Yoko Baba

Congenital Cytomegalovirus Infection Diagnosed by Polymerase Chain Reaction With the Use of Preserved Umbilical Cord in Sensorineural Hearing Loss Children

Objectives/Hypothesis: Congenital cytomegalovirus (CMV) infection is estimated to account for 30% of sensorineural hearing loss (SNHL) cases. Differences in clinical characteristics between CMV‐related and unrelated SNHL cases were scrutinized.

Outcome of cochlear implantation in children with congenital cytomegalovirus infection or GJB2 mutation.

Abstract Conclusions: Outcomes following cochlear implantation in children with congenital cytomegalovirus (CMV) infection were almost equivalent to those of children with GJB2 mutation-related sensorineural hearing loss (SNHL). Although our patients with developmental disorder showed poor auditory performance and speech and language skills after cochlear implantation, SNHL with developmental disorder should not be a contraindication for the procedure. Objective: Congenital CMV infection accounts for approximately 20% of all cases of neonatal hearing loss, while the GJB2 mutation accounts for 30–50% of all cases of profound nonsyndromic hearing loss. Here, outcomes for auditory behavior and speech and language skills were compared in children with congenital CMV infection or GJB2 mutation who received cochlear implantation for profound SNHL. Methods: Five children with asymptomatic congenital CMV infection and seven children with GJB2 mutation-related SNHL, with and without developmental disorder, underwent cochlear implantation. Hearing level and speech and language development were evaluated post-implantation using IT-MAIS, MUSS, and S-S method. Results: The IT-MAIS and MUSS scores of the congenital CMV infection group and the GJB2 mutation group continued to increase for 4 years after implantation. The S-S method score in both groups gradually increased, although the scores for children with mental retardation were low.

Presence of cytomegalovirus in the perilymphatic fluid of patients with profound sensorineural hearing loss caused by congenital cytomegalovirus infection

Abstract Conclusion: Not all patients diagnosed with congenital infection using umbilical cord assay were found to be positive for CMV-DNA by perilymphatic fluid assay. In addition, a CMV-DNA-positive result was observed in one patient who had not been diagnosed with congenital infection. Sampling of perilymphatic fluid from a large population of patients with congenital SNHL caused by congenital CMV infection or of unknown etiology is required to determine the prevalence of CMV-related profound HL. Objectives: Sensorineural hearing loss (SNHL) is one of the most frequent manifestations in patients with congenital cytomegalovirus (CMV) infection. Using dried umbilical cord, a PCR-based assay was recently developed for the retrospective detection of congenital CMV infection. This study analyzed the presence of CMV in the perilymphatic fluid and evaluated differences in the effect of cochlear implantation between CMV-positive and -negative groups. Method: Perilymphatic fluid was collected from each patient at the time of cochlear implantation and analyzed for the presence of CMV using a PCR method. Results: The perilymphatic fluid in two of the five patients suffering from congenital CMV infection and in one of the 17 patients without congenital CMV infection was found to be positive for CMV.

Temporal Bone Pathology in Syphilitic Osteitis

This study aimed at demonstrating the typical temporal bone histopathology of a 74-year-old female with syphilitic osteitis. There was osteitic fistulization between lateral semicircular canal and antrum and contact of the dilated saccular wall with the medial surface of the footplate. This case most likely would have had not only a positive fistula sign caused by fistulization, but also Hennebert’s sign caused by the contact of the saccular wall with the footplate.