Background Cochlear implant surgery is a complex procedure influenced by the anatomical structures of the temporal bone. Preoperative imaging using CT and MRI can provide critical insights into the surgical challenges that may be encountered. This study aims to evaluate the role of CT and MRI in preoperative assessment to predict the difficulty of cochlear implant surgery in terms of surgical time. Materials and methods A retrospective observational study was conducted at Saveetha Medical College and Hospital, Chennai, from April 2022 to September 2023. Ninety patients with severe to profound sensorineural hearing loss who underwent cochlear implantation were included. Preoperative high-resolution CT (HRCT) and MRI of the temporal bone were performed to assess various anatomical parameters. Surgical difficulty was evaluated intraoperatively and correlated with preoperative imaging findings. Data were analyzed using IBM SPSS Statistics for Windows, V. 21.0 (IBM Corp., Armonk, NY). Results The mean age of participants was 7.4±10.9 years, with the majority (66.7%) in the 1-5-year age group. Out of 90 participants, 50 were male and 40 were female. HRCT and MRI revealed that 35.6% of participants had hypo-/non-pneumatized mastoids, 3.3% had narrow facial recesses, and 3.3% had high-riding jugular bulbs. Significant correlations were found between surgical time and associated congenital (p=0.006) and acquired (p=0.0001) anomalies of the temporal bone, as well as the total difficulty score (p=0.0001). The mean surgical time was 103.97±25.2 minutes, with a range from 45 to 220 minutes. Conclusion Preoperative HRCT and MRI are valuable tools in predicting the degree of difficulty in cochlear implant surgery. Specific anatomical features identified in imaging studies can significantly influence the surgical approach and duration. These findings underscore the importance of detailed preoperative imaging to enhance surgical planning and outcomes in cochlear implant procedures.

Chudley-McCullough syndrome (CMS) is a rare autosomal recessive disorder characterized by sensorineural hearing loss and cerebral abnormalities, including ventriculomegaly and partial dysgenesis of the corpus callosum. CMS is caused by two inactivating mutations of the G protein signaling modulator 2 (GPSM2), which maintains inner hair cell polarity and spindle orientation. Since its initial description, CMS has been reported approximately 30 times in the medical literature with several individuals undergoing cochlear implantation to restore their hearing. Interestingly, within the past two years, we encountered two cases of CMS in our hospital, which primarily serves patients within a 30-mile radius. To our knowledge, the literature has yet to evaluate two unrelated cases of CMS occurring in such close succession. This case report describes two successful cases of bilateral cochlear implantation in two children with CMS. Notably, these individuals have no family history of consanguinity or prior hearing loss.

Usher Syndrome (USH) is a genetically inherited condition characterized by congenital sensorineural hearing loss and progressive vision loss secondary to retinitis pigmentosa. Patients may also display vestibular areflexia and balance issues secondary to inner ear damage. Usher Syndrome is the most commonly diagnosed syndrome within the blind-deaf community, and it accounts for a significant portion of the hearing and visual deficit cases among patients younger than 65 years of age. Due to the reported prevalence of Usher Syndrome in the United States, it appears there is chronic underdiagnosis in clinical settings throughout the country. A possible explanation for this is the visual deficits of Usher syndrome do not appear until later in life and thus inappropriately lower the index of suspicion for this diagnosis in young children with hearing deficits. This case study highlights a healthy newborn who failed the universal newborn hearing screening (UNHS) bilaterally and a follow-up hearing screening in a pediatrician\'s office. Auditory brainstem response (ABR) later confirmed bilateral severe-to-profound sensorineural hearing loss. Upon genetic testing, an abnormality in the Unconventional Myosin VII-A (MYO7) gene was discovered and consistent with Usher syndrome Type 1B (USH1B). Usher Syndrome should be considered on the differential for patients with congenital hearing loss. Genetic counseling should be used if no other cause of sensorineural hearing loss is identified. Due to the progressive nature of this condition and the physical and developmental deficits that will transpire without treatment, a genetic panel for hearing loss should be prioritized to determine the presence of genetic mutations suggesting Usher syndrome.

Introduction The \'hearing aid effect\' is a negative perception toward individuals using hearing assistive devices (HADs), which is a primary reason for parents and children refusing to use them. We aimed to determine the current perception toward individuals using HADs and the associated factors. Methods A 30-item photo-based survey was conducted to analyze the participants\' perception toward individuals using HADs as compared to healthy (H) individuals and individuals with disabilities (D). The survey was validated with an intrarater reliability of 86%. A cross-sectional study was conducted by approaching individuals who visited one of the largest shopping centers in a metropolitan city to participate in the survey. Demographic information, including age, gender, and educational background, was collected. Results A total of 517 participants completed the survey. Nearly two-thirds of the participants (59.7%) did not consider individuals using HADs as those who needed assistance as compared to H individuals. Interestingly, Generation X and Z participants had a significantly better perception toward individuals using HADs (63.1% and 59%, respectively) as compared to participants of the Baby Boomers generation (54.3%). The majority of participants who considered HD use a handicap compared to healthy individuals (79.9%) did not have a family member that used a HAD. Conclusion The stigma of wearing a HAD is significantly reducing with time, and the younger generations are not considering it as a disability. This is an important point that can be highlighted while counseling parents and young adults who are candidates for HAD use.

A cochlear implant (CI) is a device that restores hearing using an electrode array that is surgically placed in the cochlea. After implantation, the CI is programmed to attempt to optimize hearing outcome. Currently, we are testing an image-guided CI programming (IGCIP) technique we recently developed that relies on knowledge of relative position of intracochlear anatomy to implanted electrodes. IGCIP is enabled by a number of algorithms we developed that permit determining the positions of electrodes relative to intra-cochlear anatomy using a pre- and a post-implantation CT. One issue with this technique is that it cannot be used for many subjects for whom a pre-implantation CT was not acquired. Pre-implantation CT has been necessary because it is difficult to localize the intra-cochlear structures in post-implantation CTs alone due to the image artifacts that obscure the cochlea. In this work, we present an algorithm for automatically segmenting intra-cochlear anatomy in post-implantation CTs. Our approach is to first identify the labyrinth and then use its position as a landmark to localize the intra-cochlea anatomy. Specifically, we identify the labyrinth by first approximately estimating its position by mapping a labyrinth surface of another subject that is selected from a library of such surfaces and then refining this estimate by a standard shape model-based segmentation method. We tested our approach on 10 ears and achieved overall mean and maximum errors of 0.209 and 0.98 mm, respectively. This result suggests that our approach is accurate enough for developing IGCIP strategies based solely on post-implantation CTs.