Central auditory development in children with bilateral cochlear implants.

OBJECTIVE: To examine the time course of maturation of P1 latencies in infant sequential and simultaneous bilateral cochlear implant recipients. DESIGN: Retrospective case series. SETTING: Pediatric collaborative cochlear implant program. PATIENTS: Four children who received bilateral cochlear implants prior to age 2 years. INTERVENTION: Cortical auditory evoked potential was completed to determine the latency of the P1 response in 4 children with bilateral cochlear implants. MAIN OUTCOME MEASURES: Longitudinal development of the latency of the P1 cortical auditory evoked potential in children who received bilateral cochlear implants prior to age 2 years. RESULTS: In 2 patients who received sequential bilateral implants, P1 latencies recorded from the first implanted ear were within normal limits after 3 to 6 months of implant use. By comparison, P1 latencies from the second implanted ear reached normal limits as early as 1 month after implant use. In 2 patients who received simultaneous bilateral implants, P1 latencies from both ears were also within normal limits in a very short time frame (ie, by 1 month poststimulation). CONCLUSIONS: Our data suggest a high degree of plasticity of the central auditory pathways after early bilateral implantation. We find that P1 latencies provide a clinically useful biomarker of central auditory system development in children after cochlear implantation.

Sialoblastoma: a rare submandibular gland neoplasm.

A sialoblastoma is a rare congenital epithelial tumor that arises in a major salivary gland. To our knowledge, only 24 cases of sialoblastoma have been previously reported in the English-language literature. We report a new case, that of a 15-month-old boy who presented with a submandibular mass. Surgical excision of the mass was undertaken. Intraoperatively, the mass appeared to be adjacent to the submandibular gland, but it had not invaded it. The mass was excised, and the submandibular gland was left in place. Pathology identified the tumor as a sialoblastoma. However, pathology also revealed that residual tumor was present at the surgical margin. The patient was returned to the operating room for excision of the left submandibular gland and the level I lymph nodes. Following revision surgery, the surgical margins were negative. The patient remained disease-free at the 1-year follow-up. Despite the need for revision surgery, this case provides support for the idea that surgery alone is sufficient for curative treatment.

Cytomegalovirus in the perilymphatic fluid.

OBJECTIVE: The incidence of congenital cytomegalovirus (CMV) infection is approximately 1% of neonates. Ninety percent of congenitally infected infants are "asymptomatic;" they have no signs or symptoms at birth. The prevalence of congenital CMV in the profoundly deaf population and the pathogenesis of deafness from CMV are unknown. The objective of this study is to determine whether CMV can be demonstrated and quantified in perilymphatic fluid of patients with congenital CMV infection and sensorineural hearing loss (SNHL) using a quantitative real-time polymerase chain reaction (QRTPCR). STUDY DESIGN: Prospective case series. METHODS: Perilymphatic fluid was collected at the time of cochlear implantation from children with known or radiologic evidence of congenital CMV infection and analyzed for the presence of CMV using QRTPCR. Blood was collected and analyzed for CMV using QRTPCR, serology, and culture. CMV was quantified in perilymphatic fluid and compared with that present in the patient's blood. RESULTS: Perilymphatic fluid and blood was collected from six children. QRTPCR was positive for CMV in the perilymphatic fluid of four patients. Blood analyzed with QRTPCR, and culture was negative in all patients. CONCLUSIONS: CMV can be demonstrated and quantified in perilymphatic fluid using QRTPCR. Refinements in our technique and sampling of perilymphatic fluid from a large population of children with congenital SNHL and unknown etiology can determine the prevalence of CMV-mediated profound HL.

Clinical results with the Med-El compressed and split arrays in the United States.

OBJECTIVE: To determine the indications for which the Med-El compressed and split arrays have been used in the United States, to assess the success achieved in insertion, to determine hearing outcomes, and assess complications associated with using these arrays. STUDY DESIGN: Retrospective review of data gathered during the US clinical trial. METHODS: Data were extracted concerning indications for use, depth of insertion, number of electrodes in use, 3 and 6 month performance outcomes, and complications. The results obtained in postmeningitic patients using the compressed array were compared with results of patients using the split array. RESULTS: Six adults and 22 children have been implanted with the compressed array. The principal indications have been postmeningitis or congenital cochlear malformations. The average improvement seen in the Meaningful Auditory Integration Scale scores was 17.6 for the compressed array group and 13.9 for the split array group. The same number of electrodes was inserted on average for both groups, with comparable numbers of electrodes in use. CONCLUSIONS: The Med-El compressed array is an alternative to a standard or split array in managing patients with postmeningitic deafness or congenital cochlear malformations. Benefits to these patients are most clearly seen in subjective measures.

The effect of GJB2 allele variants on performance after cochlear implantation.

OBJECTIVES/HYPOTHESIS: It has been hypothesized that etiology of hearing loss may serve as an independent variable in performance after cochlear implantation. To test this hypothesis, the authors identified pediatric cochlear implant recipients with gap junction protein beta2 (GJB2)-related deafness. The study examines performance outcomes associated with GJB2 deafness-causing allele variants. STUDY DESIGN: Pediatric cochlear implant patients were screened for GJB2 allele variants; statistical comparisons were made with prospectively obtained performance measures. METHODS: From 181 children who participated in a nationwide cochlear implant research program, 122 children were identified with congenital nonsyndromic sensorineural hearing loss and invited to participate. Screening for GJB2 allele variants was completed for 55 children. The children were homogeneous with respect to age (8 or 9 y) and age at implant (before age 5 y). All patients have previously undergone a prospective regimented battery of performance measures. RESULTS: Performance measures were compared between 22 children with and 33 children without mutations to determine whether GJB2 status was a significant predictor of cochlear implant outcomes. Reading and cognitive outcomes were significantly dependent on connexin status. The group of children who tested positive for GJB2-related deafness scored significantly higher on a nonverbal cognitive measure, Block Design, and on a measure of reading comprehension. CONCLUSION: The isolated insult to the cochlea created by GJB2 allele variants allows for preservation of central cognitive function. Better reading performance is seen in children with GJB2-related deafness.

Cochlear implantation in children with CHARGE association.

OBJECTIVE: To explore the anomalies of the temporal bone found on radiologic examination, technical challenges in cochlear implantation, and audiologic benefit derived from cochlear implantation in a series of children with CHARGE association. DESIGN: Case series report. SETTING: Tertiary referral children's hospital pediatric cochlear implant program. PATIENTS: Six children with CHARGE association and sensorineural hearing loss. INTERVENTION: All patients were evaluated and followed up by the cochlear implant team. Cochlear implantation was attempted in all 6 children. MAIN OUTCOME MEASURES: Computed tomographic scans and cochlear implantation operative records were reviewed, and their findings were correlated. Audiometric and speech perception data before and after cochlear implantation were compared. RESULTS: Five children with CHARGE association received implants. A sixth child did not because of an aberrant course to the facial nerve. The 5 children receiving implants obtained varying degrees of measurable benefit from their implants. All 6 children had temporal bone abnormalities seen on their computed tomographic scans and documented at the time of surgery. CONCLUSIONS: Variations in the temporal bone anatomy of patients with CHARGE association can lead to increased technical challenges and risk to the facial nerve during cochlear implantation. Individual outcomes after implantation may vary; our patients receiving implants obtained benefit. Parents should be counseled thoroughly and have appropriate expectations before proceeding with implantation.

Mastoid subperiosteal abscess management in children.

The management of a mastoid subperiosteal abscess has traditionally required mastoidectomy. With the improvement of antibiotic therapy current literature supports the treatment of uncomplicated acute mastoiditis with myringotomy and intravenous antibiotics. Treatment of a mastoid subperiosteal abscess with tympanostomy tube insertion, intravenous antibiotics, and postauricular incision and drainage of the abscess avoids the morbidity and potential complications of mastoid surgery in young children. Three patients diagnosed with a mastoid subperiosteal abscess were managed in this way. The outcome of their treatment has been documented with lengthy otologic follow-up. Complete resolution of the acute infectious process was achieved in all cases with no evidence of recurrent disease.