The journal of international advanced otology最新文献

Bilateral congenital cholesteatoma (BCC) is rare among congenital cholesteatoma (CC) cases, and bilateral surgery poses a significant psychophysical burden on pediatric patients. Here, we present 2 pediatric cases of BCC that were successfully managed using simultaneous bilateral transcanal endoscopic ear surgery (TEES). The surgical approach provided clear visualization of the middle ear structures, enabling successful cholesteatoma removal with minimal invasiveness and a short operative time. In both cases, follow-up computed tomography showed no recurrence, and pure-tone audiometry results demonstrated good outcomes. Simultaneous bilateral TEES can be a viable option for pediatric BCC, as it reduces patient burden due to its less invasive procedure and short operative time.

Background: Intracochlear schwannomas (ICSs) are a subtype of intralabyrinthine schwannomas, completely located in the cochlear lumen. ICSs are particularly rare in the pediatric population. Putative diagnosis is made on the basis of magnetic resonance findings with signal characteristics that should remain the same at follow-up imaging.

Methods: A retrospective review was performed searching for pediatric patients affected by ICS treated at the Otolaryngology Department, Ospedale Ramazzini, Carpi (Italy), and Otolaryngology and Otoneurosurgery Department, Azienda Ospedaliero-Universitaria di Parma, (Italy). A scoping literature review of the period January 2000 - June 2024 was performed.

Results: Two cases of ICS in pediatric patients are described. Neither family history nor genetic signs of neurofibromatosis type II were found. A single report was identified in the literature review. Data analysis resumes the pooled data of the latter case and the authors' patients. The most common symptom at presentation was progressive sensorineural hearing loss (66%). Mean pure tone average at diagnosis was 74.2 dB. Intracochlear location was in the basal turn in 2 cases and in the apical and middle turns in the third patient. All cases initially underwent a "wait and scan" strategy. The mean follow-up time was 23.3 months.

Conclusion: Management planning of pediatric ICSs should be accurate as surgical removal may require partial or total cochlear demolition, resulting in vestibular dysfunction and precluding future positioning of a cochlear implant. Close clinical and radiological follow-up with serial MRI scans allows to evaluate both symptom progression and rate of growth, in order to provide patients with the best therapeutic option.

Background: Glycemic control and the efficacy of therapy in diabetic patients with type 2 diabetes during the previous 2-3 months are usually evaluated by measuring the glycated hemoglobin (HbA1c). Our aim is to study the correlation between serum glycated hemoglobin level (HbA1c) and the hearing thresholds in diabetic patients.

Methods: A case-control study was conducted in the Audio-Vestibular Medicine Unit, xxxx University on 82 subjects. The subjects were divided into 2 groups: the first group consisted of 42 diabetic patients and the second group consisted of 40 healthy subjects. All the participants underwent a pure tone audiogram and speech audiometric evaluation. All participants also underwent diabetes laboratory assessments, including fasting blood glucose serum level and serum HbA1c level. The average hearing threshold at frequencies from 250 Hz to 16 000 Hz in both groups was calculated and correlated to different variables.

Results: Diabetic patients showed higher hearing thresholds than those of the control group, with an increasing tendency of elevation of the hearing threshold levels toward the higher frequencies in both groups. There was no statistically significant difference in the hearing thresholds between patients with diabetes < 5 years (20 subjects) and those with a duration of ≤ 5 years (22 subjects). Also, there was no statistically significant difference in the average hearing thresholds among type 2 diabetic patients based on fasting blood sugar level results, except at 16 000 Hz.

Conclusion: Poor glycemic control status [Hb A1c ≥ 7%] is significantly associated with elevated hearing thresholds.

Background: When a universal newborn hearing screening program is not feasible, particularly in the early stages of its establishment when it requires a great deal of effort and resources, a smaller scale in screening, such as a specific geographical subset or targeted group, is suggested rather than doing nothing. This study aims to pilot a newborn hearing screening program at a hospital in a low- to middle-income country and determine its effectiveness and costs in the context of a lack of qualified audiologists.

Methods: All high-risk births at Hatyai Hospital were recruited for newborn hearing screening between January and December 2021. Newborns who failed 2 stages of transient evoked otoacoustic emissions screening were referred for diagnostic assessment and subsequent interventions. The program's effectiveness was assessed based on the standards of the American Academy of Pediatrics. All costs related to screening, diagnostic, and intervention stages were also evaluated.

Results: Out of 883 newborns, 792 newborns were screened, resulting in a 95.1% screening coverage. The referral rate regarding the diagnostic stage was 3.9%. Also, 28.3% and 12.9% lost-to-follow-up rates were observed in the secondscreening and diagnostic assessment stages, respectively. Ten children were confirmed as having permanent hearing loss, with a prevalence of 1.3%. The total cost was US$13 611, and the cost (for the screening stage) per case screened was US$4.

Conclusion: The program was considered effective with 2 out of 3 benchmarks achieved.

Background: Vestibular schwannomas (VS) are benign tumors arising from the eighth cranial nerve. They often cause no symptoms for a long period of time. Due to the improved availability and quality of magnetic resonance imaging diagnostics, even small tumors can be diagnosed at an early stage. The fact that the diagnosis of a VS might negatively affect a patient's quality of life (QoL) should be taken into consideration when informing patients about various treatment options. However, little is known about the level of QoL in patients with small VS prior to scheduled surgery.

Methods: One hundred forty- one patients with a T1 or T2 VS that had been scheduled for microsurgery via the middle cranial fossa (MCF) approach between 01/2013 and 12/2018 were included. Quality of life was evaluated using the Short-Form-36 (SF-36) survey. Eighty-one patients completed the questionnaire. Additionally, audiological and clinical data were collected.

Results: When compared to the German normative QoL data, VS patients showed a significant deterioration in quality of life in 6 of the 8 SF-36 subscales, whereas only 4 of 8 subscales were affected when compared to the normative group of patients with hearing loss. The analysis of predictors did not show any significant relationship between tumor volume, facial nerve function, and QoL. Only preoperative hearing status and sex had a slight impact on one subscale each (emotional health and physical health, respectively).

Conclusion: This study shows that patients with small VS have an impairment in QoL before surgical treatment. Most of the examined clinical predictors did not have a significant impact on the QoL.

Background: During hearing aid (HA) fitting, individuals may experience better speech discrimination at normal speech levels and worse discrimination at loud speech levels than without an HA. Therefore, we investigated factors that worsen speech discrimination when the speech sound level increases.

Methods: Speech discrimination was measured in patients aged >20 years who had average hearing thresholds <90 dB on pure-tone audiometry. An insufficient benefit was defined as speech discrimination being better at 65 dB sound pressure level (SPL) with HAs than without HAs and worse at 80 dB SPL.

Results: Of the 251 participants (296 ears), 229 and 25 had sufficient benefit and insufficient benefit, respectively. Functional gains (FGs) of sufficient benefit were approximately 1/3 gain (1/3 G) at 500 and 4000 Hz and slightly larger at 1000 and 2000 Hz. Functional gains of insufficient benefit were approximately 1/2 G at 1000 and 2000 Hz, smaller at 500 and 4000 Hz, and approximately 1/3 G at 250 Hz. Moreover, the difference between 1/2 G and FG was significantly larger in the sufficient benefit group at 250 500, and 4000 Hz than in the insufficient benefit group. Additionally, the average compression ratio at 50-80 dB SPL in the insufficient benefit group was approximately 1.1 to 1.2, slightly lower than the standard values.

Conclusion: Adjusting the compression ratio and lowering the FG to approximately 1.5 and 1/3 G, respectively, may help improve speech discrimination if it worsens with increasing sound levels during HA fitting.

Background: This study was designed to assess if thiol-disulfide homeostasis could be used as diagnostic biomarker in noise-induced hearing loss (NIHL) in a laboratory animal model.

Methods: The study was carried out with a total of 28 female albino rats in 4 groups: group 1 (control group) included rats that were not exposed to noise or any study treatment; in group 2, following noise exposure, rats received 2 mg of dexamethasone per kilogram of body weight via the intramuscular route for 5 days; in Group 3, rats were exposed to noise and received a saline solution for 5 days, in a volume (0.15 cc) matched to that of dexamethasone administered in group 2; and in group 4, rats were exposed to noise, and blood samples were collected during the early phase to assess thiol-disulfide homeostasis without administering any treatment. Rats in groups 2, 3, and 4 were exposed to 120 dB noise in the 4 kHz octave band for 4 hours. The auditory brainstem response (ABR) test was performed in all groups on day 1 after noise exposure and was repeated in groups 1, 2, and 3 on day 5. Auditory brainstem response thresholds were recorded at 8, 12, 16, 20, and 32 kHz frequencies. Groups 1, 2, and 3 rats were sacrificed on day 5, and group 4 rats were sacrificed by exsanguination on day 1 after noise exposure. Venous blood samples collected from the caudal vena cava were centrifuged and sent to the corresponding laboratory for thiol-disulfide homeostasis studies. After sacrificing the rats, the right and left temporal bones of each rat were removed and stained with hematoxylin eosin for histological studies to explore any pyknotic changes in spiral ganglion cells.

Results: Intergroup comparisons by frequency on day 5 of noise exposure showed statistically significantly lower responses in ABR measurements at 8 kHz, 12 kHz, and 16 kHz in group 2 compared to group 3 (P = .003, P=.006, and P=.002). Improvements were observed with dexamethasone administered for therapeutic purposes, particularly if the hearing loss was induced by low-frequency noise. In the assessment of the parameters of thiol-disulfide homeostasis, disulfide/native thiol and disulfide/total thiol ratios and ischemia-modified albumin (IMA) levels were higher in group 4 than in other groups, although only the differences between group 1 and group 4 reached statistical significance.

Conclusion: According to this study, thiol-disulfide homeostasis and IMA can be shown as diagnostic biomarkers in NIHL, especially in the early period. The results from our study suggest that these markers may be used as adjunctive diagnostic tools in NIHL, in addition to audiological studies. However, this issue can be clarified with further clinical studies.

Background: Despite cochlear microphonic's potential clinical application, especially in ANSD diagnosis, the optimal parameters to record cochlear microphonics and the effect of various stimulus parameters are not well understood yet, which makes its recording a difficult procedure. The present study was undertaken to determine the effect of stimulus polarity, rate, stimulus type, and stimulus frequency on different aspects of cochlear microphonics, which could help to decide an optimal stimulus parameter that can be used to record CM.

Methods: The study involved 32 normal-hearing adults. CM was recorded from these individuals using extratympanic CM measurement from the ear canal independently for tone burst frequencies (500 Hz, 1 kHz, 4 kHz & 8 kHz) and click stimuli having rarefaction and condensation polarity at 30.1/sec and 59.1/sec repetition rates. Amplitude and latency were measured from the recorded waveforms and compared across and between stimulus conditions.

Results: Results reveal that stimulus frequency and stimulus type have a significant effect on different parameters of CM. However, there was no significant effect of stimulus polarity and rate of stimulus on the amplitude and latency of cochlear microphonics. The amplitude and latency of the cochlear microphonics are inversely proportional to the stimulus frequency.

Conclusion: Hence, the study suggests the use of low-frequency tone burst (500 Hz/1 kHz) to elicit robust CM, which has greater application in the assessment of cochlear functioning over OAE as the latter gets affected by environmental and physiological noise and also due to middle ear pathology.

Background: Clear identification of the round window (RW) through the facial recess is a key surgical step for successful cochlear implantation (CI) surgery, which may be very challenging in some cases. Objective is to predict round window (RW) accessibility during CI surgery using high-resolution computed tomography (HRCT).

Methods: We retrospectively reviewed preoperative HRCT scans of 142 patients who underwent CI surgery via the standard posterior tympanotomy approach at our ENT Head and Neck Surgery department. Surgical accessibility of the RW was assessed according to 2 methods, similar to the ones introduced by Mandour et al and Elzayat et al. Pre-operative imaging findings were then compared to the actual surgical accessibility of the RW by reviewing surgical notes and video recordings.

Results: Difficult surgical access to the RW was correctly predicted in our series by Mandour's method in 81.8% of the cases, with a sensitivity and specificity of 56.3% and 96.4%, respectively, and by Elzayat's method in 72.2% of the cases, with a sensitivity and specificity of 50% and 94.5%, respectively. Combining both methods showed an increase in sensitivity levels (Se=71.9%). When the 2 methods both predicted difficult RW access, there was a strong probability that drilling a cochleostomy would be necessary for safe electrode insertion along the scala tympani of the basal turn of the cochlea (P < .001).

Conclusion: These 2 methods are both simple and reliable tools that can help the surgeon anticipate difficult surgical access and prepare for the potential use of alternative techniques.