective ossicular chain cases, the post-operative air-bone gap was within 20 dB in 76.9 per cent in the cartilage fascia group, as against 57.1 per cent in the temporalis fascia group. CONCLUSION: Cartilage rim augmented temporalis fascia tympanoplasty has a definite advantage over the temporalis fascia technique in terms of superior graft take up and statistically significant hearing gain in those with normal ossicular mobility.

Temporary conductive hearing loss in early life impairs spatial memory of rats in adulthood.

INTRODUCTION: It is known that an interruption of acoustic input in early life will result in abnormal development of the auditory system. Here, we further show that this negative impact actually spans beyond the auditory system to the hippocampus, a system critical for spatial memory. METHODS: We induced a temporary conductive hearing loss (TCHL) in P14 rats by perforating the eardrum and allowing it to heal. The Morris water maze and Y-maze tests were deployed to evaluate spatial memory of the rats. Electrophysiological recordings and anatomical analysis were made to evaluate functional and structural changes in the hippocampus following TCHL. RESULTS: The rats with the TCHL had nearly normal hearing at P42, but had a decreased performance with the Morris water maze and Y-maze tests compared with the control group. A functional deficit in the hippocampus of the rats with the TCHL was found as revealed by the depressed long-term potentiation and the reduced NMDA receptor-mediated postsynaptic current. A structural deficit in the hippocampus of those animals was also found as revealed the abnormal expression of the NMDA receptors, the decreased number of dendritic spines, the reduced postsynaptic density and the reduced level of neurogenesis. CONCLUSIONS: Our study demonstrates that even temporary auditory sensory deprivation in early life of rats results in abnormal development of the hippocampus and consequently impairs spatial memory in adulthood.

High-frequency conductive hearing loss as a diagnostic test for incomplete ossicular discontinuity in non-cholesteatomatous chronic suppurative otitis media.

Chronic suppurative otitis media, with or without cholesteatoma, may lead to erosion of the ossicles and discontinuity of the ossicular chain. In incomplete ossicular discontinuity (IOD), partial erosion of the ossicles occurs, but some sound transmission is noted throughout the ossicular chain. High-frequency conductive hearing loss (HfCHL) has been considered a hallmark of incomplete ossicular discontinuity. This study aims to evaluate the use of HfCHL as a preoperative predictor of IOD in patients with non-cholesteatomatous chronic suppurative otitis media. The HfCHL test was defined as the preoperative air-bone gap (ABG) at 4 kHz minus the average of the ABG at 0.25 and 0.5 kHz. The test was applied in 328 patients before surgery and compared to intraoperative findings as the gold standard. At surgery, 201 (61.3%) patients had an intact ossicular chain, 44 (13.4%) had a complete ossicular discontinuity, and 83 (25.3%) exhibited an IOD. The best cutoff level was calculated as 10 dB. The HfCHL test to diagnose IOD had a sensitivity of 83% and a specificity of 92% with a post-test probability of 78% and a likelihood ratio of 10.2. We concluded that the HfCHL test is highly effective in predicting IOD in patients with non-cholesteatomatous chronic suppurative otitis media and that it should be used routinely as a screening test prior to surgery.

Prevalence of hearing impairment in patients with rheumatoid arthritis, granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), or systemic lupus erythematosus.

Hearing loss in patients with autoimmune diseases, such as systemic lupus erythematosus (SLE), granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), or rheumatoid arthritis (RA), is controversial. Many studies lack measurements of bone-conduction thresholds to sufficiently differentiate between sensorineural hearing loss and conductive hearing loss. In addition, many studies lack control groups or comparisons to an age-related normal hearing threshold. This study investigates hearing performance with an extended audiological battery using psychoacoustic and objective measures. A total of 22 adults with RA, 16 with GPA, 20 with SLE, and two age- and gender-matched control groups (n = 34 for GPA and RA and n = 42 for SLE) were included. Pure-tone hearing thresholds, speech perception in quiet and noise, tympanometry, and high-resolution otoacoustic emissions were assessed. GPA patients exhibited impaired pure-tone hearing compared to the control group, whereas SLE and RA patients did not. In GPA patients, a larger air-bone gap indicated conductive hearing loss. In addition, speech perception was reduced exclusively in GPA patients. A significant correlation was found between hearing loss and both the cumulative steroid dose and number of organ manifestations in GPA and SLE patients. Our data indicate that GPA and SLE patients are at moderate-to-high risk of conductive hearing loss. In contrast, RA patients are at low risk of disease-associated hearing loss.

Prevalence and Nature of Hearing Loss in 22q11.2 Deletion Syndrome.

PURPOSE: The purpose of this study was to clarify the prevalence, type, severity, and age-dependency of hearing loss in 22q11.2 deletion syndrome. METHOD: Extensive audiological measurements were conducted in 40 persons with proven 22q11.2 deletion (aged 6-36 years). Besides air and bone conduction thresholds in the frequency range between 0.125 and 8.000 kHz, high-frequency thresholds up to 16.000 kHz were determined and tympanometry, acoustic reflex (AR) measurement, and distortion product otoacoustic emission (DPOAE) testing were performed. RESULTS: Hearing loss was identified in 59% of the tested ears and was mainly conductive in nature. In addition, a high-frequency sensorineural hearing loss with down-sloping curve was found in the majority of patients. Aberrant tympanometric results were recorded in 39% of the ears. In 85% of ears with a Type A or C tympanometric peak, ARs were absent. A DPOAE response in at least 6 frequencies was present in only 23% of the ears with a hearing threshold ≤30 dB HL. In patients above 14 years of age, there was a significantly lower percentage of measurable DPOAEs. CONCLUSION: Hearing loss in 22q11.2 deletion syndrome is highly prevalent and both conductive and high-frequency sensorineural in nature. The age-dependent absence of DPOAEs in 22q11.2 deletion syndrome suggests cochlear damage underlying the high-frequency hearing loss.

The management of children with Down syndrome and profound hearing loss.

INTRODUCTION: Although, the association between Down syndrome (DS) and conductive hearing loss is well recognized, the fact that a small proportion of these children may have a severe to profound sensorineural hearing loss that could benefit from cochlear implantation (CI) is less well understood. The management of significant co-morbidities in children with DS can delay initial diagnosis of hearing impairment and assessment of suitability for CI can likewise be challenging, due to difficulties conditioning to behavioural hearing tests. METHODS: We performed a retrospective case note review of three children with DS referred to the Manchester Cochlear Implant Programme. RESULTS: Three illustrative cases are described including CI in a 4 years old. Using conventional outcome measurement instruments, the outcome could be considered to be suboptimal with a Categories of Auditory Performance score of 4 at 6 months post-op and at last follow up. In part, this is likely to reflect the delay in implantation, but the role of cognitive impairment must be considered. The cases described emphasize the importance of comprehensive radiological and audiological assessment in children with DS being considered for CI. CONCLUSION: The influence of cognitive impairment upon outcome of CI must be taken into account, but should not be considered a contra-indication to implantation in children with DS. Benefit that might be considered limited when quantified using existing general outcome measurement instruments, may have a significant impact upon psychosocial development and quality of life in children with significant cognitive impairment, or other additional needs.

Acoustic Reflex Screening of Conductive Hearing Loss for Third Window Disorders.

OBJECTIVE: This study examines the effectiveness of acoustic reflexes in screening for third window disorders (eg, superior semicircular canal dehiscence) prior to middle ear exploration for conductive hearing loss. STUDY DESIGN: Case series with chart review. SETTING: Outpatient tertiary otology center. SUBJECTS AND METHODS: A review was performed of 212 ears with acoustic reflexes, performed as part of the evaluation of conductive hearing loss in patients without evidence of chronic otitis media. The etiology of hearing loss was determined from intraoperative findings and computed tomography imaging. The relationship between acoustic reflexes and conductive hearing loss etiology was assessed. RESULTS: Eighty-eight percent of ears (166 of 189) demonstrating absence of all acoustic reflexes had an ossicular etiology of conductive hearing loss. Fifty-two percent of ears (12 of 23) with at least 1 detectable acoustic reflex had a nonossicular etiology. The positive and negative predictive values for an ossicular etiology were 89% and 57% when acoustic reflexes were used alone for screening, 89% and 39% when third window symptoms were used alone, and 94% and 71% when reflexes and symptoms were used together, respectively. CONCLUSION: Acoustic reflex testing is an effective means of screening for third window disorders in patients with a conductive hearing loss. Questioning for third window symptoms should complement screening. The detection of even 1 acoustic reflex or third window symptom (regardless of reflex status) should prompt further workup prior to middle ear exploration.