The journal of international advanced otology最新文献

Endolymphatic hydrops has been documented as a possible complication of cochlear implantation; however, few studies have addressed its treatment. We describe the first case ever reported of delayed endolymphatic hydrops after a cochlear implant successfully treated with intratympanic gentamicin injection. A detailed discussion of this case with a focus on its management and outcomes will be provided in comparison with literature data. The intratympanic gentamicin injection has been demonstrated to be an effective treatment for symptomatic endolymphatic hydrops after cochlear implantation. We advocate further studies to validate this strategy as a promising alternative to surgical labyrinthectomy.

Cochlear size variation was first reported in 1884, and since then, there have been various reports confirming the same. Yet, there is no single report that has displayed the wide variations in the cochlear size in a single layout capturing the cochlea in the oblique coronal view/ cochlear view. Basal turn diameter (A-value) was measured in the oblique coronal plane using the OTOPLAN® otological preplanning tool in 104 computed tomography (CT) scans of the temporal bones of cochlear implant (CI) recipients in a tertiary CI center. All CT scans with an image resolution of at least 0.5 mm and identified as having cochleae with normal anatomy were included in this study. A 3-dimensional (3D) segmentation was performed using the 3D slicer and visualized to evaluate the impact of cochlear size on the number of turns studied. The A-value was found to vary between 7.3 mm and 10.4 mm among the studied patients. Three-dimensional segmentation of the inner ear revealed only 2 turns of the cochlea in 4 ears, with A-values of 7.3, 8.8, 7.8, and 7.7 mm. One ear had only 11 /2 turns of the cochlea, with an A-value of 7.9 mm. As a further advancement in the assessment of cochlear size as determined by the A-value, 3D segmentation of the complete inner ear provides a full picture of the number of cochlear turns. Three-dimensional segmentation of the entire inner ear could help improve the preoperative planning of CI surgery and have implications for electrode array selection. Cochlear size could be a predictor of the number of cochlear turns, even in cases that look normal from the radiological findings. The findings of this study could help in improving the preoperative planning for a more successful CI surgery by differentiating between the normal and abnormal cochlea.

Autosomal dominant hearing loss is represented by a large number of genetically determined forms. Over 50 genes associated with dominant nonsyndromic hearing impairments were described. Pathogenic variants in the CEACAM16 gene lead to the development of DFNA4B hearing loss. Currently, 8 pathogenic variants in this gene have been described. The objective of this study was to study the audiological and molecular genetic characteristics of a large family with CEACAM16-associated autosomal dominant nonsyndromic hearing loss. A detailed anamnesis was collected, and a comprehensive audiological examination was performed for 21 family members. Genetic testing was performed, including whole-genome sequencing for the proband's son and Sanger sequence analysis for the proband and for all available family members. In a large Russian family, including 5 generations, an autosomal dominant type of slowly progressing nonsyndromic late-onset hearing loss was observed. Eleven family members suffer from hearing impairment, which starts with tinnitus and threshold increase at high frequencies, since the age of 5-20 years. Hearing loss slowly progresses with age in each person and is similar to age-related hearing loss. We have detected the novel likely pathogenic variant с.419С>T (p.(Thr140Ile)) in exon 3 of the CEACAM16 gene, which segregates with late-onset nonsyndromic hearing loss in this family. The clinical data obtained in the examined family correspond with the phenotype in previously described cases. In general, the study widened the mutation spectrum of the gene, allowing to carry out medical genetic counseling and to answer the questions about the hearing impairment prognosis for future generations.

A labyrinthine fistula is a severe complication of middle ear cholesteatoma that can cause profound sensorineural hearing loss and vertigo. However, there is no consensus regarding the transition to postoperative hearing. Although hearing deteriorates gradually with a delay in some cases of labyrinthine fistula, insufficient consideration has been given to this point. We examined perioperative changes in cases of middle ear cholesteatoma with labyrinthine fistulas. We retrospectively reviewed the medical records of 578 patients with middle ear cholesteatoma who underwent tympanoplasty at our hospital between 2016 and 2021. Patients with labyrinthine fistulas were selected; their perioperative bone-conduction hearing was assessed. Fistula depth was determined following the classification reported by Dornhoffer et al. The hearing was compared preoperatively, early postoperatively (3-6 months), and 1 year postoperatively. Forty-eight patients (8.3%) had labyrinthine fistulas. Regarding depth, 21 cases were type I, 14 were type IIa, 3 were type IIb, and 10 were type III. Preoperative bone-conduction hearing was significantly poor in invasion type IIb or deeper cases. Cases with type IIb or deeper fistulas, multiple fistulas, or vertigo deteriorated postoperatively. Type III cases or those with multiple fistulas deteriorated further from the early postoperative period to 1 year postoperatively. Concerning frequency, 500 and 2000 Hz showed a delayed deterioration. This is a valuable report of delayed hearing loss after surgery in patients with a labyrinthine fistula. This change is associated with the labyrinthine fistula's depth and multiple fistulas-this is important during preoperative counseling of patients undergoing surgery.

Arsenic is frequently used in alternative medicine, and it is critical to promptly identify and treat suspected arsenic toxicity in patients. In a case study, a female patient presented with several symptoms, including nausea, vomiting, bilateral tinnitus, hearing loss, vertigo, and other associated complaints. After admission, the patient showed lethargy, and topical application of Chinese herbal medicine was found on her left breast, along with visible pigmentation on her torso. Examination revealed severe bilateral sensorineural deafness, liver and kidney injury, and pancytopenia. Due to the presence of broken skin, toxicological analysis detected elevated levels of arsenic in both blood (113 ng/mL) and urine (865.4 ng/mL). The patient was diagnosed with arsenic poisoning and received symptomatic treatment, including detoxification. Unfortunately, the patient died due to long-term exposure to arsenic. Therefore, early identification of the etiology is crucial for managing cases of arsenic poisoning.

Background:  As indications for surgical Eustachian tube (ET) procedures have been expanded, it is essential to understand the anatomy of ET surroundings for safe ET interventions.

Methods:  We evaluated the peritubal region using oblique planes of temporal computed tomography (CT) with the Valsalva maneuver and classified the peritubal region between the osseous ET and the internal carotid artery (ICA) into 5 types: 1. bony prominence; 2. air cell; 3. absence of peritubal structures (3a. thick canal [>0.5 mm], 3b. thin canal [<0.5 mm], 3c. dehiscence).

Results:  Bony prominence and air cell types were observed in 41.0% (50/122 ears) and 13.1% (16/122 ears), respectively. The ICA was located directly medial to the osseous ET in 39.4% (48/114 ears), of which thick and thin canal types were found in 23.8% and 15.6%, respectively. Internal carotid artery canal wall dehiscence was observed in 8 ears (6.6%). The shortest perpendicular distance between the osseous ET and ICA was 1.6 (range: 0.4-4.9) mm and 2.7 (range: 1.3-5.8) mm in the bony prominence and air cell types, respectively. Osseous ET-ICA distances were 1.2 (range: 0.6-3.6) mm and 0.4 (range: 0.1-0.5) mm in thick and thin canal types, respectively.

Conclusion:  Distinct peritubal structure types were observed on oblique CT planes with Vasalva maneuver. Bony prominence and air cell types provide a protective layer between the osseous ET and ICA. Imaging information on peritubal structures may help to better understand the anatomy of the ET pathway, leading to safe and accurate surgical approaches to the osseous ET.

Background:  The aim of this study was to survey the knowledge and treatment management practices for single-sided deafness (SSD) among different subspecialties of otolaryngology.

Methods:  A questionnaire was sent via Google Sheets to members of the Turkish and Egyptian Otorhinolaryngology Societies between December 2021 and February 2022. For the statistical analysis, the respondents were divided into 3 groups as otologists, non-otologists, and residents at the department of otolaryngology-head and neck department.

Results:  There were no statistically significant differences between otologists and non-otologists in radiological imaging (child P = .469, adult P = .140) and preferred treatment method (child P = .546, adult P = .106). However, otolaryngologists showed significant differences in radiological evaluation (P <.001), vestibular evaluation (P = .000), and frequency of treatment options recommended for pediatric and adult SSD patients (P = .000).

Conclusion:  There were no significant differences in SSD diagnosis, treatment, and rehabilitation between otologists and non-otologists. However, when comparing pediatric and adult patients, there was a difference in the treatment management of SSD patients.

Established treatment strategies for nontuberculous mycobacterial (NTM) infections are currently lacking, and whether surgical treatment should be applied in combination with antibiotic therapy remains debatable. Here, we report a case of bilateral otitis media caused by Mycobacterium abscessusa, a highly antibiotic-resistant bacterium. Many reported cases of NTM otitis media are unilateral, in which hearing of the contralateral ear is preserved. In the present case, strategies to improve hearing outcomes were considered, as both ears were affected. A 27-year-old woman presented with bilateral otorrhea that had lasted for the past 9 months. Bacterial culture showed M. abscessus in both ears. Based on drug sensitivity tests, clarithromycin, amikacin, and imipenem were administered. Three days after treatment initiation, diseased tissues were removed from the right middle ear, which had impaired hearing. On day 38, otorrhea stopped in both ears, and the hearing improved. Computed tomography revealed air in both middle ears. No apparent recurrence was detected. Under the same antibiotic therapy, resolution of diseased tissues and improvement in hearing were similar between the ears with and without surgery, suggesting that surgery is not always necessary. This finding may be incorporated into the treatment guidelines for NTM infections in the future.

The gene MED13 participates in transcription. The MED13L gene is a paralog of MED13 that is involved in developmental gene expression. Mutations in the gene have been shown to result in a heterogenous phenotype affecting several physiological systems. Hearing loss has been reported very rarely, and vestibular weakness has never been reported in the condition. In this report, we present a mutation of MED13L in c.1162A > T (p.Arg388Ter), where we detail and describe a cochleovestibular phenotype with objective vestibulometry for the first time. The child showed bilateral sloping sensorineural hearing loss, a bilateral vestibular weakness, and an inner ear vestibular structural abnormality on imaging. Early intervention with hearing aids and vestibular rehabilitation led to a favorable outcome in terms of speech, communication, and balance. We emphasize the importance of comprehensive audiovestibular assessment in children diagnosed with MED13L mutations for effective management of these children.

Cochlear implantation has become a standard of care for a child diagnosed with bilateral profound sensorineural hearing loss with a structured surgical standard operating procedure. A 3-year-old boy with bilateral profound prelingual sensorineural deafness underwent a Med-EL Sonata Ti100 implant. We faced a peculiar situation intraoperatively after inserting the electrodes and closing the wound. The impedance recording indicated high ground path impedance with short-circuiting of few electrodes. As a bionic implant, its electronic components may at times malfunction both intraoperatively and/or postoperatively; therefore, neural response telemetry (NRT) was invented to check it. By using NRT and a few milliliters of normal saline, we were able to diagnose as well as rectify the malfunctioning of the implant.