Lucia Belen Musumano

Background/Objectives: Hyperventilation-induced nystagmus (HVIN) has been described as a sensitive clinical sign in vestibular schwannoma (VS), potentially reflecting a reversible conduction block in partially demyelinated vestibular nerve fibers. However, its relationship with tumor size and instrumental vestibular deficits remains controversial. This study aimed to investigate the association between HVIN, tumor size, clinical presentation, and vestibular function assessed by the video Head Impulse Test (vHIT) and functional Head Impulse Test (fHIT). Methods: Eighty consecutive patients with unilateral VS were retrospectively evaluated. All underwent bedside vestibular examination, the vHIT, the fHIT, and the Hyperventilation Test (HVT). Tumors were classified according to the modified Koos–Sanna grading system. Associations between HVIN (presence and direction), demographic and clinical variables, vestibular deficits, and tumor size were analyzed using binary logistic regression, Fisher’s exact test, Welch’s independent samples t-test, and the Mann–Whitney U test. Results: HVIN was observed in 73% of patients. Among the patients with HVIN, ipsilesional horizontal nystagmus occurred in 57% of cases, 41% of the subjects showed contralesional nystagmus and one patient had downbeat nystagmus. vHIT abnormalities were identified in 54% of patients, while 30% of these also demonstrated fHIT deficits. No patient presented isolated fHIT abnormality. HVIN was detected even in patients with intracanalicular or small tumors and in some asymptomatic individuals. No statistically significant correlations were found between tumor size and HVIN presence, HVIN direction, or vestibular deficits in the vHIT/fHIT (all p > 0.05). Conclusions: HVIN is frequently observed in patients with VS, including those with small or asymptomatic lesions. However, neither its presence nor direction correlates with tumor size or objective vestibular deficit. The Hyperventilation Test should not be considered a prognostic tool but may serve as a simple and valuable adjunctive bedside examination in the early diagnostic suspicion of vestibular schwannoma.

An increasing number of studies highlight the role of saccadic remodulation in compensatory mechanisms following vestibular injury, and the reappearance of SHIMP saccades correlates with symptom improvement measured by the Dizziness Handicap Inventory (DHI). To investigate the influence of attentional processes and working memory on visuo-vestibular interaction, three independent but interrelated experiments were conducted. In the first two experiments, healthy subjects and patients with unilateral or bilateral vestibular deficits underwent vHIT in SHIMP mode and the Functional Head Impulse Test (fHIT), performed first separately and subsequently simultaneously. Mean latency and clustering of SHIMP saccades, together with Landolt C recognition rates, were analyzed. Differences between separate and combined protocols were assessed, and, in patients, correlated with symptom severity measured by the DHI, to determine whether the near-simultaneous execution of tasks mediated by shared parietal cortical substrates influenced performance. In the third experiment, vHIT in HIMP mode and fHIT were performed using separate and combined protocols to evaluate whether recognition-related cognitive load affected recovery saccade latency and clustering. Results suggest that visual recognition modulates visuo-vestibular interaction, supporting integrated dual-task protocols for ecological balance assessment and helping explain clinical discrepancies.

Background: Cochlear implantation (CI) following vestibular schwannoma (VS) resection, traditionally controversial, is increasingly accepted due to advances in surgical techniques. Current evidence is limited to small, short-term studies. This report presents the largest consecutive single-center cohort and offers long-term audiological outcomes. Material and methods: This retrospective study analyzed 73 consecutive patients who underwent VS resection with either simultaneous (97%) or delayed (3%) CI at the Gruppo Otologico between 1985 and 2025, among more than 4000 patients who underwent VS removal during the same period. Sixty-three patients (86%) had sporadic VS, and 10 (14%) had neurofibromatosis type 2 (NF2). Tumor removal was performed using the enlarged translabyrinthine approach (ETLA). Outcomes were assessed through pure-tone average (PTA), speech discrimination scores (SDS), and open-set speech recognition. Results: After a median 35-month follow-up, 48 patients (66%) were active CI users; 20 (27%) were nonusers or nonresponders. Five patients were “removed/non activated” (never activated due to postoperative complications or device malfunction or later explanted despite initial benefit) and were therefore not included in postoperative audiological outcome tables. Due to complications requiring explantation. Among users, the median postoperative PTA was 45.0 dB HL (IQR: 40.0–61.3), and the median SDS was 60.0% (IQR: 20.0–70.0). Open-set performance demonstrated high-to-median scores across word, sentence, and comprehension tests. Performance group analysis revealed that 52% of patients achieved intermediate-to-high outcomes, including 75% of NF2 patients. Conclusions: Performing CI immediately after VS resection is feasible and offers long-term functional benefits for most patients, including carefully selected NF2 cases. Although results can vary, more than half of the patients attain meaningful speech perception. This extensive, long-term, single-center experience strongly supports the feasibility of CI in this context, emphasizing the importance of surgical technique, nerve preservation, and precise patient selection.

Objectives: The present study aims to identify potential predictive factors for developing sensorineural hearing loss (SNHL) in individuals with congenital Cytomegalovirus (cCMV) infection. Methods: A retrospective study was performed on 50 subjects with cCMV infection (symptomatic and asymptomatic), followed at the Audiology Service of Sant’Anna Hospital (University Hospital of Ferrara). The following data were analyzed: the type of maternal Cytomegalovirus (CMV) infection (primary versus non-primary), time of in utero infection, systemic signs and symptoms or laboratory test anomalies due to cCMV infection, and signs and symptoms of central nervous system (CNS) involvement at birth. In particular, brain ultrasonography and encephalic magnetic resonance imaging (MRI) were evaluated, searching for possible links between imaging findings and SNHL. Results: The statistical analysis showed a significantly higher risk of developing SNHL in subjects with signs and symptoms of CNS involvement at birth (p = 0.009 *). The presence of brain MRI abnormalities significantly influenced the onset of SNHL in patients with symptomatic cCMV infection (p = 0.012 *). Brain ultrasonography, the type of maternal CMV infection, systemic signs/symptoms and laboratory test anomalies at birth, and sex resulted in nonsignificant correlations in the analysis. Conclusions: The presence of neurological symptoms at birth and of detectable abnormalities in brain MRI are predictors of SNHL developing in symptomatic cCMV infection. Further investigation on this topic is necessary.

Objectives To analyze the overall long-term outcome of surgically treated skull base and temporal bone chondrosarcomas. Methods The medical records of patients with surgically treated skull base and temporal bone chondrosarcomas between 1983 and 2024 were thoroughly evaluated. Results Out of a total of over 5000 skull base surgeries performed at our center, only 29 patients had histopathologically confirmed chondrosarcomas of the skull base and temporal bone. The mean of patients age was 45.6, and the male-to-female ratio was 1.9:1. The most common symptoms included hearing loss (58.6%), tinnitus (41.4%), diplopia (31%), dysphonia (24.1%), dysphagia (20.7%), vertigo (10.3%), and dizziness (10.3%). The most frequent locations of lesions among the 29 patients are as follows: petroclival region (34.5%), jugular foramen (27.6%), petrous apex (17.2%), middle ear (13.8%), others (3.4%). TO, IFTA, IFTB, IFTC, POTS, and combined surgical approaches were commonly used. The rate of gross total removal and recurrence was 82.6% and 13.8% respectively. The follow-up duration of 6 patients was more than five years and less than ten years whereas ten patients had more than ten years of follow-up. Conclusions Chondrosarcoma of the skull base and temporal bone is a very rare pathology. Due to its multiple potential sites of origin and histological specificity, it presents us with significant challenges. Gross total removal is the primary treatment for chondrosarcoma of the skull base and temporal bone. Personalized decision-making should be considered based on the following aspects: tumor, patient, and surgeon’s factors. Postoperative radiation therapy is complementary to surgical treatment in grades II and III lesions to achieve long-term survival.

The Cochlear™ Osia® System is a hearing system indicated for patients with conductive and mixed hearing loss or unilateral deafness. Given its recent introduction, this is one of the first cases in the literature of implant dehiscence with treatment using local flaps with excellent aesthetic and functional outcomes.

Background: While gender differences of several diseases have been already described in the literature, studies in the area of hyperacusis are still scant. Despite the fact that hyperacusis is a condition that severely affects the patient’s quality of life, it is not well investigated; a comprehensive understanding of its features, eventually including gender differences, could be a valuable asset in developing clinical intervention strategies. Aim: To evaluate gender differences among subjects affected by hyperacusis. Methods: A literature search was conducted focused on adult patients presenting hyperacusis, using the MedLine bibliographic database. Relevant peer-reviewed studies, published in the last 20 years, were sought. A total of 259 papers have been identified, but only 4 met the inclusion criteria. The review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Results: The four selected papers included data from 604 patients; of these, 282 subjects resulted as affected by hyperacusis (125 females and 157 males). Questionnaires for analyzing factors affecting the attentional, social and emotional variance of hyperacusis (such as VAS, THI, TSCH, MASH) were administered to all included subjects. The data suggest that there are no hyperacusis gender-specific differences in the assessed population samples. Conclusions: The literature data suggest that males and females exhibit a similar level of hyperacusis. However, in light of the subjective nature of this condition, the eventual set up of further tests to assess hyperacusis features could be very helpful in the near future.

White Sutton Syndrome is a rare autosomal dominant disorder resulting from a de novo mutation of Pogo Transposable Element Derived with Zinc Finger domain gene. The phenotype is characterized by a wide spectrum of cognitive dysfunction and developmental delays. Hearing loss is frequently mentioned as one of the symptoms of this rare disease, but details are usually scant. We report a case of a male child affected by White Sutton Syndrome and sensorineural hearing loss, with audiological findings of an auditory neuropathy spectrum disorder, a dysfunction of the auditory pathway with preserved cochlear outer hair cell function. Up to date, the present case is the first description of hearing loss due to an auditory neuropathy spectrum disorder in White Sutton Syndrome. A comprehensive audiological assessment is therefore mandatory in all White Sutton Syndrome patients in order to recognize a possible auditory neuropathy disorder and then avoid misdiagnosis, or erroneous clinical management. Cite this article as: Belen Musumano L, Fancello V, Negossi L, Ballardini E, Bigoni S, Ciorba A. Auditory neuropathy spectrum disorder in the white sutton syndrome. J Int Adv Otol. 2023;19(3):260-262.