Search

Objective: (1) Compare the non-device-related costs of bilateral simultaneous cochlear Implantation (BSICI) vs. unilateral, and (2) establish a dollar amount for potential cost savings in direct costs for BSiCI vs. sequential cochlear implantation (CI). Methods: Data from all CI cases performed during the fiscal year 2010-2011 were retrospectively reviewed without exception. Fifty-four patients received unilateral CI and 36 received BSiCI. Demographics and data regarding direct costs associated with the operation and immediate In hospital post-operative period were collected. Results: The total operating room (OR) time for unilateral and BSiCI was 3 hours 00 minutes and 4 hours 37 minutes, respectively, with a mean difference of 1 hour and 36 minutes (SD = 0 hours:06 minutes). The cost of OR and post-anesthetic care unit supporting staff was $3102 and $4240 for unilateral and BSiCI, respectively, with a mean difference of $1138 (SD = 216). Unilateral CI supplies totaled $1348 compared to $1822 for BSICI supplies with an average difference of $438 (SD = 123). Conclusion: The total direct costs for a unilateral CI (excluding implantable device and surgeon fees) were $4362 compared to $5823 dollars for BSiCI. Simultaneous implantation can lead to a potential saving of $2901 compared to sequential Implantation. [ABSTRACT FROM AUTHOR]

The objective of this study was to evaluate the critical time period between the onset of sensorineural hearing loss and cochlear implantation with respect to normal voice production in children with post-meningitic hearing loss. Acoustic measures of voice production were obtained from ten paediatric cochlear implant recipients with post-meningitic hearing loss. Acoustic measures were obtained utilising the Multi-Dimensional Voice Program and Computerized Speech Laboratory (Kay Elemetrics Corp.). Measures were based on sustained phonation of the vowel /a/. Acoustic parameters included fundamental frequency, short- and long-term frequency perturbation, and short- and long-term amplitude perturbation. Measures of fundamental frequency and short-term frequency and amplitude perturbation were comparable to values of children with normal hearing. Long-term control of frequency was within normal limits for subjects with a period of auditory deprivation of less than four months. Measures of long-term amplitude perturbation were normal for all patients except those with cochlear ossification. Early restoration of auditory feedback with cochlear implantation, the absence of cochlear ossification, residual aided hearing following meningitis, and auditory-verbal therapy were identified as factors in preserving the long-term control of frequency and amplitude in the setting of post-meningitic hearing loss. [ABSTRACT FROM AUTHOR]

Objective: To describe the cochleovestibular apparatus on computed tomography (CT) imaging in patients with cholesteatoma. We asked whether cochleovestibular anomalies coexist in individuals with cholesteatoma. Study Design: Randomized, controlled, prospective measurement. Methods: A database search yielded 145 children with cholesteatoma: 31 met inclusion criteria by not having sensorineural hearing loss, not having an associated syndrome, and having digitally stored temporal bone CT imaging available. Prospective measurement of 31 individuals (62 ears) with unilateral cholesteatoma and 32 normally hearing nonsyndromic controls (64 ears) was performed by a neuroradiologist blinded to the study objective. Twenty-six temporal bone aspects on axial imaging were evaluated (16 measurement, 10 calculated from measurement). Results: The cholesteatoma group had a larger endolymphatic fossa and vestibular aqueduct, and there was a trend for the lateral semicircular canal vestibule to be smaller as compared with controls. Subgroup analysis revealed a gradient in prevalence of these findings being most common in the congenital cholesteatoma group, intermediate in the acquired cholesteatoma group, and least common in controls. There were no differences in measurements between ears with cholesteatoma and contralateral disease-free temporal bones. Conclusions: Children with cholesteatoma have abnormal vestibular anatomy. The gradient in prevalence of these findings may suggest a relationship between congenital and acquired cholesteatoma. These may include a generalized temporal bone anomaly that predisposes to cholesteatoma formation, or a third variable such as genetic mutation may predispose to both anomalous cochleovestibular formation and cholesteatoma. [ABSTRACT FROM AUTHOR]

Objective: To describe temporal bone findings on computed tomography (CT) imaging in GJB2-related hearing loss (HL). We asked whether evaluation of the temporal bone is required in individuals with biallelic GJB2 mutations.Study Design: Randomized, blinded, controlled, prospective measurement.Methods: Blood from 264 pediatric cochlear implant users was analyzed for mutations in the GJB2 gene. Thirty-six aspects of the temporal bone on CT imaging were evaluated in 53 individuals (106 ears) with biallelic disease causing GJB2 mutations. A subset of patients was age matched and compared with normally hearing individuals. Subjects with biallelic GJB2 mutations were tested for mutations in the SLC26A4 gene to rule out Pendred syndrome as a confounding cause of large vestibular aqueduct syndrome.Results: Approximately 53% of ears of subjects (72% of subjects) with biallelic GJB2 mutations had at least one temporal bone anomaly. The most common findings were 1) dilated endolymphatic fossa (28%); 2) hypoplastic modiolus (25%); 3) large vestibular aqueduct (8%); 4) hypoplastic horizontal semicircular canal (8%); 5) hypoplastic cochlea (4%). Compared with normally hearing individuals, the GJB2 group had hypoplasia of the cochlear nerve canal, lateral semicircular canal vestibule, internal auditory canal (t tests, P < .001), and were 11 times more likely to have a hypoplastic modiolus. Dilated endolymphatic fossae were 1.4 times more common in the GJB2 group, and large vestibular aqueducts were 3 times more common in the GJB2 group, as compared with normally hearing controls.Conclusions: Temporal bone anomalies are common in GJB2-related HL, and imaging of the temporal bone should be included in routine evaluation of these individuals. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: Middle and external ear anomalies are well recognized in Down syndrome (DS, trisomy 21). Inner ear anomalies are much less frequently described. This study reviews inner ear morphology on imaging to determine the prevalence of cochlear and vestibular anomalies in children with DS. Study Design: The authors conducted a retrospective review of imaging features of (DS) inner ear structures. Methods: Fifty-nine sequential patients with DS with imaging of the inner ear were identified by a radiology report text search program. Quantitative biometric assessment of the inner ear was performed on patients with high-resolution computed tomography or magnetic resonance images of the petrous bone. Petrous imaging was performed for evaluation of inflammatory disease or hearing loss. Spinal imaging, which included petrous views, was performed in most cases to exclude C1 to 2 dislocation, a potential complication of DS. Measurements were compared with normative data. Results: Inner ear dysplasia is much more common in DS than previously reported. Inner ear structures are universally hypoplastic. Vestibular malformations are particularly common and a small bony island of the lateral semicircular canal (<3 mm in diameter) appears highly typical. Additional findings in some patients were persistent lateral semicircular anlage with fusion of the lateral semicircular canal and vestibule into a single cavity, vestibular aqueduct and endolymphatic sac fossa enlargement, cochlear nerve canal hypoplasia, and stenosis or duplication of the internal auditory canal. Stenosis of the external meatus, poor mastoid pneumatization, middle ear and mastoid opacification, and cholesteatoma were common, as expected. [ABSTRACT FROM AUTHOR]

Objective/hypothesis: It is reasonable to suppose that the pattern of sensorineural damage along the length of the cochlea depends on the etiology of a hearing loss (HL). In GJB2-related deafness, we hypothesize that gap junction deficits are uniformly distributed and will result in similar damage along the length of the cochlea as compared with non-GJB2 subjects. We assessed this by measuring patterns of neural activity and hearing from apical versus basal cochlear implant electrode regions.Study Design: This was a prospective, blind, controlled study.Methods: Blood from 301 pediatric cochlear implant users was analyzed for mutations in GJB2 by direct sequencing. After exclusion of patients with monoallelic GJB2 mutations, associated syndromes, or risk factors for HL that were not congenital, 39 children with biallelic GJB2 mutations and 58 without GJB2 mutations were evaluated. Hearing was measured before implantation at frequencies ranging from 250 Hz to 8 kHz. After implantation, neural activity at the apical and basal ends of the implanted array was measured using electrically evoked compound action potentials of the auditory nerve (ECAPs) and evoked stapedius reflexes (ESRs).Results: GJB2 and non-GJB2 groups were not significantly different with respect to sex, age at implantation, duration of auditory deprivation, hearing aid use, duration of aided hearing, ear implanted, implant model, or depth of insertion (P>.05). Children with GJB2-related HL had greater similarities between low- and high-frequency residual hearing and between neural activity electrically evoked at apical and basal regions of the cochlea as compared with children with non-GJB2-related HL who demonstrated larger deficits in basal regions.Conclusion: Results suggest more consistent spiral ganglion survival along the length of the cochlea in GJB2-related HL as compared with non-GJB2-related HL, which appears to involve a decreasing gradient of spiral ganglion survival from the apex to the base of the cochlea. Our findings support our premise that in GJB2-related HL, dysfunction of gap junctions likely occurs to a similar degree in the apical and basal regions of the cochlea. This knowledge might be used to customize implantable devices for patients with HL in the future. [ABSTRACT FROM AUTHOR]

Objectives: Early presentation of congenitally deaf children for cochlear implantation is leading to surgery in younger candidates. The safety of cochlear implantation in children aged 12 months and younger is reviewed with radiologic assessment of mastoid bone anatomy and surgical outcome data. Study Design: Analysis of case records and temporal bone computed tomography (CT) scans with description of surgical technique in infants. Methods: Chart analysis of children aged 12 months or younger at cochlear implantation. Mastoid bone anatomy was compared with older children (mean age 2 years) using CT scans. Results: Twenty-five infants received implants at 7 to 12 months of age because of meningitis (n = 4) or early detection of deafness (n = 21). Mastoid marrow content on CT scan was significantly greater in this age group (P < .001 Mann-Whitney rank sum test), but pneumatization was always adequate for safe identification of surgical landmarks. The smaller size of the mastoid bone was not restrictive. An extended postauricular approach was used in the first 11 cases and a 2.5 cm hair-line incision in the remainder. Ligature tie-down of the device was completed in all cases. No complications occurred. All are full-time implant users, except one with other neurologic sequelae of preoperative meningitis. Conclusions: In our experience, cochlear implant surgery is safe in children aged 7 to 12 months with appropriate anesthetic and postoperative support. The small incision technique is particularly suited to this age group. Ligature fixation of the device is considered advisable because of the increased risk of displacement from frequent falls when learning to walk. [ABSTRACT FROM AUTHOR]

The effect of blindness on sound source identification was studied. Four groups of normally-hearing adults, two sighted and two blind, participated. Subjects were tested using arrays of four and eight loudspeakers, surrounding them in the horizontal plane. One sighted group was tested in quiet. The other groups were tested in continuous 60-dB SPL white noise. Three 75-dB SPL 300-ms stimuli were localized: one-third octave noise bands, centered at 0.5 and 4 kHz, and broadband noise. Broadband noise was easiest to localize (both binaural and spectral cues available), and the 0.5-kHz noise band was the most difficult (primarily interaural temporal difference cue available). Subjects with late-onset blindness achieved significantly higher scores than the early blind and blindfolded sighted subjects. The percentage correct decreased with an increase in the number of speakers, but background noise had no effect. The results attest to the benefit of early visual experience for spatial hearing in adulthood, and demonstrate the negative impact of sudden loss of sight. [ABSTRACT FROM AUTHOR]

Titanium osseointegrated implants for bone-anchored hearing aids (BAHAs) have been in use since 1977. A series of 32 children who received implantation since 1990 is reported. The report focuses on the surgical aspects of BAHAs, predisposing factors, and prevention of complications in an unusual pediatric population. The records of 32 children who had undergone two-stage implantation of a BAHA were retrospectively reviewed. The majority of the patients had craniofacial abnormalities. Of the 32 implantees, 29 use their BAHA at present. Five children failed to achieve osseointegration, and eight patients have had revision surgery for lost abutments, trauma, or chronic skin problems. There were no differences between preimplantation and postimplantation bone or air conduction thresholds. The pediatric BAHA carries with it a unique set of challenges and problems but can be successfully implanted and maintained. [ABSTRACT FROM AUTHOR]

We propose a method of obtaining audible and comfort-able stimulation levels with the use of objective measures when reliable behavioral testing is not possible. Electrically evoked compound action potentials of the auditory nerve (ECAPs) and stapedius reflex (ESR) thresholds were measured in 68 children who use Nucleus devices, beginning at implantation in the operating room (OR) and continuing over the first year of implant use at three implant electrodes. Generic correction factors based on group data were subtracted from ECAP thresh-olds obtained in the OR. The correction factors were specified for two groups of electrodes and two age groups of children. Objective thresholds exceeded behavioral values in 75%, of children and were lower than minimum ESR thresholds in 85% of children. Mean dynamic ranges between the two objective measures spanned 23--36 Clinical Units across the implanted array. Maximum levels, based on ESR thresholds measured in the OR, can be globally adjusted to maintain comfort-able stimulation levels. [ABSTRACT FROM AUTHOR]