Faculty Bibliography

OBJECTIVE AND IMPORTANCE: Preoperative chronic otitis media (COM) is a risk factor for postoperative infection after cochlear implantation (CI), but its management varies by surgeon. Our case highlights a strategy for implant preservation in a patient with a history of recurrent cochlear implant infection. CLINICAL PRESENTATION: A 70-year-old woman with a history of chronic lymphocytic leukemia presented in 2005 with bilateral COM and sensorineural hearing loss meeting CI candidacy. Four months after left mastoid obliteration with abdominal fat graft and external auditory canal closure, a left CI was placed. Subsequent postauricular cellulitis resolved with oral antibiotics. A similar two-stage CI was performed on the right without complication. During the following year, numerous left-sided infections and fluid collections developed but were treated unsuccessfully with intravenous (IV) antibiotics and operative debridement. With concern for biofilm colonization, the implant was explanted and the electrode left in the cochlea. After reimplantation in 2010, infections resumed despite long-term IV antibiotics and incision and drainage. Intervention and technique In 2012, the left mastoid cavity was exteriorized and converted to standard canal wall-down anatomy. Bone pate was placed over the electrode, followed by cadaveric acellular dermis and a split-thickness skin graft. After more than 2 years, her better-performing CI remains infection-free. CONCLUSION: After 6 years of postoperative infections unresponsive to aggressive medical management, surgical interventions, and period of device removal, our patient's infections resolved after mastoid exteriorization and multilayered protection of the electrode. This strategy may enable implant preservation in patients with recurrent post-CI infection in an obliterated cavity.

Hearing impairment is a significant disability. According to the World Health Organization (WHO), more than 80% of the world's approximately 120 million people with hearing impairment live in developing countries. Cochlear implant is the only therapeutic intervention for those with severe-profound sensorineural hearing loss. We are reporting an interesting case of the very first cochlear implant operation carried out in Uganda. The patient was a 23 year old male whose presenting complaint was inability to hear in the left ear for three and a half years and in the right ear for one year. He had been treated for TB(Tuberculosis) mastoiditis. After the 8 months of treatment, the otorrhea persisted and he underwent a tympanomastoidectomy on the same ear. He reported no familial history of hearing loss. On examination, ENT examination revealed a small pars flaccida retration pocket of the right tympanic membrane with cholesteatoma. The left ear had an intact tympanic membrane. Pure tone audiometry revealed profound sensorineural hearing loss in both ears (see attached PTA results), CT scan of the temporal bone showed normal inner ear anatomy bilaterally and mild sclerotic changes in both mastoid bones. He then had surgery on his right ear which included cochlear implantation. The cochlear implant (CI) was activated on the first postoperative day remotely via internet with the help of the cochlear implant team at New York University Cochlear Implant Center and the patient was immediately able to appreciate some sounds. He received a pneumococcal vaccine on the first postoperative day and was discharged the following day.

OBJECTIVE: The aim of this study was to develop a multidimensional metric for assessing quality of life (QoL) in patients with neurofibromatosis type 2 (NF2). STUDY DESIGN: Electronically distributed questionnaire. SETTING: University tertiary care hospital, NF2 support groups. SUBJECTS AND METHODS: Structured interviews with NF2 providers and patients identified relevant domains. Items in each domain were extracted from validated QoL modules, then combined with items unique to NF2 and pretested on NF2 providers and patients. The final 61-item questionnaire was administered electronically to patients with NF2 (N = 118). The form assessed overall QoL and 11 additional domains, including hearing, balance, facial function, vision, oral intake, future uncertainty, psychosocial, cognition, sexual activity, pain, and vocal communication. Responses were compared with reference values for the general population, patients with head and neck cancer, and patients with brain cancer. RESULTS: Overall, QoL in patients with NF2 was lower than that of the general population (P < .01) and similar to that of patients with cancer. Patients with more facial weakness, hearing loss, and imbalance reported significantly lower QoL. However, domains most predictive of overall QoL were psychosocial, future uncertainty, and pain. Compared with patients with head and neck and brain cancer, patients with NF2 demonstrated significantly higher levels of psychosocial stressors, including disease-related anxiety, personal and financial stress, and lack of social support (P < .01). CONCLUSION: Psychosocial stress and pain significantly affect QoL in NF2, indicating that mental health, pain management, and financial counseling could have an important impact on QoL in this population.

OBJECTIVES: X-linked deafness is a potential etiology of sensorineural hearing loss characterized by bulbous dilatation of the fundus of the internal auditory canal (IAC) and the absence of the bony plates separating the basal turn of the cochlea and IAC. These malformations predispose patients to IAC insertion during cochlear implantation (CI). Our objective is to describe the surgical technique, audiometric and speech performance outcomes in a group of patients with this unique cochlear malformation. METHODS: A retrospective chart review was performed of all patients at a tertiary care facility who underwent CI between January 2006 and July 2011. RESULTS: A total of five patients were identified with radiographic findings characteristic of X-linked deafness, specifically a deficient modiolous, absent lamina cribrosa and bulbous IAC. A modified cochleostomy was utilized to ensure electrode insertion within the scala tympani avoiding the IAC. In each case, fluoroscopy was utilized to visual electrode progression during insertion and complete insertion was accomplished. All patients demonstrated improved speech performance following implantation. CONCLUSIONS: Utilizing the techniques described, patients with these unique radiographic findings consistent with X-linked deafness may successfully undergo CI with excellent potential for auditory rehabilitation.

Objective Preservation of residual low-frequency hearing has become a priority in cochlear implantation. The purpose of this study was to compare rates of hearing preservation and effects on performance of loss of low-frequency acoustic hearing with two different length electrodes. Study design Retrospective chart review. Setting Tertiary Care Hospital. Patients Twelve patients were implanted with the CI422 a slim-straight electrode; the second group consisted of 10 patients implanted with the Hybrid-L, a shorter hearing preservation electrode. Main outcome measure Audiometric thresholds and speech perception measures. Results At 1 year, 3/10 (30%) patients with the Hybrid-L and 7/12 (58%) patients with the CI422 lost residual acoustic hearing resulting in a profound hearing loss in the implanted ear. In comparing these patients in particular, mean CNC words in the implanted ear were 72% in the CI422 electrode group and 15% in the Hybrid-L electrode group at 1 year (P = 0.03). While hearing preservation rates with the Hybrid-L tended to be better, among recipients who lost residual hearing, speech perception was better in those with the longer CI422 electrode. Conclusions With emphasis on preservation of residual hearing, patients need to be counseled regarding possible outcomes and options should loss of residual hearing occur following implantation. While shorter electrodes may have better rates of hearing preservation, the patients with the longer straight electrode in our study had significantly better speech understanding following the loss of residual hearing.

OBJECTIVE: To validate a method of measuring angular depth of insertion (aDOI) as well as positional depth of each electrode contact in a cochlear implant by using intraoperative postinsertion skull radiographs. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: Intraoperative postinsertion radiographs obtained from 18 cochlear implant recipients were chosen for analysis. One high-resolution computer tomography scan of the head with the electrode in place was also analyzed. One cadaveric temporal bone with an inserted electrode provided additional data for analysis. INTERVENTION: aDOI and position of each electrode contact were measured from the radiographs using readily available software. High-resolution computer tomography imaging of the cochlea and electrode were reconstructed in three dimensions and used to simulate head rotation during intraoperative radiographs. The cadaveric temporal bone was imaged by x-ray at various acquisition angles. MAIN OUTCOME MEASURES: We evaluated the error introduced in measuring aDOI by assessing intra- and inter-rater variability. We also evaluated the error introduced by x-ray acquisition at nonstandardized angles by analyzing the three-dimensional construct and the cadaveric temporal bone. RESULTS: The concordance correlation coefficients for intrarater (0.991) and inter-rater (0.996) variability in aDOI measurement were excellent. The error introduced by nonstandardized x-ray acquisition angles was only -12.5 degrees to +15.8 degrees even at the limits of clinically relevant head rotation. CONCLUSIONS: The intraoperative postinsertion radiograph is sufficient for estimating positional depth of electrode contacts and the aDOI. This measure is robust in the face of nonstandardized x-ray acquisition angles, and shows good intra- and inter-rater variability.

OBJECTIVES: To examine the variables affecting outcomes for sequential bilateral cochlear implantation patients in the adolescent population. STUDY DESIGN: Retrospective chart review at tertiary care center. METHODS: Main outcome measures were open set speech recognition tests at the word (Consonant-Nucleus-Consonant/Phonetically Balanced Kindergarten List Test [CNC/PBK]) and sentence levels in noise (Hearing in Noise Test-Noise [HINT-N]) in different test conditions with respect to the age at first and sequential implantation, as well as the interval between implants. RESULTS: Despite a mean age at sequential implantation of 13.5 years, sequential bilateral implanted adolescents revealed significant improvement in the sequential cochlear implant (CI2) ear. The mean time interval between implants was 8.2 years. A wide range of performance was noted, and age at implantation and interval between first cochlear implant (CI1) and CI2 did not predict outcome. Mean CNC/PBK score with CI1 alone was 83.0%, with the CI2 alone was 56.5%, and with bilateral implants was 86.8%. Sentence scores (HINT-N) were 89.5% for CI1, 74.2% for CI2, and 94.4% for bilateral CI condition. The clinical relevance of these enhanced perception abilities requires attention to individual device use, performance with the first implant, and subjective benefits reported by patients. CONCLUSIONS: Bilateral sequential cochlear implantation leads to improved speech perception in the adolescent population and should be considered in this population, even after a long period of deafness and despite a prolonged interval between implants. Numerous factors affect the ability to predict performance, but age at implantation and interimplant interval were not correlated with performance measures. Extensive preoperative counseling and individualized evaluation are critical to ensure that patients and families understand the range of possible outcomes. LEVEL OF EVIDENCE: 6. Laryngoscope, 2015.

OBJECTIVES: Commercially available cochlear implant systems attempt to deliver frequency information going down to a few hundred Hertz, but the electrode arrays are not designed to reach the most apical regions of the cochlea, which correspond to these low frequencies. This may cause a mismatch between the frequencies presented by a cochlear implant electrode array and the frequencies represented at the corresponding location in a normal-hearing cochlea. In the following study, the mismatch between the frequency presented at a given cochlear angle and the frequency expected by an acoustic hearing ear at the corresponding angle is examined for the cochlear implant systems that are most commonly used in the United States. DESIGN: The angular insertion of each of the electrodes on four different electrode arrays (MED-EL Standard, MED-EL Flex28, Advanced Bionics HiFocus 1J, and Cochlear Contour Advance) was estimated from X-ray. For the angular location of each electrode on each electrode array, the predicted spiral ganglion frequency was estimated. The predicted spiral ganglion frequency was compared with the center frequency provided by the corresponding electrode using the manufacturer's default frequency-to-electrode allocation. RESULTS: Differences across devices were observed for the place of stimulation for frequencies below 650 Hz. Longer electrode arrays (i.e., the MED-EL Standard and Flex28) demonstrated smaller deviations from the spiral ganglion map than the other electrode arrays. For insertion angles up to approximately 270 degrees , the frequencies presented at a given location were typically approximately an octave below what would be expected by a spiral ganglion frequency map, while the deviations were larger for angles deeper than 270 degrees . For frequencies above 650 Hz, the frequency to angle relationship was consistent across all four electrode models. CONCLUSIONS: A mismatch was observed between the predicted frequency and the default frequency provided by every electrode on all electrode arrays. The mismatch can be reduced by changing the default frequency allocations, inserting electrodes deeper into the cochlea, or allowing cochlear implant users to adapt to the mismatch. Further studies are required to fully assess the clinical significance of the frequency mismatch.

OBJECTIVE: To assess whether recombinant growth factor (hGH) therapy has an effect on cochlear implant (CI) performance. PATIENTS: Two pediatric CI recipients (S1, S2) who underwent treatment with hGH for short stature were identified for review. S1 has bilateral labyrinthine dysplasia and received implants at ages 10 months (right) and 4 years 3 months (left). S2 was diagnosed with severe to progressive sensorineural hearing loss bilaterally and received a CI at age 9 years 10 months (left). INTERVENTION(S): Case series. MAIN OUTCOME MEASURE(S): Cochlear implant, hGH, and speech perception data were collected. Phonetically Balanced Kindergarten (PBK) and Consonant Nucleus Consonant (CNC) word recognition scores were reviewed to assess auditory perception. Electrode impedances, threshold levels, and comfort levels were also reviewed. RESULTS: After 4 months of hGH, word recognition scores for S1 were observed to decrease from 90 to 72% (right) and were stable at 40% (left). Despite troubleshooting, performance continued to decline bilaterally to 52% (right) and 28% (left), and the decision was made to discontinue hGH. One month after cessation of hGH, word recognition scores began improving to 74% (right) and 68% (left). Word recognition scores for S2 were observed to have decreased from 92% the previous year to 82% after taking hGH for 2 months. Given both our previous experience with S1 and discussions with S2's parents, hGH was discontinued after 10 months of therapy. Two months after cessation of hGH, S2's word recognition had improved to 86% (left). CONCLUSIONS: Our case studies illustrate that implanted children undergoing treatment with hGH may experience a decrease in speech perception, which recovers after the cessation of treatment. Since hGH use has become more prevalent in recent years, it is important to inquire whether children undergoing, or who have undergone, implantation are receiving hGH so that they may be appropriately monitored.