Background/Objectives: Cytomegalovirus (CMV)-associated hearing loss is common in non-genetic congenital hearing loss. Despite this high prevalence, a wide range of clinical characteristics exists, and the pattern of hearing loss remains unknown. This study aims to describe the clinical manifestations in children with CMV-associated hearing loss and to clarify the timing of hearing level change and the degree of hearing level fluctuation. Methods: A total of 54 patients with hearing loss due to congenital CMV infection were included. Hearing loss type (congenital or later onset), hearing loss laterality (unilateral or bilateral), severity at first and last visit, hearing progression and timing, and the difference between patients with intellectual disability and without intellectual disability were assessed. Results: The number of patients with congenital hearing loss and later onset hearing loss were 19 patients and 13 patients, respectively. Seventy-four percent (14/19) of the congenital hearing loss patients and 62% (8/13) of the later onset hearing loss patients eventually progressed to severe to profound hearing loss bilaterally. Progression occurred in less than 1 year (9 cases), between 1 and 3 years (7 cases), between 3 and 7 years (4 cases), or more than 8 years (1 case). Multiple progression events occurred in 11 cases. Conclusions: Sixty-one percent of patients had progression of hearing loss. Several cases experienced progression over more than one year and showed multiple progression events. CMV patients without intellectual disability tended to suffer later onset hearing loss. Sixty-nine percent of the patients eventually progressed to bilateral severe to profound hearing loss, which means that continuous long-term follow-up is required.

Background/Objectives: Frequency-to-place mismatch between cochlear implant (CI) electrodes and cochlear tonotopy has been suggested to affect postoperative speech perception. This study aimed to examine the associations between frequency-to-place mismatch and speech perception outcomes across multiple linguistic levels in patients with CI and to assess how these associations change over time using postoperative computed tomography. Methods: This retrospective cohort study included 44 postlingually deafened adults who underwent unilateral cochlear implantation with a Flex28 electrode by a single surgeon at a tertiary care hospital. Speech perception was assessed using CI-2004, a Japanese speech perception test consisting of monosyllables, consonants, words, and sentences, in quiet settings at 3, 6, and 12 months after CI activation. Partial correlation analyses between frequency-to-place mismatch and postoperative speech perception scores were performed in 35 of the 44 patients, controlling for age and mean preoperative pure-tone thresholds. Results: Negative associations were observed between frequency-to-place mismatch and CI-2004 scores, particularly for monosyllable and consonant perception in uncorrected analyses. After correction for multiple comparisons, only consonant perception at 3 months after CI activation remained significant (r = −0.52, p = 0.002). Similar patterns were observed for other speech measures and at later time points, although these did not remain significant after correction. Conclusions: Frequency-to-place mismatch was associated with postoperative speech perception outcomes, particularly those involving phoneme-level recognition. After correction for multiple comparisons, only consonant perception at 3 months after CI activation remained significant.

Objectives: The middle cranial fossa (MCF) approach is valued for preserving hearing while accessing the internal auditory canal (IAC), petrous apex, inner ear, and related structures. This study evaluated its clinical outcomes across otolaryngological diseases, focusing on postoperative complications, hearing preservation, and the effect of IAC manipulation on auditory function. Methods: We retrospectively analyzed 35 patients who underwent MCF otologic surgery at a single center over twelve years. We calculated the proportion of MCF cases among all otologic surgeries and assessed postoperative complications and hearing changes (bone conduction thresholds). Outcomes were compared between patients with and without IAC manipulation. Results: MCF procedures comprised 1.4% of all otologic surgeries. Petrous bone cholesteatoma was the leading indication (15 cases). Intracranial complications occurred in 4 patients (11.4%): seizures, epidural abscess, and cerebral infarction. Facial nerve paralysis occurred in 3 (10.7%) patients without any cerebrospinal fluid leaks. In patients without IAC manipulation, hearing functions were preserved (22.3 ± 7.8 dB HL pre- vs. 25.7 ± 9.5 dB HL postoperatively), whereas those with IAC manipulation showed significantly greater deterioration. Conclusions: The middle cranial fossa approach, though technically demanding and infrequently used, offers a safe and effective option across various otolaryngological diseases. This approach achieved favorable hearing preservation with a low complication rate, particularly when intradural manipulation of the IAC was not required.

Cancer originating from the external auditory canal (EAC) is rare, which precludes a clear understanding of the disease and its management approaches. We present an extremely rare case of bilateral auditory canal carcinoma that occurred heterochronously. Bilateral treatments resulted in good disease control and EAC structure preservation, which could accommodate various hearing aid options after cancer treatment. While disease control is the most crucial aspect to be considered when choosing a treatment of EAC cancer, one should also consider potential hearing ability in patients post-surgery, particularly in those with bilateral lesions, as it greatly influences the quality of life.

Vestibular involvement in CDH23-related nonsyndromic hearing loss remains incompletely understood, particularly prior to cochlear implantation. To evaluate vestibular function prior to cochlear implantation in patients with severe to profound non-syndromic hearing loss caused by CDH23 mutations. We retrospectively evaluated vestibular function in seven patients with severe to profound non-syndromic hearing loss caused by CDH23 mutations who were scheduled for cochlear implantation. Vestibular assessment included caloric testing, ACS-cVEMP, BCV-oVEMP, and damped rotation testing, depending on age. Six patients had homozygous missense mutations, and one had compound heterozygous mutations in CDH23. Caloric testing revealed unilateral reduced responses in two patients (28.6%). Damped rotation testing, performed in four pediatric patients, showed a unilateral reduced response in one patient (25%). ACS-cVEMP demonstrated a unilateral reduced response in one patient (14.3%) and an absent response in another (14.3%). BCV-oVEMP, conducted in three patients, revealed a unilateral absent response in one patient (33.3%). Overall, unilateral vestibular dysfunction was identified in four of seven patients (57.1%). More than half of patients with CDH23-related non-syndromic deafness exhibited unilateral vestibular dysfunction prior to cochlear implantation, suggesting that preoperative vestibular assessment may be clinically relevant in this population.