Transmastoid Facial Nerve Decompression for Craniometaphyseal Dysplasia.

OBJECTIVE: We document the first successful transmastoid surgical treatment of facial nerve palsy for a patient with craniometaphyseal dysplasia (CMD), a rare genetic disease. PATIENT: A 9-month-old girl with bilateral facial nerve palsies and conductive hearing loss. Genetic testing made a diagnosis of CMD, and imaging showed narrowing of the facial nerve canals and ossicular fixation. INTERVENTION: Right transmastoid facial nerve decompression and ossicular chain reconstruction. MAIN OUTCOME MEASURE: Facial nerve function (House-Brackmann grade). RESULTS: Facial nerve function initially worsened, then improved within 12 months from House-Brackmann grade IV-V to grade III. CONCLUSION: Surgical cranial nerve decompression of and ossicular chain reconstruction may be effective treatments for patients with CMD.

Combined surgical repair and venous sinus stenting for patients with skull base encephaloceles secondary to dural venous sinus stenosis.

BACKGROUND: Chronically elevated intracranial pressure (ICP) seen in idiopathic intracranial hypertension (IIH) can cause the development of skull base encephaloceles and cerebrospinal fluid (CSF) leaks. Surgical repair and ventriculoperitoneal shunt (VPS) placement are mainstays of treatment. Venous sinus stenting (VSS) is a newly accepted treatment modality. The goal of this study was thus to determine if VSS can be used to treat symptoms and prevent recurrence after surgical encephalocele repair. METHODS: Retrospective chart review of patients that had surgical repair of encephaloceles followed by VSS for symptomatic stenosis with elevated pressure gradient. RESULTS: A total of 13 patients underwent a combined encephalocele repair and VSS. Seventy-two percent were female; 46% had headaches, 69% pulsatile tinnitus, and 92% CSF rhinorrhea or otorrhea. One had seizures. Mean lumbar opening pressure was 23.3 ± 2.6 cm H2O; the average sagittal-to-jugular pressure gradient was 12.7 ± 1.8 cmH2O and was elevated in all patients. Four patients had middle fossa craniotomy for repair of tegmen defect (one bilateral); one had a retrosigmoid craniotomy for repair of a sigmoid plate defect. Eight had an endoscopic endonasal repair for sphenoid or cribriform plate encephalocele. There were no VSS procedural complications or complications associated with dual antiplatelet therapy. One patient had meningitis after endoscopic repair that was treated with antibiotics. One patient had recurrence of both CSF leak and venous stenosis adjacent to the stent requiring repeat repair and VSS. There was no further recurrence. CONCLUSION: In patients with dural sinus stenosis and encephaloceles requiring repair, VSS can be performed safely within weeks of surgery for relief of symptoms, resolution of underlying pathology, and prevention of CSF leak recurrence.

Long-Term Effects of Hearing Aids on Hearing Ability in Patients with Sensorineural Hearing Loss.

BACKGROUND: A frequent concern surrounding amplification with hearing aids for patients with sensorineural hearing loss is whether these devices negatively affect hearing ability. To date, there have been few studies examining the long-term effects of amplification on audiometric outcomes in adults. PURPOSE: In the present study, we examined how hearing aids affect standard audiometric outcomes over long-term periods of follow-up. RESEARCH DESIGN: We retrospectively collected audiometric data in adults with sensorineural hearing loss, constructing a model of long-term outcomes. STUDY SAMPLE: This retrospective cohort study included 802 ears from 401 adult patients with bilateral sensorineural hearing loss eligible for amplification with hearing aids at a single institution. INTERVENTION: Of the eligible patients, 88 were aided bilaterally, and 313 were unaided. DATA COLLECTION AND ANALYSIS: We examined the standard three-frequency pure-tone average (PTA3-Freq), a novel extended pure-tone average (PTAExt), and word recognition score (WRS) per-ear at each encounter. We then modeled the association between the use of hearing aids for 5 years and these audiometric outcomes using targeted maximum likelihood estimation. RESULTS: In comparing aided and unaided ears at the end of 5 years, there were discernible effects for all measurements. The PTA3-Freq was 5 dB greater in aided ears (95% CI: 1.37-8.64, p = 0.007), WRS was 4.5 percentage points lower in aided ears (95% CI: -9.14 to 0.15, p = 0.058), and PTAExt was 5 dB greater in aided ears (95% CI: 2.18-7.82, p < 0.001), adjusting for measured confounders. CONCLUSION: Our analysis revealed discernible effects of 5 years of hearing aid use on hearing ability, specifically as measured by the PTA3-Freq, novel PTAExt, and WRS, suggesting a greater decline in hearing ability in patients using hearing aids. Future studies are needed to examine these effects between treatment groups over longer periods of time and in more heterogeneous populations to improve clinical practice guidelines and safety of both prescriptive fitting nonprescriptive amplification.

Comparison of Cochlear Implant Device Fixation-Well Drilling Versus Subperiosteal Pocket. A Cost Effectiveness, Case-Control Study.

OBJECTIVE: To compare surgical characteristics and complications between well drilling (WD) and subperiosteal pocket techniques (SPT) for receiver/stimulator (R/S) fixation of cochlear implant (CI), and conduct cost-effectiveness analysis. STUDY DESIGN: Retrospective clinical study, decision-analysis model. SETTING: Tertiary referral center. PATIENTS: Three-hundred and eighty-eight CI recipients with a minimum of 6-months follow-up. INTERVENTIONS: CI surgery using either WD or SPT for R/S fixation. A decision-analysis model was designed using data from a systematic literature review. MAIN OUTCOME MEASURES: Surgical operation time, rates of major and minor long-term complications were compared. Incremental cost-effectiveness was also estimated, comparing the two methods of fixation. RESULTS: We compared 179 WD with 209 SPT. Surgery time was significantly shorter in SPT (148 versus 169 min, p = 0.001) and remained significant after adjustment for possible confounders. Higher rates of major complications requiring surgical intervention were found with SPT (10.5% versus 4.5%, p = 0.042), however, the difference was not significant after adjusting for follow-up time (47.8 versus 32.5 months for SPT, WD respectively; p < 0.001). The incremental cost-effectiveness ratio for WD (compared with SPT) was $48,795 per major complication avoided, which was higher than the willingness-to-pay threshold of $47,700 (average cost of 2 h revision surgery). CONCLUSIONS: SPT was found to be faster but potentially risks more complications, particularly relating to device failure. Further long-term studies are required to validate these differences. Based on data from the current literature, neither of the methods is compellingly cost-effective over the other, and surgeons can base their choice on personal preference, comfort, and previous training.

Comparison of Speech Performance in Bimodal versus Bilateral Cochlear Implant Users.

OBJECTIVE: To assess whether listening with two cochlear implants (bilateral) offers significant benefits in terms of speech perception over listening with one cochlear implant and one hearing aid (bimodal). METHODS: Retrospective review of bilateral cochlear implant recipients (24 pediatric and 26 adult). Bimodal listening was compared to bilateral listening in terms of speech perception performance at 1-year post second implant under three listening conditions: 50 dBHL, 35 dBHL, and 50 dBHL+5 SNR. Changes in speech performance from bimodal (before second implant) to bilateral (after second implant) listening were determined within subjects and compared to a separate control group of bimodal users matched for age of first implantation who never received a second implant (10 pediatric and 20 adult). RESULTS: In the pediatric group, compared to bimodal listening prior to a second implant, speech perception scores with bilateral implants increased significantly when measured at 50 dBHL, 35 dBHL, and 50 dBHL+5 SNR. By contrast, pediatric bimodal controls who never received a second implant failed to demonstrate similar improvement over 1 year's time. In the adult group, compared to bimodal listening prior to a second implant, speech perception scores with bilateral implants increased when measured at 50 dBHL, but were not significantly different at 35 dBHL and 50 dBHL + 5 SNR. Adult bimodal controls who never received a second implant failed to demonstrate significant improvement in all conditions over 1 year's time. CONCLUSION: Bilateral listening with two cochlear implants improved speech perception performance relative to bimodal listening in the pediatric population. Improvement in the adult population was not as significant. LEVEL OF EVIDENCE: 4, Retrospective Chart Review. Laryngoscope, 131:E1322-E1327, 2021.

Venous sinus stenting for the treatment of isolated pulsatile tinnitus: Results of a prospective trial.

OBJECTIVES: This prospective study evaluates the effectiveness and safety of venous sinus stenting for patients with isolated pulsatile tinnitus and lateral sinus stenosis. METHODS: Patients with isolated pulsatile tinnitus and lateral sinus stenosis with a minimum trans stenotic gradient of 4 mm Hg were treated with stenting. Pulsatile tinnitus before and after treatment was assessed with the Tinnitus Handicap Inventory (THI). Periprocedural adverse events, neurological complications, clinical and radiographic follow-up were also recorded. RESULTS: A total of 42 patients (41 females and 1 male) were included in the study (median age of 37.5 years). Thirty patients had post-stenotic fusiform and 12 had post-stenotic saccular venous sinus aneurysm. In addition to stenting, coils were used to treat the patients with saccular venous aneurysms. The median follow-up was 5 months (range 1 to 34 months). Most patients had complete (39/42) or near-complete (2/42) resolution of their pulsatile tinnitus post-procedure. There were no serious adverse events. CONCLUSION: Stenting of the lateral venous sinus is a safe and effective treatment for patients with isolated pulsatile tinnitus due to venous sinus stenosis.

Multicenter US Clinical Trial With an Electric-Acoustic Stimulation (EAS) System in Adults: Final Outcomes.

OBJECTIVE: To demonstrate the safety and effectiveness of the MED-EL Electric-Acoustic Stimulation (EAS) System, for adults with residual low-frequency hearing and severe-to-profound hearing loss in the mid to high frequencies. STUDY DESIGN: Prospective, repeated measures. SETTING: Multicenter, hospital. PATIENTS: Seventy-three subjects implanted with PULSAR or SONATA cochlear implants with FLEX electrode arrays. INTERVENTION: Subjects were fit postoperatively with an audio processor, combining electric stimulation and acoustic amplification. MAIN OUTCOME MEASURES: Unaided thresholds were measured preoperatively and at 3, 6, and 12 months postactivation. Speech perception was assessed at these intervals using City University of New York sentences in noise and consonant-nucleus-consonant words in quiet. Subjective benefit was assessed at these intervals via the Abbreviated Profile of Hearing Aid Benefit and Hearing Device Satisfaction Scale questionnaires. RESULTS: Sixty-seven of 73 subjects (92%) completed outcome measures for all study intervals. Of those 67 subjects, 79% experienced less than a 30 dB HL low-frequency pure-tone average (250-1000 Hz) shift, and 97% were able to use the acoustic unit at 12 months postactivation. In the EAS condition, 94% of subjects performed similarly to or better than their preoperative performance on City University of New York sentences in noise at 12 months postactivation, with 85% demonstrating improvement. Ninety-seven percent of subjects performed similarly or better on consonant-nucleus-consonant words in quiet, with 84% demonstrating improvement. CONCLUSION: The MED-EL EAS System is a safe and effective treatment option for adults with normal hearing to moderate sensorineural hearing loss in the low frequencies and severe-to-profound sensorineural hearing loss in the high frequencies who do not benefit from traditional amplification.

Method to estimate the complete and two-turn cochlear duct length.

HYPOTHESIS: Using a linear measurement of the cochlea on a single radiographic image can reliably estimate the complete and two-turn cochlear duct length (CDL) in a normal human temporal bone. BACKGROUND: CDL is measured from the middle of the round window to the helicotrema. Histologic studies have shown the length of the organ of Corti (OC) to range from 25 to 35 mm. CDL measurements, performed either radiographically or histologically, are quite tedious and time-consuming. We propose equations that can reliably estimate both two-turn and complete CDL using a single computed tomography (CT) image. METHODS: Prior studies of CDL, measured either histologically or radiographically, were reviewed, which yielded distributions of CDL measured at the OC and the lateral wall of the cochlea. Using Escudé's third equation as a basis, we were able to extrapolate complete and two-turn CDL based on a CT scan measurement of the diameter of the basal turn (A). RESULTS: Using measurement A, the relationship of two-turn CDL measured at the OC is 2TL(oc) = 3.65(A-1) and for 2TL(i) = 3.65(A-0.7). The equation for estimation of complete CDL is CDL(oc) = 4.16A - 4 and for CDL(i) = 4.16A - 2.7. CONCLUSION: Using a single linear measurement from a CT scan image can reliably estimate the two-turn and complete CDLs in human temporal bones. The two-turn length represents the best compromise of cochlear coverage while minimizing intracochlear trauma for electrode insertions.

Diagnostic and prognostic utility of measuring tumor necrosis factor in the peripheral circulation of patients with immune-mediated sensorineural hearing loss.

OBJECTIVES: To characterize levels of tumor necrosis factor (TNF; formerly known as tumor necrosis factor α), a well-established proinflammatory cytokine, in patients with immune-mediated sensorineural hearing loss (IM-SNHL) and to determine the role of this cytokine in identifying steroid-responsive hearing loss. DESIGN: Prospective case-control study. SETTING: Tertiary care academic medical center. PATIENTS: A total of 11 control subjects and 85 patients with clinical and audiometric characteristics of IM-SNHL (autoimmune inner ear disease and sudden SNHL combined) treated with corticosteroids were enrolled in the study. Patients were categorized as steroid responders (n = 47) and steroid nonresponders (n = 38). Peripheral venous blood was used to determine the total amount of plasma TNF by enzyme-linked immunosorbent assay. Peripheral blood mononuclear cells (PBMCs) were isolated and treated with in vitro dexamethasone. Treated and untreated PBMCs were then analyzed for release of soluble TNF protein into conditioned supernatants as well as expression of TNF messenger RNA (mRNA). MAIN OUTCOME MEASURES: Mean plasma levels of TNF, unstimulated and dexamethasone-stimulated PBMC-secreted levels of TNF, and TNF mRNA levels in unstimulated and dexamethasone-stimulated PBMCs. RESULTS: Steroid nonresponders had the highest mean baseline plasma levels of TNF compared with steroid responders and control subjects (27.6, 24.1, and 14.4 pg/mL, respectively) (P = .03). For patients with IM-SNHL with a high baseline plasma levels of TNF (>14.4 pg/mL), the mean TNF secreted by PBMCs was 59.1 pg/mL, which decreased to 7.2 pg/mL with in vitro dexamethasone stimulation in the responder group, while the mean TNF secreted by PBMCs was 11.2 pg/mL, which slightly increased to 11.7 pg/mL with in vitro dexamethasone stimulation in the nonresponder group (P = .04). CONCLUSIONS: The level of TNF can be used as both a diagnostic and prognostic cytokine for IM-SNHL. For patients presenting with a sudden change in hearing threshold, a high baseline plasma TNF from the peripheral circulation is supportive of the diagnosis if it is greater than 18.8 pg/mL, with a positive predictive value higher than 97%. In addition, this study demonstrates that for patients with IM-SNHL and high plasma levels of TNF, their clinical response to oral glucocorticoids can be predicted by their in vitro PBMC response to dexamethasone. This algorithm may further guide optimal medical treatment and possibly avoid the deleterious adverse effects of administering glucocorticoids to those patients who would not benefit from their effect.

Revision cochlear implant surgery in patients with suspected soft failures.

OBJECTIVE: To review our patient series who underwent revision cochlear implantation surgery, with special emphasis on the "soft failure" group. STUDY DESIGN: Retrospective chart review of cochlear implant revision surgeries from 1979 to 2008. An extensive review of these patients' medical, audiologic, and radiographic histories was performed. SETTING: Two tertiary care hospitals and 1 academic cochlear implant center. INTERVENTION: Explantation and reimplantation of cochlear implant, explanted device analysis, speech perception testing. MAIN OUTCOME MEASURES: Postoperative speech performance. RESULTS: Approximately 1,500 cochlear implant surgeries were performed from 1979 to 2008. Of these, 113 (7.53%) procedures in 98 patients were revision cases. The underlying reason for revision surgery was divided into 4 categories: 26 hard failures (23%), 31 medical failures (27.4%), 14 soft failures (12.4%), and 42 (37.2%) not classified/ambiguous cases. The last group was not categorized because of lack of available medical documentation or because of an ambiguous device failure analysis. The top 3 most common causes of hard failure were loss of hermiticity (8 patients [30.8%]), Vendor B defects (7 patients [26.9%]), and cracked casing (4 patients [15.4%]). The most common cause of medical failure was device extrusion (11 patients [35.5%]) followed by head trauma to the site of implantation (11 patients [35.5%]), and wound infection (5 patients [16.1%]). Fourteen patients (14.2%) were categorized as soft failures. All soft failure patients demonstrated a deterioration in pure-tone average and speech perception. Of the soft failure group, time to revision surgery was 4.7 years in contrast to 4.2 years for the hard failure group and 4.3 years for all revisions. An extensive review of patients' medical histories showed that 4 (28.5%) of the 14 patients had a previous diagnosis of meningitis. Two patients (14.3%) had evidence of inner ear malformations, and 2 patients (14.3%) had history of asthma. CONCLUSION: Our cochlear revision series are comparable to what is reported in the literature. However, an unexpected relationship between meningitis was identified among our soft failure group. More than one-quarter carried a history of meningitis. Moreover, nearly one-half of all soft failures had some form of inflammatory derangement. We used the soft failure criteria established by the 2005 Consensus Development Conference for our population analysis. Although we agree that audiologic data often are essential for defining soft failure, multiple patients in our series experienced pain that was severe enough to prevent a complete audiometric evaluation, therefore not rigorously fulfilling the criteria set forth by the 2005 Consensus. However, because their symptoms resolved after reimplantation, and their speech performance restored, we propose modifications of the current definition of "soft failure" to include these patients.

Otologics fully implantable hearing system: Phase I trial 1-year results.

OBJECTIVE: To assess the safety of the Otologics fully implantable hearing system after 1 year of use in a Phase I clinical trial. STUDY DESIGN: Repeated-measures within-subjects design. SETTING: Procedures were performed in a variety of facilities, including a university, military, and private hospital's ambulatory surgical center and outpatient clinical audiologic test facilities. PATIENTS: Adult patients with bilateral moderate to severe sensorineural hearing loss. INTERVENTION(S): Surgical insertion of this prosthesis included an atticotomy to expose the incus, securing the transducer to the mastoid bone, attaching the transducer tip to the incus via insertion into a laser-drilled hole, and postauricular implantation of the microphone/battery/electronics capsule. MAIN OUTCOME MEASURE(S): Subjective patient benefit, aided sound field thresholds, and speech discrimination with the subject's own, appropriately fit, walk-in hearing aid(s) and the prosthesis were assessed. RESULTS: There were no pre-post-implant differences noted for bone conduction: slight differences were noted in the pre-post-implant air conduction results (p < 0.05). These differences were attributed to the healing process and reversed to almost preimplant assessment levels by the third-month evaluation. Pure-tone averages and monaural word recognition scores were slightly better for the walk-in-aided condition (p < 0.05), whereas the patient benefit scales favored the postoperative implant-aided conditions.Adverse effects of the implant were encountered on 14 occasions after the implantation of the 20 subjects. With the exception of partial device extrusions (that occurred later), all were rectified by the time of initial activation. At the 12-month data collection point, problems that had been encountered by subjects included 1) partial device extrusion (3 subjects), necessitating explantation in 2; 2) loss of external communication (2 subjects), resulting in 1 explantation; and 3) increased charging times beyond 1.5 hours (7), resulting in 3 explantations and 2 patients not using their device while awaiting explantation. CONCLUSION: Phase I trial results provide evidence that this fully implantable device can provide sound amplification to sensorineural hearing loss patients, with performance results similar to the patients' walk-in hearing aids.

Glomus tumors in patients of advanced age: a conservative approach.

OBJECTIVES: Identify and discuss controversies in the management of paragangliomas in elderly patients. Assess and evaluate a conservative treatment strategy involving limited surgical resection and vigilant monitoring of the outcome measures of tumor control, peritreatment morbidity, symptom resolution, and hearing preservation. STUDY DESIGN: Retrospective case review. METHODS: All of the patients in this study were over age 60 with temporal bone glomus tumors. Primary outcome assessment included length of hospitalization, perioperative morbidity, symptom resolution, hearing preservation, and long-term tumor control. RESULTS: Twelve female patients with mean age of 74.5 years (range 61-85 years) with follow-up from 24 months to 33 years (mean/median: 5/7.8 years) were identified. Nine (75%) of the patients presented with pulsatile tinnitus. Seven patients (58%) underwent surgical excision of the middle ear component of the paraganglioma. Tumors extending to the jugular foramen were purposely not resected. Five patients (45%) had relative or absolute contraindications to surgical resection and were treated with observation or primary radiation therapy. Post-treatment audiometric evaluation confirmed stable or improved hearing. Pulsatile tinnitus resolved in all patients. No patient experienced cranial nerve deficits, extended hospitalization, or blood transfusions. All patients were followed closely with radiological imaging. The majority of patients demonstrated no disease or stable disease, while two patients demonstrated tumor growth 6 years after diagnosis. CONCLUSION: A prolonged natural history and the morbidity associated with surgical intervention have led to controversies in the treatment of glomus tumors in an elderly population. Our experience supports recent limited reports advocating conservative surgical excision and vigilant long-term monitoring in this population.

U.S. Phase I preliminary results of use of the Otologics MET Fully-Implantable Ossicular Stimulator.

OBJECTIVES: The safety of the Otologics Fully-Implantable MET Ossicular Stimulator was assessed in adult patients with bilateral moderate to severe sensorineural hearing loss. METHODS: Surgical implantation of the ossicular stimulator was performed. A repeated-measure, within-subjects design assessed safety and aided sound field thresholds and speech performances with the subject's own, appropriately fitted, walk-in hearing aid(s) and the Otologics Fully-Implantable MET Ossicular Stimulator. RESULTS: Twenty patients were implanted and activated as part of the Phase I clinical trial. Results demonstrated 10-20 dB of functional gain across audiometric frequencies. Pure tone averages and monaural word recognition scores were slightly better for the walk-in-aided condition, while patient benefit scales favored the postoperative implant-aided conditions. CONCLUSION/SIGNIFICANCE: Although monaural word scores and aided thresholds favored the walk-in-aided condition, preliminary results indicate that the Otologics MET Fully-Implantable Ossicular Stimulator is an alternative to currently available hearing aids in patients with sensorineural hearing loss.

Cranial nerve preservation in surgery for large acoustic neuromas.

Facial nerve outcomes and surgical complication rates for other cranial nerves were evaluated retrospectively after the resection of large acoustic neuromas. The charts of all patients who underwent surgical removal of an acoustic neuroma between 1992 and 2001 at New York University Medical Center were reviewed. Fifty-four patients with tumors measuring 3 cm or larger were included in the study. Four patients had neurofibromatosis type 2, two of whom underwent bilateral removal of acoustic neuromas. Translabyrinthine microsurgical removal of tumor was performed in 47 of 56 cases (84%). In all cases, EMG monitoring, improved sharp microdissection, and ultrasonic aspiration were employed. Facial nerve function was assessed using the House-Brackmann facial nerve grading system immediately after surgery and at follow-up visits. A House-Brackmann grade III or better was achieved in 90% of patients, and a grade II or better was achieved in 84% of patients. Ultimate facial nerve outcome was excellent after the surgical resection of large acoustic neuromas. Preoperative cranial nerve palsies also improved after surgery. The translabyrinthine approach for tumor removal is our treatment of choice for acoustic neuromas 3 cm or larger.

Fluoroscopically assisted cochlear implantation.

HYPOTHESIS: Real-time intraoperative fluoroscopy is a useful adjunct to cochlear implantation in selected cases. The advantages include the avoidance of complications such as extracochlear array placement, intrameatal array insertion, and avoidance of significant bending or kinking. This is particularly useful when implanting a severely abnormal cochlea. BACKGROUND: The technique was initially developed for laboratory study of electrode prototypes in cadaver temporal bones to evaluate insertion dynamics and mechanisms of intracochlear trauma. The technique was subsequently adapted for use in live surgeries. METHODS: Live surgeries were performed using fluoroscopic guidance on nine patients. RESULTS: Five patients were implanted with the Nucleus 24 RCS during preclinical trials. Two patients with severe cochlear malformations were implanted with a Nucleus CI24M straight array. Two patients with severe cochlear ossification were implanted with the Nucleus CI24 double array. Appropriate insertions were achieved without electrode damage in all cases. CONCLUSIONS: Intraoperative fluoroscopy is a useful adjunct to cochlear implantation, which can be performed with minimum risk to the patient and operating room staff if the outlined precautions are taken. Intraoperative fluoroscopy is indicated in cases where the intracochlear behavior of the electrode array cannot be predicted, a condition encountered when implanting new electrode designs, cases with severely malformed inner ears, or cases of severe intraluminal obstruction requiring a double-array insertion.

Cochlear implantation in human immunodeficiency virus-infected patients.

OBJECTIVE: To evaluate the efficacy of cochlear implants in human immunodeficiency virus-infected individuals and correlate these results with a proposed pathophysiological mechanism of human immunodeficiency virus-associated hearing loss. STUDY DESIGN: Retrospective case series and temporal bone analysis of deceased human immunodeficiency virus-positive patients. SETTING: Tertiary care hospital. PATIENTS: Seven human immunodeficiency virus-positive individuals with profound sensorineural hearing loss. INTERVENTION: Cochlear implantation at New York University Medical Center. METHODS: The surgical outcomes and complications were analyzed. Additionally, electron microscopic and immunohistochemical findings of cadaver temporal bone specimens of other known human immunodeficiency virus-positive individuals were reviewed. The performance results of the human immunodeficiency virus-positive cochlear implant patients were then correlated with the previously hypothesized pathophysiological mechanism of human immunodeficiency virus-associated hearing loss. RESULTS: The patients had a varied performance with cochlear implantation, and as a group performance was good. There were no surgical complications or postoperative complications. The good performance of these patients supports the hypothesis that the mechanism of human immunodeficiency virus-associated deafness involves infiltration, malfunction, and premature degeneration of the hair cells and supportive cells of the cochlea. CONCLUSIONS: Human immunodeficiency virus-positive individuals benefit from cochlear implantation without increased surgical risk.