Predictors of Hearing Functional Outcome following Surgery for Cerebellopontine Angle Meningioma.

OBJECTIVE Cerebellopontine angle (CPA) meningioma can affect hearing function and require expeditious treatment to prevent permanent hearing loss. The authors sought to determine the factors associated with hearing functional outcome in CPA meningioma patients treated with surgery and/or radiation therapy either stereotactic radiosurgery or stereotactic radiation therapy. METHODS Consecutive patients with CPA meningioma who had presented at our hospital from 2008 to 2018 were identified through retrospective chart review. Hearing function-pure tone audiogram (PTA) and speech discrimination score (SDS)-was assessed before and after surgery for CPA meningioma. Hearing function with PTA>50 dB and SDS<69% were defined as poor hearing functional outcome. Multivariable Cox Proportional Hazards Regression model was used to assess the associations between pre-operative hearing functional assessment and post-operative hearing functional outcomes. RESULTS The study cohort included 31 patients (80.6% females, with a mean age of 61.3±15.2 years) with a median clinical follow-up of 5 months (range, 1 week-98 months). The mean pre-operative PTA and SDS were 23.8±11.2 dB and 64.4±22.2% respectively. At the last visit, there was a significant hearing recovery, with an improvement of 29.7±18.0 dB (p<0.001) and 87.6±17.8% (p<0.001) in PTA and SDS respectively. Multivariable cox proportional hazards regression model was conducted after adjusting for age, gender, tumor volume, location, and classification of the tumor, which revealed that patients undergoing surgery through retro sigmoid approach [Hazards Ratio (HR): 32.1, 95% Confidence Interval (CI): 2.11-491.0, p=0.01] and gross total resection (GTR) (HR: 2.99, 95%CI: 1.09-9.32, p=0.05) had significantly higher risk of poor hearing functional outcome. Moreover, patients with poor preoperative hearing had 85% higher chances of poor hearing functional outcome post operatively (HR: 0.15, 95%CI: 0.03-0.59, p=0.007). CONCLUSION Postoperative improvement in hearing is a reasonable expectation following surgery for CPA meningioma. Preoperative hearing, surgical approach and extent of surgical resection are predictive of postoperative hearing function outcome and can identify patients at higher risk of hearing loss.

Investigation of the Interaction between Hearing Function and Comorbidities in Adults Living with Human Immunodeficiency Virus

Adults living with the human immunodeficiency virus (HIV) have a high prevalence of co-existing comorbidities. While research indicates that adults living with HIV are at risk of developing hearing impairment, limited research exists on the interaction between hearing function and comorbidities in this population. The objective of this study was to determine and compare the hearing function of a group of adults living with HIV and comorbidities and those without comorbidities. A sample of 132 adults living with HIV underwent a basic audiological test battery to assess their hearing function. Participants with comorbidities were 1.23 times more likely to develop hearing loss, with crude odds of 1.236 (95%CI 0.5467 to 2.795), while those with three comorbidities were 2.52 times more likely to develop hearing loss. Participants with hypertension were 93% more likely to develop hearing loss when compared to nonhypertensive participants (OR = 1.928; 95%CI: 0.7856 to 4.7345). There was only a marginal association between hypercholesterolemia and sensorineural hearing loss (SNHL), with no association between other comorbidities and the type of hearing loss. The current findings raise a need for prioritizing patients with comorbidities in audiological assessment and monitoring in resource-constrained contexts, where capacity versus demand challenges might prevent the provision of audiological services to all adults living with HIV. These findings also highlight the importance of preventive care in this population with regard to the burden of the disease, as it may lead to worse ear and hearing outcomes for affected individuals.

Lineage-tracing and translatomic analysis of damage-inducible mitotic cochlear progenitors identifies candidate genes regulating regeneration

Cochlear supporting cells (SCs) are glia-like cells critical for hearing function. In the neonatal cochlea, the greater epithelial ridge (GER) is a mitotically quiescent and transient organ, which has been shown to nonmitotically regenerate SCs. Here, we ablated Lgr5+ SCs using Lgr5-DTR mice and found mitotic regeneration of SCs by GER cells in vivo. With lineage tracing, we show that the GER houses progenitor cells that robustly divide and migrate into the organ of Corti to replenish ablated SCs. Regenerated SCs display coordinated calcium transients, markers of the SC subtype inner phalangeal cells, and survive in the mature cochlea. Via RiboTag, RNA-sequencing, and gene clustering algorithms, we reveal 11 distinct gene clusters comprising markers of the quiescent and damaged GER, and damage-responsive genes driving cell migration and mitotic regeneration. Together, our study characterizes GER cells as mitotic progenitors with regenerative potential and unveils their quiescent and damaged translatomes.

Efficacy of the Suboccipital Paracondylar-Lateral Cervical Approach: The Series of 64 Jugular Foramen Tumors Along With Follow-Up Data

ObjectiveComplete resection of jugular foramen tumors with minimal cranial nerve complications remains challenging even for skilled neurosurgeons. Here, we introduce a modified paracondylar approach, named the suboccipital paracondylar-lateral cervical (SPCLC) approach for this purpose. We also share the follow-up data of our series and discuss the advantages and limitations of this modified paracondylar approach.MethodsWe included 64 patients with jugular foramen tumors who underwent surgery by the same senior neurosurgeon between November 2011 and August 2020. All patients were treated with the SPCLC approach, which aimed for gross total tumor removal in a single-stage operation. The clinical characteristics, including preoperative and postoperative neurological status, the extent of surgical resection, and follow-up data were retrospectively acquired and evaluated.ResultsThere were 48 schwannomas, nine meningiomas, three paragangliomas, one hemangiopericytoma, one chordoma, one endolymphatic sac tumor, and one Langerhans’ cell histiocytosis. The median age of our patients was 43 years (range: 21–77 years). Dysphagia, hoarseness, and tongue deviation were observed in 36, 26, and 28 patients, respectively. Thirty-two patients had hearing function impairments, including hearing loss or tinnitus. Gross total resection was achieved in 59 patients (59/64, 92.2%). Gamma Knife treatment was used to manage residual tumors in five patients. Postoperatively, new-onset or aggravative dysphagia and hoarseness occurred in 26 and 18 cases, respectively. Nine patients developed new-onset facial palsy, and one patient developed new-onset hearing loss. There were no cases of intracranial hematoma, re-operation, tracheostomy, or death. At the latest follow-up, hearing loss and tinnitus had improved in 20 cases (20/32, 62.5%), dysphagia alleviated in 20 cases (20/36, 55.6%), and hoarseness improved in 14 cases (14/26, 53.8%). Over a mean follow-up period of 27.8 ± 19.5 months (range: 3–68 months), tumor recurrence was observed in one patient.ConclusionThe SPCLC approach, modified from the paracondylar approach, and was less invasive, safe, and efficient for certain jugular foramen tumors. Taking advantage of the anatomic understanding, clear operational vision, and appropriate surgical skills, it is possible to achieve gross total tumor removal and the preservation of neurological function.

Management of Panfacial Trauma: Sequencing and Pitfalls

Panfacial trauma refers to high-energy mechanism injuries involving two or more areas of the craniofacial skeleton, the frontal bone, the midface, and the occlusal unit. These can be distracting injuries in an unstable patient and, as in any trauma, Advanced Trauma Life Support (ATLS) protocols should be followed. The airway should be secured, bleeding controlled, and sequential examinations should take place to avoid overlooking injuries. When indicated, neurosurgery and ophthalmology should be consulted as preservation of brain, vision, and hearing function should be prioritized. Once the patient is stabilized, reconstruction aims to reduce panfacial fractures, restore the horizontal and vertical facial buttresses, and resuspend the soft tissue to avoid the appearance of premature aging. Lost or comminuted bone can be replaced with bone grafts, although adequate reduction should be ensured prior to any grafting. Operative sequencing can be performed from top-down and outside-in or from bottom-up and inside-out depending on patient presentation. All protocols can successfully manage panfacial injuries, and the emphasis should be placed on a systematic approach that works from known areas to unknown areas.

On the hearing effects of a cholesteatoma growing: A biomechanical study

Chronic otitis media enables the appearance of a benign middle ear tumor, known as a cholesteatoma, that may compromise hearing. To evaluate the influence of a cholesteatoma growth on the hearing function, a computational middle ear model based on the finite element method was used and three different size of cholesteatoma were modeled. The cholesteatoma solidification and the consequent degradation of the ossicles were also simulated as two condition that commonly occurs during cholesteatoma evolution. A sound pressure level of 80 dB SPL was applied in the tympanic membrane and a steady state analysis was performed for frequencies from 100 Hz to 10 kHz. The displacements of both the tympanic membrane and the stapes footplate were measured. The results were compared with a healthy case and it was shown that the cholesteatoma development leads to a decrease in the umbo and stapes displacements. The ossicles degradation simulation showed the higher difference comparing with the cholesteatoma in an initial stage, with lower displacements in the stapes footplate mainly for high frequencies. The observed displacement differences are directly connected to hearing loss, being possible to conclude that cholesteatoma evolution in the middle ear will lead to hearing problems, mainly in an advanced stage.

Potentially predictive factors for hearing function improvement in pediatric cytomegalovirus infection therapy

Background Symptomatic congenital cytomegalovirus (CMV) infection has an impact mainly on neurological sequelae, including sensorineural deafness. Because of the long-term impact, early treatment of CMV infection is mandatory. However, predictive factors for hearing function improvement in CMV infection therapy remain unexamined. Objective To evaluate potential predictive factors for hearing improvement in pediatric CMV infection therapy. Methods All medical record data of patients aged 0-6 years with CMV infection who completed a 6-week course of ganciclovir therapy or a combination of a 4-week course of ganciclovir and a 2-week course valganciclovir from January 2013 to December 2017 were collected. Age at onset of therapy, gender, gestational age, nutritional status, multi-organ involvement, and neurological symptoms were studied as potential predictive factors of hearing improvement in CMV therapy. The effectiveness of CMV infection therapy on improving hearing function was measured with the brainstem evoked response audiometry (BERA) test. Results BERA tests proportion in the right, left, and best ear showed significant improvement after therapy. All variables analyzed were not statistically significant as predictive factors for hearing improvement in CMV infection therapy. Conclusion Ganciclovir/valganciclovir therapy in CMV infection patients accounted for the improvement of hearing impairment. However, none of the assessed factors were considered predictive for improving hearing function in CMV infection therapy.

Genetic Determinants of Non-Syndromic Enlarged Vestibular Aqueduct: A Review

Hearing loss is the most common sensorial deficit in humans and one of the most common birth defects. In developed countries, at least 60% of cases of hearing loss are of genetic origin and may arise from pathogenic sequence alterations in one of more than 300 genes known to be involved in the hearing function. Hearing loss of genetic origin is frequently associated with inner ear malformations; of these, the most commonly detected is the enlarged vestibular aqueduct (EVA). EVA may be associated to other cochleovestibular malformations, such as cochlear incomplete partitions, and can be found in syndromic as well as non-syndromic forms of hearing loss. Genes that have been linked to non-syndromic EVA are SLC26A4, GJB2, FOXI1, KCNJ10, and POU3F4. SLC26A4 and FOXI1 are also involved in determining syndromic forms of hearing loss with EVA, which are Pendred syndrome and distal renal tubular acidosis with deafness, respectively. In Caucasian cohorts, approximately 50% of cases of non-syndromic EVA are linked to SLC26A4 and a large fraction of patients remain undiagnosed, thus providing a strong imperative to further explore the etiology of this condition.

OTOACOUSTIC EMISSION REGISTRATION CHARACTERISTICS IN CHILDREN OF THE FIRST 3 MONTHS OF LIFE AFTER ACUTE OTITIS MEDIA

Data on the frequency and nature of hearing impairment in newborns and children during the first months of life after acute otitis media are contradictory. Otoacoustic emission is one of the methods for assessing the hearing function in infants. The main advantages of this method are objectivity, non-invasiveness, speed and accuracy. Objectives. To evaluate the features of otoacoustic emission registration at the frequency of the product distortion in children of the first 3 months of life after an acute inflammation of the middle ear. Material and methods. Distortion-product otoacoustic emission was performed in 36 children after complete recovery from acute otitis media (main group) and in 32 children without otitis media (comparison group). The estimation of the amplitude and spectrum of the response received was performed. The analysis of the otoacoustic response at frequencies of 1 kHz, 1.5 kHz, 2 kHz, 3 kHz, 4 kHz, 5 kHz, 6 kHz, 8 kHz was carried out. Results. Otoacoustic emission was registered in 74.2% of investigations in the comparison group, in 56.0% of observations in otoscopically healthy ears in case of unilateral inflammation, in 35.1% of cases after a non-suppurative form of the disease, and only in 15.4% of cases after a purulent form of acute otitis media. Statistically significant differences were revealed in the frequency of registration of otoacoustic emission in children of the examined groups. The greatest amplitude of the signal/noise relationship was recorded at frequencies of 5 and 6 kHz, and the smallest one at the frequency of 1 kHz in all studied groups. Conclusions. A decrease in auditory function is observed in children of the first 3 months of life after past acute otitis media. This is confirmed by significantly higher rate of absence of otoacoustic emission in this group of patients. Distortion-product otoacoustic emission in children of the first 3 months of life is recommended to be carried out at frequencies of 1.5 kHz and higher.