Occurrence of mucosa-affecting diseases of the upper airways in middle ear cholesteatoma patients: a nationwide case-control study.

PURPOSE: Exploring a possible link between upper airway inflammation and the development of cholesteatoma by studying the association between mucosa-affecting diseases of the upper airways and cholesteatoma surgery. METHODS: This is a nationwide case-control study of 10,618 patients who underwent surgery for cholesteatoma in Sweden between 1987 and 2018. The cases were identified in the National Patient Register and 21,235 controls matched by age, sex and place of residency were included from national population registers. Odds ratios (OR) and corresponding 95% confidence intervals were used to assess the association between six types of mucosa-affecting diseases of the upper airways and cholesteatoma surgery. RESULTS: Chronic rhinitis, chronic sinusitis and nasal polyposis were more common in cholesteatoma patients than in controls (OR 1.5 to 2.5) as were both adenoid and tonsil surgery (OR > 4) where the strongest association was seen for adenoid surgery. No association was seen between allergic rhinitis and cholesteatoma. CONCLUSION: This study supports an association between mucosa-affecting diseases of the upper airways and cholesteatoma. Future studies should aim to investigate the mechanisms connecting mucosa-affecting diseases of the upper airways and cholesteatoma formation regarding genetic, anatomical, inflammatory and mucosa properties.

Asymmetry in Cortical Thickness of the Heschl's Gyrus in Unilateral Ear Canal Atresia.

HYPOTHESIS: Unilateral congenital conductive hearing impairment in ear canal atresia leads to atrophy of the gray matter of the contralateral primary auditory cortex or changes in asymmetry pattern if left untreated in childhood. BACKGROUND: Unilateral ear canal atresia with associated severe conductive hearing loss results in deteriorated sound localization and difficulties in understanding of speech in a noisy environment. Cortical atrophy in the Heschl's gyrus has been reported in acquired sensorineural hearing loss but has not been studied in unilateral conductive hearing loss. METHODS: We obtained T1w and T2w FLAIR MRI data from 17 subjects with unilateral congenital ear canal atresia and 17 matched controls. Gray matter volume and thickness were measured in the Heschl's gyrus using Freesurfer. RESULTS: In unilateral congenital ear canal atresia, Heschl's gyrus exhibited cortical thickness asymmetry (right thicker than left, corrected p = 0.0012, mean difference 0.25 mm), while controls had symmetric findings. Gray matter volume and total thickness did not differ from controls with normal hearing. CONCLUSION: We observed cortical thickness asymmetry in congenital unilateral ear canal atresia but no evidence of contralateral cortex atrophy. Further research is needed to understand the implications of this asymmetry on central auditory processing deficits.

A pilot study on spatial hearing in children with congenital unilateral aural atresia.

Despite normal hearing in one ear, individuals with congenital unilateral aural atresia may perceive difficulties in everyday listening conditions typically containing multiple sound sources. While previous work shows that intervention with bone conduction devices may aid spatial hearing for some children, testing conditions are often arranged to maximize any benefit and are not very similar to daily life. The benefit from amplification on spatial tasks has been found to vary between individuals, for reasons not entirely clear. This study has sought to expand on the limited knowledge on how children with unilateral aural atresia recognize speech masked by competing speech, and how horizontal sound localization accuracy is affected by the degree of unilateral hearing loss and by amplification using unilateral bone conduction devices when fitted before 3 years of age. In a within-subject, repeated measures design, including 11 children (mean age = 7.9 years), bone conduction hearing device (BCD) amplification did not negatively affect horizontal sound localization accuracy. The effect on speech recognition scores showed greater inter-individual variability. No benefit from amplification on a group level was found. There was no association between age at fitting and the benefit of the BCD. For children with poor unaided sound localization accuracy, there was a greater BCD benefit. Unaided localization accuracy increased as a function of decreasing hearing thresholds in the atretic ear. While it is possible that low sound levels in the atretic ear provided access to interaural localization cues for the children with the lowest hearing thresholds, the association has to be further investigated in a larger sample of children.

The Risk of Cholesteatoma in Individuals With First-degree Relatives Surgically Treated for the Disease.

Importance: Cholesteatoma in the middle ear is not regarded as a hereditary disease, but case reports of familial clustering exist in the literature, as well as observed familial cases in the clinical work. However, the knowledge regarding cholesteatoma as a hereditary disease is lacking in the literature. Objective: To assess the risk of cholesteatoma in individuals with a first-degree relative surgically treated for the same disease. Design, Setting, and Participants: In this nested case-control study in the Swedish population between 1987 and 2018 of first-time cholesteatoma surgery identified from the Swedish National Patient Register, 2 controls per case were randomly selected from the population register through incidence density sampling, and all first-degree relatives for cases and controls were identified. Data were received in April 2022, and analyses were conducted between April and September 2022. Exposure: Cholesteatoma surgery in a first-degree relative. Main Outcomes and Measures: The main outcome was first-time cholesteatoma surgery. The association between having a first-degree relative with cholesteatoma and the risk of cholesteatoma surgery in the index persons was estimated by odds ratios (ORs) and 95% CIs through conditional logistic regression analysis. Results: Between 1987 and 2018, 10 618 individuals with a first-time cholesteatoma surgery (mean [SD] age at surgery, 35.6 [21.5] years; 6302 [59.4%] men) were identified in the Swedish National Patient Register. The risk of having a cholesteatoma surgery was almost 4 times higher in individuals having a first-degree relative surgically treated for the disease (OR, 3.9; 95% CI, 3.1-4.8), but few cases were exposed overall. Among the 10 105 cases with at least 1 control included in the main analysis, 227 (2.2%) had at least 1 first-degree relative treated for cholesteatoma, while the corresponding numbers for controls were 118 of 19 553 control patients (0.6%). The association was stronger for individuals under the age of 20 years at first surgery (OR, 5.2; 95% CI, 3.6-7.6) and for a surgery involving the atticus and/or mastoid region (OR, 4.8; 95% CI, 3.4-6.2). There was no difference in the prevalence of having a partner with cholesteatoma between cases and controls (10 cases [0.3%] and 16 controls [0.3%]; OR, 0.92; 95% CI, 0.41-2.05), which implies that increased awareness does not explain the association. Conclusions and Relevance: In this Swedish case-control study using nationwide register data with high coverage and completeness, the findings suggest that the risk of cholesteatoma in the middle ear is strongly associated with a family history of the condition. Family history was nevertheless quite rare and can therefore only explain a limited number of all cases; these families could be an important source for information regarding the genetic background for cholesteatoma disease.

Automatic segmentation of the core of the acoustic radiation in humans.

Introduction: Acoustic radiation is one of the most important white matter fiber bundles of the human auditory system. However, segmenting the acoustic radiation is challenging due to its small size and proximity to several larger fiber bundles. TractSeg is a method that uses a neural network to segment some of the major fiber bundles in the brain. This study aims to train TractSeg to segment the core of acoustic radiation. Methods: We propose a methodology to automatically extract the acoustic radiation from human connectome data, which is both of high quality and high resolution. The segmentation masks generated by TractSeg of nearby fiber bundles are used to steer the generation of valid streamlines through tractography. Only streamlines connecting the Heschl's gyrus and the medial geniculate nucleus were considered. These streamlines are then used to create masks of the core of the acoustic radiation that is used to train the neural network of TractSeg. The trained network is used to automatically segment the acoustic radiation from unseen images. Results: The trained neural network successfully extracted anatomically plausible masks of the core of the acoustic radiation in human connectome data. We also applied the method to a dataset of 17 patients with unilateral congenital ear canal atresia and 17 age- and gender-paired controls acquired in a clinical setting. The method was able to extract 53/68 acoustic radiation in the dataset acquired with clinical settings. In 14/68 cases, the method generated fragments of the acoustic radiation and completely failed in a single case. The performance of the method on patients and controls was similar. Discussion: In most cases, it is possible to segment the core of the acoustic radiations even in images acquired with clinical settings in a few seconds using a pre-trained neural network.

Adults with unilateral congenital ear canal atresia - sound localization ability and recognition of speech in competing speech in unaided condition.

BACKGROUND: Individuals with unilateral hearing loss show poor spatial hearing, but individual variability is high. AIMS/OBJECTIVES: To investigate if the degree of hearing loss in unilateral ear canal atresia affects horizontal sound localization and speech recognition. MATERIALS AND METHODS: Twelve subjects with unilateral ear canal atresia without childhood hearing intervention. Previously published data from eight normal-hearing subjects in normal binaural as well as experimentally induced unilateral hearing loss served as a reference. Horizontal sound localization and recognition of speech in spatially separate competing speech were assessed. RESULTS: Linear regression analysis demonstrated a relationship between sound localization accuracy (SLA) and the air conduction pure tone average of the atretic ear (r = 0.85, p=.007). The large proportion of variability in SLA (72%) explained by the degree of hearing loss of the atretic ear indicates that binaural processing is possible. SLA was worse than for normal hearing individuals (p<.0001), and comparable to moderate simulated unilateral hearing loss (p=.13). Speech discrimination was significantly worse than normal (p<.0001) and not dependent on degree of hearing loss of the atretic ear. CONCLUSIONS AND SIGNIFICANCE: Individuals with congenital unilateral ear canal atresia show impaired horizontal SLA and recognition of speech in competing speech.

Improving Clinical Outcomes in Cochlear Implantation Using Glucocorticoid Therapy: A Review.

Cochlear implant surgery is a successful procedure for auditory rehabilitation of patients with severe to profound hearing loss. However, cochlear implantation may lead to damage to the inner ear, which decreases residual hearing and alters vestibular function. It is now of increasing interest to preserve residual hearing during this surgery because this is related to better speech, music perception, and hearing in complex listening environments. Thus, different efforts have been tried to reduce cochlear implantation-related injury, including periprocedural glucocorticoids because of their anti-inflammatory properties. Different routes of administration have been tried to deliver glucocorticoids. However, several drawbacks still remain, including their systemic side effects, unknown pharmacokinetic profiles, and complex delivery methods. In the present review, we discuss the role of periprocedural glucocorticoid therapy to decrease cochlear implantation-related injury, thus preserving inner ear function after surgery. Moreover, we highlight the pharmacokinetic evidence and clinical outcomes which would sustain further interventions.

Local treatment of the inner ear: a study of three different polymers aimed for middle ear administration.

CONCLUSION: A formulation based on sodium hyaluronate (NaHYA) was the most promising candidate vehicle for intra-tympanic drug administration regarding conductive hearing loss, inflammatory reactions, and elimination. OBJECTIVES: Recent advances in inner ear research support the idea of using the middle ear cavity for drug administration to target the inner ear. This paper presents rheological and safety assessments of three candidate polymer formulations for intra-tympanic drug administration. METHOD: The formulations were based on sodium carboxymethyl cellulose (NaCMC), sodium hyaluronate (NaHYA), and poloxamer 407 (POL). Rheological studies were performed with a controlled rate instrument of the couette type. Safety studies were performed in guinea pigs subjected to an intra-tympanic injection of the formulations. Hearing function was explored with ABR before and 1, 2, and 3 weeks after the injection. Elimination of the formulations marked with coal was explored with an endoscopic digital camera 1, 2, and 3 weeks after injection. Middle and inner ear morphology was examined with light microscopy 6 days after injection. RESULTS: The results speak in favor of NaHYA, since it did not cause prolonged hearing threshold elevations. The results of the elimination and morphological investigations support the conclusion of NaHYA being the most promising candidate for intra-tympanic administration.